diff --git a/.search_history.json b/.search_history.json
index cb93abd..3560342 100644
--- a/.search_history.json
+++ b/.search_history.json
@@ -15,6 +15,23 @@
}
]
},
+ {
+ "label": "'Hypotensive' (exact)",
+ "queries": [
+ {
+ "qval": "Hypotensive",
+ "mode": "exact",
+ "targets": [
+ "Title",
+ "Content",
+ "Breadcrumbs",
+ "Keywords",
+ "Category",
+ "Type"
+ ]
+ }
+ ]
+ },
{
"label": "'polycystic live' (exact)",
"queries": [
diff --git a/Dockerfile b/Dockerfile
new file mode 100644
index 0000000..237c422
--- /dev/null
+++ b/Dockerfile
@@ -0,0 +1,18 @@
+FROM python:3.10-slim
+
+WORKDIR /app
+
+# Install system dependencies if any are needed
+RUN apt-get update && apt-get install -y --no-install-recommends \
+ build-essential \
+ && rm -rf /var/lib/apt/lists/*
+
+COPY requirements.txt /app/
+
+RUN pip install --no-cache-dir -r requirements.txt
+
+COPY . /app
+
+EXPOSE 8000
+
+CMD ["uvicorn", "scrapers.cache_server:app", "--host", "0.0.0.0", "--port", "8000"]
diff --git a/docker-compose.yml b/docker-compose.yml
new file mode 100644
index 0000000..26ca028
--- /dev/null
+++ b/docker-compose.yml
@@ -0,0 +1,13 @@
+version: '3.8'
+
+services:
+ cache-server:
+ build: .
+ container_name: statdx-cache-server
+ ports:
+ - "8000:8000"
+ volumes:
+ - .:/app
+ environment:
+ - PYTHONUNBUFFERED=1
+ restart: unless-stopped
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\ No newline at end of file
diff --git a/docs_md/_images_index.json b/docs_md/_images_index.json
index ae06817..a04b809 100644
--- a/docs_md/_images_index.json
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+---
+title: "Abdominal Aorta and Visceral Vasculature Anatomy"
+docid: "341c0af5-bfe8-4755-a401-39b7b9f2d9b9"
+authors:
+ - key: "91e93745-f376-45a8-9b33-eae419cd3322"
+ value: "T. Gregory Walker, MD, FSIR"
+breadcrumbs:
+ -
+ name: "Vasculature"
+ slug: "vasculature"
+ treeNodeId: "6de1ee4d-afe9-419c-a868-d4074ec0fb7e"
+ -
+ name: "Anatomy"
+ slug: "anatomy"
+ treeNodeId: "3bbfaa8b-2dbc-41eb-b3da-3bd83a9737c4"
+ -
+ name: "Abdominal Aorta and Visceral Vasculature Anatomy"
+ slug: "abdominal-aorta-and-visceral-vascu-"
+ treeNodeId: null
+category: "Vasculature"
+documentVersionId: "110784af-0b71-416f-9b1e-1e6b77953a53"
+imageCount: 8
+lastUpdated: "02/21/24"
+pageDescription: "Abdominal Aorta and Visceral Vasculature Anatomy"
+pageKeywords: "Vasculature, Anatomy, Abdominal Aorta and Visceral Vasculature Anatomy"
+pageTitle: "Abdominal Aorta and Visceral Vasculature Anatomy | STATdx"
+enhancedTitle: "Abdominal Aorta and Visceral Vasculature Anatomy"
+type: "ANATOMY"
+references: true
+breadcrumbs:
+ - "Vasculature"
+ - "Anatomy"
+ - "Abdominal Aorta and Visceral Vasculature Anatomy"
+---
+## GROSS ANATOMY
+
+- ### Abdominal Aorta
+
+
+ - Begins at level of diaphragmatic crura and terminates at bifurcation into common iliac arteries
+ - Abdominal aorta and its branches supply arterial perfusion to all structures below diaphragm
+ - Major branches supply abdominal viscera and kidneys and also yield multiple parietal branches
+ - Lies slightly left of midline; courses anterior to vertebral bodies, parallel to inferior vena cava (IVC)
+ - Progressively decreases in caliber as it yields branches
+- ### Visceral Branches of Abdominal Aorta
+
+
+ - Celiac artery (a.k.a. celiac axis/trunk): Arises anteriorly from abdominal aorta below diaphragmatic hiatus; divides into 3 large branches
+ - Left gastric artery: Courses superiorly to supply distal esophagus and gastric cardia; anastomoses with short gastric branches from splenic artery
+ - Continues along lesser curvature of stomach giving branches anteriorly and posteriorly; terminally anastomoses with right gastric artery
+ - Splenic artery: Largest celiac branch; tortuous course to left side to supply pancreas, spleen, and stomach
+ - Pancreatic branches: Multiple branches, including dorsal pancreatic and pancreatica magna arteries, supply pancreatic body and tail
+ - Splenic branches: Splenic artery divides in hilum of spleen into multiple branches
+ - Short gastric arteries: Supply fundus of stomach
+ - Left gastroepiploic artery: Supplies left side of greater curvature of stomach, anastomoses with right gastroepiploic artery
+ - Common hepatic artery: Has multiple branches that supply liver, stomach, duodenum, gallbladder, and pancreas; becomes proper hepatic artery after yielding gastroduodenal artery
+ - Gastroduodenal artery: Bifurcates into right gastroepiploic and superior pancreaticoduodenal arteries; supplies duodenum, pancreas, and stomach
+ - Cystic artery: Typically arises from right hepatic artery (70%); supplies cystic duct and gallbladder
+ - Right gastric artery: Usually arises from proper hepatic artery; supplies lesser gastric curvature and anastomoses terminally with left gastric artery
+ - Right hepatic artery: Supplies right hepatic lobe (hepatic segments 5-8)
+ - Left hepatic artery: Supplies left hepatic lobe (hepatic segments 1-4)
+ - Middle hepatic artery: Anatomic variant; when present, typically supplies hepatic segment 4
+ - Superior mesenteric artery (SMA): Arises anteriorly from aorta just below celiac artery; supplies bowel from 2nd portion of duodenum as far distally as splenic flexure of transverse colon
+ - Inferior pancreaticoduodenal arteries: Supplies duodenum (distal to bile duct), pancreas, and spleen
+ - Anastomoses with superior pancreaticoduodenal arteries to form arterial arcade
+ - Middle colic artery: Supplies proximal 2/3 of transverse colon up to splenic flexure
+ - May have separate right and left branches that supply respective regions of transverse colon
+ - Jejunal and ileal branches: Supply respectively named segments of small intestine
+ - Form anastomotic loops (arterial arcades), which give off vasa recta (straight arteries)
+ - Right colic artery: Supplies ascending colon and proximal transverse colon
+ - Ileocolic artery: Supplies terminal ileum, cecum, appendix, and proximal ascending colon
+ - Middle adrenal arteries: Supply adrenal glands
+ - Arise directly from aorta near origin of celiac artery; 1 or more adrenal arteries on either side
+ - Gonadal arteries: Supply ovaries and fallopian tubes (females) or testes and spermatic cords (males)
+ - Originate inferior to renal arteries but superior to inferior mesenteric artery (IMA)
+ - May arise at different levels on either side
+ - IMA: Supplies distal 1/3 of transverse colon to proximal rectum
+ - Left colic artery: Supplies splenic flexure (distal transverse colon) and descending and sigmoid colon
+ - Sigmoidal arteries: Supply sigmoid colon
+ - Superior rectal (hemorrhoidal) artery: Terminal branch of IMA; supplies proximal rectum
+ - Remainder of rectum is supplied by middle and inferior rectal arteries, which are small branches that arise from internal iliac arteries bilaterally
+ - Marginal artery of Drummond: Anastomotic artery coursing along mesenteric border of colon
+ - Immediately adjacent to colon; gives off vasa recta
+- ### Renal Arteries
+
+
+ - Described in "Renal Vasculature Anatomy" document
+- ### Parietal Branches of Abdominal Aorta
+
+
+ - Inferior phrenic arteries: Paired vessels originating anteriorly from aorta; supply diaphragm from below
+ - May have separate origins or short common trunk; may also arise from celiac artery
+ - Both right and left inferior phrenic arteries give rise to multiple superior adrenal arteries
+ - Lumbar arteries: Paired vessels arising posteriorly from aorta; supply abdominal wall and spinal cord
+ - Anastomose with lower intercostal, iliolumbar, deep circumflex iliac, and inferior epigastric arteries
+ - Median sacral artery: Single midline vessel arising posteriorly from distal aorta above bifurcation; supplies lower lumbar spine, sacrum, and coccyx
+ - Anastomoses with iliolumbar and lateral sacral branches; also provides small branches to rectum
+- ### Arcade Arrangement of Visceral Vessels
+
+
+ - Most visceral organs have 2 or more sources of arterial blood supply and venous drainage; important sources of collateral circulation
+ - Left gastric to right gastric arcade
+ - Connects celiac artery with distal hepatic artery
+ - Left gastroepiploic to right gastroepiploic arcade
+ - Connects common hepatic and splenic arteries
+ - Form arc of Barkow via branches (right and left epiploic) in posterior omental layer
+ - Pancreatic arcades
+ - Superior and inferior arcades connect celiac artery and SMA via gastroduodenal artery
+ - Arc of Bühler, if present, connects celiac axis and SMA
+ - Superior to inferior mesenteric arcades
+ - Marginal artery of Drummond, coursing along descending colon, anastomoses with middle colic artery of transverse colon in splenic flexure region
+ - Arc of Riolan runs cephalad within mesentery (rather than along colonic margin) and connects left colic artery with middle colic artery
+- ### Variant Anatomy of Aortic Branches
+
+
+ - Aberrant or replaced artery: Anatomic variation in which entire vessel arises from different parent vessel
+ - Replaced right hepatic artery: Arises from SMA rather than from proper hepatic artery
+ - Most common variant in hepatic arterial anatomy; 9-15% incidence
+ - Replaced left hepatic artery: Arises from left gastric artery rather than from proper hepatic artery; 3-10% incidence
+ - Replaced common hepatic artery: Entire hepatic blood supply can arise from SMA; 2-4% incidence
+ - Common hepatic artery may arise directly from aorta rather than from celiac artery in 2% of cases
+ - Accessory artery: Anatomic variant wherein additional vessel supplies territory usually supplied by 1 artery
+ - Accessory right hepatic artery arises from SMA in 1-7% of individuals
+ - Accessory left hepatic artery arises from left gastric artery in 8-13% of individuals
+ - Celiacomesenteric trunk: Single common origin to celiac and SMA; occurs in < 1% of individuals
+ - Dorsal pancreatic artery: Usually arises from proximal splenic artery; several well-known vascular variants
+ - May arise directly from celiac artery
+ - May arise from common hepatic artery
+ - May give rise to left branch of middle colic artery or entire middle colic artery
+- ### Venous Drainage of Abdominal Viscera
+
+
+ - Portal vein: 1 of 2 separate venous systems providing drainage for abdominal and pelvic viscera, formed by union of splenic and superior mesenteric vein; accounts for ~ 70% of hepatic blood supply
+ - Splenic vein: Drains spleen, stomach, colon, and pancreas via multiple tributaries
+ - Inferior mesenteric vein: Drains descending colon, sigmoid colon, and rectum via left colic, sigmoid, and superior rectal veins
+ - Pancreatic veins: Drain pancreatic tail and body
+ - Left gastroepiploic vein: Drains stomach inferiorly
+ - Short gastric veins: Drain gastric fundus
+ - Superior mesenteric vein: Drains jejunum, ileum, appendix, cecum, and ascending/transverse colon
+ - Ileocolic vein: Drains terminal ileum, appendix, cecum, and lower ascending colon
+ - Right colic vein: Drains ascending colon
+ - Jejunal and ileal veins: Drain respectively named segments of small intestine
+ - Right gastroepiploic vein: Drains greater curvature of stomach along with left gastroepiploic vein
+ - Inferior pancreaticoduodenal vein: Drains pancreatic head via arcade formed with superior pancreaticoduodenal vein
+ - Veins directly entering portal vein
+ - Left gastric (coronary) vein: Provides drainage of lesser curvature of stomach and lower esophagus
+ - Superior pancreaticoduodenal vein: Drains duodenum and pancreatic head
+ - Cystic vein: Drains gallbladder
+ - IVC: Other venous system with multiple tributaries providing drainage to abdominal and pelvic viscera; also drains lower extremities
+ - Common iliac veins: Provide outflow drainage for both lower extremities and various pelvic viscera
+ - Lower extremity and pelvic venous drainage described separately in "Venous Anatomy" document
+ - Lumbar veins
+ - Interconnected on either side by vertically coursing ascending lumbar veins; latter connect with azygos vein (on right) and hemiazygos vein (on left)
+ - Also anastomose with tributaries of epigastric veins
+ - Right gonadal vein: Drains right ovary and fallopian tube (females) or right testis and spermatic cord (males)
+ - Left gonadal vein drains into left renal vein
+ - Renal veins
+ - Described in "Renal Vasculature Anatomy" document
+ - Right adrenal vein: Drains right adrenal grand
+ - Left adrenal vein drains into left renal vein; shares common trunk with left inferior phrenic vein
+ - Right inferior phrenic vein: Drains hemidiaphragm
+ - Left inferior phrenic vein drains into left renal vein
+ - Hepatic veins: Provide venous drainage for entire liver; enter IVC just below diaphragm
+ - Right, middle, and left hepatic veins constitute normal venous anatomy
+
+ 2bdf4346-c344-4446-ae81-7fc9245122fc
+
+## References
+
+## Selected References
+
+1. [Ibukuro K et al: Spatial relationship between the hepatic artery and portal vein based on the fusion image of CT angiography and CT arterial portography: the left hemiliver. AJR Am J Roentgenol. 200(5):1160-6, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23617505%5Bpmid%5D)
+1. [Kiyosue H et al: Multidetector CT anatomy of drainage routes of gastric varices: a pictorial review. Radiographics. 33(1):87-100, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23322829%5Bpmid%5D)
+1. [Chen H et al: Anatomic variation of the celiac trunk with special reference to hepatic artery patterns. Ann Anat. 191(4):399-407, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19540742%5Bpmid%5D)
+1. [Walker TG: Mesenteric vasculature and collateral pathways. Semin Intervent Radiol. 26(3):167-74, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=21326561%5Bpmid%5D)
+1. [Chaib E et al: The main hepatic anatomic variations for the purpose of split-liver transplantation. Hepatogastroenterology. 54(75):688-92, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17591042%5Bpmid%5D)
+1. [Song SY et al: Nonhepatic arteries originating from the hepatic arteries: angiographic analysis in 250 patients. J Vasc Interv Radiol. 17(3):461-9, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16567670%5Bpmid%5D)
+1. [Gourley EJ et al: The meandering mesenteric artery: a historic review and surgical implications. Dis Colon Rectum. 48(5):996-1000, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15933893%5Bpmid%5D)
+1. [Nonent M et al: Celiac-bimesenteric trunk: anatomic and radiologic description--case report. Radiology. 220(2):489-91, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11477258%5Bpmid%5D)
+1. [Amonoo-Kuofi HS et al: Anomalous origins of colic arteries. Clin Anat. 8(4):288-93, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7552967%5Bpmid%5D)
+1. [Fisher DF Jr et al: Collateral mesenteric circulation. Surg Gynecol Obstet. 164(5):487-92, 1987](http://www.ncbi.nlm.nih.gov/pubmed/?term=3554567%5Bpmid%5D)
+1. [Kuhns LR et al: Normal roentgen variant: aberrant right hepatic artery on computed tomography. Radiology. 135(2):392, 1980](http://www.ncbi.nlm.nih.gov/pubmed/?term=7367634%5Bpmid%5D)
+1. [Michels NA: Newer anatomy of the liver and its variant blood supply and collateral circulation. Am J Surg. 112(3):337-47, 1966](http://www.ncbi.nlm.nih.gov/pubmed/?term=5917302%5Bpmid%5D)
+
+
+## Images
+
+
+### Abdominal Aorta and Branches
+
+
+*Graphic shows the abdominal aorta and its major branches. The abdominal aorta begins at diaphragmatic level and lies on the left of midline, coursing anterior to the vertebral bodies and parallel to the inferior vena cava. The abdominal aorta decreases in caliber as it yields various branches and terminates at its bifurcation into the common iliac arteries. The major abdominal aortic branches supply the abdominal viscera and kidneys. The aorta also yields multiple parietal branches (inferior phrenic, lumbar, and median sacral arteries).*
+
+
+*Graphic shows the abdominal aorta and its major branches. The abdominal aorta begins at diaphragmatic level and lies on the left of midline, coursing anterior to the vertebral bodies and parallel to the inferior vena cava. The abdominal aorta decreases in caliber as it yields various branches and terminates at its bifurcation into the common iliac arteries. The major abdominal aortic branches supply the abdominal viscera and kidneys. The aorta also yields multiple parietal branches (inferior phrenic, lumbar, and median sacral arteries).*
+
+
+*Graphic shows the abdominal aorta and its major branches. The abdominal aorta begins at diaphragmatic level and lies on the left of midline, coursing anterior to the vertebral bodies and parallel to the inferior vena cava. The abdominal aorta decreases in caliber as it yields various branches and terminates at its bifurcation into the common iliac arteries. The major abdominal aortic branches supply the abdominal viscera and kidneys. The aorta also yields multiple parietal branches (inferior phrenic, lumbar, and median sacral arteries).*
+
+
+*Graphic shows the abdominal aorta and its major branches. The abdominal aorta begins at diaphragmatic level and lies on the left of midline, coursing anterior to the vertebral bodies and parallel to the inferior vena cava. The abdominal aorta decreases in caliber as it yields various branches and terminates at its bifurcation into the common iliac arteries. The major abdominal aortic branches supply the abdominal viscera and kidneys. The aorta also yields multiple parietal branches (inferior phrenic, lumbar, and median sacral arteries).*
+
+
+*Abdominal aorta DSA shows that the celiac, middle adrenal, superior mesenteric, gonadal, and inferior mesenteric arteries are the major visceral arteries arising from the aorta. The celiac, superior, and inferior mesenteric arteries supply the gastrointestinal system distal to the esophagus, and the liver, spleen, and pancreas. The middle adrenal arteries that supply the adrenal glands are supplemented by the superior and inferior adrenal arteries that arise as branches of the inferior phrenic and renal arteries, respectively. The gonadal arteries supply the ovaries and fallopian tubes (females) or testes and spermatic cords (males).*
+
+
+*Abdominal aorta DSA shows that the celiac, middle adrenal, superior mesenteric, gonadal, and inferior mesenteric arteries are the major visceral arteries arising from the aorta. The celiac, superior, and inferior mesenteric arteries supply the gastrointestinal system distal to the esophagus, and the liver, spleen, and pancreas. The middle adrenal arteries that supply the adrenal glands are supplemented by the superior and inferior adrenal arteries that arise as branches of the inferior phrenic and renal arteries, respectively. The gonadal arteries supply the ovaries and fallopian tubes (females) or testes and spermatic cords (males).*
+
+
+*Abdominal aorta DSA shows that the celiac, middle adrenal, superior mesenteric, gonadal, and inferior mesenteric arteries are the major visceral arteries arising from the aorta. The celiac, superior, and inferior mesenteric arteries supply the gastrointestinal system distal to the esophagus, and the liver, spleen, and pancreas. The middle adrenal arteries that supply the adrenal glands are supplemented by the superior and inferior adrenal arteries that arise as branches of the inferior phrenic and renal arteries, respectively. The gonadal arteries supply the ovaries and fallopian tubes (females) or testes and spermatic cords (males).*
+
+
+### Celiac Artery Anatomy
+
+
+*Graphic shows normal anatomy of the celiac artery and its branches. The celiac artery supplies blood to the liver, stomach, lower esophagus, spleen, proximal duodenum, and pancreas. The first major division of the celiac artery is the left gastric artery, which supplies the distal esophagus and gastric cardia. It anastomoses with short gastric branches from the splenic artery, which is the largest celiac branch, and supplies the pancreas, spleen, and stomach. The common hepatic artery supplies the liver, stomach, duodenum, gallbladder, and pancreas. It becomes the proper hepatic artery after yielding the gastroduodenal artery.*
+
+
+*Graphic shows normal anatomy of the celiac artery and its branches. The celiac artery supplies blood to the liver, stomach, lower esophagus, spleen, proximal duodenum, and pancreas. The first major division of the celiac artery is the left gastric artery, which supplies the distal esophagus and gastric cardia. It anastomoses with short gastric branches from the splenic artery, which is the largest celiac branch, and supplies the pancreas, spleen, and stomach. The common hepatic artery supplies the liver, stomach, duodenum, gallbladder, and pancreas. It becomes the proper hepatic artery after yielding the gastroduodenal artery.*
+
+
+*Graphic shows normal anatomy of the celiac artery and its branches. The celiac artery supplies blood to the liver, stomach, lower esophagus, spleen, proximal duodenum, and pancreas. The first major division of the celiac artery is the left gastric artery, which supplies the distal esophagus and gastric cardia. It anastomoses with short gastric branches from the splenic artery, which is the largest celiac branch, and supplies the pancreas, spleen, and stomach. The common hepatic artery supplies the liver, stomach, duodenum, gallbladder, and pancreas. It becomes the proper hepatic artery after yielding the gastroduodenal artery.*
+
+
+*The celiac artery, the 1st major abdominal aortic branch, arises at the level of the upper margin of the 1st lumbar vertebra. Celiac artery DSA shows variant arterial anatomy, as the origin of the left hepatic artery is replaced to the left gastric artery. This is a common anatomic variant that occurs in up to 10% of individuals. Variations in the hepatic arterial anatomy may be seen in 40-45% of cases, of which the most frequent is replacement of the right hepatic artery origin to the superior mesenteric artery.*
+
+
+*The celiac artery, the 1st major abdominal aortic branch, arises at the level of the upper margin of the 1st lumbar vertebra. Celiac artery DSA shows variant arterial anatomy, as the origin of the left hepatic artery is replaced to the left gastric artery. This is a common anatomic variant that occurs in up to 10% of individuals. Variations in the hepatic arterial anatomy may be seen in 40-45% of cases, of which the most frequent is replacement of the right hepatic artery origin to the superior mesenteric artery.*
+
+
+*The celiac artery, the 1st major abdominal aortic branch, arises at the level of the upper margin of the 1st lumbar vertebra. Celiac artery DSA shows variant arterial anatomy, as the origin of the left hepatic artery is replaced to the left gastric artery. This is a common anatomic variant that occurs in up to 10% of individuals. Variations in the hepatic arterial anatomy may be seen in 40-45% of cases, of which the most frequent is replacement of the right hepatic artery origin to the superior mesenteric artery.*
+
+
+### Superior and Inferior Mesenteric Arteries
+
+
+*Superior mesenteric artery DSA shows normal vascular anatomy. The artery arises from the abdominal aorta, below the celiac artery origin, usually at the 1st lumbar vertebral level. It supplies the bowel from the lower duodenum through the splenic flexure of the transverse colon and also supplies the pancreas. The middle, right, and ileocolic branches of the superior mesenteric artery anastomose along the mesenteric border of the colon.*
+
+
+*Superior mesenteric artery DSA shows normal vascular anatomy. The artery arises from the abdominal aorta, below the celiac artery origin, usually at the 1st lumbar vertebral level. It supplies the bowel from the lower duodenum through the splenic flexure of the transverse colon and also supplies the pancreas. The middle, right, and ileocolic branches of the superior mesenteric artery anastomose along the mesenteric border of the colon.*
+
+
+*Superior mesenteric artery DSA shows normal vascular anatomy. The artery arises from the abdominal aorta, below the celiac artery origin, usually at the 1st lumbar vertebral level. It supplies the bowel from the lower duodenum through the splenic flexure of the transverse colon and also supplies the pancreas. The middle, right, and ileocolic branches of the superior mesenteric artery anastomose along the mesenteric border of the colon.*
+
+
+*DSA shows both normal and variant anatomy of the inferior mesenteric artery, which arises from the infrarenal abdominal aorta above the aortic bifurcation. It supplies the left colon from the splenic flexure through the upper rectum. In the splenic flexure, the left colic branch of the inferior mesenteric artery anastomoses with the middle colic artery via the marginal artery of Drummond, thus connecting the inferior and superior mesenteric arteries. In this example, however, the left branch of the middle colic artery arises from the dorsal pancreatic artery and connects the inferior mesenteric and celiac arteries. The major inferior mesenteric artery branches are the left colic, sigmoidal, and superior rectal arteries.*
+
+
+*DSA shows both normal and variant anatomy of the inferior mesenteric artery, which arises from the infrarenal abdominal aorta above the aortic bifurcation. It supplies the left colon from the splenic flexure through the upper rectum. In the splenic flexure, the left colic branch of the inferior mesenteric artery anastomoses with the middle colic artery via the marginal artery of Drummond, thus connecting the inferior and superior mesenteric arteries. In this example, however, the left branch of the middle colic artery arises from the dorsal pancreatic artery and connects the inferior mesenteric and celiac arteries. The major inferior mesenteric artery branches are the left colic, sigmoidal, and superior rectal arteries.*
+
+
+*DSA shows both normal and variant anatomy of the inferior mesenteric artery, which arises from the infrarenal abdominal aorta above the aortic bifurcation. It supplies the left colon from the splenic flexure through the upper rectum. In the splenic flexure, the left colic branch of the inferior mesenteric artery anastomoses with the middle colic artery via the marginal artery of Drummond, thus connecting the inferior and superior mesenteric arteries. In this example, however, the left branch of the middle colic artery arises from the dorsal pancreatic artery and connects the inferior mesenteric and celiac arteries. The major inferior mesenteric artery branches are the left colic, sigmoidal, and superior rectal arteries.*
+
+
+### Portal Venous Anatomy
+
+
+*The portal vein, which is formed by the confluence of the superior mesenteric and splenic veins, also receives blood from the inferior mesenteric, gastric, and cystic veins. Immediately before reaching the liver, the portal vein divides into right and left branches that divide into smaller venous branches and ultimately portal venules. Each venule courses alongside a hepatic arteriole, and the 2 vessels form the vascular components of the portal triad. These vessels empty into the hepatic sinusoids to supply blood to the liver. The portal vein drains blood from the gastrointestinal tract and spleen and provides ~ 70% of the hepatic blood supply.*
+
+
+*The portal vein, which is formed by the confluence of the superior mesenteric and splenic veins, also receives blood from the inferior mesenteric, gastric, and cystic veins. Immediately before reaching the liver, the portal vein divides into right and left branches that divide into smaller venous branches and ultimately portal venules. Each venule courses alongside a hepatic arteriole, and the 2 vessels form the vascular components of the portal triad. These vessels empty into the hepatic sinusoids to supply blood to the liver. The portal vein drains blood from the gastrointestinal tract and spleen and provides ~ 70% of the hepatic blood supply.*
+
+
+*The portal vein, which is formed by the confluence of the superior mesenteric and splenic veins, also receives blood from the inferior mesenteric, gastric, and cystic veins. Immediately before reaching the liver, the portal vein divides into right and left branches that divide into smaller venous branches and ultimately portal venules. Each venule courses alongside a hepatic arteriole, and the 2 vessels form the vascular components of the portal triad. These vessels empty into the hepatic sinusoids to supply blood to the liver. The portal vein drains blood from the gastrointestinal tract and spleen and provides ~ 70% of the hepatic blood supply.*
+
+
+*The portal vein normally branches into the right and left portal veins. The right portal vein divides into anterior and posterior branches, with the former supplying segments 5 and 8, and the latter supplying segments 6 and 7. The left portal vein usually supplies hepatic segments 2, 3, and 4. Anatomic variants are seen in 20-35% of individuals, as in this portal venogram, wherein the segment 4 branches arise from the right rather than the left portal vein. Portal vein variants increase the risk of bile duct hilar anatomical variation.*
+
+
+*The portal vein normally branches into the right and left portal veins. The right portal vein divides into anterior and posterior branches, with the former supplying segments 5 and 8, and the latter supplying segments 6 and 7. The left portal vein usually supplies hepatic segments 2, 3, and 4. Anatomic variants are seen in 20-35% of individuals, as in this portal venogram, wherein the segment 4 branches arise from the right rather than the left portal vein. Portal vein variants increase the risk of bile duct hilar anatomical variation.*
+
+
+*The portal vein normally branches into the right and left portal veins. The right portal vein divides into anterior and posterior branches, with the former supplying segments 5 and 8, and the latter supplying segments 6 and 7. The left portal vein usually supplies hepatic segments 2, 3, and 4. Anatomic variants are seen in 20-35% of individuals, as in this portal venogram, wherein the segment 4 branches arise from the right rather than the left portal vein. Portal vein variants increase the risk of bile duct hilar anatomical variation.*
+
diff --git a/docs_md/articles/abnormal-shape-configuration-of-corpus-callosum_c75baee2-7a4c-4fd8-9b3f-cc662d0f1c18.md b/docs_md/articles/abnormal-shape-configuration-of-corpus-callosum_c75baee2-7a4c-4fd8-9b3f-cc662d0f1c18.md
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@@ -0,0 +1,505 @@
+---
+title: "Abnormal Shape/Configuration of Corpus Callosum"
+docid: "c75baee2-7a4c-4fd8-9b3f-cc662d0f1c18"
+authors:
+ - key: "47381de4-c9fd-4999-8dd0-1808cd72db6b"
+ value: "Luke L. Linscott, MD"
+breadcrumbs:
+ -
+ name: "Brain"
+ slug: "brain"
+ treeNodeId: "0361042c-9c90-48e4-864e-a2e6428ee9c9"
+ -
+ name: "Differential Diagnosis"
+ slug: "differential-diagnosis"
+ treeNodeId: "0de9c040-6d20-4d13-a2bb-35101f8d7945"
+ -
+ name: "Supratentorial Brain Parenchyma"
+ slug: "supratentorial-brain-parenchyma"
+ treeNodeId: "35b2ee23-3033-430f-ac69-474732630009"
+ -
+ name: "Anatomically Based Differentials"
+ slug: "anatomically-based-differentials"
+ treeNodeId: "ff7f1675-c8d2-41af-8821-3f7eca8fc964"
+ -
+ name: "Abnormal Shape/Configuration of Corpus Callosum"
+ slug: "abnormal-shapeconfiguration-of-cor-"
+ treeNodeId: null
+category: "Brain"
+documentVersionId: "3f5b6df0-45ee-4b5b-8da1-73e1402d8857"
+imageCount: 53
+lastUpdated: "02/01/23"
+pageDescription: "Abnormal Shape/Configuration of Corpus Callosum"
+pageKeywords: "Brain, Differential Diagnosis, Supratentorial Brain Parenchyma, Anatomically Based Differentials, Abnormal Shape/Configuration of Corpus Callosum"
+pageTitle: "Abnormal Shape/Configuration of Corpus Callosum | STATdx"
+enhancedTitle: "Abnormal Shape/Configuration of Corpus Callosum"
+type: "DDX"
+references: true
+breadcrumbs:
+ - "Brain"
+ - "Differential Diagnosis"
+ - "Supratentorial Brain Parenchyma"
+ - "Anatomically Based Differentials"
+ - "Abnormal Shape/Configuration of Corpus Callosum"
+---
+## ESSENTIAL INFORMATION
+
+- ### Key Differential Diagnosis Issues
+
+
+ - Clinical features to consider
+ - Normal corpus callosum (CC) varies in thickness & shape
+ - Associated anomalies portend worse prognosis
+ - If not congenital anomaly, clinical history is crucial
+ - Prior surgical history, prematurity, etc.
+ - Corpus callosotomy, shunt placement, endoscopic 3rd ventriculostomy
+ - Imaging features to consider
+ - Isolated callosal dysgenesis is not common → additional malformations in > 50%
+ - Malformations of cortical development
+ - Noncallosal midline anomalies
+ - Abnormal brainstem or cerebellum
+ - Look for parenchymal abnormalities to identify etiology
+ - White matter (WM) volume loss, prior infarction, diffuse axonal injury
+ - Ventricular abnormalities are common
+ - Colpocephaly → CC agenesis/dysgenesis, Chiari 2
+ - Enlarged, angular ventricles → periventricular leukomalacia
+ - Modality considerations
+ - CT: Helpful to distinguish cellular vs. inflammatory
+ - ↑ density → cellular mass, hemorrhage
+ - ↓ density → edema, inflammation
+ - MR: Best spatial & contrast resolution
+ - Sagittal imaging is critical for evaluation of CC
+ - 3D acquisitions allow multiplanar reconstructions
+- ### Helpful Clues for Common Diagnoses
+
+
+ - **Normal Variant**
+ - Immature CC is thin
+ - Gradually thickens with progressive myelination
+ - Size, shape, & thickness of normal CC vary
+ - Splenium & genu are largest parts of CC
+ - Normal narrowing at junction of body & splenium ("isthmus")
+ - Dorsal surface of fully developed, normally myelinated CC is often "wavy"
+ - **Thin Corpus Callosum**
+ - Many causes (congenital, acquired)
+ - All may result in focal or diffuse callosal thinning
+ - **Periventricular leukomalacia**
+ - Premature infant is at greatest risk
+ - Acute findings: US → ↑ periventricular WM echogenicity
+ - MR: Diffusion restriction, ↑ T1, ↓ T2
+ - Subacute findings: Cavitation, periventricular cysts
+ - Chronic findings: ↓ volume of periventricular WM
+ - Thin posterior body & splenium are most common
+ - Ventricular enlargement shows angular margins
+ - **Hypoxic-ischemic encephalopathy**
+ - Loss of cerebral WM → thin CC
+ - May occur with profound or partial prolonged injury
+ - Profound: Often perirolandic → posterior body CC
+ - Partial prolonged: Watershed injury → entire CC
+ - **Chronic cerebral infarction**
+ - Axonal loss → focal/diffuse thinning of CC
+ - **Obstructive hydrocephalus**
+ - Look for obstructing lesion (e.g., tumor, aqueductal stenosis) or sequelae of prior hemorrhage/infection
+ - Acute: CC stretched, bowed upward
+ - Chronic: Thinned, irregular (sequela of CC impingement against falx & chronic WM injury from hydrocephalus)
+ - **Chemotherapy & radiation therapy**
+ - WM injury with volume loss
+ - e.g., chronic methotrexate toxicity
+ - **Postsurgical Defects**
+ - **Corpus c****allosotomy**
+ - Surgical disruption for intractable epilepsy
+ - Isolated callosotomy or part of functional hemispherotomy
+ - Often imaged in postoperative setting to detect residual neuronal connections across midline
+ - Best seen on sagittal or coronal MR
+ - **Ventricular drainage catheter tract**
+ - Small defect in paramidline CC
+ - Typically with overlying WM parenchymal tract & postoperative skull focus
+ - May see hypointense intracranial catheter ± hyperintense fluid-filled extracranial components
+ - **Endoscopic 3rd ventriculostomy**
+ - Small defect in CC represents scope tract, typically with overlying WM parenchymal tract & postoperative skull focus
+- ### Helpful Clues for Less Common Diagnoses
+
+
+ - **Callosal Agenesis**
+ - Absent WM bridging cerebral hemispheres
+ - Absent septum pellucidum
+ - Absent cingulate gyrus with vertically oriented parasagittal sulci radiating to high-riding 3rd ventricle
+ - Lateral ventricles: Colpocephaly, upturned frontal horns
+ - Probst bundles (WM tracts that would have formed CC) lie along medial aspects of lateral ventricles
+ - **Primary Callosal Dysgenesis**
+ - Absence of 1 or all segments
+ - Rostrum & splenium are most likely to be deficient
+ - Remnants vary in size, shape, configuration
+ - Look for other associated malformations
+ - "Micro" CC: Small but well formed, often syndromic
+ - "Mega" CC: Megalencephalic (bulky WM) vs. small to normal brain (syndromic)
+ - **Chiari 2 Malformation**
+ - Constellation of intracranial findings secondary to open neural tube defect (e.g., myelomeningocele)
+ - Small posterior fossa, towering cerebellum that wraps around dorsal brainstem, small elongated 4th ventricle, vermian/tonsillar extension into upper cervical spine, "beaked" tectum, scalloped clivus
+ - Degree of callosal dysgenesis is highly variable
+ - Correlates with severity of hydrocephalus
+ - **Neoplasm**
+ - **Glioblastoma**
+ - Common in adults, uncommon in children
+ - Butterfly glioma crosses CC
+ - Central necrosis + thick, irregular rim enhancement
+ - **Lymphoma**
+ - NECT: Hyperdense
+ - Strong, uniform enhancement
+ - **Pericallosal Lipoma**
+ - 40-50% occur in interhemispheric fissure
+ - Almost always located in subarachnoid space; blood vessels & cranial nerves course through lipoma
+ - 2 morphologic types
+ - Bulky, mass-like ("tubulonodular" type)
+ - Thin, dorsal to body/splenium ("curvilinear" type)
+ - Midline lipomas may be part of more general midline developmental disorder; CC is often deficient
+ - **Neurofibromatosis Type 1 (NF1)**
+ - Patients with NF1 have ↑ CC volume
+ - Sometimes markedly so & qualitatively evident
+ - Nonenhancing lesions of NF1 can occur in CC
+ - If focal lesions of CC enhance, suggest low-grade tumor
+ - **Holoprosencephaly**
+ - Alobar
+ - CC absent
+ - "Pancake" anterior cerebral tissue
+ - Monoventricle with large dorsal "cyst"
+ - Semilobar
+ - Frontal lobe fusion/hypoplasia; caudate head fusion
+ - Splenium may be present
+ - Lobar
+ - Genu sometimes present; GM often crosses with genu
+ - Absent anterior midline falx & fissure
+ - Middle interhemispheric variant (a.k.a. syntelencephaly)
+ - Splenium & genu present, body deficient
+ - Middle CC body "dips"
+ - GM crosses midline in expected location of CC body
+ - ± bilateral perisylvian polymicrogyria
+- ### Helpful Clues for Rare Diagnoses
+
+
+ - **Hypomyelination**
+ - Primary pathologic hypomyelination is rare
+ - e.g., Pelizaeus-Merzbacher, *TUBB4A* disorders
+ - **Inherited Metabolic Leukodystrophies**
+ - **Metachromatic leukodystrophy**
+ - Entire CC affected, genu & splenium worst
+ - **X-linked adrenoleukodystrophy**
+ - Most commonly involves splenium
+ - **Alexander disease**
+ - Frontal lobe & genu involvement
+ - **Krabbe disease**
+ - Central WM + deep gray nuclei (especially thalamus)
+
+## References
+
+## Selected References
+
+1. [Moradi B et al: Fetal corpus callosum abnormalities: ultrasound and magnetic resonance imaging role. J Clin Ultrasound. 50(7):989-1003, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35488776%5Bpmid%5D)
+1. [Shwe WH et al: Outcome of agenesis of the corpus callosum diagnosed by fetal MRI. Pediatr Neurol. 135:44-51, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35988419%5Bpmid%5D)
+1. [ENSO Working Group.: Role of prenatal magnetic resonance imaging in fetuses with isolated anomalies of corpus callosum: multinational study. Ultrasound Obstet Gynecol. 58(1):26-33, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33596324%5Bpmid%5D)
+1. [Al-Hashim AH et al: Corpus callosum abnormalities: neuroradiological and clinical correlations. Dev Med Child Neurol. 58(5):475-84, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26661037%5Bpmid%5D)
+1. [Edwards TJ et al: Clinical, genetic and imaging findings identify new causes for corpus callosum development syndromes. Brain. 137(Pt 6):1579-613, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24477430%5Bpmid%5D)
+1. [Battal B et al: Corpus callosum: normal imaging appearance, variants and pathologic conditions. J Med Imaging Radiat Oncol. 54(6):541-9, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=21199431%5Bpmid%5D)
+1. [Bourekas EC et al: Lesions of the corpus callosum: MR imaging and differential considerations in adults and children. AJR Am J Roentgenol. 179(1):251-7, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12076946%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+**Normal Variant**
+*Midline sagittal T1 MR in a normal term neonate shows a thin, unmyelinated corpus callosum (CC)
. The CC will gradually thicken as it myelinates from posterior to anterior. Note that the entire pituitary gland normally shows T1 shortening
in the 1st few weeks of life.*
+
+
+**Normal Variant**
+*Midline sagittal T1 MR in a normal term neonate shows a thin, unmyelinated corpus callosum (CC)
. The CC will gradually thicken as it myelinates from posterior to anterior. Note that the entire pituitary gland normally shows T1 shortening
in the 1st few weeks of life.*
+
+
+**Normal Variant**
+*Midline sagittal T1 MR in a normal term neonate shows a thin, unmyelinated corpus callosum (CC)
. The CC will gradually thicken as it myelinates from posterior to anterior. Note that the entire pituitary gland normally shows T1 shortening
in the 1st few weeks of life.*
+
+
+**Normal Variant**
+*Midline sagittal T1 MR in a normal term neonate shows a thin, unmyelinated corpus callosum (CC)
. The CC will gradually thicken as it myelinates from posterior to anterior. Note that the entire pituitary gland normally shows T1 shortening
in the 1st few weeks of life.*
+
+
+**Normal Variant**
+*Midline sagittal T1 MR in a 13-year-old with headaches shows a normal variant morphology of the CC with relative thinning of the posterior body
. This should not be mistaken for a sign of white matter (WM) volume loss.*
+
+
+**Periventricular Leukomalacia**
+*Axial FLAIR MR in a 7-year-old with a history of prematurity & periventricular leukomalacia (PVL) shows severe WM volume loss
with relatively little signal abnormality. Also note the angular margins
of the expanded ventricular occipital horns, consistent with PVL related to extreme prematurity.*
+
+
+**Periventricular Leukomalacia**
+*Midline sagittal T1 MR in the same patient shows marked thinning of the posterior body & splenium of the CC
due to WM volume loss. This is the most common area of CC involvement in PVL.*
+
+
+**Periventricular Leukomalacia**
+*Midline sagittal T1 MR in the same patient shows marked thinning of the posterior body & splenium of the CC
due to WM volume loss. This is the most common area of CC involvement in PVL.*
+
+
+**Hypoxic-Ischemic Encephalopathy**
+*Axial T2 MR in a 9-year-old with a history of hypoxic-ischemic encephalopathy (HIE) at birth shows extensive gliosis & encephalomalacia causing WM volume & signal abnormality in a watershed distribution
. This results in marked CC thinning.*
+
+
+**Hypoxic-Ischemic Encephalopathy**
+*Midline sagittal T1 MR in the same patient shows marked thinning of the CC
secondary to WM loss as a consequence of the remote HIE injury.*
+
+
+**Hypoxic-Ischemic Encephalopathy**
+*Midline sagittal T1 MR in the same patient shows marked thinning of the CC
secondary to WM loss as a consequence of the remote HIE injury.*
+
+
+**Obstructive Hydrocephalus**
+*Midline sagittal T2 MR in a neonate with posthemorrhagic hydrocephalus shows a stretched & thinned CC
. Note the enlarged lateral
, 3rd
, & 4th
ventricles as well as thin T2 hypointensity
along the brainstem, consistent with hemosiderin deposition.*
+
+
+**Obstructive Hydrocephalus**
+*Midline sagittal T2 MR in a neonate with posthemorrhagic hydrocephalus shows a stretched & thinned CC
. Note the enlarged lateral
, 3rd
, & 4th
ventricles as well as thin T2 hypointensity
along the brainstem, consistent with hemosiderin deposition.*
+
+
+**Obstructive Hydrocephalus**
+*Midline sagittal T1 MR in the same patient 1 year after shunting shows a thinned & dysmorphic CC
as well as numerous thin, pencil-like gyri (stenogyria)
.*
+
+
+**Obstructive Hydrocephalus**
+*Midline sagittal T1 MR in the same patient 1 year after shunting shows a thinned & dysmorphic CC
as well as numerous thin, pencil-like gyri (stenogyria)
.*
+
+
+**Corpus Callosotomy**
+*Coronal FLAIR MR shows changes of a left functional hemispherotomy with a WM disconnection
& insular decortication
. Corpus callosotomy may be performed in isolation or as part of a more extensive functional hemispherotomy, as in this patient.*
+
+
+**Corpus Callosotomy**
+*Coronal FLAIR MR shows changes of a left functional hemispherotomy with a WM disconnection
& insular decortication
. Corpus callosotomy may be performed in isolation or as part of a more extensive functional hemispherotomy, as in this patient.*
+
+
+**Corpus Callosotomy**
+*Coronal T2 MR shows absence of the midline CC
with persistent paramidline callosal tissue
, consistent with an isolated surgical callosotomy.*
+
+
+**Corpus Callosotomy**
+*Coronal T2 MR shows absence of the midline CC
with persistent paramidline callosal tissue
, consistent with an isolated surgical callosotomy.*
+
+
+**Ventricular Drainage Catheter Tract**
+*Paramidline sagittal T1 MR in a 12-year-old with Chiari 2 malformation shows a ventricular shunt catheter tract
in the anterior body of the CC. Note the caudal migration of the cerebellum & brainstem
, consistent with Chiari 2.*
+
+
+**Endoscopic 3rd Ventriculostomy**
+*Paramidline sagittal T2 MR in a teenager with a history of a prior endoscopic 3rd ventriculostomy shows a linear defect
in the parasagittal body of the CC. The defect represents the site of surgical access for the scope to enter the 3rd ventricle.*
+
+
+**Endoscopic 3rd Ventriculostomy**
+*Paramidline sagittal T2 MR in a teenager with a history of a prior endoscopic 3rd ventriculostomy shows a linear defect
in the parasagittal body of the CC. The defect represents the site of surgical access for the scope to enter the 3rd ventricle.*
+
+
+**Callosal Agenesis**
+*Coronal T2 MR in a 4-year-old with callosal agenesis shows widely spaced, upturned lateral ventricular frontal horns
, a high-riding 3rd ventricle
, & bilateral Probst bundles
. Also note the extensive periventricular gray matter (GM) heterotopia
.*
+
+
+**Callosal Dysgenesis**
+*Midline sagittal T1 MR in a 5-month-old with isolated callosal dysgenesis shows a very short & thin CC
with no evident rostrum or splenium. Isolated callosal dysgenesis is uncommon. Associated anomalies should be carefully sought.*
+
+
+**Callosal Dysgenesis**
+*Midline sagittal T1 MR in a 7-year-old with multiple anomalies shows a short, thin, & dysmorphic CC with a poorly formed splenium
& rostrum
.*
+
+
+**Chiari 2 Malformation**
+*Midline sagittal T2 MR in a child with a repaired myelomeningocele & Chiari 2 malformation (with beaked tectum
, small 4th ventricle
, & scalloped clivus
) shows a thinned & dysmorphic CC
.*
+
+
+**Chiari 2 Malformation**
+*Midline sagittal T2 MR in a child with a repaired myelomeningocele & Chiari 2 malformation (with beaked tectum
, small 4th ventricle
, & scalloped clivus
) shows a thinned & dysmorphic CC
.*
+
+
+**Glioblastoma**
+*Coronal T2 MR in a 10-year-old with glioblastoma IDH-wildtype shows mass-like infiltrative signal
crossing the midline through an expanded CC. Infiltrative high-grade glial neoplasms should be considered whenever such a finding is encountered, as they commonly spread along WM tracts, such as the CC.*
+
+
+**Lymphoma**
+*Midline sagittal T2 MR shows expansion & increased signal in the rostrum & anterior genu of the CC
, consistent with tumor infiltration/edema in this patient with CNS lymphoma.*
+
+
+**Lymphoma**
+*Midline sagittal T2 MR shows expansion & increased signal in the rostrum & anterior genu of the CC
, consistent with tumor infiltration/edema in this patient with CNS lymphoma.*
+
+
+**Pericallosal Lipoma**
+*Midline sagittal T1 MR in a 4-month-old shows a T1-hyperintense lipoma
along the dorsal CC with associated absence of the splenium
.*
+
+
+**Neurofibromatosis Type 1**
+*Midline sagittal T1 MR in a 15-year-old with neurofibromatosis type 1 (NF1) shows diffuse, marked thickening of the entire CC, a finding that can be seen in NF1. Look for associated findings of NF1, such as nonenhancing signal abnormalities of the globus pallidus & medial cerebellum, optic pathway gliomas, & plexiform neurofibromas.*
+
+
+**Holoprosencephaly**
+*Midline sagittal T2 MR shows absence of the CC in a patient with alobar holoprosencephaly. There is continuity of frontal WM & GM across the midline with a large dorsal cyst
that communicates with a monoventricle
. Note the lack of a vermian primary fissure due to associated rhombencephalosynapsis.*
+
+
+**Holoprosencephaly**
+*Midline sagittal T2 MR shows absence of the CC in a patient with alobar holoprosencephaly. There is continuity of frontal WM & GM across the midline with a large dorsal cyst
that communicates with a monoventricle
. Note the lack of a vermian primary fissure due to associated rhombencephalosynapsis.*
+
+
+**Holoprosencephaly**
+*Midline sagittal T1 MR in a 2-year-old with semilobar holoprosencephaly shows absence of a normal CC & extension of cortical GM
across the midline.*
+
+
+**Holoprosencephaly**
+*Midline sagittal T1 MR in a teenager with the middle interhemispheric variant of holoprosencephaly shows an intact CC anteriorly
& posteriorly
but abnormal extension of GM
across the midline in the expected location of the CC body. The abnormal body of the CC typically "dips" down toward the interthalamic adhesion.*
+
+
+**Holoprosencephaly**
+*Midline sagittal T1 MR in a teenager with the middle interhemispheric variant of holoprosencephaly shows an intact CC anteriorly
& posteriorly
but abnormal extension of GM
across the midline in the expected location of the CC body. The abnormal body of the CC typically "dips" down toward the interthalamic adhesion.*
+
+
+**Holoprosencephaly**
+*Coronal T2 MR in the same patient with syntelencephaly shows abnormal GM
crossing the midline along the CC WM
. Also note the azygous internal carotid artery (ICA)
.*
+
+
+**Holoprosencephaly**
+*Coronal T2 MR in the same patient with syntelencephaly shows abnormal GM
crossing the midline along the CC WM
. Also note the azygous internal carotid artery (ICA)
.*
+
+
+**Metachromatic Leukodystrophy**
+*Coronal T2 MR in a 13-year-old girl with metachromatic leukodystrophy shows symmetric extensive WM signal abnormality
with preservation of the subcortical WM
. Note the marked thinning of the CC
.*
+
+
+**X-Linked Adrenoleukodystrophy**
+*Axial FLAIR MR in a 14-year-old boy with X-linked adrenoleukodystrophy (ALD) shows symmetric increased FLAIR signal intensity
that crosses the splenium
of the CC. This is the most common distribution of signal abnormality in X-linked ALD.*
+
+
+### Additional Images
+
+
+**Normal Variant**
+*Midline sagittal 3D SSFP MR with a close-up view of the CC shows normal "wavy" dorsal surface. Note the focal thinning along the posterior body
, a common normal finding.*
+
+
+**Normal Variant**
+*Midline sagittal T1 MR shows a normal neonatal CC
, thin due to an age-appropriate lack of myelin. The cingulate gyrus
is normal.*
+
+
+**Periventricular Leukomalacia**
+*Midline sagittal T1 MR shows diffuse thinning of the posterior CC
, greater than typically seen. The thinning of the CC is secondary to loss of commissural fibers, damaged by PVL.*
+
+
+**Periventricular Leukomalacia**
+*Axial T2 MR in the same child shows marked loss of the right periventricular parenchyma
at the site of a prior grade 4 hemorrhage. The posterior white matter loss correlates with the focal corpus callosum atrophy
.*
+
+
+**Periventricular Leukomalacia**
+*Axial T2 MR in the same child shows marked loss of the right periventricular parenchyma
at the site of a prior grade 4 hemorrhage. The posterior white matter loss correlates with the focal corpus callosum atrophy
.*
+
+
+**Periventricular Leukomalacia**
+*Midline sagittal T1 MR shows marked callosal thinning
in a child whose hydrocephalus follows unilateral grade 4 intraventricular hemorrhage. Note the more severe callosal volume loss posteriorly
.*
+
+
+**Periventricular Leukomalacia**
+*Midline sagittal T1 MR shows marked callosal thinning
in a child whose hydrocephalus follows unilateral grade 4 intraventricular hemorrhage. Note the more severe callosal volume loss posteriorly
.*
+
+
+**Chronic Cerebral Infarction**
+*Midline sagittal T1 MR shows thinning
of the body & splenium of the CC following neonatal parietooccipital ischemia & gliosis from a combination of hypoxic ischemic encephalopathy & hypoglycemia.*
+
+
+**Chronic Cerebral Infarction**
+*Coronal T2 MR shows parietal ulegyria
& marked thinning of the posterior CC
.*
+
+
+**Obstructive Hydrocephalus**
+*Midline sagittal T2 MR shows mild stretching & thinning of the CC due to hydrocephalus. There is obstruction of the aqueduct of Sylvius by a tectal glioma
.*
+
+
+**Chemotherapy & Radiation Therapy**
+*Coronal FLAIR MR shows thinning & gliosis of the CC
& surrounding white matter following therapy for acute lymphoblastic leukemia (ALL).*
+
+
+**Postsurgical Defects**
+*Midline sagittal T1 MR shows a focal defect at the junction of the genu & body of the CC
, which had been the site of a prior surgical approach to this child's suprasellar tumor
.*
+
+
+**Corpus Callosotomy**
+*Paramidline sagittal T1 MR in a 7-year-old with intractable epilepsy shows near-complete absence of the CC
due to surgical discontinuity.*
+
+
+**Corpus Callosotomy**
+*Midline sagittal T2 MR in an 11-year-old with intractable epilepsy who had undergone an isolated corpus callosotomy shows absence of the CC
but presence of a cingulate gyrus
. The presence of a cingulate gyrus would not be expected with congenital agenesis of the CC.*
+
+
+**Callosal Agenesis**
+*Midline sagittal T1 MR shows complete absence of the CC with associated absence of the cingulate gyrus. Note the radial arrangement of parasagittal gyri/sulci
, which point toward the 3rd ventricle.*
+
+
+**Callosal Agenesis**
+*Axial T1 MR in a patient with callosal agenesis shows parallel lateral ventricles with colpocephaly
, resulting in a typical tear-drop shape.*
+
+
+**Callosal Agenesis**
+*Axial T1 MR in a patient with callosal agenesis shows parallel lateral ventricles with colpocephaly
, resulting in a typical tear-drop shape.*
+
+
+**Primary Callosal Dysgenesis**
+*Midline sagittal T1 MR shows only a residual genu
of the CC with absence of the body & splenium as well as truncation of the rostrum.*
+
+
+**Primary Callosal Dysgenesis**
+*Midline sagittal T1 MR in a child with severe microcephaly shows a short, thick CC
.*
+
+
+**Chiari 2 Malformation**
+*Midline sagittal T1 MR shows an abnormal CC with an absent rostrum, small deformed genu, thick body
, & absent splenium in this child with a Chiari 2 malformation due to a myelomeningocele. Note the prominent massa intermedia
, inferiorly beaked tectum
, & caudally displaced elongated 4th ventricle with flattening of the fastigium
.*
+
+
+**Chiari 2 Malformation**
+*Axial T2 MR shows a prominent massa intermedia
& colpocephalic lateral ventricles with periventricular white matter deficiency in Chiari 2. The genu
of the CC, usually seen on axial images, is absent.*
+
+
+**Chiari 2 Malformation**
+*Midline sagittal T2 MR in a 15-month-old with Chiari 2 malformation shows a severely thinned & dysmorphic CC
. Note the typical Chiari 2 features, including a small posterior fossa with caudal herniation of the brainstem & cerebellum, clival scalloping
, elongated 4th ventricle
, & beaked tectum
.*
+
+
+**Glioblastoma**
+*Coronal T1 C+ MR shows a classic "butterfly" glioblastoma multiforme of the corpus callosum
. Central necrosis with an irregular rind of enhancing tumor is typical.*
+
+
+**Glioblastoma**
+*Coronal T1 C+ MR shows a classic "butterfly" glioblastoma multiforme of the corpus callosum
. Central necrosis with an irregular rind of enhancing tumor is typical.*
+
+
+**Lymphoma**
+*Axial T1 C+ MR shows a primary CNS lymphoma involving the splenium of the CC. There is avid, solid enhancement of the tumor
with extension into the adjacent parenchymal white matter.*
+
+
+**Lymphoma**
+*Coronal oblique T1 C+ MR in an 11-year-old with CNS lymphoma shows bifrontal areas of enhancement
, which corresponded to hyperdense areas on CT (not shown). Note the abnormally thickened CC
that is infiltrated by a nonenhancing tumor.*
+
+
+**Pericallosal Lipoma**
+*Midline sagittal T1 MR shows a large pericallosal lipoma with severe dysgenesis of the CC
.*
+
+
+**Pericallosal Lipoma**
+*Axial FLAIR MR shows a large midline lipoma. Two smaller lipomatous masses
protrude into the lateral ventricles.*
+
+
+**Pericallosal Lipoma**
+*Axial FLAIR MR shows a large midline lipoma. Two smaller lipomatous masses
protrude into the lateral ventricles.*
+
+
+**Holoprosencephaly**
+*Axial T1 MR in a patient with holoprosencephaly shows the lack of a midline fissure. WM
is in continuity across the midline. The small basal ganglia
approximate each other. Note the monoventricle
communicating with a dorsal cyst
.*
+
+
+**Holoprosencephaly**
+*Midline sagittal T1 MR shows both WM & GM
crossing midline anterior & posterior to the "dip"
in the CC, where only GM traverses. This is a middle interhemispheric variant of holoprosencephaly (syntelencephaly).*
+
+
+**Holoprosencephaly**
+*Axial T1 MR in the same patient shows GM & WM traversing the midline
in the expected location of the splenium. GM also protrudes
into the ventricular system. The septum pellucidum is absent.*
+
diff --git a/docs_md/articles/aneurysmal-subarachnoid-hemorrhage_9109b698-5ee5-49c4-ba0c-1a86f1fbede4.md b/docs_md/articles/aneurysmal-subarachnoid-hemorrhage_9109b698-5ee5-49c4-ba0c-1a86f1fbede4.md
new file mode 100644
index 0000000..68b14c7
--- /dev/null
+++ b/docs_md/articles/aneurysmal-subarachnoid-hemorrhage_9109b698-5ee5-49c4-ba0c-1a86f1fbede4.md
@@ -0,0 +1,514 @@
+---
+title: "Aneurysmal Subarachnoid Hemorrhage"
+docid: "9109b698-5ee5-49c4-ba0c-1a86f1fbede4"
+authors:
+ - key: "8d5254e9-8dda-478b-8f08-bdee97a32c79"
+ value: "Karen L. Salzman, MD, FACR"
+ - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
+ value: "Anne G. Osborn, MD, FACR"
+breadcrumbs:
+ -
+ name: "Brain"
+ slug: "brain"
+ treeNodeId: "0361042c-9c90-48e4-864e-a2e6428ee9c9"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "948c6f1e-8e18-45f3-bd9d-7a04ab0b8708"
+ -
+ name: "Pathology-Based Diagnoses"
+ slug: "pathology-based-diagnoses"
+ treeNodeId: "d60fe914-7897-46a0-9cd2-f88ffb322f16"
+ -
+ name: "Subarachnoid Hemorrhage and Aneurysms"
+ slug: "subarachnoid-hemorrhage-and-aneury-"
+ treeNodeId: "ba407b7b-06e9-4df6-8644-e26706599742"
+ -
+ name: "Subarachnoid Hemorrhage"
+ slug: "subarachnoid-hemorrhage"
+ treeNodeId: "6ba0efe4-e14d-4374-b6b0-441f5010d9a3"
+ -
+ name: "Aneurysmal Subarachnoid Hemorrhage"
+ slug: "aneurysmal-subarachnoid-hemorrhage"
+ treeNodeId: null
+category: "Brain"
+documentVersionId: "41cd30e1-7d72-49a7-8742-164e0133fc5d"
+imageCount: 35
+lastUpdated: "09/04/25"
+pageDescription: "Aneurysmal Subarachnoid Hemorrhage"
+pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Subarachnoid Hemorrhage and Aneurysms, Subarachnoid Hemorrhage, Aneurysmal Subarachnoid Hemorrhage"
+pageTitle: "Aneurysmal Subarachnoid Hemorrhage | STATdx"
+enhancedTitle: "Aneurysmal Subarachnoid Hemorrhage"
+type: "DX"
+references: true
+breadcrumbs:
+ - "Brain"
+ - "Diagnosis"
+ - "Pathology-Based Diagnoses"
+ - "Subarachnoid Hemorrhage and Aneurysms"
+ - "Subarachnoid Hemorrhage"
+ - "Aneurysmal Subarachnoid Hemorrhage"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - SAH caused by ruptured aneurysm (aSAH)
+ - Saccular (SA) > > dissecting aneurysm (DA)
+- ### Imaging
+
+
+ - CT/CTA
+ - Hyperdense cisterns/sulci on NECT
+ - Within 6 hours after ictus, sensitivity ~ 100%
+ - Distribution varies with aneurysm location
+ - Suprasellar cistern (IC-PCoA, ACoA aneurysms)
+ - Sylvian fissure [middle cerebral artery (MCA) bifurcation]
+ - Prepontine, CPA cisterns [posterior inferior cerebellar artery (PICA), BA bifurcation SA, or vertebral DA]
+ - CTA 90-95% positive if aneurysm ≥ 2 mm
+ - MR/MRA
+ - FLAIR-hyperintense sulci, cisterns (nonspecific)
+ - Blooms on T2* GRE
+ - TOF MRA 85-95% sensitive for aneurysms ≥ 3 mm
+ - DSA
+ - Use if CTA negative or endovascular treatment considered
+ - Low yield if NECT, neurologic examination normal
+ - DSA negative in 15% of aSAH; repeat positive < 5%
+- ### Top Differential Diagnoses
+
+
+ - Nonaneurysmal SAH
+ - Reversible cerebral vasoconstriction syndrome, vasculitis
+ - Pseudo-SAH
+- ### Clinical Issues
+
+
+ - "Thunderclap/worst headache of life"
+ - 50% mortality
+ - Vasospasm 1-3 weeks post aSAH
+ - 20% rebleed within first 2 weeks
+- ### Diagnostic Checklist
+
+
+ - Diffuse, low-density brain makes normal arteries look hyperdense, can mimic aSAH
+
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - Aneurysmal subarachnoid hemorrhage (aSAH)
+- ### Definitions
+
+
+ - Extravasation of blood into subarachnoid space
+ - Usually from ruptured saccular aneurysm (SA)
+ - Less common: Intracranial dissection, dissecting aneurysm (DA)
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Hyperdense basal cisterns, sulci on NECT
+ - #### Location
+
+
+ - Suprasellar, basal, sylvian, interhemispheric cisterns
+ - ± intraventricular hemorrhage (IVH)
+ - aSAH distribution depends on location of SA
+ - aSAH highest near site of rupture
+ - Anterior communicating artery (ACoA) aneurysm → anterior interhemispheric fissure
+ - Middle cerebral artery (MCA) aneurysm → sylvian fissure
+ - Basilar tip, superior cerebellar artery (SCA), posterior inferior cerebellar artery (PICA) SA, or vertebral artery (VA) DA → prepontine cistern, foramen magnum, 4th ventricle
+ - "Culprit" aneurysm sometimes seen as filling defect within hyperdense aSAH
+ - SAs typically located at bifurcation points along intradural internal carotid artery (ICA), circle of Willis (COW), MCA
+ - **90%** located on **anterior circulation**: ACoA, posterior communicating artery (PCoA), MCA, carotid terminus, carotid-ophthalmic, superior hypophyseal
+ - **10%** on **posterior** circulation: Basilar tip, PICA, anterior inferior cerebellar artery (AICA), SCA
+ - DAs: Intradural V4 VA segment most common
+ - Blood blister aneurysm (BBA)
+ - Dorsal variant supraclinoid ICA
+ - Rarely MCA, basilar artery
+- ### CT Findings
+
+
+ - #### NECT
+
+
+ - Hyperdense basal cisterns/sylvian fissure
+ - Within 6 hours after ictus, sensitivity ~ 100%
+ - 93% at 24 hours, < 60% after 5 days
+ - "Effaced" basal cisterns/sylvian fissure if subacute (filled with isodense SAH)
+ - Hydrocephalus common, may occur early
+ - ± intraparenchymal hemorrhage at site of ruptured aneurysm
+ - #### CTA
+
+
+ - 90-95% positive if aneurysm ≥ 2 mm
+ - Low yield in patients with acute headache, normal neurologic examination, normal NECT
+- ### MR Findings
+
+
+ - #### T1WI
+
+
+ - Acute aSAH is isointense to CSF
+ - CSF may appear mildly hyperintense ("dirty")
+ - #### T2WI
+
+
+ - Difficult to see (hyperintense)
+ - #### FLAIR
+
+
+ - Hyperintense
+ - More sensitive than CT but less specific
+ - #### T2* GRE
+
+
+ - Striking sulcal blooming
+ - #### DWI
+
+
+ - May see foci of restricted diffusion if vasospasm
+ - #### MRA
+
+
+ - TOF MRA 85-95% sensitive for aneurysms ≥ 3 mm
+- ### Ultrasonographic Findings
+
+
+ - Transcranial Doppler (TCD) may be helpful in evaluating vasospasm
+- ### Angiographic Findings
+
+
+ - CTA has high sensitivity, specificity for detecting ruptured aneurysm(s)
+ - Best in initial diagnosis of SAH, patient triage
+ - 4-vessel DSA still gold standard
+ - Must image
+ - Both ICA circulations
+ - Both VAs or dominant VA + reflux to contralateral PICA
+ - **SA**
+ - Saccular outpouching at arterial branch point
+ - Look for Murphy teat = site of rupture
+ - Look for additional aneurysms (20% multiple)
+ - If > 1 aneurysm, then biggest, most irregular ± adjacent vasospasm is likely source of bleed
+ - **DA**
+ - Irregular ± dilated or stenotic V4 segment of VA
+ - **Blood blister-like aneurysm**
+ - Smooth/irregular bleb-/dome-shaped outpouching
+ - Not associated with major vessel branch point
+ - Most common along supraclinoid ICA
+ - DSA negative in 15% of aSAH; repeat positive < 5%
+ - Evaluate external carotid arteries (ECAs) [to exclude dural arteriovenous fistula (dAVF)]
+ - SA may not be seen on initial DSA if optimal projection not obtained, spontaneous partial or complete aneurysm thrombosis, &/or presence of vasospasm
+ - Consider repeating DSA in 5-7 days
+- ### Imaging Recommendations
+
+
+ - #### Best imaging tool
+
+
+ - NECT + multiplanar CTA
+ - #### Protocol advice
+
+
+ - Proceed to DSA if NECT consistent with aSAH but CTA negative
+ - Consider MR if DSA + CTA negative
+ - Likelihood of aneurysm in patient who is CT negative, LP positive, CTA negative is very low
+
+## DIFFERENTIAL DIAGNOSIS
+
+- ### Nonaneurysmal Subarachnoid Hemorrhage
+
+
+ - [Perimesencephalic SAH](/document/perimesencephalic-nonaneurysmal-su-/09db329f-e08a-4075-9a94-4fcc9dfee765)
+ - Small SAH, localized to interpeduncular and prepontine cisterns
+ - Presumed venous etiology with low recurrence rate
+ - [Traumatic SAH](/document/traumatic-subarachnoid-hemorrhage/3697dced-cf23-47db-bdbd-0b941d63cf42)
+ - Adjacent to contusions, subdural hematomas
+ - Rarely from intracranial dissection or rupture of traumatic pseudoaneurysm
+ - SAH, not otherwise specified
+ - Vascular malformation: Arteriovenous malformation (AVM), cavernous hemangioma
+- [Reversible Cerebral Vasoconstriction Syndrome](/document/reversible-cerebral-vasoconstricti-/c3a19be2-1f91-4dcb-b63a-cbfdaeaca5c1)
+ - Clinical: Thunderclap headache
+ - SAH typically in cortical sulci vs. basal cisterns with aSAH
+- ### Vasculitis
+
+
+ - May see SAH and small infarcts
+ - Intracranial vascular irregularities, stenoses, and occlusions
+- ### Pseudosubarachnoid Hemorrhage
+
+
+ - Hypodense brain: Severe cerebral edema
+ - Hyperdense CSF: Intrathecal contrast; meningitis
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Etiology
+
+
+ - **SAs**
+ - Berry aneurysms: Congenital deficiency of internal elastic lamina and tunica media at arterial branch points → focal vessel wall weakness
+ - ↑ risk: Familial intracranial aneurysms (5% of cases), adult polycystic kidney disease, aortic coarctation
+ - May be related to high-flow arteriopathy along feeding vessel of AVM or, less commonly, dAVF
+ - ↑ aneurysm rupture risk if female, smoker, hypertension
+ - **Fusiform aneurysms**
+ - Dissection from trauma, hypertension, atherosclerotic vascular disease (ASVD)
+ - Underlying arteriopathy, including fibromuscular dysplasia (FMD), Marfan, Ehlers-Danlos, infection
+ - Mycotic
+ - **BBA**: All layers absent (contained by fibrous cap)
+ - #### Associated abnormalities
+
+
+ - Vasospasm
+ - Caused by blood breakdown products, apolipoprotein-E genotype, endothelin-1 release from CSF leukocytes
+ - 70% develop angiographic evidence of vasospasm
+ - 30% have clinically apparent vasospasm
+ - Starts ~ day 3-4, peaks ~ day 7-9, and lasts ~ 12-16 days post SAH
+ - Cerebral salt-wasting syndrome
+ - Excessive renal Na+ excretion → hyponatremia, hypovolemia
+ - Terson syndrome
+ - Initially defined as vitreous hemorrhage associated with SAH; now defined as hemorrhages into any of retinal spaces
+ - Up to 1/3 of aSAH patients; visual symptoms usually absent
+ - 50% bilateral asymmetric or unilateral
+- ### Staging, Grading, & Classification
+
+
+ - Clinical grading: Hunt and Hess (H&H) grades 0-5
+ - 0: No SAH (unruptured aneurysm)
+ - 1: No symptoms, minimal headache, slight nuchal rigidity
+ - 2: Moderate to severe headache, nuchal rigidity
+ - No neurologic deficit except cranial nerve palsy
+ - 3: Drowsy, minimal neurologic deficit
+ - 4: Stuporous, moderate/severe hemiparesis
+ - 5: Coma, decerebrate rigidity, moribund appearance
+ - WFNS clinical grading system: Based on GCS and presence/absence of major focal neurologic deficit
+ - Fisher CT grading
+ - 1: No SAH visible
+ - 2: Diffuse, thin layer (< 1 mm)
+ - 3: Localized clot or thick layer (> 1 mm)
+ - 4: Intraventricular blood
+- ### Gross Pathologic & Surgical Features
+
+
+ - Blood in basal cisterns, sulci, and ventricles
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - Sudden "thunderclap/worst headache of life"
+ - 10% preceded by "sentinel hemorrhage"
+ - Self-limiting SAH + headache in preceding days/weeks
+ - ↑ likelihood of death/disability 4x
+- ### Demographics
+
+
+ - #### Age
+
+
+ - Peak = 40-60 years
+ - #### Sex
+
+
+ - M:F = 1:2
+ - #### Epidemiology
+
+
+ - Aneurysms cause 85% of spontaneous SAHs
+ - Incidence ~ 9.9 per 100,000 population
+ - 15-30% of deaths happen before hospital admission, so real incidence likely higher
+- ### Natural History & Prognosis
+
+
+ - 50% mortality; 20% rebleed within first 2 weeks
+ - Clinical outcome inversely proportional to initial H&H or WFNS grade
+ - Vasospasm + ischemia → delayed morbidity, mortality
+ - Severity correlates with amount of SAH (Fisher CT grade); inverse correlation with patient age
+ - 90% hydrocephalus at presentation
+ - ~ 10% require permanent CSF diversion
+- ### Treatment
+
+
+ - Ruptured aneurysm
+ - Coil embolization ("coiling"), if anatomy favorable
+ - Lower rates of acute adverse events; long term, all cause morbidity
+ - Microneurosurgical clipping
+ - Proven effective over decades but invasive, higher morbidity/mortality compared with coiling
+ - One study: Death or dependence at 1 year = 23.7% with coiling vs. 30.7% with clipping
+ - Vasospasm
+ - Ca⁺⁺ antagonists, "triple-H" therapy (hypervolemia, hemodilution, hypertension)
+ - Endovascular: Intraarterial Ca⁺⁺ antagonist ("chemical angioplasty"), balloon angioplasty
+ - Lumbar drain often placed to ↓ blood and debris from subarachnoid space
+ - Hydrocephalus
+ - Temporary or permanent CSF diversion
+ - Cerebral salt-wasting syndrome
+ - Na+ tablets or IV hypertonic saline
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - Nonaneurysmal SAH if characteristic blood distribution [e.g., perimesencephalic SAH, reversible cerebral vasoconstriction syndrome (RCVS)]
+ - Look for multiple aneurysms (~ 20%)
+
+ bfc1737e-c900-4e6d-aadd-e4abf5e85c4b
+
+## References
+
+## Selected References
+
+1. [Allaw S et al: A review of intracranial aneurysm imaging modalities, from CT to state-of-the-art MR. AJNR Am J Neuroradiol. 46(6):1082-92, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=39443148%5Bpmid%5D)
+1. [Cinar C et al: Endovascular treatment of small-parent artery aneurysms: mid-term results of the silk vista baby flow diverter. Neuroradiology. ePub, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=40402211%5Bpmid%5D)
+1. [Dodier P et al: Conservative management of 661 patients with unruptured intracranial aneurysms: an observational study over 4 decades. J Neurosurg. 1-13, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=40446344%5Bpmid%5D)
+1. [GBD 2021 Global Subarachnoid Hemorrhage Risk Factors Collaborators et al: Global, regional, and national burden of nontraumatic subarachnoid hemorrhage: the global burden of disease study 2021. JAMA Neurol. ePub, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=40406922%5Bpmid%5D)
+1. [Pando A et al: Flow diverter assisted embolization of ruptured aneurysms is associated with increased hemorrhagic complications: prognostic factors and outcomes in neuroendovascular treatment of subarachnoid hemorrhages. World Neurosurg. 124061, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=40349917%5Bpmid%5D)
+1. [Thaler C et al: Risk factors for unfavorable functional outcome after endovascular treatment of cerebral vasospasm following aneurysmal subarachnoid hemorrhage. AJNR Am J Neuroradiol. 46(3):495-501, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=40015973%5Bpmid%5D)
+1. [Wenz F et al: Lumbar puncture or external ventricular drainage as initial treatment for acute hydrocephalus in aneurysmal subarachnoid hemorrhage-a 2-center cohort study. Neurosurgery. ePub, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=40445007%5Bpmid%5D)
+1. [Heitkamp C et al: CTA supplemented by CTP increases interrater reliability and endovascular treatment use in patients with aneurysmal SAH. AJNR Am J Neuroradiol. 45(3):284-290, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38238090%5Bpmid%5D)
+1. [Moser MM et al: Agreement between CT-angiography and digital subtraction angiography in predicting angiographic vasospasm in patients with subarachnoid hemorrhage. J Clin Med. 13(13), 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38999308%5Bpmid%5D)
+1. [Panicker S et al: CT imaging computed tomography/computed tomography angiography/perfusion in acute ischemic stroke and vasospasm. neuroimaging Clin N Am. 34(2):175-89, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38604703%5Bpmid%5D)
+1. [Toi H et al: Clinical features of cytotoxic lesions of the corpus callosum associated with aneurysmal subarachnoid hemorrhage. AJNR Am J Neuroradiol. 42(6):1046-51, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33664118%5Bpmid%5D)
+1. [Ditz C et al: Cerebral vasospasm after spontaneous subarachnoid hemorrhage: angiographic pattern and its impact on the clinical course. World Neurosurg. 46(6):1082-92, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32247799%5Bpmid%5D)
+1. [Kang HM et al: Clinical characteristics of asymptomatic Terson syndrome in the patients with aneurysmal subarachnoid hemorrhage. Int J Ophthalmol. 13(2):292-300, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32090040%5Bpmid%5D)
+1. [Neulen A et al: Automated grading of cerebral vasospasm to standardize computed tomography angiography examinations after subarachnoid hemorrhage. Front Neurol. 11:13, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32082241%5Bpmid%5D)
+1. [Sharma S et al: The role of transcranial Doppler in cerebral vasospasm: a literature review. Acta Neurochir Suppl. 127:201-5, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31407086%5Bpmid%5D)
+1. [Shi Z et al: Artificial intelligence in the management of intracranial aneurysms: current status and future perspectives. AJNR Am J Neuroradiol. 41(3):373-9, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32165361%5Bpmid%5D)
+1. [Caton MT Jr et al: Non-traumatic subdural hemorrhage: beware of ruptured intracranial aneurysm. Emerg Radiol. 26(5):567-71, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31037589%5Bpmid%5D)
+1. [Fragata I et al: Imaging predictors of outcome in acute spontaneous subarachnoid hemorrhage: a review of the literature. Acta Radiol. 60(2):247-59, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=29792042%5Bpmid%5D)
+1. [Hackenberg KAM et al: Common data elements for radiological imaging of patients with subarachnoid hemorrhage: proposal of a multidisciplinary research group. Neurocrit Care. 30(Suppl 1):60-78, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31115823%5Bpmid%5D)
+1. [Howard BM et al: Comprehensive review of imaging of intracranial aneurysms and angiographically negative subarachnoid hemorrhage. Neurosurg Focus. 47(6):E20, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31786554%5Bpmid%5D)
+1. [Hsu CC et al: High-resolution MRI vessel wall imaging in acute aneurysmal subarachnoid hemorrhage: spatiotemporal pattern and clinicoradiologic implications. Clin Neuroradiol. 30(4):801-10, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31667525%5Bpmid%5D)
+1. [Rouanet C et al: Aneurysmal subarachnoid hemorrhage: current concepts and updates. Arq Neuropsiquiatr. 77(11):806-14, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31826137%5Bpmid%5D)
+1. [Alons IME et al: Yield of computed tomography (CT) angiography in patients with acute headache, normal neurological examination, and normal non contrast CT: a meta-analysis. J Stroke Cerebrovasc Dis. 27(4):1077-84, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29277281%5Bpmid%5D)
+1. [Gonçalves B et al: Effect of early brain infarction after subarachnoid hemorrhage: a systematic review and meta-analysis. World Neurosurg. 115:e292-8, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29660554%5Bpmid%5D)
+1. [Ho AL et al: Practical pearl: Use of MRI to differentiate pseudo-subarachnoid hemorrhage from true subarachnoid hemorrhage. Neurocrit Care. 29(1):113-8, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29948997%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+*Axial graphic at the midbrain depicts subarachnoid hemorrhage (SAH) in red throughout the basal cisterns. Given the diffuse distribution of SAH without focal hematoma, the most likely location of the ruptured aneurysm is the ACoA or PCoA, the most common location for aneurysms.*
+
+
+*Axial NECT in a 51-year-old man "found down" shows diffuse SAH filling the basal cisterns and sylvian fissures. SAH distribution depends on the aneurysm location and is highest near the site of rupture.*
+
+
+*Axial NECT in a 63-year-old shows SAH filling the basal cisterns and sylvian fissures as well as a large amount in the anterior interhemispheric fissure
suggesting an anterior circulation aneurysm, likely ACoA. The low density within the hyperdense hemorrhage
may represent the aneurysm.*
+
+
+*Axial CTA MIP shows the ACoA aneurysm
within the interhemispheric fissure hemorrhage
. Anterior circulation aneurysms are most common, representing ~ 90% of aneurysms.*
+
+
+*Axial NECT in a 52-year-old with severe headache shows SAH
in the basal cisterns, right sylvian fissure, and a right temporal parenchymal hematoma
. Hyperdensity is noted in the left sylvian fissure
.*
+
+
+*Axial CTA shows bilateral MCA aneurysms
. The right aneurysm is larger and mildly irregular and lies adjacent to the hemorrhage
. Occasionally, MCA aneurysms will result in parenchymal hemorrhage in the temporal lobe, as in this patient. Multiple aneurysms are noted in 20% of patients.*
+
+
+*Axial NECT in a 49-year-old with "worst headache of life" shows diffuse SAH
throughout the basal cisterns. Early hydrocephalus is noted with dilatation of temporal horns
. SAH was also in the prepontine cistern suggesting a posterior circulation aneurysm, such as a basilar tip, superior cerebellar artery, or posterior inferior cerebellar artery aneurysm or vertebral artery dissecting aneurysm.*
+
+
+*Coronal shaded surface display of the DSA in the same patient shows the complex, multilobular basilar tip aneurysm
.*
+
+
+*Axial NECT in a 48-year-old with a possible stroke shows focal SAH in the parietal lobe
with adjacent edema. Traumatic SAH is the most common cause of SAH. Ruptured aneurysm is the 2nd most common cause.*
+
+
+*Axial CTA MIP shows a small mycotic aneurysm
within a hematoma
in the right parietal lobe, just inferior to the SAH. Mycotic aneurysms are uncommon and often relate to cardiac valve disease, as in this patient with mitral valve disease with associated septic emboli.*
+
+
+### Additional Images
+
+
+*Axial T1 MR shows no obvious abnormality in this patient with a "thunderclap" headache and lumbar puncture that showed mildly bloody CSF.*
+
+
+*Axial FLAIR MR in the same patient shows widespread high signal intensity within the cerebral sulci caused by aneurysmal SAH (aSAH).*
+
+
+*Axial NECT in an 83-year-old man found down shows diffuse SAH, seen here as hyperdense fluid
in virtually all the visualized sulci. CSF-blood level
is also present in the lateral ventricles.*
+
+
+*Axial NECT in a 73-year-old man with thunderclap headache shows diffuse SAH in the suprasellar cistern
and sylvian fissures
. Note focal hemorrhage in the left inferomedial frontal lobe
. This suggests an ACoA "culprit" aneurysm that ruptured superolaterally. Early severe obstructive hydrocephalus is also present.*
+
+
+*More cephalad NECT shows SAH in the sulci
. Moderately severe hydrocephalus is present. Bilateral choroid plexus hemorrhage
is also present.*
+
+
+*CTA in the same patient shows an ACoA aneurysm
.*
+
+
+*DSA in the same patient shows a large, multilobulated ACoA aneurysm
with a "tit"
at its anterosuperior aspect, which is likely the rupture site that caused the intraparenchymal hematoma seen on NECT.*
+
+
+*Axial FLAIR MR obtained 2 days later in the same patient shows diffuse sulcal hyperintensity
and normally suppressed CSF in the lateral ventricles with blood-fluid level
.*
+
+
+*Axial GRE MR in the same patient shows blood in the sulci as "blooming" hypointensities
. The blood-CSF level
in the lateral ventricles is also clearly seen.*
+
+
+*Sagittal T1 MR shows typical findings of acute aSAH. Note "dirty" CSF
that appears isointense with adjacent brain. The normal basilar artery flow void
is surrounded by the SAH.*
+
+
+*Axial T1 MR in the same patient shows a nice contrast between the isointense (with brain) "dirty" CSF
and the more normal-appearing, hypointense ("dark") CSF in the cistern
and temporal horns
.*
+
+
+*Axial T2 MR in the same patient shows that the hyperintense SAH is difficult to distinguish from the normal "bright" CSF
. The SAH
is very slightly less hyperintense than the adjacent CSF.*
+
+
+*Axial FLAIR MR in the same patient shows CSF in the suprasellar cistern
is abnormally hyperintense. Sulcal-cisternal hyperintensity is also seen in the left perimesencephalic and superior cerebellar cisterns as well as the parietooccipital subarachnoid spaces
. Normal CSF suppresses on FLAIR.*
+
+
+*Axial NECT shows the typical appearance of aSAH. Acute subarachnoid blood is seen as hyperdensity
in the basal cisterns, sylvian fissures, perimesencephalic cisterns, and interhemispheric fissure. Ruptured ACoA aneurysm was found on CTA (not shown).*
+
+
+*Coronal CTA shows a large basilar tip aneurysm as the cause of this patient's aSAH. The location of aSAH distribution depends on the location of saccular aneurysm. In this case, the blood was present in the prepontine cistern as well as all of the basal cisterns.*
+
+
+*Axial NECT in a 63-year-old man found down in a parking lot shows diffuse SAH
throughout the basal cisterns. Note the enlargement of both temporal horns of the lateral ventricles
, consistent with early extraventricular obstructive hydrocephalus.*
+
+
+*Coronal MIP CTA in the same patient shows a saccular aneurysm
projecting superiorly from the ACoA, one of the most common locations for intracranial aneurysms.*
+
+
+*Coronal shaded surface display of the DSA in the same patient nicely demonstrates the "culprit" aneurysm
. The lesion was successfully coiled after this diagnostic DSA was performed.*
+
+
+*Axial NECT in a 58-year-old man with thunderclap hemorrhage shows diffuse SAH in the basal and perimesencephalic cisterns
. Note mild enlargement of the temporal horns, consistent with early obstructive hydrocephalus
.*
+
+
+*Sagittal NECT shows hemorrhage in the prepontine and suprasellar cisterns
. A small amount of hemorrhage is present in the perimesencephalic cistern
and occipital sulci
. Emergent CTA and DSA (not shown) were negative for aneurysm, vasospasm.*
+
+
+*Sagittal T1 MR shows isointense (with brain) subarachnoid blood filling the prepontine, interpeduncular, and suprasellar cisterns
.*
+
+
+*Axial T1 MR shows the suprasellar cistern
and interpeduncular notch
are filled with blood that is almost perfectly isointense with the adjacent brain.*
+
+
+*Axial T2 MR shows blood in the suprasellar cistern is mildly hypointense
, while blood in the dependent interpeduncular notch is much more hypointense
. A hematocrit level
seems to be present within the subarachnoid space.*
+
+
+*FLAIR MR shows mixed signal intensity in the suprasellar cistern
, while the surface sulci are hyperintense
. DSA was repeated 2 weeks later. This was found to be angiogram-negative SAH, presumably from a blood blister-like aneurysm that was too small to be identified.*
+
+
+*Coronal shaded surface display of the DSA in a patient with SAH centered in the anterior interhemispheric fissure shows an irregular "culprit" aneurysm
involving the ACoA. The lesion was successfully coiled.*
+
diff --git a/docs_md/articles/carbon-monoxide-poisoning_827ae14b-3d0f-4c3a-937a-e450a7eec716.md b/docs_md/articles/carbon-monoxide-poisoning_827ae14b-3d0f-4c3a-937a-e450a7eec716.md
new file mode 100644
index 0000000..ff791c4
--- /dev/null
+++ b/docs_md/articles/carbon-monoxide-poisoning_827ae14b-3d0f-4c3a-937a-e450a7eec716.md
@@ -0,0 +1,475 @@
+---
+title: "Carbon Monoxide Poisoning"
+docid: "827ae14b-3d0f-4c3a-937a-e450a7eec716"
+authors:
+ - key: "a25c450b-3d34-4f64-bba3-cc0834813df6"
+ value: "Miral D. Jhaveri, MD, MBA"
+breadcrumbs:
+ -
+ name: "Brain"
+ slug: "brain"
+ treeNodeId: "0361042c-9c90-48e4-864e-a2e6428ee9c9"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "948c6f1e-8e18-45f3-bd9d-7a04ab0b8708"
+ -
+ name: "Pathology-Based Diagnoses"
+ slug: "pathology-based-diagnoses"
+ treeNodeId: "d60fe914-7897-46a0-9cd2-f88ffb322f16"
+ -
+ name: "Acquired Toxic/Metabolic/Degenerative Disorders"
+ slug: "acquired-toxicmetabolicdegenerativ-"
+ treeNodeId: "80461866-16fe-4ccf-b9b8-034117c0a1e6"
+ -
+ name: "Toxic, Metabolic, Nutritional, Systemic Diseases With CNS Manifestations"
+ slug: "toxic-metabolic-nutritional-system-"
+ treeNodeId: "9f567059-7d25-4aca-9920-93171423ef5e"
+ -
+ name: "Carbon Monoxide Poisoning"
+ slug: "carbon-monoxide-poisoning"
+ treeNodeId: null
+category: "Brain"
+documentVersionId: "5654d554-8b46-4a4f-b4fb-a850bf0d44e6"
+imageCount: 25
+lastUpdated: "08/07/25"
+pageDescription: "Carbon Monoxide Poisoning"
+pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Acquired Toxic/Metabolic/Degenerative Disorders, Toxic, Metabolic, Nutritional, Systemic Diseases With CNS Manifestations, Carbon Monoxide Poisoning"
+pageTitle: "Carbon Monoxide Poisoning | STATdx"
+enhancedTitle: "Carbon Monoxide Poisoning"
+type: "DX"
+references: true
+breadcrumbs:
+ - "Brain"
+ - "Diagnosis"
+ - "Pathology-Based Diagnoses"
+ - "Acquired Toxic/Metabolic/Degenerative Disorders"
+ - "Toxic, Metabolic, Nutritional, Systemic Diseases With CNS Manifestations"
+ - "Carbon Monoxide Poisoning"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Anoxic-ischemic encephalopathy, usually with bilateral lesions, caused by inhalation of carbon monoxide (CO) gas
+- ### Imaging
+
+
+ - Best diagnostic clue: Globi pallidi (GP) T2/FLAIR hyperintensity
+ - T1 MR: Both hypointensity in GP (likely necrosis) and hyperintensity in GP (likely hemorrhage) reported
+ - T2 MR: Ischemia/infarct of GP
+ - Cerebral hemispheric white matter (WM): Bilateral confluent hyperintense WM (periventricular, centrum semiovale)
+ - Cortical hyperintensity (commonly temporal lobe)
+ - Medial temporal lobe hyperintensity (uncommon despite frequent pathologic findings)
+ - DWI MR: Acute restriction is common
+ - MRS: Progressively ↓ NAA/Cr with time; ↑ Cho/Cr
+ - Progressively ↑ Lac/Cr with time
+ - MR more sensitive than CT
+- ### Top Differential Diagnoses
+
+
+ - Hypoxic-ischemic encephalopathy
+ - Drug abuse
+ - Wilson disease
+ - Japanese encephalitis (JE)
+ - Creutzfeldt-Jakob disease (CJD)
+- ### Pathology
+
+
+ - CO-Hgb impairs erythrocyte oxygen transport, reducing cellular oxygen and causing hypoxia
+ - Demyelination, edema, and hemorrhagic necrosis
+- ### Clinical Issues
+
+
+ - Acute toxicity: Nausea, vomiting, headache
+ - Neuropsychologic sequelae
+ - Delayed neurologic sequelae (10-30% of victims)
+ - Early administration of 100% oxygen, hyperbaric oxygen (HBO) therapy
+
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - Carbon monoxide (CO) poisoning (COP)
+- ### Definitions
+
+
+ - Anoxic-ischemic encephalopathy, usually with bilateral lesions, caused by inhalation of CO gas
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Globi pallidi (GP) hyperintensity on T2 MR or hypodensity on CT
+ - #### Location
+
+
+ - GP: Most common site of abnormality
+ - Cerebral white matter (WM): 2nd most common
+ - Putamen, caudate nucleus, thalamus, substantia nigra, corpus callosum, fornix, hippocampus: Less common
+ - #### Size
+
+
+ - ↓ (hippocampal and generalized cerebral atrophy)
+ - #### Morphology
+
+
+ - Typically oval lesions confined to GP
+ - Severe changes show loss of gray-white differentiation due to diffuse edema
+- ### CT Findings
+
+
+ - #### NECT
+
+
+ - Symmetric hypodensity in GP and symmetric diffuse hypodensity in cerebral WM
+- ### MR Findings
+
+
+ - #### T1WI
+
+
+ - In GP, both T1 hypointensity (likely due to necrosis) and T1 hyperintensity (likely due to hemorrhage) reported
+ - #### T2WI
+
+
+ - Ischemia/infarct of GP
+ - Bilateral T2 hyperintensities of GP surrounded by hypointense rim (likely due to hemosiderin)
+ - Caudate nucleus and putamen may be affected, either alone or in addition to GP abnormality
+ - Cerebral hemispheric WM
+ - Bilateral, confluent, T2-hyperintense WM (periventricular, centrum semiovale)
+ - Reflects diffuse demyelination
+ - Reported in delayed encephalopathy
+ - Abnormal signal in cerebral cortex (less frequent)
+ - Cortical hyperintensity: Most common pattern with predilection for temporal lobe
+ - Abnormalities in perisylvian cortex, anterior temporal lobe, and insular cortex
+ - Medial temporal lobe in region of hippocampus (uncommon despite frequent pathologic findings)
+ - Asymmetrical, diffuse cortical hyperintensity affecting parietal and occipital lobes also possible
+ - Diffuse, bilateral high signal within cerebellar hemispheres, affecting cortex and WM
+ - Not seen in acute setting; develops later
+ - Delayed encephalopathy 2-3 weeks after recovery
+ - Additional high intensity in corpus callosum, subcortical U fibers, internal and external capsules
+ - Associated with low intensity in thalamus and putamen (due to iron deposition)
+ - #### PD/intermediate
+
+
+ - High signal in GP
+ - #### FLAIR
+
+
+ - Same as T2WI
+ - Additional periventricular high signal in acute COP
+ - May not be visible on conventional T2 FSE
+ - #### DWI
+
+
+ - Early (acute) stage of COP
+ - Symmetric DWI hyperintensity in GB
+ - Diffuse symmetric DWI hyperintensity in subcortical hemispheric WM (restricted diffusion due to cytotoxic edema)
+ - WM may appear normal on FLAIR, particularly in low-dose exposure
+ - Low ADC values in same regions
+ - May see subtle cortical lesions ± BG, thalami, periventricular WM, or hippocampus
+ - Delayed stage of COP (weeks post exposure)
+ - High-signal area in cerebral WM
+ - ± abnormal WM findings on T2WI
+ - Low ADC values persist at this stage
+ - Chronic stage of COP
+ - Gradual ↑ in ADC values, consistent with macrocystic encephalomalacia
+ - Hyperintense WM areas on T2WI and symmetric bright lesions in GP
+ - Diffusion tensor imaging (DTI): Fractional anisotropy (FA) values ↓ in deep WM, including centrum semiovale
+ - High correlation between FA and Mini-Mental State Examination
+ - Diffusion kurtosis
+ - WM mean kurtosis tend to ↑ from acute to delayed neuropsychiatric phases, ↓ in chronic phase
+ - GM mean kurtosis shows constant decline
+ - #### T1WI C+
+
+
+ - Variable enhancement in GP, often in patients with acute COP
+ - #### MRS
+
+
+ - Serial ¹H-MRS scans performed after appearance of delayed sequel in COP, disturbances of neuronal function
+ - Persistently ↑ Cho/Cr at DWI abnormal WM site
+ - Progressively ↓ NAA/Cr with time
+ - ↓ NAA suggests neuron and axon degeneration
+ - Progressively ↑ Lac/Cr with time, often seen in high-dose exposure cases
+ - Reflects developmental process of WM lesions
+ - WM demyelination progresses to neuronal necrosis
+- ### Nuclear Medicine Findings
+
+
+ - SPECT studies show cerebral hypoperfusion deficits
+ - ↓ regional cerebral blood flow in frontal and temporal cortices and diffuse hypoperfusion defects reported
+- ### Imaging Recommendations
+
+
+ - #### Best imaging tool
+
+
+ - MR more sensitive than CT
+ - DWI best for lesion detection in acute stage of COP
+ - ADC value ↓ progressively and persists much longer than acute cerebral infarction
+ - MRS and DTI under investigation
+ - #### Protocol advice
+
+
+ - Multiplanar MR, including DWI
+
+## DIFFERENTIAL DIAGNOSIS
+
+- [Hypoxic-Ischemic Encephalopathy](/document/adult-hypoxic-ischemic-injury/91ac293f-161c-4b3b-81e5-740f831eaa5d)
+ - Common pathophysiology
+ - Imaging findings often overlap
+ - Generally affects entire BG and hippocampi, less often only GP or only WM
+- [Drug Abuse](/document/drug-abuse/48859403-0b26-44d8-ba74-e0919e4c3147)
+ - Overlapping imaging findings due to common pathophysiology
+ - May see symmetric GB, WM lesions
+- [Wilson Disease](/document/wilson-disease/b89eef10-ea47-4ca9-a3b7-b8aeeca86802)
+ - WM-gray matter (GM) lesions, involving BG, dentate nucleus, pons, mesencephalon
+ - T1-hypointense (occasionally hyperintense) lesions
+ - Variably T2 hyperintense/hypointense
+- [Japanese Encephalitis](/document/miscellaneous-encephalitis/7e3dd11d-21c7-468a-a227-b363d595bbce)
+ - Homogeneous T2 hyperintensities in BG and thalami
+ - Most characteristic finding in Japanese encephalitis (JE)
+ - Bilateral thalamic hyperintensities ± hemorrhage
+ - JE is meningoencephalitis → meningeal enhancement
+- [Arteriolosclerosis](/document/arteriolosclerosis/07e561a5-0554-4867-b811-448c36890ee3)
+ - Focal hyperintensities in corona radiata, centrum semiovale
+ - BG lacunae: Typically asymmetric, multifocal
+- [Creutzfeldt-Jakob Disease](/document/creutzfeldt-jakob-disease-cjd/30a88a01-b24d-476d-a933-48aabcdb6f95)
+ - Progressively symmetric hyperintense changes in BG, thalami, cerebral cortex
+ - DWI and FLAIR most sensitive
+- ### Leigh Syndrome
+
+
+ - Symmetric spongiform brain lesions with onset in infancy/early childhood
+ - Lesions predominantly in brainstem, BG (particularly putamen), and cerebral WM
+ - Focal, bilateral, and symmetric T2-hyperintense lesions
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Etiology
+
+
+ - CO: Colorless, odorless, tasteless gas
+ - Produced by incomplete combustion of carbonaceous material
+ - 200x affinity for hemoglobin than O₂
+ - Brain and heart damage once CO-Hgb level exceeds 20%
+ - Mechanisms of brain injury
+ - CO-Hgb impairs erythrocyte oxygen transport, reducing cellular oxygen and causing hypoxia
+ - Lipid peroxidation leading to oxidative injury
+ - Peroxynitrite damage vascular endothelium
+ - Excitotoxicity, apoptosis
+ - #### Associated abnormalities
+
+
+ - CO-induced parkinsonism
+ - GP lesions after COP or periventricular and deep WM hyperintensities without BG lesions
+ - Extrapyramidal syndrome may be due to lesions of WM areas containing BG output &/or input
+ - Improvement usually accompanied by ↓ extent and signal intensity of WM abnormalities, especially in frontoparietal centrum semiovale
+ - Microstructural WM pathology is likely related to delayed cognitive impairment
+ - Bilateral necrosis of GP
+ - Multifocal areas of demyelination in periventricular WM, sparing of subcortical arcuate U fibers ("Grinker myelinopathy")
+- ### Staging, Grading, & Classification
+
+
+ - 4 main pathologic types
+ - GP lesions: Variable degree of necrosis
+ - WM lesions: Scattered/focal areas of necrosis or confluent areas of demyelination
+ - Cortical lesions: Spongiform changes, degeneration, and neuronal loss
+ - Hippocampal lesions: Coagulative necrosis
+- ### Gross Pathologic & Surgical Features
+
+
+ - GP necrosis, WM pallor
+- ### Microscopic Features
+
+
+ - Demyelination, edema, and hemorrhagic necrosis
+ - Necrotic lesions in GP, other BG, hippocampus, cortex, and cerebellum
+ - WM lesions: Foci of necrosis or demyelination
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - Nonspecific symptoms; controversial association of specific symptoms with known CO-Hgb levels
+ - Acute toxicity: Nausea, vomiting, headache
+ - Confusion, cognitive impairment, loss of consciousness, seizures, coma, death
+ - Neuropsychologic sequelae
+ - Dementia, memory deficits, ↓ attention, irritability, mood and personality disturbance
+ - Gait disturbance, parkinsonian-like symptoms, apraxia, convulsive disorders, visual-spatial and speech impairment
+ - #### Clinical profile
+
+
+ - Depends on duration and intensity of exposure
+- ### Demographics
+
+
+ - #### Age
+
+
+ - Equivalent age-specific fatality rates in adults, death rates from COP: ↑ in patients > 65 years
+ - Neonates and in utero fetuses most vulnerable
+ - #### Ethnicity
+
+
+ - For unintentional COP, race-specific death rates for African Americans are 20% higher than for Caucasians
+ - Race-specific death rates for minority racial groups are 87% lower than for Caucasians (cultural partiality to this form of suicide)
+ - #### Epidemiology
+
+
+ - Most common cause of accidental poisoning
+ - Every year, ~ 50,000 people in USA visit emergency departments, and at least 430 people die from accidental COP
+ - ↑ prevalence of COP during winter months
+ - Can be fire-related smoke inhalation or nonfire related (poorly functioning heating systems, improperly vented fuel-burning devices)
+- ### Natural History & Prognosis
+
+
+ - Persistent neurologic sequelae: Occur immediately following COP and persist over time
+ - Delayed neurologic sequelae (10-30% of victims)
+ - Occur weeks after initial recovery from acute COP
+ - 2 categories with regard to outcome
+ - Normal/mild functional impairment: No or minimal abnormality on brain MR
+ - Death/severe functional impairment (coma): Diffuse brain damage on MR
+- ### Treatment
+
+
+ - Hyperbaric oxygen (HBO) therapy: Treatment of choice in acute COP (within 6 hours for best effect)
+ - Early administration of 100% oxygen or HBO may prevent long-term neuropsychiatric sequelae
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - MR to monitor progression/resolution of lesions
+
+ 321d103f-179a-495d-9321-12fc0c3f07dd
+
+## References
+
+## Selected References
+
+1. [Li W et al: Carbon monoxide poisoning with hippocampi lesions on MRI: cases report and literature review. BMC Neurol. 24(1):159, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38734615%5Bpmid%5D)
+1. [Chenoweth JA et al: Carbon monoxide poisoning. Crit Care Clin. 37(3):657-72, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34053712%5Bpmid%5D)
+1. [Chou MC et al: Longitudinal white matter changes following carbon monoxide poisoning: a 9-month follow-up voxelwise diffusional kurtosis imaging study. AJNR Am J Neuroradiol. 40(3):478-82, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30765380%5Bpmid%5D)
+1. [Vamadevan T et al: Imaging appearances of toxic and acquired metabolic encephalopathic disorders. Br J Hosp Med (Lond). 80(7):372-6, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31283394%5Bpmid%5D)
+1. [Zhang Y et al: Cerebral damage after carbon monoxide poisoning: a longitudinal diffusional kurtosis imaging study. AJNR Am J Neuroradiol. 40(10):1630-7, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31558500%5Bpmid%5D)
+1. [Godinho MV et al: Hypoxic, toxic, and acquired metabolic encephalopathies at the emergency room: the role of magnetic resonance imaging. Semin Ultrasound CT MR. 39(5):481-94, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30244762%5Bpmid%5D)
+1. [Jeon SB et al: Acute brain lesions on magnetic resonance imaging and delayed neurological sequelae in carbon monoxide poisoning. JAMA Neurol. 75(4):436-43, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29379952%5Bpmid%5D)
+1. [Lee JJ et al: Diffusion kurtosis imaging as a neuroimaging biomarker in patients with carbon monoxide intoxication. Neurotoxicology. 68:38-46, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30017424%5Bpmid%5D)
+1. [Beppu T: The role of MR imaging in assessment of brain damage from carbon monoxide poisoning: a review of the literature. AJNR Am J Neuroradiol. 35(4):625-31, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=23598831%5Bpmid%5D)
+1. [Betterman K et al: Neurologic complications of carbon monoxide intoxication. Handb Clin Neurol. 120:971-9, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24365364%5Bpmid%5D)
+1. [Mizuno Y et al: Delayed leukoencephalopathy after carbon monoxide poisoning presenting as subacute dementia. Intern Med. 53(13):1441-5, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24990337%5Bpmid%5D)
+1. [Wu PE et al: Carbon monoxide poisoning. CMAJ. 186(8):611, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24396094%5Bpmid%5D)
+1. [Hampson NB et al: Toxic CO-ingestions in intentional carbon monoxide poisoning. J Emerg Med. 44(3):625-30, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23137961%5Bpmid%5D)
+1. [Huzar TF et al: Carbon monoxide and cyanide toxicity: etiology, pathophysiology and treatment in inhalation injury. Expert Rev Respir Med. 7(2):159-70, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23547992%5Bpmid%5D)
+1. [Guzman JA: Carbon monoxide poisoning. Crit Care Clin. 28(4):537-48, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22998990%5Bpmid%5D)
+1. [Hampson NB et al: Practice recommendations in the diagnosis, management, and prevention of carbon monoxide poisoning. Am J Respir Crit Care Med. 186(11):1095-101, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=23087025%5Bpmid%5D)
+1. [Katner A et al: Emergency department visits for carbon monoxide poisoning in LA. J La State Med Soc. 164(6):306-10, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=23431671%5Bpmid%5D)
+1. [Kondziella D et al: 1H MR spectroscopy of gray and white matter in carbon monoxide poisoning. J Neurol. 256(6):970-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19252780%5Bpmid%5D)
+1. [Lin WC et al: White matter damage in carbon monoxide intoxication assessed in vivo using diffusion tensor MR imaging. AJNR Am J Neuroradiol. 30(6):1248-55, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19270104%5Bpmid%5D)
+1. [Prockop LD et al: Carbon monoxide intoxication: an updated review. J Neurol Sci. 262(1-2):122-30, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17720201%5Bpmid%5D)
+1. [Prockop LD: Carbon monoxide brain toxicity: clinical, magnetic resonance imaging, magnetic resonance spectroscopy, and neuropsychological effects in 9 people. J Neuroimaging. 15(2):144-9, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15746226%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+*Axial graphic shows the typical involvement of the brain by carbon monoxide (CO) poisoning. The globi pallidi (GP)
are most affected, followed by the cerebral white matter (WM). Pathologically, there is necrosis of the GP with variable areas of necrosis and demyelination in the WM.*
+
+
+*Axial graphic shows the typical involvement of the brain by carbon monoxide (CO) poisoning. The globi pallidi (GP)
are most affected, followed by the cerebral white matter (WM). Pathologically, there is necrosis of the GP with variable areas of necrosis and demyelination in the WM.*
+
+
+*Axial FLAIR MR in a 22-year-old man with acute CO poisoning demonstrates symmetric hyperintensity
in the GP bilaterally. Note relatively normal-appearing WM.*
+
+
+*Axial DWI MR in the same patient shows extensive symmetric hyperintensities involving the GP
, cerebral WM
, and corpus callosum
.*
+
+
+*Axial ADC map in the same patient shows corresponding low signal in the GP
, cerebral WM
, and corpus callosum
consistent with restricted diffusion. Diffusion is the most sensitive sequence for early detection of lesions in suspected CO poisoning.*
+
+
+*Axial T2 MR in a patient with CO poisoning demonstrates symmetric T2 hyperdensity
in the GP.*
+
+
+*Axial T1 MR in the same patient demonstrates central hypointensity and rim of hyperintensity
involving the medial GP bilaterally. T1 hyperintensity is caused by hemorrhage or coagulative necrosis. GP are exquisitely sensitive to hypoxia, which is hallmark of acute CO poisoning.*
+
+
+*Axial DWI MR image in a patient with acute CO poisoning demonstrates symmetric restricted diffusion
in the GP bilaterally. Note central low signal in the GP
.*
+
+
+*Axial SWI image in the same patient shows central areas of low signal
due to hemorrhagic necrosis and corresponds to the low signal seen on the diffusion image. Ten to thirty percent of victims with CO poisoning develop delayed neurologic sequelae.*
+
+
+*Axial NECT shows the appearance of chronic CO poisoning involving the GP with fairly symmetric hypodensities
.*
+
+
+*Axial T1 MR in the same patient shows low signal intensity lesions
with a subtle T1-hyperintense rim in the GP bilaterally due to necrosis. Drug abuse often shows overlapping imaging findings with CO poisoning due to common pathophysiology.*
+
+
+### Additional Images
+
+
+*Gross autopsy that shows bilateral GP necrosis
secondary to CO inhalation. (Courtesy R. Hewlett, MD.)*
+
+
+*Axial T2 MR shows hyperintense lesions within GP in a patient in the acute stage of CO poisoning.*
+
+
+*Axial T2 MR in the same patient a few months later shows mild decrease in the size of GP lesions and development of a rim of hypointense signal.*
+
+
+*Axial FLAIR MR in a different patient with CO poisoning shows hyperintense lesions within GP.*
+
+
+*Axial FLAIR MR in a patient with CO poisoning shows hyperintense signal in both insulae
.*
+
+
+*Axial FLAIR MR in a different patient with CO poisoning shows bilateral diffuse hyperintensities in centrum semiovale with sparing of subcortical U fibers.*
+
+
+*Axial FLAIR MR in this patient with acute CO poisoning shows the classic appearance with symmetric GP hyperintensity. Note the additional involvement of the posterior temporal cortex
and hippocampi
, a less common finding.*
+
+
+*Axial DWI MR in the same patient shows areas of restricted diffusion involving the posterior temporal cortex
, hippocampal tail, and insular cortex
bilaterally. DWI often shows the affected areas more readily than corresponding T2 or FLAIR MR.*
+
+
+*Axial FLAIR MR in the same patient shows symmetric hyperintensity involving the bilateral hippocampi
and posterior temporal cortex
.*
+
+
+*Axial T1 MR of a patient with acute CO poisoning shows heterogeneous signal in the GP bilaterally with areas of central hypointensity and a surrounding rim of hyperintensity
. The hyperintensity is likely related to blood products.*
+
+
+*Axial T1 C+ FS MR in the same patient shows heterogeneous enhancement of the GP bilaterally
. Enhancement is variably seen in CO poisoning.*
+
+
+*Axial T1 MR shows the appearance of chronic CO poisoning involving the basal ganglia. There are bilateral, nonenhancing, CSF intensity lesions present within the GP
.*
+
+
+*Axial T2 MR in the same patient shows symmetric, bilateral, CSF intensity lesions within the GP
. Often, there is a rim of hypointensity related to hemosiderin surrounding the injured deep gray nuclei. Up to 30% of patients with CO poisoning have delayed neurologic sequelae.*
+
+
+*Axial T2 MR shows symmetric, bilateral GP hyperintensities
and diffuse hyperintensity throughout the WM
with sparing of the subcortical U fibers.*
+
+
+*Axial T2 MR in the same patient shows bilateral diffuse hyperintensity throughout the WM
with typical sparing of the subcortical U fibers. The WM hyperintensity is related primarily to demyelination with variable amounts of necrosis. The hyperintensity typically shows diffusion restriction.*
+
diff --git a/docs_md/articles/carotid-pseudoaneurysm-extracranial_a7b63123-7069-4528-aa11-698d458b5c34.md b/docs_md/articles/carotid-pseudoaneurysm-extracranial_a7b63123-7069-4528-aa11-698d458b5c34.md
new file mode 100644
index 0000000..6cca3db
--- /dev/null
+++ b/docs_md/articles/carotid-pseudoaneurysm-extracranial_a7b63123-7069-4528-aa11-698d458b5c34.md
@@ -0,0 +1,396 @@
+---
+title: "Carotid Pseudoaneurysm, Extracranial"
+docid: "a7b63123-7069-4528-aa11-698d458b5c34"
+authors:
+ - key: "99f10fe6-7f91-4026-bc60-d769c5d7c7c4"
+ value: "C. Douglas Phillips, MD, FACR"
+breadcrumbs:
+ -
+ name: "Vasculature"
+ slug: "vasculature"
+ treeNodeId: "6de1ee4d-afe9-419c-a868-d4074ec0fb7e"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "53de565c-37fe-4b4b-802e-7ea3610cc666"
+ -
+ name: "Extracranial Cerebral Arteries"
+ slug: "extracranial-cerebral-arteries"
+ treeNodeId: "f83006c9-b9f0-4c4b-a4ad-92ec5a46547f"
+ -
+ name: "Carotid Pseudoaneurysm, Extracranial"
+ slug: "carotid-pseudoaneurysm-extracranial"
+ treeNodeId: null
+category: "Vasculature"
+documentVersionId: "a1e19b90-b529-4fe2-a80e-8fe26667b4eb"
+imageCount: 16
+lastUpdated: "01/31/23"
+pageDescription: "Carotid Pseudoaneurysm, Extracranial"
+pageKeywords: "Vasculature, Diagnosis, Extracranial Cerebral Arteries, Carotid Pseudoaneurysm, Extracranial"
+pageTitle: "Carotid Pseudoaneurysm, Extracranial | STATdx"
+enhancedTitle: "Carotid Pseudoaneurysm, Extracranial"
+type: "DX"
+references: true
+ddx: true
+cases: 2
+breadcrumbs:
+ - "Vasculature"
+ - "Diagnosis"
+ - "Extracranial Cerebral Arteries"
+ - "Carotid Pseudoaneurysm, Extracranial"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Paravascular cavitated thrombus in continuity with carotid artery
+ - Not contained by true vessel wall (tunica intima and media), hence "pseudo" or "false" aneurysm
+- ### Imaging
+
+
+ - Round/ovoid/lobulated iso- to slightly hyperdense carotid space (CS) mass ± local bony expansion or remodeling if above skull base
+ - CTA useful as 1st-line imaging tool for both extra- and intracranial carotid injury
+ - Classic imaging appearance: CS mass that communicates directly with carotid artery
+- ### Top Differential Diagnoses
+
+
+ - Dissecting aneurysm
+ - Arteriovenous fistula
+ - Fibromuscular dysplasia
+ - True carotid artery aneurysm
+- ### Pathology
+
+
+ - May result from dissection from any etiology (trauma, iatrogenic, fibromuscular dysplasia, etc.)
+ - In absence of embolic complications, may be more benign than previously assumed
+ - Up to 25% may resolve, ~ 15% decrease in size, and ~ 60% do not change without therapy
+- ### Clinical Issues
+
+
+ - Can be occult, asymptomatic (2.5% of patients with blunt injury)
+- ### Diagnostic Checklist
+
+
+ - True cervical carotid aneurysms are rare
+ - Look for associated trauma/dissection
+ - Multiplanar CTA reformats is usually diagnostic
+ - Look for associated stenosis, contained thrombus
+
+## TERMINOLOGY
+
+- ### Synonyms
+
+
+ - False carotid aneurysm
+- ### Definitions
+
+
+ - Paravascular cavitated thrombus in continuity with carotid artery lumen
+ - Not contained by true vessel wall (tunica intima and media), hence "pseudo" or "false" aneurysm
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Carotid space (CS) mass commonly at skull base with luminal dilatation and concentric mural hematoma
+ - Perivascular cavity communicates with true lumen of native carotid artery
+ - #### Location
+
+
+ - Common carotid artery (CCA) and cervical segment of internal carotid artery (ICA) > petrous > cavernous > intradural ICA
+ - #### Morphology
+
+
+ - May be fusiform dilatation vs. saccular outpouching
+ - Classic imaging appearance: CS mass that communicates directly with carotid artery
+- ### CT Findings
+
+
+ - #### NECT
+
+
+ - Round/ovoid/lobulated iso- to slightly hyperdense CS mass ± local bony expansion or remodeling if above skull base
+ - May contain mural thrombus of varying ages
+ - #### CECT
+
+
+ - Irregular widening of vessel contour
+ - Outpouching of lumen from carotid artery with discernible neck
+ - May compress carotid artery true lumen
+ - Enhances to same degree as opacified lumen of other vascular structures
+ - #### CTA
+
+
+ - Multiplanar reformats ± 3D reconstructions provide excellent visualization of pseudoaneurysm size + orientation of connection with carotid artery lumen ± contained thrombus
+- ### MR Findings
+
+
+ - #### T1WI
+
+
+ - Heterogeneous signal (usually isointense ± flow void, phase artifact)
+ - #### T2WI
+
+
+ - Heterogeneous signal (mixed hypo/hyperintense ± flow void)
+ - #### FLAIR
+
+
+ - Useful for detection of end-organ (brain) injury due to thromboemboli from pseudoaneurysm sac or hemodynamic compromise from compression of true lumen
+ - #### T2* GRE
+
+
+ - Significant blooming artifact from mural hematoma
+ - #### DWI
+
+
+ - Most sensitive for acute ischemia or infarction from thromboemboli or hypoperfusion
+ - #### MRA
+
+
+ - Visualization depends on flow dynamics within and adjacent to pseudoaneurysm
+ - Turbulent flow may result in signal loss and reduce conspicuity
+ - Gadolinium-enhanced MRA may provide superior depiction of lumen and aneurysm
+- ### Ultrasonographic Findings
+
+
+ - #### Color Doppler
+
+
+ - Duplex ultrasound is useful as screening tool
+ - Turbulent flow, pseudoaneurysm ± compression (stenosis) of carotid artery
+- ### Angiographic Findings
+
+
+ - Conventional
+ - DSA remains gold standard for detection and characterization of carotid artery injury; useful for treatment planning: Surgical vs. endovascular vs. conservative/medical
+ - Arch aortogram, arteriogram of carotid bifurcation should be performed prior to more distal selective catheterization of injured carotid artery
+ - Imaging of contralateral carotid ± vertebral artery/arteries should be considered if concurrent injury is suspected in case of multitrauma; necessary if carotid sacrifice is being considered as therapeutic option
+ - Demonstration of aneurysm morphology, aneurysm neck, size ± compression/stenosis of carotid artery lumen
+- ### Imaging Recommendations
+
+
+ - #### Best imaging tool
+
+
+ - CTA useful as 1st-line imaging tool for both extra- and intracranial carotid injuries
+ - Duplex sonography useful as rapid screening examination in acute setting of suspected cervical vascular injury
+
+## DIFFERENTIAL DIAGNOSIS
+
+- ### Dissecting Aneurysm
+
+
+ - Intramural hematoma from rupture of vasa vasorum or intimal tear dilates and weakens vessel wall
+ - May see intimal flap between true and false lumens
+- [Arteriovenous Fistula](/document/arteriovenous-fistula/6d20b0cb-4346-4ccb-936c-357888a432fa)
+ - May occur as rare complication of carotid pseudoaneurysm rupture → arteriovenous shunt to neck veins or cavernous sinus
+ - More commonly associated with penetrating neck injury
+- ### Fibromuscular Dysplasia
+
+
+ - Irregular outpouchings from vessel lumen, usually along upper cervical ICA segment, bilateral in 60%
+ - Occurs in carotid circulation > vertebral arteries
+ - Renal artery involvement most common
+ - Often asymptomatic finding
+ - M:F = 1:3
+ - Classic = "string of beads" with alternating segments of dilatation and strictures
+ - Less symmetric forms also occur
+- ### True Carotid Artery Aneurysm
+
+
+ - Contained by all layers of vessel wall as opposed to pseudoaneurysm
+ - CCA and cervical ICA locations are extremely rare
+ - True cavernous ICA aneurysms are associated with fibromuscular dysplasia and Ehlers-Danlos syndrome
+ - Supraclinoid ICA blister and berry aneurysms may cause subarachnoid hemorrhage
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Etiology
+
+
+ - May result from dissection from any etiology (trauma, iatrogenic, fibromuscular dysplasia, etc.)
+ - Represents contained vessel rupture in soft tissues
+ - Carotid dissection may result in characteristic pseudoaneurysm at skull base
+ - In absence of embolic complications, may be more benign than previously assumed
+ - Up to 25% may resolve, ~ 15% decrease in size, and ~ 60% do not change without therapy
+ - Rarely occurs as sequela of deep neck space infection with necrosis of vessel wall (mycotic pseudoaneurysm)
+ - Carotid blowout syndrome
+ - Carotid pseudoaneurysm ± active bleeding into neck soft tissues or oral cavity
+ - Related to aggressive primary and salvage surgery for head and neck cancer, irradiation
+ - Requires emergent endovascular vessel occlusion or placement of covered stent
+ - Complication of head and neck cancer with vascular involvement
+ - #### Associated abnormalities
+
+
+ - Injury to other vessels possible due to traumatic etiology; should be sought on CTA/DSA
+ - > 80% of all cervical vascular injuries involve carotid arteries
+ - Vessel wall disruption results in periluminal hemorrhage with contained extravasation
+ - Paravascular thrombus forms, cavitates, communicates with parent vessel
+- ### Gross Pathologic & Surgical Features
+
+
+ - Bluish-purple paravascular CS mass contained by fascia, organized hematoma ± adventitia
+- ### Microscopic Features
+
+
+ - Wall of pseudoaneurysm does not contain intima, internal elastic lamina, muscularis layers; adventitia may be present
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - Can be occult, asymptomatic (2.5% of patients with blunt injury)
+ - 50-60% have palpable cervical mass (± pulsation)
+ - 40% neurologic symptoms
+ - Horner syndrome
+ - CN9-CN11 palsy
+ - Jaw pain
+ - Cerebral ischemia/infarction
+ - Cavernous ICA pseudoaneurysm rupture may cause
+ - Massive epistaxis (rupture into sphenoid sinus)
+ - May be life threatening
+ - Direct carotid cavernous fistula (rupture into cavernous sinus) → ophthalmoplegia, proptosis, chemosis
+- ### Demographics
+
+
+ - #### Epidemiology
+
+
+ - 1/3 of cervical ICA vascular injuries caused by penetrating trauma
+- ### Natural History & Prognosis
+
+
+ - Variable; may enlarge, resolve spontaneously, undergo thrombosis, rupture
+ - 45% combined stroke/mortality rate after ligation of parent vessel
+ - 23% stroke/mortality with observation only
+ - < 5% major morbidity/mortality if parent vessel is repaired or reconstructed endovascularly
+- ### Treatment
+
+
+ - Goal is occlusion of pseudoaneurysm with preservation of ICA (constructive)
+ - Alternative is therapeutic carotid sacrifice (occlusion) after ICA balloon test occlusion (destructive)
+ - Stent-supported coil embolization of pseudoaneurysm sac or placement of covered stent to exclude sac is usually curative
+ - Placement of noncovered stent may change hemodynamics within sac sufficiently to promote thrombosis without need for additional embolization with coils
+ - Endovascular embolization with Onyx (liquid embolic material) may also be curative
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - History of trauma, surgery ± radiation therapy for head and neck cancer
+- ### Image Interpretation Pearls
+
+
+ - True cervical carotid aneurysms are rare
+ - Look for associated trauma/dissection
+ - Multiplanar CTA reformats is usually diagnostic
+ - Look for associated stenosis, contained thrombus
+ - Thromboembolic events best seen with MR (DWI for acute; FLAIR for chronic)
+
+ b46d38c0-4d60-4e2c-9816-b5e601a8f175
+
+## References
+
+## Selected References
+
+1. [Bridge KI et al: Images in vascular medicine. Delayed Horner's syndrome as a presenting symptom of traumatic internal carotid artery dissection and pseudoaneurysm. Vasc Med. 16(2):159-60, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21511675%5Bpmid%5D)
+1. [Herrera DA et al: Endovascular treatment of penetrating traumatic injuries of the extracranial carotid artery. J Vasc Interv Radiol. 22(1):28-33, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21109458%5Bpmid%5D)
+1. [Asma A et al: Massive epistaxis secondary to pseudoaneurysm of internal carotid artery. Med J Malaysia. 61(1):84-7, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16708739%5Bpmid%5D)
+1. [Kaviani A et al: Infected carotid pseudoaneurysm and carotid-cutaneous fistula as a late complication of carotid artery stenting. J Vasc Surg. 43(2):379-82, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16476618%5Bpmid%5D)
+1. [Madan A et al: Traumatic carotid artery-cavernous sinus fistula treated with a covered stent. Report of two cases. J Neurosurg. 104(6):969-73, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16776343%5Bpmid%5D)
+1. [Gorriz-Gomez E et al: [Internal carotid artery pseudoaneurysm and stenosis: treatment with stents and coils.] Neurocirugia (Astur). 16(6):528-32, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16378136%5Bpmid%5D)
+1. [Mordekar SR et al: Occult carotid pseudoaneurysm following streptococcal throat infection. J Paediatr Child Health. 41(12):682-4, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16398875%5Bpmid%5D)
+1. [Koyanagi M et al: Stent-supported coil embolization for carotid artery pseudoaneurysm as a complication of endovascular surgery--case report. Neurol Med Chir (Tokyo). 44(10):544-7, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15633468%5Bpmid%5D)
+1. [Alexander MJ et al: Treatment of an iatrogenic petrous carotid artery pseudoaneurysm with a Symbiot covered stent: technical case report. Neurosurgery. 50(3):658-62, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=11841739%5Bpmid%5D)
+1. [Reisner A et al: Endovascular occlusion of a carotid pseudoaneurysm complicating deep neck space infection in a child. Case report. J Neurosurg. 91(3):510-4, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10470831%5Bpmid%5D)
+
+## Differential diagnosis
+
+### Carotid Space Lesion
+DDX:d413c68a-935b-41cb-a6d4-78c43acd0261
+
+### Horner Syndrome
+DDX:74926a92-b99e-40c0-a2e6-da909a3369de
+
+### Hyperdense Neck Lesion (CT)
+DDX:b9cff55e-ac3e-44b6-b1c6-799e93f449e3
+
+### Carotid Artery Lesion
+DDX:1a02ab19-467c-4d4b-95f8-0d7470357899
+
+## Cases
+
+- {'cases': [{'authors': [{'key': '890a0a02-eacf-42d9-b319-c41e515fc82e', 'value': 'Michelle A. Michel, MD'}, {'key': '624acd80-0502-4325-be71-e68fec740eb3', 'value': 'Richard H. Wiggins, III, MD, CIIP, FSIIM, FAHSE, FACR'}], 'caseVersionId': 'c6c54f38-8826-4d46-9ca6-cbb6c6f3efb3', 'description': 'Axial T2 (#1) and FLAIR (#2) MR images demonstrate abnormal hyperintense signal, consistent with mural thrombus in the wall of an internal carotid artery (ICA) pseudoaneurysm (arrows). A flow void (curved arrow) can be seen in the vessel of origin (ICA) posterior to the thrombus. A source image from an MRA examination (#3) shows flow-related signal in the distal left ICA (curved arrow) with mural thrombus anteriorly (arrows). The flow within the ICA is slightly deformed. The pseudoaneurysm is difficult to appreciate on the maximi intensity projection (MIP) MR image (#4). There is focal widening of the flow-related signal in the vessel proximally (arrows, #4), but it is poorly delineated. Conventional angiogram images (#5-8) show focal irregularity and dilatation of the ICA at the CAPA site (open arrows, #5) and better filling of the pseudoaneurysm (arrows) on delayed images (#6-8).', 'history': None, 'imagePoolId': 'f3a8b226-34f7-4ae8-b6a7-cea1775e21a4', 'name': 'Distal ICA', 'teachingPoint': 'This case shows the MR and angiographic features of a carotid artery pseudoaneurysm (CAPA) just below the skull base.'}, {'authors': [{'key': '492a5336-3f0a-4e41-9824-96828aafab79', 'value': 'Rebecca S. Cornelius, MD, FACR'}], 'caseVersionId': 'f04aee82-1efb-4caa-84d2-ab5cb6362318', 'description': 'This is a typical CTA case of bilateral internal carotid artery fibromuscular dysplasia with a small pseudoaneurysm.\n\nCoronal (#1) and sagittal (#2-3) CTA reconstructions show diffuse wall irregularity in both internal carotid arteries (arrows, #1-3). A subtle pseudoaneurysm is visible in the high cervical left internal carotid artery (curved arrow, #1). CTA volume-rendered coronal oblique (#4) and coronal (#5) images better demonstrate the diffuse bilateral internal carotid artery irregularity (arrows, #4-5) with some areas showing the classic "string of beads" appearance (open arrows, #4). Note the greater conspicuity of the small pseudoaneurysm (curved arrow, #5) on the coronal volume-rendered sequence.', 'history': 'Patient presented with neck pain and headache. Initial head CT and brain MRI were performed; questioned abnormality on brain MRI prompted CTA study.', 'imagePoolId': '040bffd1-f86b-41b0-a2b5-f597632d027c', 'name': 'Bilateral internal carotid artery involvement', 'teachingPoint': None, 'demographics': '44 Years old female'}, {'authors': [{'key': '890a0a02-eacf-42d9-b319-c41e515fc82e', 'value': 'Michelle A. Michel, MD'}, {'key': '624acd80-0502-4325-be71-e68fec740eb3', 'value': 'Richard H. Wiggins, III, MD, CIIP, FSIIM, FAHSE, FACR'}], 'caseVersionId': '9594240a-d5bc-4791-aa9a-0fc11e380dd9', 'description': 'Axial CT image (#1) obtained during CT angiography shows a strongly enhancing, well-defined mass (arrows) in the right carotid space just below the skull base. The normal ICA cannot be identified and the lesion enhances even more strongly than adjacent venous structures (curved arrows). An image from a digital subtraction angiogram (#2) during injection of the ICA demonstrates contrast filling a large saccular pseudoaneurysm (arrows). The pattern of filling in the aneurysm is somewhat circular and it has not completely filled with contrast in this early arterial phase of the injection (note phase of filling of cerebral artery branches). There is mass effect upon the internal carotid artery medial to the CAPA. There is focal irregular dilatation of the ICA (open arrow) at the neck of the CAPA.', 'history': None, 'imagePoolId': 'f717223f-0cab-48d2-a082-3137bdddd831', 'name': 'Large, ICA', 'teachingPoint': 'This case shows the CT and angiographic features of a carotid artery pseudoaneurysm (CAPA) of the internal carotid artery (ICA) located below the skull base.'}, {'authors': [{'key': '890a0a02-eacf-42d9-b319-c41e515fc82e', 'value': 'Michelle A. Michel, MD'}, {'key': '6651ae1c-5f55-4d2e-9f68-46223037c90a', 'value': ' , '}], 'caseVersionId': '86e05f80-9925-459c-af43-ffd26b11ab6f', 'description': 'This case demonstrates the classic CT features of a carotid artery pseudoaneurysm (CAPA).\n\nAxial CECT images (#1-3) show a mixed density mass in the right neck below the angle of the mandible (white arrows). The lesion is located within the right carotid space medial to the internal jugular vein (black arrow). The common carotid artery (CCA) cannot be identified on this side (note normal carotid anatomy on the left, open arrows). The central portion of the lesion enhances to the same degree as other vasculature consistent with flow within the CAPA and it is surrounded by mural thrombus of soft tissue attenuation. A punctate focus of vessel wall calcification is noted medially (curved arrow, #1).\n\nComment: CAPA may have a saccular or fusiform morphology. This lesion, near the bifurcation, is more fusiform in appearance. The enhancement similar to that of other vasculature and the presence of calcification in the vessel wall are important clues to the diagnosis. In this location, a traumatic or atherosclerotic etiology would be suspected.', 'history': None, 'imagePoolId': '453d099c-04bb-43e8-a4d9-264cc2f66677', 'name': 'Fusiform, near the bifurcation', 'teachingPoint': None, 'demographics': '49 Years old male'}], 'caseType': 'typical', 'name': 'TYPICAL'}
+- {'cases': [{'authors': [{'key': '33151213-01b2-4542-9105-342e006b3915', 'value': 'H. Ric Harnsberger, MD'}], 'caseVersionId': 'aef69931-2070-487a-b949-62f1168d360d', 'description': 'Variant CECT case of internal carotid artery pseudoaneurysm after a stenting procedure.\n\nAxial CT angiography images presented from superior to inferior (#1-7) reveal a high density (contrast-filled) carotid artery pseudoaneurysm (curved arrow) with subintimal clot seen as low density crescentic areas in the wall. The pseudoaneurysm is connected superiorly with the internal carotid artery (arrow, #1-4). Coronal (#8-9) and sagittal (#10) reformations show the common carotid stent (open arrow) inferiorly with the pseudoaneurysm perched on its superior margin (curved arrow) and the internal carotid artery emerging from the superomedial wall (arrow, #8).', 'history': 'Patient presents after carotid artery stenting for atherosclerotic narrowing with new neck mass and a pulsatile sound in the right ear.', 'imagePoolId': '5b9216f7-7ca3-4705-b623-5966ca4848eb', 'name': 'Post-stenting dissection with pseudoaneurysm', 'teachingPoint': None, 'demographics': '66 Years old male'}, {'authors': [{'key': '890a0a02-eacf-42d9-b319-c41e515fc82e', 'value': 'Michelle A. Michel, MD'}, {'key': '33151213-01b2-4542-9105-342e006b3915', 'value': 'H. Ric Harnsberger, MD'}], 'caseVersionId': '34ab1059-6ffd-43e9-aabb-a7f8269d0fbf', 'description': 'This is a case of a large, complex carotid artery pseudoaneurysm after prior head and neck surgery demonstrated with CT and angiography.\n\nAxial contrast-enhanced CT images (#1-8) demonstrate a large, complex pseudoaneurysm (arrows) arising from the distal right common carotid artery (open arrows, #2-4). There is irregular filling of the patent pseudoaneurysm lumen with a significant amount of thrombus filling portion of the sac. The thrombus has a slightly lamellar appearance on the inferior images (#7-8). Images obtained during conventional angiography (#9-12) show the large pseudoaneurysm (arrows) with irregular, lobular filling of multiple portions of the lumen. There is irregularity and dilatation in the vessel just distal to the origin of the aneurysm (open arrow, #12). The intra-arterial study evaluates only the patent portions of the sac and underestimates the overall size of the lesion. This patient had previous surgery for head and neck cancer surgery with a mandibular resection and flap reconstruction. Damage to the artery during the time of tumor resection or from invasion by the previous tumor, or damage to the vessel during post-operative radiation therapy could have contributed to the development of this lesion.', 'history': None, 'imagePoolId': 'f80f2549-4cf0-4e1f-ba59-84e0a3dd8ca8', 'name': 'Large, therapy related', 'teachingPoint': None}, {'authors': [{'key': '890a0a02-eacf-42d9-b319-c41e515fc82e', 'value': 'Michelle A. Michel, MD'}, {'key': '94f835c8-fa13-4e8a-995b-53048e6b0605', 'value': 'Philip R. Chapman, MD'}], 'caseVersionId': 'fa2614fb-42e3-49f5-95a9-c6d679c1c9b6', 'description': 'This case shows the CT and MR imaging features of a carotid artery pseudoaneurysm (CAPA) located at the bifurcation.\n\nAn axial T1 MR image (#1) demonstrates a flow void (arrow) within the distal right common carotid artery (CCA) surrounded with intermediate signal material (open arrows) consistent with mural thrombus within the CAPA. The mural thrombus (open arrows) is hypointense on T2 MR (#2) and shows little enhancement on the post-gadolinium axial (#3) and coronal (#4) T1 MR images with fat suppression. The wall of the pseudoaneurysm enhances and contrast is also noted within the central aspect of the vessel. The CAPA was further evaluated with contrast-enhanced CT (#5-12). The axial images (#5-10) show the largely thrombosed pseudoaneurysm (arrows) with a central enhancing lumen. The coronal (#11) and sagittal (#12) reformatted images demonstrate the thrombus (open arrows) surrounding the lumen (curved arrow). Calcifications are appreciated in the wall of the vessel and given the patients history of cardiovascular risk factors, the etiology of this CAPA was likely atherosclerotic.', 'history': 'Patient with a palpable right neck mass. Patient had a long history of hypertension, smoking, and elevated cholesterol.', 'imagePoolId': 'ef9679ac-d123-43de-9147-49bd0c66791c', 'name': 'Thrombosed, atherosclerotic', 'teachingPoint': None, 'demographics': '68 Years old female'}], 'caseType': 'variant', 'name': 'VARIANT'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*A 41-year-old patient, in a car accident 4 weeks earlier, presenting with a small right middle cerebral artery (MCA) infarction at the time of initial trauma. Axial CTA of the neck shows tapering occlusion of the right internal carotid artery (ICA)
and relatively normal lumen of the left ICA near the skull base
.*
+
+
+*A 41-year-old patient, in a car accident 4 weeks earlier, presenting with a small right middle cerebral artery (MCA) infarction at the time of initial trauma. Axial CTA of the neck shows tapering occlusion of the right internal carotid artery (ICA)
and relatively normal lumen of the left ICA near the skull base
.*
+
+
+*Axial T1WI FS MR obtained with presaturation pulses at inferior and superior margin of slab shows hyperintense crescentic mural hematoma on the right
. ICA was occluded just superior to this section.*
+
+
+*Axial CTA of the same patient with ICA occlusion
secondary to dissection and contralateral pseudoaneurysm shows that the left ICA lumen is irregularly dilated near the carotid canal
, representing ICA pseudoaneurysm.*
+
+
+*Oblique MRA (same patient) demonstrates tapering occlusion of the right ICA just above the bifurcation
. The left ICA is remarkable for ICA pseudoaneurysm at the skull base, just proximal to the carotid canal
. The pseudoaneurysm was subsequently embolized.*
+
+
+*Axial CECT of a 36-year-old patient with asymmetric palate and neck fullness shows a carotid space mass that represents markedly enlarged right ICA with normal enhancement in a dilated lumen
(compare with normal left ICA at the bulb just above the bifurcation
).*
+
+
+*Axial T2WI MR at a similar level (same patient) with hypointense signal of lumen of ICA pseudoaneurysm
reveals little thrombus in the wall of this pseudoaneurysm with only marginal T2 hyperintensity.*
+
+
+*Axial T1WI C+ FS MR with enhancement of the lumen
of the dilated pseudoaneurysm of the right ICA reveals no obvious mural thrombus. The left ICA
has a normal caliber.*
+
+
+*Axial CECT in a 74-year-old patient with longstanding pulsatile mass of the left neck shows peripheral calcifications
as well as laminated layers of mural thrombus
along the anterior and medial aspect of heterogeneous carotid space mass. The lumen is opacified with contrast and markedly dilated
.*
+
+
+*Axial T1WI MR (same patient) shows a carotid space mass with a thickened irregular wall and laminated mural thrombus
. The patent lumen with flow is hypointense and irregularly ovoid
, as in the prior image.*
+
+
+*Oblique DSA from a left common carotid artery injection, with a pseudoaneurysm sac
filling slowly with contrast, shows the lumen of the ICA proximal and distal to the aneurysm with an irregular string of pearls appearance
secondary to fibromuscular disease.*
+
+
+### Additional Images
+
+
+*Axial CTA shows an enhancing carotid space mass consistent with a large saccular pseudoaneurysm of the cervical ICA
with compression of the native ICA vessel lumen
.*
+
+
+*AP right CCA DSA shows a large pseudoaneurysm of the cervical ICA
. Note slow flow ± thrombus within the pseudoaneurysm with mass effect and displacement of the adjacent ICA
and ECA
.*
+
+
+*Left ICA DSA shows a boot-shaped saccular pseudoaneurysm
with associated luminal irregularity of the adjacent parent vessel, probably the sequela of previous dissection
.*
+
+
+*Left ICA DSA after stent-supported coil embolization shows preservation of the parent vessel lumen by the stent
, and coil packing of the pseudoaneurysm lumen
.*
+
+
+*3D DSA shows a large cavernous ICA pseudoaneurysm
in a patient who underwent transsphenoidal pituitary surgery complicated by massive epistaxis. This was treated with a covered stent.*
+
+
+*Lateral CCA DSA shows a fusiform pseudoaneurysm of the distal cervical ICA
in a patient with prior closed head injury. Irregularity of its wall
is suggestive of contained thrombus.*
+
diff --git a/docs_md/articles/central-skull-base_8887bf04-6027-42cb-9a34-3e15c6cc27ff.md b/docs_md/articles/central-skull-base_8887bf04-6027-42cb-9a34-3e15c6cc27ff.md
new file mode 100644
index 0000000..968ef4f
--- /dev/null
+++ b/docs_md/articles/central-skull-base_8887bf04-6027-42cb-9a34-3e15c6cc27ff.md
@@ -0,0 +1,284 @@
+---
+title: "Central Skull Base"
+docid: "8887bf04-6027-42cb-9a34-3e15c6cc27ff"
+authors:
+ - key: "a25c450b-3d34-4f64-bba3-cc0834813df6"
+ value: "Miral D. Jhaveri, MD, MBA"
+ - key: "94f835c8-fa13-4e8a-995b-53048e6b0605"
+ value: "Philip R. Chapman, MD"
+breadcrumbs:
+ -
+ name: "Head and Neck"
+ slug: "head-and-neck"
+ treeNodeId: "5c1f8e17-7acd-48d8-9d55-f9f8c2cad850"
+ -
+ name: "Anatomy"
+ slug: "anatomy"
+ treeNodeId: "5deb3a75-762a-49d7-8d1c-dffda4a1b190"
+ -
+ name: "Skull Base"
+ slug: "skull-base"
+ treeNodeId: "d613fd1d-ce43-4be4-85b1-508426706997"
+ -
+ name: "Central Skull Base"
+ slug: "central-skull-base"
+ treeNodeId: null
+category: "Head and Neck"
+documentVersionId: "427f951f-e43a-476d-b6d9-23745e9c66c4"
+imageCount: 25
+lastUpdated: "01/30/24"
+pageDescription: "Central Skull Base"
+pageKeywords: "Head and Neck, Anatomy, Skull Base, Central Skull Base"
+pageTitle: "Central Skull Base | STATdx"
+enhancedTitle: "Central Skull Base"
+type: "ANATOMY"
+breadcrumbs:
+ - "Head and Neck"
+ - "Anatomy"
+ - "Skull Base"
+ - "Central Skull Base"
+---
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - Anterior, central, posterior skull base (ASB, CSB, PSB)
+ - Greater, lesser wings of sphenoid (GWS, LWS)
+- ### Definitions
+
+
+ - CSB: Skull base posterior to LWS/planum sphenoidale & anterior to petrous ridge/dorsum sella
+
+## IMAGING ANATOMY
+
+- ### Overview
+
+
+ - CSB is floor of middle cranial fossa & roof of sphenoid sinus
+ - Bones forming CSB
+ - Sphenoid bone, basisphenoid, & GWS
+ - Temporal bone anterior to petrous ridge
+ - Boundaries of CSB
+ - Anteriorly boundary: Planum sphenoidale posterior margin (limbus sphenoid) medially & LWS laterally
+ - Posterior boundary: Dorsum sella medially & petrous ridges laterally
+ - Lateral boundary: Squamous temporal bone & GWS
+ - Relationships of CSB
+ - Superior: Pituitary, cavernous sinus, Meckel cave, CNI-IV, CNVI, CNV1-3, temporal lobe
+ - Inferior: Anterior roof of pharyngeal mucosal space, masticator, parotid & parapharyngeal spaces
+- ### Bony Landmarks of Central Skull Base
+
+
+ - **Sella turcica**: Contains pituitary gland
+ - Latin term for Turkish saddle
+ - **Anterior clinoid processes**: Extend posteromedially off LWS
+ - Anteriorly and superiorly, merges with upper flat surface of LWS
+ - Anteriorly and inferiorly, merges with optic strut that becomes lateral margin of optic canal
+ - Variant: Posterior inferior strut fuses to sphenoid body creating complete bony ring around cavernous Internal carotid
+ - **Posterior clinoid processes**: Extend posterolaterally off dorsum sellae; attachment for tentorium cerebelli
+ - **Chiasmatic sulcus**: Shallow groove between posterior margin of planum sphenoidale and tuberculum sella
+ - Optic chiasm is not in sulcus, but sits posterior and superior to sulcus
+ - Some authors prefer term "prechiasmatic sulcus" since it is actually anterior to chiasm
+ - Chiasmatic sulcus is shallow trough that extends transversely between medial optic nerve canals
+ - **Tuberculum sellae**: Anterosuperior margin of sella turcica
+- ### Foramina and Fissures of Central Skull Base
+
+
+ - **Optic canal**
+ - Transmits: CNII with dura, arachnoid & pia, CSF & ophthalmic artery
+ - Formed by LWS, superomedial to superior orbital fissure
+ - **Superior orbital fissure (SOF)**
+ - Transmits: CNIII, CNIV, CNV1, & CNVI, superior ophthalmic vein
+ - Formed by cleft between LWS & GWS
+ - SOF is separated from optic canal by optic strut, variably pneumatized extension from sphenoid body
+ - **Inferior orbital fissure**
+ - Transmits: Infraorbital artery, vein, & nerve (CNV2)
+ - Formed by cleft between body of maxilla & GWS
+ - **Carotid canal**
+ - Transmits: Internal carotid artery & sympathetic plexus
+ - Formed by GWS & temporal bone
+ - **Foramen rotundum**
+ - Transmits: CNV2, artery of foramen rotundum, & emissary veins
+ - Within sphenoid bone; superolateral to vidian canal
+ - Provides direct connection to pterygopalatine fossa
+ - **Foramen ovale**
+ - Transmits: CNV3, lesser petrosal nerve, accessory meningeal branch of maxillary artery, & emissary vein
+ - Within GWS, connection to masticator space
+ - **Foramen spinosum**
+ - Transmits: Middle meningeal artery & vein, meningeal branch of CNV3
+ - Within GWS, posterolateral to foramen ovale
+ - **Foramen lacerum**
+ - Not true foramen, between temporal & sphenoid bones
+ - Cartilaginous floor of medial part of horizontal petrous internal carotid artery canal
+ - **Vidian canal**
+ - Transmits: Vidian artery and nerve
+ - Inferomedial to foramen rotundum
+- ### Development of Central Skull Base
+
+
+ - CSB formed by > 25 ossification centers
+ - Ossification occurs from posterior to anterior
+ - **Important ossification centers**: Orbitosphenoids, alisphenoids, pre- and postsphenoid, basiocciput
+ - **Orbitosphenoids** → LWS, **alisphenoids** → GWS
+ - **Presphenoid** and **postsphenoid** fuse at ~ 3 months
+ - **Postsphenoid** and **basiocciput** fuse → clivus
+ - **Sphenooccipital synchondrosis**
+ - Between postsphenoid and basiocciput
+ - Responsible for most of postnatal skull base growth
+ - One of last sutures of skull base to fuse
+ - Open until 14 years, fuses by ~ 16 years in girls & ~ 18 years in boys
+- ### Variant Anatomy
+
+
+ - **Palatovaginal canal**
+ - Between vaginal process of sphenoid bone & sphenoid process of palatine bone
+ - May appear as a groove instead of complete canal (15%)
+ - Connect nasopharynx to pterygopalatine fossa
+ - Inferomedial to vidian canal
+ - Contains pterygovaginal artery, posterior branch of internal maxillary artery & pharyngeal nerve
+ - **Vomerovaginal canal**
+ - Variable, may communicate with palatovaginal canal
+ - Between alae of the vomer & vaginal process of sphenoid body
+ - **Persistent craniopharyngeal canal**
+ - Remnant of Rathke pouch or vascular channel formed during osteogenesis
+ - Vertical cleft in sphenoid body
+ - At site of fusion of pre- & postsphenoid
+ - Extends from floor of sella turcica to nasopharynx
+ - Classification of craniopharyngeal canal
+ - Type 1: Incidental canal
+ - Type 2: Medium-sized canal, contains ectopic adenohypophysis
+ - Type 3A: Contain encephalocele
+ - Type 3B: Contain tumors (pituitary adenoma, craniopharyngioma, dermoid, teratoma & glioma)
+ - Type 3C: Features of both type 3A & 3B
+ - **Extensive pneumatization of sphenoid sinus**
+ - Can cause endosinal vidian canals & foramen rotundum
+ - Pneumatized clinoid processes
+ - **Canaliculus innominatus**
+ - Medial between foramen ovale & foramen spinosum
+ - Variant canal for lesser superficial petrosal nerve
+ - **Foramen of Vesalius**
+ - Anteromedial to foramen ovale, transmits emissary vein from cavernous sinus to pterygoid plexus
+ - Frequently symmetric when present
+ - Asymmetry usually result of pathologic process (carotid-cavernous fistula, tumor invasion)
+ - **Canalis basilaris medianus**
+ - Vestige of cephalic portion of notochordal canal or remnant of emissary vein
+ - Well-corticated channel along midline basiocciput
+ - Subdivided into complete & incomplete channels
+ - Typically asymptomatic, can be associated with meningitis
+ - **Fossa navicularis magna**
+ - Osseous defect along anterior aspect of clivus
+ - Contains lymphoid tissue, loose connective tissue, or gliotic tissue
+ - **Sternberg canal**
+ - Osseous defect between sphenoid body & lesser wing
+ - Medial to SOF & foramen rotundum
+ - Rare etiologic factor for spontaneous CSF leak & lateral sphenoid meningoencephalocele
+
+## ANATOMY IMAGING ISSUES
+
+- ### Imaging Pitfalls
+
+
+ - Beware sphenoid MR signal changes
+ - Sphenoid sinus: Low-signal cartilage until 2 years → high-signal fat until 6 years → low-signal air (adult)
+ - Clivus low signal until 25 years, then high-signal fat
+ - "Don't touch me" lesion: Arrested pneumatization of sphenoid, persistent atypical fatty marrow
+ - Do not confuse pneumatized clinoid processes with vascular flow voids on MR
+
+ 6adf598e-e074-4bfd-ba8c-957cb8dcecc0
+
+
+## Images
+
+
+### Graphics
+
+
+*Graphic of the central skull base (CSB) from above shows important nerves on the left. The numerous fissures & foramina of CSB are shown on the right. Greater wing of sphenoid forms anterior wall of middle cranial fossa. The posterior limit of the CSB is the dorsum sella medially & petrous ridge laterally.*
+
+
+*Graphic of the central skull base (CSB) from above shows important nerves on the left. The numerous fissures & foramina of CSB are shown on the right. Greater wing of sphenoid forms anterior wall of middle cranial fossa. The posterior limit of the CSB is the dorsum sella medially & petrous ridge laterally.*
+
+
+*Sagittal graphic through the central & anterior skull base depicts the trigeminal nerve branches & exiting foramina. Ophthalmic division of CNV exits into orbit via the superior orbital fissure. Maxillary division of CNV exits via foramen rotundum to become infraorbital nerve as well as give rise to the greater & lesser palatine nerves inferiorly to provide sensation for the hard & soft palates. Mandibular division of CNV exits through foramen ovale, then divides into 2 main trunks, lingual & inferior alveolar nerves. Note the vidian nerve in vidian canal.*
+
+
+*Graphic of CSB from above shows its many ossification centers. Between the ossification centers of presphenoid is a cartilaginous gap called the olivary eminence, which is obliterated shortly after birth. A persistent cleft, called the craniopharyngeal canal, can also be variably seen in intersphenoid synchondrosis. Do not confuse these variants with pathology.*
+
+
+*Lateral graphic of CSB shows major ossification centers & the location of sutures. Intersphenoidal suture closes at ~ 3 months age. At ~ 2 years of age, the presphenoid begins to demineralize & become pneumatized. Pneumatization progresses posteriorly into postsphenoid until ~ 5-7 years of age. Sphenooccipital synchondrosis is one of the last sutures to fuse at ~ 16 years of age. It is the suture most responsible for growth of the skull base.*
+
+
+### Axial Bone CT
+
+
+*First of 9 axial bone CT images of the CSB presented from superior to inferior is shown. Note that the posterior clinoids merge with the dorsum sella. The optic canal is bound by the sphenoid sinus medially and the anterior clinoid process laterally. Inferolateral to optic canal is the superior orbital fissure.*
+
+
+*At the level of the sella turcica, the superior orbital fissure is seen as the medial opening of the orbit into the middle cranial fossa. It lies below the optic canal, between the greater wing of the sphenoid and the sphenoid body. The sella turcica is bound by the dorsum sella posteriorly.*
+
+
+*In this image, the body of the sphenoid bone is seen to be made up of the sphenoid sinus, sella turcica, and dorsum sella. Anterior to the sphenoid bone is the ethmoid bone.*
+
+
+*In this image, the clivus can be seen forming the medial posterior boundary of CSB, while the petrous ridge defines its lateral posterior margin.*
+
+
+*This image shows the inferior orbital fissure along the floor of the orbit inferior to the superior orbital fissure. It is bounded superiorly by the greater wing of sphenoid, inferiorly by the maxilla and orbital process of palatine bone, and laterally by the zygomatic bone.*
+
+
+*At the level of the foramen rotundum, both pterygopalatine fossae are clearly visible. The maxillary division of the trigeminal nerve (CNV2) exits the skull base through the foramen rotundum & continues as the infraorbital nerve into orbit via the inferior orbital fissure. Malignant tumors of the skin of the cheek, orbit, & sinonasal area may all use CNV2 as a perineural route to gain intracranial access. Note the foramen rotundum empties anteriorly into the pterygopalatine fossa, which connects laterally with the masticator space through the pterygomaxillary fissure.*
+
+
+*In this image, the vidian canal is visible connecting the pterygopalatine fossa anteriorly to the carotid canal floor (foramen lacerum) posteriorly. A malignant tumor that has accessed the pterygopalatine fossa may reach the carotid canal of the skull base via perineural spread on the vidian nerve in the vidian canal. There is a medial connection between the pterygopalatine fossa & nose, the sphenopalatine foramen. Juvenile angiofibroma begins along the nasal margin of this foramen.*
+
+
+*In this image, note that the foramen ovale is located in the greater wing of the sphenoid bone. Extracranial perineural malignancy on CNV3 enters the intracranial area via the foramen ovale.*
+
+
+*In this image, note the foramen spinosum is posterolateral to the foramen ovale in the greater wing of the sphenoid bone. The middle meningeal artery passes intracranially via the foramen spinosum. The inconstant foramen of Vesalius is anteromedial to the foramen ovale. The inconstant canaliculus innominatus is between the foramen ovale and foramen spinosum.*
+
+
+### Coronal Bone CT
+
+
+*First of 3 coronal bone CT images of the CSB presented from posterior to anterior is shown. The foramen lacerum is seen as a large defect between the greater wing of the sphenoid bone and the sphenoid body. The foramen lacerum is not a true foramen; it represents the cartilaginous floor of the anteromedial horizontal segment of the petrous internal carotid artery canal.*
+
+
+*In this image, the foramen ovale is evident lateral to the vidian canal and anterolateral to the foramen lacerum. It transmits CNV3 from the middle cranial fossa to the masticator space.*
+
+
+*More anteriorly, the foramen rotundum and vidian canal are both seen running in the transverse plane. Both the foramen rotundum and vidian canal open into the pterygopalatine fossa. Also note the pterygoid plates inferiorly.*
+
+
+### Axial T1 C+ MR
+
+
+*First of 6 axial T1 C+ MR images of the CSB presented from superior to inferior is shown. The enhancing venous plexus of the cavernous sinus is seen surrounding the cavernous internal carotid artery. Medially, the enhancing pituitary gland in the sella turcica is bound by the dorsum sella posteriorly and the sphenoid sinus anteriorly.*
+
+
+*In this image, the upper basisphenoid part of the clivus is seen. Cerebrospinal fluid-filled Meckel cave is seen along the posterior border of the cavernous sinus.*
+
+
+*In this image, the basiocciput part of the clivus is visible. The upper clivus above the fused sphenooccipital synchondrosis is part of the sphenoid bone, while the lower clivus is part of the occipital bone. Notice the marrow space of the clivus enhances.*
+
+
+*Image through the superior pterygopalatine fossa shows its anterolateral connection to the inferior orbital fissure. The anteriorly projecting foramen rotundum can also be seen. The sphenoid bone is partially pneumatized (sphenoid sinus).*
+
+
+*In this image, the maxillary nerve (CNV2) is seen as a linear low-intensity structure in the foramen rotundum on the right. On the left, this same nerve can be seen exiting the foramen rotundum into the pterygopalatine fossa.*
+
+
+*At the level of the foramen ovale, the mandibular nerve (CNV3) is seen bilaterally. Also note the middle meningeal artery passing through the foramen spinosum. The vidian canal is clearly visible medial to the foramen ovale. The clival occipital bone should be distinguished from the body of the sphenoid bone even though the sphenooccipital fissure cannot be discerned.*
+
+
+### Sagittal T1 & T2 MR, Development
+
+
+*Sagittal T2 MR of the CSB in a newborn shows the important synchondroses of this area. The intersphenoidal suture separates presphenoid from postsphenoid while the sphenooccipital synchondrosis separates postsphenoid from basiocciput.*
+
+
+*Sagittal T1 MR shows the CSB at 6 months. The intersphenoidal suture closes at ~ 3 months of age, resulting in formation of the sphenoid body from the presphenoid and postsphenoid. There is normal high-signal fat within what used to be presphenoid. The sphenooccipital synchondrosis will remain open until adolescence.*
+
+
+*Sagittal T2 MR shows the CSB in an adult. Typically, pneumatization extends throughout the entire sphenoid body up to the fused sphenooccipital synchondrosis. The sphenooccipital synchondrosis is one of last sutures of the skull base to close. It fuses completely by ~ 16-18 years of age.*
+
diff --git a/docs_md/articles/cerebral-amyloid-disease-inflammatory_23aa6e14-b5eb-48c9-8191-e53042e55d51.md b/docs_md/articles/cerebral-amyloid-disease-inflammatory_23aa6e14-b5eb-48c9-8191-e53042e55d51.md
new file mode 100644
index 0000000..44ed050
--- /dev/null
+++ b/docs_md/articles/cerebral-amyloid-disease-inflammatory_23aa6e14-b5eb-48c9-8191-e53042e55d51.md
@@ -0,0 +1,386 @@
+---
+title: "Cerebral Amyloid Disease, Inflammatory"
+docid: "23aa6e14-b5eb-48c9-8191-e53042e55d51"
+authors:
+ - key: "2bca6b86-1eca-4e93-b997-4e18913686a7"
+ value: "Hediyeh Baradaran, MD, MS"
+ - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
+ value: "Anne G. Osborn, MD, FACR"
+ - key: "8d5254e9-8dda-478b-8f08-bdee97a32c79"
+ value: "Karen L. Salzman, MD, FACR"
+breadcrumbs:
+ -
+ name: "Brain"
+ slug: "brain"
+ treeNodeId: "0361042c-9c90-48e4-864e-a2e6428ee9c9"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "948c6f1e-8e18-45f3-bd9d-7a04ab0b8708"
+ -
+ name: "Pathology-Based Diagnoses"
+ slug: "pathology-based-diagnoses"
+ treeNodeId: "d60fe914-7897-46a0-9cd2-f88ffb322f16"
+ -
+ name: "Stroke"
+ slug: "stroke"
+ treeNodeId: "7a135176-0a69-4fc9-b200-59569fbf5166"
+ -
+ name: "Nonatheromatous Vasculopathy"
+ slug: "nonatheromatous-vasculopathy"
+ treeNodeId: "2ccf261b-3d7a-42a7-8041-8ed08dd81bff"
+ -
+ name: "Cerebral Amyloid Disease, Inflammatory"
+ slug: "cerebral-amyloid-disease-inflammat-"
+ treeNodeId: null
+category: "Brain"
+cmeTopicId: "696f3910-8e80-41a8-84c1-355f32266e29"
+documentVersionId: "92035be9-42ab-4ff8-a45b-5722ae6f53e7"
+imageCount: 19
+lastUpdated: "08/19/25"
+pageDescription: "Cerebral Amyloid Disease, Inflammatory"
+pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Stroke, Nonatheromatous Vasculopathy, Cerebral Amyloid Disease, Inflammatory"
+pageTitle: "Cerebral Amyloid Disease, Inflammatory | STATdx"
+enhancedTitle: "Cerebral Amyloid Disease, Inflammatory"
+type: "DX"
+references: true
+breadcrumbs:
+ - "Brain"
+ - "Diagnosis"
+ - "Pathology-Based Diagnoses"
+ - "Stroke"
+ - "Nonatheromatous Vasculopathy"
+ - "Cerebral Amyloid Disease, Inflammatory"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Cerebral amyloid angiopathy (CAA)
+ - No inflammation
+ - CAA-related inflammation (CAA-RI)
+ - Perivascular inflammation
+ - Amyloid β-related angiitis (ABRA)
+ - Angiodestructive inflammation
+- ### Imaging
+
+
+ - Best diagnostic clue: Infiltrative white matter changes with leptomeningeal enhancement and cerebral microbleeds
+ - Diffuse lobar edema
+ - Less common: Focal, mass-like ± parenchymal enhancement
+ - Intracerebral lobar hemorrhage more frequent in CAA without inflammation
+ - Cortical-subcortical microbleeds, superficial siderosis
+ - CAA-RI and ABRA have overlapping imaging features
+- ### Top Differential Diagnoses
+
+
+ - Diffusely infiltrating glioma
+ - Microbleeds less common
+ - Other types of vasculitis
+ - Often slightly younger patients
+ - Amyloid-related imaging abnormalities (ARIA)
+- ### Pathology
+
+
+ - CAA: Amyloid-β deposition in media, adventitia of cortical and leptomeningeal vessels
+ - CAA-RI: Perivascular inflammatory reaction around amyloid-laden vessels
+ - ABRA: Vasculitic transmural, often granulomatous, inflammatory infiltration
+ - Angiodestructive with necrosis, variable multinucleated giant cells
+- ### Clinical Issues
+
+
+ - CAA-RI, ABRA respond to immunosuppressive treatment
+ - If biopsied, include leptomeningeal and gray-white junction
+ - Patients with CAA-RI, ABRA tend to be younger than CAA
+- ### Diagnostic Checklist
+
+
+ - CAA-RI and ABRA are not distinguishable by imaging
+
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - Cerebral amyloid angiopathy-related inflammation (CAA-RI)
+ - Amyloid-β related angiitis (ABRA)
+- ### Synonyms
+
+
+ - Cerebral amyloid inflammatory vasculopathy
+ - Cerebral amyloid-β angiitis
+- ### Definitions
+
+
+ - CAA: No inflammation
+ - CAA-RI: Perivascular inflammatory reaction around amyloid-laden vessels
+ - ABRA: Vasculitic transmural, often granulomatous, inflammatory infiltration
+ - Angiodestructive with necrosis, variable multinucleated giant cells
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Patient with CAA, presenting with asymmetric patchy or confluent subcortical FLAIR hyperintensities (vasogenic edema) with microhemorrhages
+ - May have sulcal or leptomeningeal enhancement, lobar hemorrhage, cortical superficial siderosis
+ - CAA-RI and ABRA imaging features overlap
+ - #### Location
+
+
+ - Typically supratentorial subcortical white matter (WM)
+ - Leptomeningeal involvement
+ - #### Size
+
+
+ - Asymmetric WM hyperintensities can be large
+ - Microbleeds are smaller, noted on GRE/SWI imaging
+ - #### Morphology
+
+
+ - Subcortical WM, can have cortical involvement
+- ### CT Findings
+
+
+ - #### NECT
+
+
+ - Asymmetric subcortical low attenuation
+ - May have other similar findings as noninflammatory CAA
+ - Parenchymal volume loss common
+- ### MR Findings
+
+
+ - #### T2WI
+
+
+ - Subcortical and cortical T2 hyperintensity
+ - Usually asymmetric
+ - #### FLAIR
+
+
+ - Subcortical and cortical T2 hyperintensity
+ - Usually asymmetric
+ - Can have some mild mass effect
+ - Sulcal failure of CSF suppression
+ - #### T2* GRE
+
+
+ - Microbleeds
+ - Often in region of abnormal WM signal
+ - Diffuse, bilateral and peripheral in location
+ - Often more than in noninflammatory CAA
+ - Cortical superficial siderosis
+ - #### DWI
+
+
+ - Ischemic infarcts seen in ~ 30% of patients
+ - Usually small
+ - #### T1WI C+
+
+
+ - Leptomeningeal enhancement in regions of WM hyperintensity
+ - Can have focal mass-like enhancement
+ - #### MRA
+
+
+ - Usually normal
+- ### Imaging Recommendations
+
+
+ - #### Best imaging tool
+
+
+ - MR with FLAIR, SWI, and T1 C+
+ - #### Protocol advice
+
+
+ - SWI and T1 C+ will aid in confirming diagnosis
+
+## DIFFERENTIAL DIAGNOSIS
+
+- ### Vasculitis
+
+
+ - May be difficult to distinguish on imaging
+ - Vasculitis patients often younger
+ - Vasculitis may have fewer peripheral microbleeds
+ - May have small infarcts and subarachnoid hemorrhage
+ - DSA: Multifocal vascular narrowing of small & medium-sized vessels
+- ### Neoplasm
+
+
+ - Neoplasms will have more mass effect, usually fewer diffuse microbleeds
+- ### Amyloid-Related Imaging Abnormalities
+
+
+ - Must have history of anti-amyloid immunotherapy
+ - Similar imaging features
+- ### Posterior Reversible Encephalopathy Syndrome
+
+
+ - Fewer microbleeds and no superficial siderosis
+ - Typically less confluent and more symmetric
+ - Inflammatory CAA lacks typical posterior reversible encephalopathy syndrome (PRES) predisposing factors
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - Amyloid-β deposition in cortical or leptomeningeal vessels leading to inflammatory response
+- ### Staging, Grading, & Classification
+
+
+ - CAA-RI: Nondestructive perivascular inflammation
+ - ABRA: Transmural vascular inflammation with granulomatous inflammation and angiodestruction
+- ### Gross Pathologic & Surgical Features
+
+
+ - Lobar hemorrhage, edema
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - Cognitive decline, focal neurologic deficits, encephalopathy
+ - Seizures, headaches, focal neurologic deficits
+ - #### Other signs/symptoms
+
+
+ - Seizures, headache, behavioral changes
+- ### Demographics
+
+
+ - #### Age
+
+
+ - Typically present in 60s and 70s
+ - Typically younger than CAA alone
+ - #### Sex
+
+
+ - No strong predilection
+ - #### Epidemiology
+
+
+ - Estimated incidence of 0.13 in 100,000, though likely underestimated
+ - Association with APOE4 allele
+- ### Natural History & Prognosis
+
+
+ - Early immunosuppressive therapy improves prognosis, though, even with treatment, there is high morbidity and mortality
+- ### Treatment
+
+
+ - Immunosuppressive therapy
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - T2*/SWI imaging helpful
+- ### Image Interpretation Pearls
+
+
+ - Consider inflammatory CAA in cases of asymmetric WM hyperintensity and cerebral microbleeds
+- ### Reporting Tips
+
+
+ - CAA-RI and ABRA are not distinguishable by imaging
+
+ 383584de-6762-4ebb-9b2e-16e4d9459b54
+
+## References
+
+## Selected References
+
+1. [Koemans EA et al: Cerebral amyloid angiopathy: one single entity? Curr Opin Neurol. 38(1):29-34, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=39760721%5Bpmid%5D)
+1. [Panteleienko L et al: Cerebral amyloid angiopathy-related inflammation in iatrogenic cerebral amyloid angiopathy. Eur J Neurol. 32(5):e70198, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=40345981%5Bpmid%5D)
+1. [Tang M et al: Severe cerebral amyloid angiopathy related inflammation (CAA-ri) associated with vaccination: case report and literature review. J Neuroimmunol. 394:578406, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=39094435%5Bpmid%5D)
+1. [de Souza A et al: Inflammatory cerebral amyloid angiopathy: a broad clinical spectrum. J Clin Neurol. 19(3):230-41, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37151140%5Bpmid%5D)
+1. [Theodorou A et al: Clinical, neuroimaging, and genetic markers in cerebral amyloid angiopathy-related inflammation: a systematic review and meta-analysis. Stroke. 54(1):178-88, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36453271%5Bpmid%5D)
+1. [Corovic A et al: Cerebral amyloid angiopathy associated with inflammation: a systematic review of clinical and imaging features and outcome. Int J Stroke. 13(3):257-67, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29134927%5Bpmid%5D)
+1. [Kusakabe K et al: Cerebral amyloid angiopathy-related inflammation with epilepsy mimicking a presentation of brain tumor: a case report and review of the literature. Int J Surg Case Rep. 48:95-100, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29883923%5Bpmid%5D)
+1. [Espinoza Marcos MA et al: Inflammatory variant of cerebral amyloid angiopathy. Radiologia. 59(6):544-6, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28734389%5Bpmid%5D)
+1. [Auriel E et al: Validation of clinicoradiological criteria for the diagnosis of cerebral amyloid angiopathy-related inflammation. JAMA Neurol. 73(2):197-202, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26720093%5Bpmid%5D)
+1. [Salvarani C et al: Imaging findings of cerebral amyloid angiopathy, aβ-related angiitis (ABRA), and cerebral amyloid angiopathy-related inflammation: a single-institution 25-year experience. Medicine (Baltimore). 95(20):e3613, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27196463%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+*Axial FLAIR MR in a 70-year-old with cognitive decline shows cortical and subcortical hyperintensity in the right occipital lobe
. Note subtle lack of FLAIR suppression
in the right hemisphere sulci. Postcontrast T1 MR showed enhancement in the regions of sulcal hyperintensity.*
+
+
+*Axial FLAIR MR in a 70-year-old with cognitive decline shows cortical and subcortical hyperintensity in the right occipital lobe
. Note subtle lack of FLAIR suppression
in the right hemisphere sulci. Postcontrast T1 MR showed enhancement in the regions of sulcal hyperintensity.*
+
+
+*Axial SWI MIP in the same patient shows multiple foci of hypointensity
related to microbleeds in this patient with inflammatory amyloid. Inflammatory amyloid often presents in younger patients than classic amyloid angiopathy.*
+
+
+*Axial T1 C+ FS MR in an 83-year-old with confusion shows striking sulcal enhancement over the left hemisphere
and more subtle enhancement in the right hemisphere
.*
+
+
+*Axial SWI MIP MR shows cortical superficial siderosis
and innumerable blooming "black dots" representing cortical microbleeds
. The patient improved significantly after steroids and immunosuppression. Clinical and imaging features are consistent with inflammatory cerebral amyloid angiopathy (amyloid β-related angiitis).*
+
+
+*Axial FLAIR MR in a 68-year-old presenting with acute encephalopathy shows multifocal failure of CSF suppression
within the sulci and patchy cortical and subcortical hyperintensities
. There was no DWI restriction (not shown).*
+
+
+*Axial T1 C+ MR in the same patient shows multifocal leptomeningeal enhancement bilaterally
. Imaging differential considerations include leptomeningeal processes, including meningitis and vasculitis as well as leptomeningeal carcinomatosis.*
+
+
+*Axial FLAIR MR in the same patient a few weeks later shows a left frontal parenchymal hematoma
with surrounding vasogenic edema. SWI showed multiple microhemorrhages (not shown). Findings suggested inflammatory CAA. Biopsy confirmed the diagnosis.*
+
+
+*Axial NECT in a 61-year-old man with confusion and dysarthria who was admitted to the ER for evaluation of a stroke shows a hypodense left parietal lesion
causing effacement of the superficial sulci and gray-white matter interface.*
+
+
+*Axial T2 MR in the same patient shows confluent subcortical and deep white matter hyperintensity
. Numerous other patchy white matter hyperintensities
are present in both hemispheres.*
+
+
+*Axial T2* GRE shows multiple blooming "black dots" in the adjacent cortex
. The imaging diagnosis of inflammatory CAA was confirmed by biopsy. GRE or SWI are key to identifying microbleeds, which allow an accurate imaging diagnosis of inflammatory CAA.*
+
+
+### Additional Images
+
+
+*Axial FLAIR in an 82-year-old man with speech difficulties and cognitive decline shows right temporal lobe volume loss with abnormal cortical/subcortical hyperintensity. The left temporal lobe appears diffusely edematous with confluent FLAIR hyperintensity.*
+
+
+*Axial T1 C+ FS MR shows no enhancement within the mass itself
, but some subtle, adjacent sulcal enhancement
is present.*
+
+
+*Close-up view of axial T1 C+ FS MR "black blood" study shows diffuse leptomeningeal enhancement
with striking vessel wall enhancement
. There is CAA-related inflammation (CAA-RI). Differential considerations in this case include amyloid-β related angiitis (ABRA) and other vasculitis and amyloid-related imaging abnormalities (ARIA), which has similar imaging, but a completely different clinical history. (Courtesy S. McNally, MD., PhD.)*
+
+
+*Axial T1 MR in an 83-year-old woman with progressive confusion and right-sided weakness shows sulcal effacement and indistinct gray-white differentiation
in the left hemisphere.*
+
+
+*Axial FLAIR MR in the same patient shows striking cortical and subcortical hyperintensity in the left hemisphere
. Note FLAIR signal in the left paramedian sulci
. More subtle parenchymal and
sulcal abnormalities
are present in the right hemisphere. SWI confirmed multiple microhemorrhages in this patient with inflammatory amyloid.*
+
+
+*Coronal T2 MR in an older adult with cognitive changes shows hyperintensity
in the occipital regions bilaterally involving the cortex and subcortical white matter. Imaging mimics posterior reversible encephalopathy syndrome (PRES). Inflammatory amyloid hyperintensities is more often asymmetric, as in this patient.*
+
+
+*Axial T1 C+ MR in a patient with inflammatory amyloid shows leptomeningeal enhancement
and more focal enhancement
related to a recent hemorrhage. SWI confirmed multiple microhemorrhages.*
+
+
+*Axial T1 C+ MR in the same patient shows the leptomeningeal enhancement
in the right parietal lobe.*
+
+
+*Axial C+ VWI shows vessel wall enhancement
and leptomeningeal enhancement
in a patient with inflammatory amyloid, ABRA. Pathologically, CAA-RI shows perivascular inflammation, while ABRA shows angiodestructive inflammation. Imaging of inflammatory amyloid, CAA-RI vs. ABRA is often indistinguishable.*
+
diff --git a/docs_md/articles/cerebral-amyloid-disease_18edc9f3-9218-410c-8280-29c3e6df4c91.md b/docs_md/articles/cerebral-amyloid-disease_18edc9f3-9218-410c-8280-29c3e6df4c91.md
new file mode 100644
index 0000000..bff984e
--- /dev/null
+++ b/docs_md/articles/cerebral-amyloid-disease_18edc9f3-9218-410c-8280-29c3e6df4c91.md
@@ -0,0 +1,163 @@
+---
+title: "Cerebral Amyloid Disease"
+docid: "18edc9f3-9218-410c-8280-29c3e6df4c91"
+authors:
+ - key: "2bca6b86-1eca-4e93-b997-4e18913686a7"
+ value: "Hediyeh Baradaran, MD, MS"
+ - key: "8d5254e9-8dda-478b-8f08-bdee97a32c79"
+ value: "Karen L. Salzman, MD, FACR"
+breadcrumbs:
+ -
+ name: "Brain"
+ slug: "brain"
+ treeNodeId: "0361042c-9c90-48e4-864e-a2e6428ee9c9"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "948c6f1e-8e18-45f3-bd9d-7a04ab0b8708"
+ -
+ name: "Pathology-Based Diagnoses"
+ slug: "pathology-based-diagnoses"
+ treeNodeId: "d60fe914-7897-46a0-9cd2-f88ffb322f16"
+ -
+ name: "Stroke"
+ slug: "stroke"
+ treeNodeId: "7a135176-0a69-4fc9-b200-59569fbf5166"
+ -
+ name: "Nonatheromatous Vasculopathy"
+ slug: "nonatheromatous-vasculopathy"
+ treeNodeId: "2ccf261b-3d7a-42a7-8041-8ed08dd81bff"
+ -
+ name: "Cerebral Amyloid Disease"
+ slug: "cerebral-amyloid-disease"
+ treeNodeId: null
+category: "Brain"
+documentVersionId: "baa1c081-4b9f-4952-a6ba-5c385507a3c9"
+imageCount: 20
+lastUpdated: "08/19/25"
+pageDescription: "Cerebral Amyloid Disease"
+pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Stroke, Nonatheromatous Vasculopathy, Cerebral Amyloid Disease"
+pageTitle: "Cerebral Amyloid Disease | STATdx"
+enhancedTitle: "Cerebral Amyloid Disease"
+type: "DX"
+references: true
+breadcrumbs:
+ - "Brain"
+ - "Diagnosis"
+ - "Pathology-Based Diagnoses"
+ - "Stroke"
+ - "Nonatheromatous Vasculopathy"
+ - "Cerebral Amyloid Disease"
+---
+## KEY FACTS
+
+- ### Terminology
+- ### Imaging
+- ### Top Differential Diagnoses
+- ### Clinical Issues
+
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - Cerebral amyloid angiopathy (CAA)
+ - CAA-related inflammation (CAA-RI)
+ - Amyloid β-related angiitis (ABRA)
+- ### Synonyms
+
+
+ - "Congophilic angiopathy," cerebral amyloidosis
+- ### Definitions
+
+
+ - CAA is common cause of "spontaneous" lobar hemorrhage in older adults
+ - Cerebral amyloid deposition occurs in 3 morphologic varieties
+ - CAA (common)
+ - Inflammatory CAA: Diffuse white matter (WM) inflammatory involvement (uncommon)
+ - ABRA, CAA-RI
+ - Amyloidoma (rare)
+
+## IMAGING
+
+- ### General Features
+- ### CT Findings
+- ### MR Findings
+- ### Nuclear Medicine Findings
+- ### Angiographic Findings
+- ### Imaging Recommendations
+
+## DIFFERENTIAL DIAGNOSIS
+
+## PATHOLOGY
+
+- ### General Features
+- ### Staging, Grading, & Classification
+- ### Gross Pathologic & Surgical Features
+- ### Microscopic Features
+
+## CLINICAL ISSUES
+
+- ### Presentation
+- ### Demographics
+- ### Natural History & Prognosis
+- ### Treatment
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+ f7fe54fe-a8d3-46a8-90b5-63ff6946e395
+
+## References
+
+## Selected References
+
+1. [Panteleienko L et al: Cerebral amyloid angiopathy-related inflammation in iatrogenic cerebral amyloid angiopathy. Eur J Neurol. 32(5):e70198, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=40345981%5Bpmid%5D)
+1. [Banerjee G et al: Clinical considerations in early-onset cerebral amyloid angiopathy. Brain. 146(10):3991-4014, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37280119%5Bpmid%5D)
+1. [Szidonya L et al: Cerebral amyloid angiopathy. Radiol Clin North Am. 61(3):551-62, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36931769%5Bpmid%5D)
+1. [Charidimou A et al: The Boston criteria version 2.0 for cerebral amyloid angiopathy: a multicentre, retrospective, MRI-neuropathology diagnostic accuracy study. Lancet Neurol. 21(8):714-25, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35841910%5Bpmid%5D)
+1. [Malhotra K et al: Prevalence of clinical and neuroimaging markers in cerebral amyloid angiopathy: a systematic review and meta-analysis. Stroke. 53(6):1944-53, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35264008%5Bpmid%5D)
+1. [Gatti L et al: Understanding the pathophysiology of cerebral amyloid angiopathy. Int J Mol Sci. 21(10), 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32414028%5Bpmid%5D)
+1. [Gurol ME et al: Advanced neuroimaging to unravel mechanisms of cerebral small vessel diseases. Stroke. 51(1):29-37, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31752614%5Bpmid%5D)
+1. [Gurol ME et al: Multiple faces of cerebral small vessel diseases. Stroke. 51(1):9-11, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31752615%5Bpmid%5D)
+1. [Kuhn J et al: Cerebral amyloid angiopathy. StatPearls, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32310565%5Bpmid%5D)
+1. [Tsai HH et al: Superficial cerebellar microbleeds and cerebral amyloid angiopathy: a magnetic resonance imaging/positron emission tomography study. Stroke. 51(1):202-8, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31726962%5Bpmid%5D)
+1. [Akers C et al: Atypical clinical manifestations of cerebral amyloid angiopathy. Curr Neurol Neurosci Rep. 19(9):64, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31352553%5Bpmid%5D)
+1. [Chen SJ et al: Advances in cerebral amyloid angiopathy imaging. Ther Adv Neurol Disord. 12:1756286419844113, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31105769%5Bpmid%5D)
+1. [Greenberg SM et al: Diagnosis of cerebral amyloid angiopathy: evolution of the Boston criteria. Stroke. 49(2):491-7, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29335334%5Bpmid%5D)
+1. [Wermer MJH et al: The growing clinical spectrum of cerebral amyloid angiopathy. Curr Opin Neurol. 31(1):28-35, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29120920%5Bpmid%5D)
+1. [Beitzke M et al: Contribution of convexal subarachnoid hemorrhage to disease progression in cerebral amyloid angiopathy. Stroke. 46(6):1533-40, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25953372%5Bpmid%5D)
+1. [Charidimou A et al: Cerebral amyloid angiopathy with and without hemorrhage: evidence for different disease phenotypes. Neurology. 84(12):1206-12, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25716356%5Bpmid%5D)
+1. [Ding J et al: Risk factors associated with incident cerebral microbleeds according to location in older people: the age, gene/environment susceptibility (AGES)-Reykjavik study. JAMA Neurol. 72(6):682-8, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25867544%5Bpmid%5D)
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+1. [Yamada M: Cerebral amyloid angiopathy: emerging concepts. J Stroke. 17(1):17-30, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25692104%5Bpmid%5D)
+1. [Milner E et al: Cerebral amyloid angiopathy increases susceptibility to infarction after focal cerebral ischemia in Tg2576 mice. Stroke. 45(10):3064-9, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25190447%5Bpmid%5D)
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+1. [Alcalay RN et al: MRI showing white matter lesions and multiple lobar microbleeds in a patient with reversible encephalopathy. J Neuroimaging. 19(1):89-91, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18494780%5Bpmid%5D)
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+1. [Kinnecom C et al: Course of cerebral amyloid angiopathy-related inflammation. Neurology. 68(17):1411-6, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17452586%5Bpmid%5D)
+1. [Arboix A et al: New concepts in lacunar stroke etiology: the constellation of small-vessel arterial disease. Cerebrovasc Dis. 17 Suppl 1:58-62, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14694281%5Bpmid%5D)
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+1. [Dichgans M et al: Cerebral microbleeds in CADASIL: a gradient-echo magnetic resonance imaging and autopsy study. Stroke. 33(1):67-71, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=11779891%5Bpmid%5D)
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+1. [Caulo M et al: Cerebral amyloid angiopathy presenting as nonhemorrhagic diffuse encephalopathy: neuropathologic and neuroradiologic manifestations in one case. AJNR Am J Neuroradiol. 22(6):1072-6, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11415900%5Bpmid%5D)
+1. [Fazekas F et al: Histopathologic analysis of foci of signal loss on gradient-echo T2*-weighted MR images in patients with spontaneous intracerebral hemorrhage: evidence of microangiopathy-related microbleeds. AJNR Am J Neuroradiol. 20(4):637-42, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10319975%5Bpmid%5D)
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+1. [McCarron MO et al: Cerebral amyloid angiopathy-related hemorrhage. Interaction of APOE epsilon2 with putative clinical risk factors. Stroke. 30(8):1643-6, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10436115%5Bpmid%5D)
+1. [Miller JH et al: Intracerebral haemorrhage and cerebral amyloid angiopathy: CT features with pathological correlation. Clin Radiol. 54(7):422-9, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10437691%5Bpmid%5D)
diff --git a/docs_md/articles/cerebral-contusion_ac1add00-0c2a-4818-8c79-310fa74e2331.md b/docs_md/articles/cerebral-contusion_ac1add00-0c2a-4818-8c79-310fa74e2331.md
new file mode 100644
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+++ b/docs_md/articles/cerebral-contusion_ac1add00-0c2a-4818-8c79-310fa74e2331.md
@@ -0,0 +1,592 @@
+---
+title: "Cerebral Contusion"
+docid: "ac1add00-0c2a-4818-8c79-310fa74e2331"
+authors:
+ - key: "cdbaaa96-a7b4-4498-a355-a2823a7d9e26"
+ value: "Kelly A. Dahlstrom, DO"
+ - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
+ value: "Anne G. Osborn, MD, FACR"
+breadcrumbs:
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+ name: "Brain"
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+ slug: "diagnosis"
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+ -
+ name: "Cerebral Contusion"
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+imageCount: 44
+lastUpdated: "10/06/25"
+pageDescription: "Cerebral Contusion"
+pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Trauma, Primary Effects of CNS Trauma, Cerebral Contusion"
+pageTitle: "Cerebral Contusion | STATdx"
+enhancedTitle: "Cerebral Contusion"
+type: "DX"
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+cases: 2
+breadcrumbs:
+ - "Brain"
+ - "Diagnosis"
+ - "Pathology-Based Diagnoses"
+ - "Trauma"
+ - "Primary Effects of CNS Trauma"
+ - "Cerebral Contusion"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Brain surface injuries involving gray matter and contiguous subcortical white matter
+ - Coup: Direct injury to brain **beneath** impact site
+ - Contrecoup: Injury **opposite** impact site (often more severe than coup)
+- ### Imaging
+
+
+ - NECT
+ - Patchy hemorrhages within edematous background
+ - Adjacent to irregular bony protuberance or dural fold
+ - 40% anteroinferior frontal, 30% anteroinferior temporal lobes; 20-25% both
+ - **Nearly 75% exhibit expansion on follow-up CT**
+ - "Blossoming" contusions = ↑ hemorrhage, edema, mass effect
+ - Correlates with initial hemorrhage volume (larger more likely to progress, at faster rate)
+ - Note: Comparison between autopsy, antemortem CT has high specificity (≥ 80%) for most findings
+ - MR
+ - FLAIR: Best for hyperintense cortical edema (nonhemorrhagic contusion) and subarachnoid hemorrhage
+ - SWI > > GRE: Hypointense hemorrhagic foci bloom
+ - Best imaging tool
+ - CT to detect acute hemorrhagic contusions, other intracranial lesions, and herniations
+ - MR to detect presence and delineate extent of lesions
+- ### Top Differential Diagnoses
+
+
+ - Infarct
+ - Venous sinus thrombosis
+ - Cerebritis
+ - Low-grade neoplasm
+ - Transient postictal changes
+- ### Pathology
+
+
+ - Inflammation → worsening/enlarging lesions
+ - Neuroinflammation ongoing long after initial injury
+- ### Clinical Issues
+
+
+ - Initial symptom: Confusion → obtundation
+ - Central goal: Prevent and treat secondary injury
+ - Mass effect and herniation may require evacuation
+
+## TERMINOLOGY
+
+- ### Definitions
+
+
+ - Brain surface injuries involving gray matter and contiguous subcortical white matter
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Patchy hemorrhages within edematous background
+ - #### Location
+
+
+ - Adjacent to irregular bony protuberance or dural fold
+ - 40% anterior inferior frontal lobes, 30% anterior inferior temporal lobes (20-25% both)
+ - 25% parasagittal ("gliding" contusions)
+ - < 5% each parietal/occipital/brainstem
+ - > 95% concurrent extraaxial hemorrhage [most common = traumatic subarachnoid hemorrhage (tSAH)]
+ - Coup: Direct injury to brain beneath impact site
+ - Contrecoup: Injury opposite impact site; usually more severe than coup
+ - #### Morphology
+
+
+ - Early: Patchy, ill-defined, superficial foci of punctate or linear hemorrhage along gyral crests
+ - 24-48 hours: Existing lesions enlarge and become more hemorrhagic; new lesions may appear
+ - Chronic: Encephalomalacia with volume loss
+ - Multiple bilateral lesions in 90% of cases
+- ### CT Findings
+
+
+ - #### NECT
+
+
+ - Comparison between autopsy, antemortem CT has high specificity (≥ 80%) for most findings
+ - Early: Patchy, ill-defined, low-density edema with small foci of hyperdense hemorrhage
+ - 24-48 hours
+ - **Nearly 75% exhibit expansion on follow-up CT**
+ - "Blossoming" contusions = ↑ hemorrhage, edema, mass effect
+ - New foci of edema and hemorrhage may also appear
+ - Predictive factors of contusion expansion: Initial hemorrhage volume (larger more likely to progress, at faster rate), location (frontal contusions and presence of contrecoup injury ↑ risk of expansion), multiplicity, and coexisting SAH and subdural hemorrhage (SDH)
+ - Chronic
+ - Become isodense, then hypodense
+ - Encephalomalacia with volume loss
+ - Secondary lesions
+ - Herniations/mass effect with secondary infarction
+ - Hydrocephalus due to hemorrhage
+- ### MR Findings
+
+
+ - #### T1WI
+
+
+ - Acute: Inhomogeneous isointensity and mass effect
+ - Chronic: Focal or diffuse atrophy
+ - #### FLAIR
+
+
+ - Acute: Best for hyperintense cortical edema (nonhemorrhagic contusion) and SAH
+ - Chronic
+ - Hyperintense demyelination and microglial scarring
+ - Hypointense hemosiderin staining
+ - Hypointense cavitation (cystic encephalomalacia)
+ - #### T2* GRE
+
+
+ - Acute: Hypointense hemorrhagic foci bloom
+ - Chronic: Hypointense hemosiderin deposition
+ - SWI is more sensitive for evaluation compared to T2*GRE
+ - #### DWI
+
+
+ - Hyperintense in areas of cell death
+ - Decreased apparent diffusion coefficient (ADC) correlates with poor outcome
+ - Diffusion tensor imaging sometimes shows white matter damage when CT, routine MR appear normal
+ - #### MRS
+
+
+ - ↓ NAA, ↑ choline
+- ### Nuclear Medicine Findings
+
+
+ - SPECT Tc-99m HMPAO imaging
+ - Depicts focal changes in 53% with mild injury
+ - Negative in 1st month predicts good outcome
+ - Positive can predict poor clinical outcome
+- ### Imaging Recommendations
+
+
+ - #### Best imaging tool
+
+
+ - CT to detect acute hemorrhagic contusions, other intracranial lesions, and herniations
+ - Clinically stable patient with mild traumatic brain injury (TBI), positive initial CT can have follow-up CT delayed up to 48 hours
+ - Consider using dual-energy CT when following up hemorrhagic contusions after contrast-enhanced whole-body trauma CT
+ - Avoids overestimation of contusion volume
+ - #### Protocol advice
+
+
+ - FLAIR for edema and SAH; GRE/SWI for hemorrhagic foci
+
+## DIFFERENTIAL DIAGNOSIS
+
+- [Infarct](/document/acute-cerebral-ischemiainfarction/7a3ed4a9-ae05-4d64-ae8e-6a30105501e1)
+ - No trauma history
+ - Characteristic acute onset of focal neurologic deficit
+ - Vascular distribution: Spares frontal and temporal poles
+- [Venous Sinus Thrombosis](/document/dural-sinus-thrombosis/9a1ac112-bd65-4306-92b0-022d16e360c3)
+ - Edema and hemorrhage adjacent to occluded sinus
+- ### Cerebritis
+
+
+ - No trauma history
+ - Herpes typically involves medial temporal lobe
+- ### Low-Grade Neoplasm
+
+
+ - No trauma history
+ - Solitary nonhemorrhagic lesion
+ - No predilection for anterior frontal or temporal lobes
+- ### Transient Postictal Changes
+
+
+ - No trauma history
+ - Preceding or ongoing seizure activity
+ - May be hyperintense on DWI; can enhance acutely
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Etiology
+
+
+ - Stationary head struck by object
+ - Direct injury beneath impact site
+ - Contusion is rare without fracture
+ - Moving head: Motor vehicle crash, falls
+ - Differential accel-/deceleration and rotational forces on portions of brain with different densities
+ - Gliding injury: Cortex anchored to dura by arachnoid granulations; subcortical tissue glides more than cortex
+ - Traffic injuries are main cause in young adults (20-40 years)
+ - Falls are main cause in infants (0-4 years) and older adults (≥ 70 years)
+ - #### Associated abnormalities
+
+
+ - Soft tissue injuries in 70% of patients
+ - tSAH, SDH, intraventricular hemorrhage
+ - ± skull fracture at coup site
+- ### Gross Pathologic & Surgical Features
+
+
+ - Contusions
+ - Edema along gyral crests
+ - Petechial hemorrhages (most evident in 24-48 hours)
+ - Small hemorrhages may coalesce into hematoma
+ - Delayed hematomas may develop 24-48 hours later
+ - Lacerations
+ - Intracerebral hematoma with "burst" lobe
+ - SDH communicates with hematoma via lacerated brain, torn pia-arachnoid
+ - Liquefaction and encephalomalacia in chronic phase
+- ### Microscopic Features
+
+
+ - Capillary disruption → blood extravasation: RBCs cause visible hemorrhage, plasma leads to edema
+ - Perivascular hemorrhage, ↑ pinocytic activity of endothelial cells, and cytotoxic edema of astroglial cells
+ - Higher levels of serum protein S100B and IL-6 correlate with ultrastructural changes of endothelial cells
+- ### Cellular Features
+
+
+ - Chemokine, nitric oxide activation occurs early
+ - Inflammatory response → neutrophil oxidative burst → proteolytic and neurotoxic enzyme release
+ - Neuroinflammation mediated by cyto-/chemokines, complement
+ - Contributes to secondary ischemic damage, contusion enlargement
+ - Continues long after initial traumatic event
+ - CNS cells synthesize distinct chemokines
+ - Chemokine CCL2 is highly expressed early in pericontusional area
+ - Chemokine CXCL8 (a.k.a. IL-8) is highly expressed as late inflammatory mediator
+ - Inflammatory processes contribute to pericontusional cytotoxic injury via astrocytic activation with capillary vessel compression and leukocyte accumulation → microvascular occlusion
+ - Blood-brain barrier failure aided by proinflammatory factor activation and matrix metalloproteinases
+ - Injured cortex upregulates peroxisome proliferator-activated receptor α (PPAR-α) binding activity and protein expression
+ - Peaks 24-72 hours post injury; PPAR-α agonists protect against excessive oxidative stress and inflammation in TBI and stroke
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - Varies with severity; from mild confusion to obtundation
+ - ± cerebral dysfunction, seizures
+- ### Demographics
+
+
+ - #### Age
+
+
+ - Children:adults = 2:1; highest risk between 15-24 years
+ - #### Sex
+
+
+ - M:F = 3:1
+ - #### Epidemiology
+
+
+ - Annual cerebral contusion incidence is 200 per 100,000 brain trauma-related hospitalizations
+ - Contusion is 2nd most common primary traumatic neuronal injury (44%); diffuse axonal injury (DAI) is most common
+ - 1.4 million suffer TBI each year in USA; 50,000 die, and 80,000 experience long-term disability
+ - TBI causes 6.5% of deaths in USA (32 per 100,000)
+- ### Natural History & Prognosis
+
+
+ - Varies with extent of primary injury
+ - Outcome is critically dependent on extent of brain damage that evolves after initial insult
+ - Secondary lesions: Hypoxia, hypotension, ischemia, brain edema, and ↑ intracranial pressure
+ - Highest mortality rate: Older adult population, larger initial hemorrhages
+ - Linear ↑ of 40-50% in odds of poor outcome for every 10 years of age
+ - 90% of patients survive injury
+ - ~ 25% have significant residual complaints
+ - Temporal and especially brainstem contusions are independent risk factors for poor outcome
+ - In severe TBI, 63% have good clinical outcome, and 32% have excellent clinical outcome
+- ### Treatment
+
+
+ - Central goal: Prevent or rapidly treat secondary injury
+ - Mass effect and herniation may require evacuation
+ - Mitigate secondary effects of ↑ intracranial pressure, perfusion disturbances
+ - Ventricular catheter to monitor and control intracranial pressure
+ - Long-term (5 days) mild hypothermia significantly improves outcome of severe TBI patients with cerebral contusion and intracranial hypertension
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - Repeat exam recommended if initial exam negative but symptoms persist for 24-48 hours
+- ### Image Interpretation Pearls
+
+
+ - Characteristic location: Anteroinferior frontal and temporal lobes
+ - Mixed-density contusions may be mistaken for common artifacts at skull base
+
+ df01a862-ae94-448e-bb1c-10548419fd45
+
+## References
+
+## Selected References
+
+1. [Smith PD et al: Predictive factors for traumatic cerebral contusion volume, expansion, and outcomes. J Neurosurg. 142(6):1616-1624, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=39752651%5Bpmid%5D)
+1. [Taddei G et al: Evidence-based indications for repeat head CT after mild traumatic brain injury: a systematic review and meta-analysis. Neurosurg Rev. 48(1):397, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=40304799%5Bpmid%5D)
+1. [Jirlow U et al: Cerebral contusions - pathomechanism, predictive factors for progression and historical and current management. Brain Spine. 4:103329, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=39281852%5Bpmid%5D)
+1. [Peng J et al: Imaging predictors of hemorrhagic progression of a contusion after traumatic brain injury: a systematic review and meta-analysis. Sci Rep. 14(1):5961, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38472247%5Bpmid%5D)
+1. [Sriyook A et al: Imaging of head trauma: pearls and pitfalls. Radiol Clin North Am. 61(3):535-49, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36931768%5Bpmid%5D)
+1. [Wang R et al: Classification, risk factors, and outcomes of patients with progressive hemorrhagic injury after traumatic brain injury. BMC Neurol. 23(1):68, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36782124%5Bpmid%5D)
+1. [Shih YJ et al: Prediction of intraparenchymal hemorrhage progression and neurologic outcome in traumatic brain injury patients using radiomics score and clinical parameters. Diagnostics (Basel). 12(7), 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35885581%5Bpmid%5D)
+1. [Adatia K et al: Contusion progression following traumatic brain injury: a review of clinical and radiological predictors, and influence on outcome. Neurocrit Care. 34(1):312-24, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=32462411%5Bpmid%5D)
+1. [Yuh EL et al: Pathological computed tomography features associated with adverse outcomes after mild traumatic brain injury: a TRACK-TBI study with external validation in CENTER-TBI. JAMA Neurol. 78(9):1137-48, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34279565%5Bpmid%5D)
+1. [Mathieu F et al: Relationship between measures of cerebrovascular reactivity and intracranial lesion progression in acute traumatic brain injury patients: a CENTER-TBI study. J Neurotrauma. 32(2):373-82, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31928143%5Bpmid%5D)
+1. [Morotti A et al: Noncontrast CT markers of intracerebral hemorrhage expansion and poor outcome: a meta-analysis. Neurology. 95(14):632-43, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32847959%5Bpmid%5D)
+1. [Zeiler FA et al: Diffuse intracranial injury patterns are associated with impaired cerebrovascular reactivity in adult traumatic brain injury: a CENTER-TBI validation study. J Neurotrauma. 37(14):1597-608, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32164482%5Bpmid%5D)
+1. [Gregson BA et al: Surgical decision making in brain hemorrhage. Stroke. 50(5):1108-5, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30932784%5Bpmid%5D)
+1. [Kulkarni P et al: Neuroradiological changes following single or repetitive mild TBI. Front Syst Neurosci. 13:34, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31427931%5Bpmid%5D)
+1. [Nagesh M et al: Role of repeat CT in mild to moderate head injury: an institutional study. Neurosurg Focus. 47(5):E2, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31675712%5Bpmid%5D)
+1. [Alexis RJ et al: Clinical profile and autopsy findings in fatal head injuries. J Emerg Trauma Shock. 11(3):205-10, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30429629%5Bpmid%5D)
+1. [Bodanapally UK et al: Dual-energy ct in hemorrhagic progression of cerebral contusion: overestimation of hematoma volumes on standard 120-kV images and rectification with virtual high-energy monochromatic images after contrast-enhanced whole-body imaging. AJNR Am J Neuroradiol. 39(4):658-62, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29439124%5Bpmid%5D)
+1. [Carnevale JA et al: Blossoming contusions: identifying factors contributing to the expansion of traumatic intracerebral hemorrhage. J Neurosurg. 129(5):1305-16, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29303442%5Bpmid%5D)
+1. [Useche JN et al: Conventional computed tomography and magnetic resonance in brain concussion. Neuroimaging Clin N Am. 28(1):15-29, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29157850%5Bpmid%5D)
+1. [Yue JK et al: Temporal lobe contusions on computed tomography are associated with impaired 6-month functional recovery after mild traumatic brain injury: a TRACK-TBI study. Neurol Res. 40(11):972-81, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30175944%5Bpmid%5D)
+1. [Panzer S et al: Traumatic brain injury: comparison between autopsy and ante-mortem CT. J Forensic Leg Med. 52:62-69, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28866283%5Bpmid%5D)
+1. [DeQuesada IM 2nd et al: Neuroimaging of acute traumatic brain injury: emphasis on magnetic resonance imaging and prognostic factors. Semin Roentgenol. 49(1):64-75, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24342676%5Bpmid%5D)
+
+## Differential diagnosis
+
+### Homonymous Hemianopsia
+DDX:1cdca8f4-95f8-4d19-b28a-19a433d1a624
+
+### Anosmia-Hyposmia
+DDX:cb90e245-060a-4489-8041-6803f8cc93e7
+
+### Intracranial Hemorrhage
+DDX:3a9cbed6-aa2c-45a0-88bf-b39f1523ee85
+
+## Anatomy
+
+### Basal Ganglia
+Brain/ANATOMY:a9de3815-ec59-4c78-adf0-94974065a7e3
+
+### Language Overview
+Brain/ANATOMY:40f2ed79-0d31-4943-aaa2-7c3244a7e87b
+
+### Brainstem Overview
+Brain/ANATOMY:bf889ffb-646b-429d-8699-d9bd34e8e193
+
+### Gyral/Sulcal Anatomy
+Brain/ANATOMY:849da2a0-4a32-4a07-8f00-c69291e59434
+
+### Gyral/Sulcal Anatomy
+Brain/ANATOMY:299a5990-1805-4018-85b5-191d8416385b
+
+### Brain
+Ultrasound/ANATOMY:080771c2-02f3-408d-ad70-04a80d849500
+
+### Attention Control Network
+Brain/ANATOMY:a1bedda5-6478-40b2-98e7-6c5f5363b06f
+
+### Default Mode Network
+Brain/ANATOMY:a29f7551-d39d-4deb-933e-b8d2816168c3
+
+### Functional Network Overview
+Brain/ANATOMY:7b97f239-0f6f-4809-ac44-594cdf4842d5
+
+### Functional Network Overview
+Brain/ANATOMY:ef0be4c8-3d36-4ca9-b4c5-f22f66d2b367
+
+### Midbrain
+Brain/ANATOMY:628d8588-6898-45ba-b946-1702bcce1366
+
+### Other Deep Gray Nuclei
+Brain/ANATOMY:aba59a23-3d05-4ef5-a004-fc928a7c40d6
+
+### Social Brain Anatomy
+Brain/ANATOMY:0352d34a-5966-494e-b9c3-c26bde257bca
+
+### Midbrain
+Ultrasound/ANATOMY:931a002c-9dcc-4b1a-81ac-a55c418aee32
+
+## Cases
+
+- {'cases': [{'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}], 'caseVersionId': 'e91d112b-6fc8-4789-b49a-a0e33e617142', 'description': 'Following assault, this patient was repeatedly imaged over several days due to a spiraling downward clinical decline. The presentation NECT (#1,2) showed diffuse cerebral edema, bitemporal and bifrontal hemorrhagic contusions (white arrows, #1-8), and subarachnoid hemorrhage (black arrows, #1-2). On day 7, images (#3-4), there is bilateral uncal herniation causing slight effacement of the basilar cisterns (curved arrows, #3) as well as slight effacement of the third ventricle (open arrow, #4), both due to mass effect from enlarging contusions (arrows, #4) and diffuse cerebral edema. On day 9, images (#5-6), the effacement of the basilar cisterns (curved arrows, #5) and third ventricle (open white arrow, #6) have worsened, the frontal contusions are again larger (arrows, #6), but quite significantly there is new hypodensity within bilateral occipital lobes from new PCA ischemia (open black arrows, #5-6). By day 17 images (#7-8), the basilar cisterns (curved arrows, #7) and third ventricle (open white arrow, #8) are completely effaced and there is significant ischemic PCA hypodensity of both occipital lobes (open black arrows, #7-8). \n\nOn day 18, Tc-99m HMPAO SPECT imaging of the brain during dynamic (#9-10) and static (#11-14) acquisition reveals a lack of parenchymal flow as the "light bulb" and "hot nose" signs of brain death.', 'history': 'Assaulted.', 'imagePoolId': '8d32551e-d4fa-4aa8-b5c6-ff11de53c49a', 'name': 'Secondary', 'teachingPoint': 'PCA ischemia is a very common form of post-traumatic cerebral ischemia and is due to compression of PCA (one or both) against a rigid tentorial edge from medial temporal lobe herniation in the setting of severe cerebral edema (and/or other causes of mass effect).', 'demographics': '42 Years old male'}, {'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}], 'caseVersionId': '8aed1a80-3666-4f96-87ff-1d0d6db783a0', 'description': 'Subcortical injury is manifested by injuries to the superior cerebellar peduncles, midbrain, right globus pallidus, left thalamus, anterior commissure, and corpus callosum (black arrows, #1-17). \n\nDAI is present in this case with three imaging appearances: (1) NECT hyperdense, FLAIR hyperintense, and GRE hypodense (white arrows, #2-3,9-10,16-17); (2) not visible by CT yet FLAIR hyperintense, and GRE hypointense (white open arrows, #8-10,15-17); and (3) only visible by GRE (curved arrows, #15-16).\n\nThere are also right temporal and right rectus gyrus frontal lobe hemorrhagic contusions (black open arrows, #5-7,11,13), calvarial fracture, pneumocephalus, intraventricular hemorrhage, and extensive subarachnoid hemorrhage.\n\nComment: This case very nicely illustrates how patients with subcortical injury have experienced severe trauma and are often highly complex cases with a multitude of abnormal findings. When you think you are finished reviewing the case - look at everything one more time!', 'history': 'Motor vehicle collision.', 'imagePoolId': 'cad21136-7023-47dc-b8e7-a0331bff15fb', 'name': 'Extensive', 'teachingPoint': None, 'demographics': '20 Years old female'}, {'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}], 'caseVersionId': '9c853284-99d1-461b-9282-1f53c1fd4662', 'description': 'Post-trauma imaging reveals subcortical injury in the form of deep DAI as CT hypodense and T2/FLAIR hyperintense abnormalities involving the right middle cerebellar peduncle/pons (white arrows, #1,4,8), the midbrain (open white arrows, #2,5,9), the right thalamus (curved white arrows, #6,10), and the body of the corpus callosum (black arrows, #3,7,11). Also note the extensive CT hypodense and T2/FLAIR hyperintense contusions involving the right temporal, bilateral frontal, and left occipital lobes (open black arrows, #1-2,5-6,9-10). Lastly, subarachnoid hemorrhage is evident by CT and FLAIR (curved black arrows, #3, 6-7).\n\nComment: Subcortical injury has a poor prognosis, is associated with a very low Glasgow Coma Scale rating, and has affiliated traumatic abnormalities such as intracranial hemorrhage, contusion, &/or ischemia.', 'history': 'Motorcycle collision.', 'imagePoolId': '93216031-5e7b-4b59-a17b-8004de0d6ad0', 'name': 'Extensive', 'teachingPoint': None, 'demographics': '36 Years old male'}, {'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}, {'key': '490aa917-ad7e-4d15-84a2-762f90db39e5', 'value': 'John H. Rees, MD'}], 'caseVersionId': '9e7a06f4-7816-46b1-817b-702892384fbb', 'description': 'Typical deep cerebral contusion from falx impaction. \n\nT1 isointense, T2 hyperintense cerebral contusion with hyperacute hemorrhage (open arrows) is present from parasagittal "gliding" injury from impaction against the falx (arrows) during trauma.', 'history': None, 'imagePoolId': '7b0fde90-f054-47a2-b844-521388d378ff', 'name': 'Gliding, parasagittal', 'teachingPoint': None}, {'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}], 'caseVersionId': 'a094142e-cc8a-4450-8944-ae239c28996a', 'description': 'Initial posttrauma NECT imaging shows subarachnoid hemorrhage (arrows, #1,2), mild bilateral cisternal effacement and uncal herniation (open black arrows, #1), bifrontal and left temporal hemorrhagic contusions (open white arrows, #1-2), a small posterior falcine subdural hematoma (arrow, #3,4), and generalized sulcal effacement more anteriorly nearing the convexity, right worse than right (curved arrows, #3,4).\n\nMR imaging (FLAIR & T2) performed four days later due to a lack of patient improvement confirm the subarachnoid hemorrhage, somewhat redistributed (arrows, #5-7) as well as the bifrontal and left temporal hemorrhagic contusions (open arrows, #5,6, 9-10). MR demonstrates that the CT apparent sulcal effacement is due to gyral swelling with T2 hyperintensity secondary to hypoxia (curved arrows, #7,8,11,12).', 'history': 'Trauma.', 'imagePoolId': '1a82beba-3fd3-40d4-b6e9-4841ec8e7bfc', 'name': 'Hemorrhagic, hypoxia', 'teachingPoint': 'Trauma frequently leads to hemorrhagic contusions in expected locations wherever the brain is in close contact with skull surfaces, especially the anterior inferior frontal lobes and temporal lobes. Additionally, trauma may induce hemodynamic alterations (local, regional, or general perfusion disturbances) leading to hypoxic injury or outright infarction.', 'demographics': '11 Years old male'}, {'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}], 'caseVersionId': 'bf84d6e6-6207-4bfe-a2eb-408391f04975', 'description': 'NECT images immediately following trauma reveal a coup hemorrhagic contusion of the right cerebellar hemisphere (arrow, #1) and a larger contrecoup hemorrhagic contusion involving the left inferior and periorbital frontal brain (arrows, #3-5). There is also an associated subdural hematoma of the right tentorium, which extends to involve the posterior falx (open arrow, #2).\n\nNECT performed 20 days later following surgical evacuation of the right cerebellar hemispheric hemorrhage demonstrates expected postoperative findings, including missing calvarial bone and right cerebellar encephalomalacia (arrows, #6). The left frontal contusion grew since the initial imaging and now has a worsened mass effect; however, evolving encephalomalacia is evident (arrows, #7-10). The tentorial subdural hematoma has resolved (open arrow, #7).\n\nComment: Coup represents direct injury to brain beneath impact site whereas contrecoup injury lies opposite the impact site. Contrecoup lesions are usually more severe than coup.', 'history': 'Status post fall from a picnic table. ', 'imagePoolId': 'fcd71776-d412-4f4e-bc51-0fcbb9ab4800', 'name': 'Coup, contrecoup', 'teachingPoint': None, 'demographics': '60 Years old male'}, {'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}], 'caseVersionId': 'c74663fa-62a1-4982-bae4-3c03a4dd34d1', 'description': 'Axial NECT images show the classic findings of hemorrhagic contusions in this case involving the bitemporal and inferior right frontal lobes (arrows, #1-3), as well as subarachnoid hemorrhage (open arrows, #4).\n\nMR performed the same day better demonstrates these lesions. Although the FLAIR is a bit motion degraded it better shows the extent of the contusions (arrows, #5-6), which are not as well seen with T2 technique (arrows, #8-9). GREs nicely demonstrate the hemorrhagic components (arrows, #11-12). Note the FLAIR also confirms the subarachnoid hemorrhage as abnormal CSF hyperintensity with the sulci (open arrows, #7), but this is not evident by either T2 or GRE techniques (#10, 13).\n\nComment: Contusions are most often found where the brain parenchyma is "cupped" by immobile bone structures, such as those found surrounding the temporal lobes in the middle cranial fossa and also adjacent to the frontal lobe rectus and periorbital gyri.', 'history': 'Trauma.', 'imagePoolId': 'b1921223-1c85-471b-a5c0-9fa88fdbe6ed', 'name': 'Classic', 'teachingPoint': None, 'demographics': '41 Years old male'}, {'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}], 'caseVersionId': 'dd831056-87db-4c6f-bb5b-8c827347aa2c', 'description': 'Presentation in bone CT reveals numerous calvarial fractures (arrows, #1). NECT shows diffuse cerebral edema with tonsillar herniation filling the foramen magnum (arrows, #2) and hydrocephalus (arrows, #3). \n\nOn day 2 (#4) the patient deteriorated rapidly and was found to have a new left-sided subdural hematoma (open arrows) containing hypodense CSF and active hemorrhage. Also, in the interim, two right frontal hemorrhagic cerebral contusions became apparent (white curved arrows) as well as intraventricular hemorrhage (black curved arrows). A ventriculostomy was also placed. \n\nA CT performed on day 3 (#5) shows surgical evacuation of the subdural, persistent intraventricular hemorrhage (black curved arrows), better visualization of the right cerebral contusions (white curved arrows), and a newly apparent left frontal hemorrhagic contusion (white arrow).\n\nMR imaging performed later on day 3 (#6-8) re-demonstrates the left frontal hemorrhagic contusion (white arrows, #6,8), right frontal hemorrhagic contusions (curved white arrows), and intraventricular hemorrhage (black curved arrows, #6-10). Careful evaluation of the anterior right frontal contusion reveals vasogenic edema close to the hemorrhagic core (bright on both DWI and ADC)(white arrows, #9-10) but with restricted diffusion of local ischemia along the periphery (bright on DWI but dark on ADC)(black arrows, #9-10).\n\nComment: A variety of mechanisms accounts for post-traumatic cerebral ischemia, including a primary local affect of direct vascular compression by a mass lesion.', 'history': 'Fall from 30 feet with loss of consciousness.', 'imagePoolId': 'c185758e-6a87-4477-a556-4f680200bf13', 'name': 'Local, primary', 'teachingPoint': None, 'demographics': '27 Years old male'}, {'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}], 'caseVersionId': '0407ef27-7847-4d3a-b2ff-2eb28b2d5c15', 'description': 'Preoperative bone CT imaging following trauma shows an extensive left calvarial fracture, which results in a longitudinal left temporal bone fracture (arrow, #1), pterional fracture (arrow, #2), and left parietal fracture with an elevated fragment (arrow, #3). There is also a subdural hematoma near the convexity (arrow, #4). Postoperative bone CT shows near anatomic positioning of the previously elevated fragment (arrow, #5). Hemorrhagic contusions have become apparent on axial NECT images in a classic coup (open arrow, #7) and contrecoup (open arrow, #6) configuration. MR better defines the extent of the contusions (curved white arrows, #8-13). A small contusion can also be seen on postoperative CT and MR underlying a craniotomy site (curved black arrow, #7, 9, 11). C', 'history': 'Trauma.', 'imagePoolId': '6da0d271-674b-4538-b587-7d0ef32d275c', 'name': 'Coup, contrecoup, elevated', 'teachingPoint': 'Coup represents direct injury to the brain beneath the impact site, whereas a contrecoup injury lies opposite the impact site. Contrecoup lesions are usually more severe than coup.', 'demographics': '15 Years old male'}, {'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}], 'caseVersionId': '09b819a9-12ba-40be-b153-62c1dd3d4a64', 'description': 'SCI lesions can be found in the midbrain (arrows, #1,4,10), bilateral cerebral peduncles (arrows, #2,5,8,11), and left caudate/internal capsule/anterior lentiform nuclei (open white arrows, #3,6,9). There are also bifrontal and left temporal cortical contusions (open black arrows, #2,3,5,6,8,9) and subdural hematomas (curved arrows, images 1-11).\n\nComment: Patients with SCI have experienced severe trauma and are often highly complex cases with a multitude of abnormal findings. When you think you are finished reviewing the case - look at everything one more time!', 'history': 'Ejected from golf cart, loss of consciousness', 'imagePoolId': '2fabeb60-19b6-4af7-9af2-09f75ef18a62', 'name': 'Severe', 'teachingPoint': None, 'demographics': '58 Years old female'}, {'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}], 'caseVersionId': '09c84360-5834-4a89-8e5e-15e25daa4488', 'description': 'NECTs (#1-4) demonstrate bifrontal nonhemorrhagic contusions (arrows). \n\nComment: Although cerebral contusions are often hemorrhagic, this case nicely shows that they can be quite significant yet remain nonhemorrhagic, at least at the sensitivity level of CT.', 'history': 'Auto-pedestrian accident.', 'imagePoolId': '15866bef-634c-479e-b784-e5b2471b409e', 'name': 'Nonhemorrhagic', 'teachingPoint': None, 'demographics': '49 Years old male'}, {'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}], 'caseVersionId': '189a4fb4-52f9-428f-b6a7-777f40a12261', 'description': 'Bone CT (#1-3) performed following MVA shows a face "crush" injury with innumerable calvarial fractures. NECT (#4) at the time reveals extensive bifrontal hemorrhagic contusions (arrows). Comorbidities included diffuse cerebral edema, subarachnoid hemorrhage, and subdural hemorrhage (not shown), but there was no NECT evidence of ischemia. MR was performed six days later due to clinical deterioration which redemonstrated the contusions (white arrows, #5-6), subarachnoid hemorrhage (open arrows, #5,7) and subdural hematoma (curved arrows, #5-8). MR also revealed CT invisible DAI/Subcortical injury lesions (black arrows, #5-6,11-12) but more significantly demonstrated new bilateral ACA distribution acute ischemia (white arrows, #7-13).', 'history': 'MVA.', 'imagePoolId': 'c31ac0a6-076b-4c01-ab1d-8dd0d0bba290', 'name': 'Secondary', 'teachingPoint': 'ACA distribution ischemia is very common from diffuse cerebral edema causing cingulate gyrus compression of one or both ACAs &/or their branches.', 'demographics': '16 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '53bb19ea-ae01-4c78-9407-991bd2bfad6c', 'description': 'The series of axial NECT scans (#1-3) shows a small epidural hematoma in the right middle cranial fossa (arrow, #1), traumatic subarachnoid hemorrhage (arrows, #2-3), and a small cortical contusion (open arrows, #2-3). Bone CT (#4-7) shows severely comminuted, depressed skull fractures (arrows) that overlie the contusions and traumatic subarachnoid hemorrhage. Of interest in this patient is the lack of "contre-coup" injury to the left temporal or parietal lobes, which appear normal. It is likely that the skull absorbed most of the kinetic energy, and only the directly underlying brain appears affected.\n\nFollow-up scan 3 days later shows typical, expected enlargement and evolution of the cortical contusion (arrow, #8).', 'history': 'Unrestrained patient in high-speed MVA.', 'imagePoolId': '81b0e9a2-63e4-4609-814f-a3fbb1722769', 'name': 'Depressed, comminuted', 'teachingPoint': None, 'demographics': '52 Years old female'}, {'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}, {'key': '84820c52-dc92-42a2-93fa-61132d2af4d1', 'value': 'Karen A. Tong, MD'}], 'caseVersionId': '5a326d86-2cc9-424e-ad1b-f46ede99d653', 'description': 'Following MVA, an NECT showed 4 hemorrhagic contusions of bifrontal and biparietal lobes (arrows, #1). Subsequently, an MR was performed, confirming these same 4 contusions on T1 with hyperintense hemorrhage (arrows, #2). T2 re-demonstrates these contusions with hypointense hemorrhage (arrows, #3) but also shows additional lesions (open arrows, #3). GRE confirms all the lesions seen on T2 (arrows, #4) and, as expected, also demonstrates many other small petechial foci of susceptibility of additional lesions (too many for arrows). Lastly, susceptibility-weighted imaging (#5) reveals many, many, many more lesions than were apparent on GRE (far too many for arrows).\n\nComment: One must keep in mind that deoxyhemoglobin within venous structures will add to this SWI appearance. Regardless, the sensitivity of susceptibility-weighted imaging for detecting microhemorrhage from DAI is impressive when compared to NECT or even GRE.\n\nThese beautiful images are courtesy of Dr. Karen Tong.', 'history': 'MVA.', 'imagePoolId': 'b1ff4179-1d38-410d-9ddb-402d9cae5e67', 'name': 'SWI, extensive', 'teachingPoint': None, 'demographics': '16 Years old male'}, {'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}], 'caseVersionId': '7e9e7062-825a-46a3-b610-b8f693860386', 'description': 'The presentation in NECT shows only subtle hypodensity and slight mass effect from cerebral contusions of the left frontal, temporal, and parietal lobes (white arrows, #1-3) which are more evident as T2 hyperintensity on FLAIR technique (white arrows, #5-7). Not evident by CT (#4), but obvious by FLAIR, are hyperintense foci of DAI (white open arrows, #7-8). Subcortical injury of the left cerebral peduncle matches the patients hemiparesis (curved arrow, #6). Note the subarachnoid, interpeduncular, and intraventricular hemorrhages, also only seen by FLAIR technique (black arrows, #5-7). Lastly, a thin hyperdense/hyperintense subdural hematoma is present (open black arrows, #3,6-7).', 'history': 'Patient in motor vehicle accident with hemiparesis.', 'imagePoolId': 'f96e747d-49f0-4350-85bd-aa926fdd6814', 'name': 'Hemiparesis', 'teachingPoint': 'Subcortical injury has a poor prognosis, is associated with a very low Glasgow Coma Scale rating, and has affiliated traumatic abnormalities such as intracranial hemorrhage, contusion, &/or ischemia.', 'demographics': '2 Years old male'}, {'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}], 'caseVersionId': '82606f6f-51ec-4e91-b49f-c326e9f8b5a8', 'description': 'NECTs show the subtle loss of gray-white interface from a contrecoup, nonhemorrhagic contusion involving the left temporal lobe (arrows, #1-2). This is confirmed on MR as T1 hypointense and FLAIR/T2 hyperintense abnormality (arrows, #4-5, 7). Note the MR images demonstrate a thin subdural hematoma not visible on CT (open black arrows, #4-5, 7); the negative corresponding NECT image is #3. FLAIR technique also reveal diffuse subarachnoid hemorrhage within many sulci, some of which are annotated by the curved arrows on images #5-6. Soft tissue swelling and injury is apparent at the primary impact, or coup, site (open white arrows, #1-8).', 'history': 'MVA.', 'imagePoolId': '325d0f51-5fcf-4b3d-8ff5-e51ba6fb287b', 'name': 'Nonhemorrhagic, contrecoup', 'teachingPoint': 'Coup represents direct injury to brain beneath impact site, whereas contrecoup injury lies opposite the impact site. Contrecoup lesions are usually more severe than coup.', 'demographics': '25 Years old female'}, {'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}], 'caseVersionId': '0476b55b-6fa5-48f0-bb38-efaa6fbb0dab', 'description': 'Typical evolving and enlarging cerebral contusion.\n\nCT of a patient with closed head injury shows a small left frontal cerebral hypodense contusion with small foci of hyperdense hemorrhage.\n\nRepeat CT 24 hours later shows enlargement with clear demarcation of the hypodense contusion with interspersed foci of hyperdense hemorrhage.', 'history': None, 'imagePoolId': '8dc055c4-9f05-46e0-8d86-cb45ec231d29', 'name': 'Classic, enlarging', 'teachingPoint': None, 'demographics': '14 Years old male'}, {'authors': [{'key': '84820c52-dc92-42a2-93fa-61132d2af4d1', 'value': 'Karen A. Tong, MD'}], 'caseVersionId': 'e5b987ff-56ce-417a-bd9f-916ab5eb1c89', 'description': 'Typical case of rapidly enlarging hemorrhagic contusion.\n\nInitial axial NECT (#1) showed subtle small areas of hemorrhagic contusion along the periphery of the right frontal and temporal lobes (arrows). There is also diffuse sulcal effacement and slight leftward midline shift at the level of the septum pellucidum. A subsequent CT (#2) after a short time interval demonstrates that the small hemorrhagic contusions have progressed to a frank hematoma, with markedly worse mass effect. \n\nComment: It is not uncommon for delayed hematomas to develop 24-48 hours after injury. Even if the initial CT is negative, it is useful to consider repeating the CT in 24-48 hours.', 'history': None, 'imagePoolId': 'd60b429e-b212-44fa-b518-fb17e51a1db9', 'name': 'Massive increase', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '42b7725d-0571-4d4b-8b63-4285a000f1d1', 'description': 'NECT scan shows a left frontal hyperdensity with surrounding hypodensity typical of cortical contusion. Note effaced frontal sulci from focal mass effect.', 'history': 'Closed head injury.', 'imagePoolId': 'ee651246-b325-48ee-8fa8-723a8c901985', 'name': 'Focal gyrus, sulci involved', 'teachingPoint': None}, {'authors': [{'key': '84820c52-dc92-42a2-93fa-61132d2af4d1', 'value': 'Karen A. Tong, MD'}], 'caseVersionId': '913dd06b-571d-41b5-8810-1219a1d25251', 'description': 'Typical CT case of traumatic contrecoup hemorrhagic contusion.\n\nAxial bone CT images (#1,2) show marked left parietal and temporal scalp swelling (arrows) without evidence of fracture. Axial NECT (#3) shows a large hemorrhagic contusion in the right temporal lobe (open arrow), opposite to the side of the scalp swelling, indicative of contrecoup injury. There is also subdural blood layering along the tentorium (curved arrow). Axial NECT image near the vertex (#4) shows traumatic subarachnoid hemorrhage within multiple peripheral sulci (curved arrows), greater on the left than right. The sulci are also better visualized on the left (black arrows), indicating greater edema on the right, probably secondary to the large right temporal contusion.', 'history': None, 'imagePoolId': '940722f0-3727-4ec8-ad2d-9eadd85ae035', 'name': 'Contrecoup', 'teachingPoint': 'Direct injuries that occur ipsilateral to the site of impact are designated "coup" lesions, whereas indirect injuries that occur opposite to the impact site are designated "contrecoup" lesions. The latter injuries are induced by gyral crests striking a fixed surface (i.e., the skull).'}, {'authors': [{'key': '84820c52-dc92-42a2-93fa-61132d2af4d1', 'value': 'Karen A. Tong, MD'}], 'caseVersionId': '9c55b296-b27e-49ab-a05e-aac133df87de', 'description': 'Typical CT case of frontal and temporal contusions from acceleration/deceleration injury.\n\nAxial NECT shows several hemorrhagic contusions (arrows) in the inferior frontal lobes, anterior right temporal lobe and posterior right temporal lobe. There is also a nonhemorrhagic contusion in the anterior/inferior right frontal lobe (open arrow).', 'history': None, 'imagePoolId': '31382187-0d11-49cf-8080-4fe9d2b6b863', 'name': 'Acceleration/deceleration', 'teachingPoint': 'Contusions can occur from direct impact or acceleration/deceleration injury. The second mechanism can be associated with linear acceleration (e.g., boxing injury) or deceleration (e.g., motor vehicle accident), which causes the brain to strike the skull. In an event in which the head is in motion, cortical injury occurs adjacent to the floor of the anterior or posterior cranial fossa, the sphenoid wing, the petrous ridge, the convexity of the skull, and the falx or tentorium. The inferior frontal and temporal lobes are particularly vulnerable.'}], 'caseType': 'typical', 'name': 'TYPICAL'}
+- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '7d90ed51-c5d3-4e6b-8a11-5b72e12fcbe8', 'description': 'Axial NECT scan through the suprasellar cistern shows traumatic subarachnoid hemorrhage, with blood around the tentorium and within the interpeduncular fossa (arrow, #1). Note lack of contusions in the anterior temporal lobes and anteroinferior temporal lobes. Scans through the corpus callosum (#2-3) show an enormous midline hematoma (arrows) with some intraventricular hemorrhage (curved arrow, #2). A linear collection of smaller hemorrhages (open arrows) probably represents axonal injury.\n\nBecause of the unusual hematoma, possible traumatic pseudoaneurysm involving the anterior cerebral artery and its branches was a possible etiology. DSA of both the right (#4) and left (#5) common carotid arteries showed no evidence for aneurysm, although some areas of mild vessel narrowing (arrows) secondary to the hematoma are seen.\n\nComment: Impingement of the dorsolateral corpus callosum against the falx during closed head injury is the etiology of this unusual traumatic hematoma. Damage to the adjacent anterior cerebral arteries should be evaluated by either DSA or CTA in such cases, as continued hemorrhage from a pseudoaneurysm can occur.', 'history': 'Unrestrained victim in car roll-over at high speed.', 'imagePoolId': '5a0c24e7-ec67-4dc8-84bc-333eb68f57e0', 'name': 'Huge hematoma in dorsolateral corpus callosum', 'teachingPoint': None, 'demographics': '62 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'a5ce3a87-65da-4931-86ec-1fe6ea3f929d', 'description': 'Axial NECT scans (#1-10) show innumerable cortical contusions, many with fluid-fluid levels indicating active bleeding. Complete bilateral central descending herniation has occurred, with obliteration of all basal cisterns and sulci. The hemispheres are hypodense because of diffuse brain swelling and brain death (the patient was arrested and died soon after the scan was obtained).\n\nNote the central midbrain Duret hemorrhage (arrow, #1), a complication of severe descending herniation. Image #8 shows frankly lacerated brain (arrow) with hemorrhage extending through the right posterior frontal lobe to the subarachnoid space. Traumatic subarachnoid hemorrhage can also be seen (open arrow). A vertex venous epidural hematoma, caused by a severe fracture crossing and tearing the superior sagittal sinus, is seen on images #9 and #10 (curved arrows). Bone CT scans (#11-14) show multiple comminuted basilar and calvarial fractures.\n\nComment: Severe cortical contusions with rapid hemorrhage may show fluid-fluid levels, as happened in this case, without coagulopathy or other predisposing factors.', 'history': 'Beaten with 2 x 4 until unconscious.', 'imagePoolId': '3a7d2a2f-af8c-49ce-969c-7a8495ba7a72', 'name': 'Massive; active bleeding', 'teachingPoint': None, 'demographics': '43 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '3df363e4-a5bc-4004-817b-85b1c1c737fd', 'description': 'Axial NECT scan shows a mostly hypodense mass in the corpus callosum (arrows, #1).\n\nSagittal (#2) T1WI shows mixed iso- and hyperintense hemorrhage in the corpus callosum body (arrows). FLAIR (#3) shows mixed hyperintense mass indenting lateral ventricles (arrows). T2* scan shows some blooming (arrows, #4). T1C+ scans (#5,6) show no enhancement.', 'history': 'Trauma with persistent headaches.', 'imagePoolId': 'c5834f9f-95a0-417c-9a9d-20084d203b09', 'name': 'Dorsolateral', 'teachingPoint': 'Impaction of the corpus callosum against the inferior free margin of the falx cerebri during closed head injury may result in striking dorsolateral contusions, as occurred in this case.'}], 'caseType': 'variant', 'name': 'VARIANT'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*Coronal graphic illustrates the pathology of a closed head injury. Note the hemorrhagic foci involving gray matter of several contused gyri
, axonal and deep gray injuries, and traumatic subarachnoid hemorrhage
in the basal cisterns and sylvian fissure.*
+
+
+*Graphics depict the most common sites of cerebral contusions in red. Less common sites are shown in green. The most common locations of all are the anteroinferior frontal and temporal lobes.*
+
+
+*Gross pathology of the brain from a patient who died from a severe closed head injury shows bifrontal, temporal hemorrhagic contusions
as well as traumatic subarachnoid hemorrhage in the suprasellar cistern
. (Courtesy R. Hewlett, MD.)*
+
+
+*Sagittal NECT status post fall with traumatic hemorrhagic contusions
in characteristic locations at the left temporal pole and basal frontal regions is shown. Posterior cerebral subdural hemorrhage
and superficial scalp contusion are also demonstrated.*
+
+
+*Sagittal NECT after fall shows a small cortical contusion
at the impaction site against the bony sphenoid wing
. Cortical contusions are gyral crest injuries caused by impact against unyielding bone or dura. Traumatic subarachnoid hemorrhage is typically also present.*
+
+
+*Axial SWI MR in the same patient shows focal blooming
at the site of parenchymal acute hemorrhage. In patients with chronic injury, susceptibility signal signifies hemosiderin deposition. SWI is more sensitive for blood products than GRE.*
+
+
+*Initial sagittal NECT after a fall from the roof with a nondisplaced left calvarial fracture (not shown) shows a contrecoup right temporal hemorrhagic contusion
with surrounding edema. Contrecoup injury is usually more severe than coup.*
+
+
+*Same patient at 24-hour follow-up CT shows expansion of the hemorrhagic contusion
and surrounding edema. Decompressive craniectomy was subsequently performed due to rapid progression and increasing mass effect. Contusion progression occurs in up to 75% of TBI.*
+
+
+*Autopsy of severe head trauma shows cortical contusions seen as multiple small hemorrhages
with preferential location along the superficial cortex. Traumatic subarachnoid hemorrhage
commonly accompanies cortical contusions.*
+
+
+*Coronal T2 MR demonstrates chronic sequela of TBI with bifrontal encephalomalacia and gliosis localizing to the basal frontal lobes, namely the gyrus recti
.*
+
+
+### Additional Images
+
+
+*Axial NECT scan 24 hours after head trauma shows frontotemporal contusions
and a left inferior frontal subdural hygroma
.*
+
+
+*Axial T2W MR in the same patient obtained immediately after the CT scan shows contusions
with perilesional edema
and small bilateral subdural hygromas
.*
+
+
+*Axial T2* GRE in the same patient shows blooming of right frontotemporal contusions
. A left temporal contusion
is apparent that was not evident on the T2 MR.*
+
+
+*Axial FLAIR MR in the same patient shows multiple mixed hyper- (edema)
and hypointense (blood)
cortical contusions. Note hyperintensity in multiple sulci
. This is a traumatic subarachnoid hemorrhage, which is especially prominent adjacent to the contusions and contrecoup injury
.*
+
+
+*Cerebral contusions tend to increase with time, as illustrated by this case. NECT in a patient involved in a high-impact traffic accident shows several anteroinferior hemorrhagic foci
. The Glasgow Coma Scale (GCS) was 13 at the accident site.*
+
+
+*Several hours following admission, the same patient suddenly deteriorated and became comatose. Repeat NECT shows striking interval enlargement of the contusions. Mixed density of the clots suggests rapid bleeding.*
+
+
+*Sagittal midline autopsied brain in severe trauma shows dorsolateral corpus callosum contusions
. During severe traumatic impact, the corpus callosum can be forced against the inferior margin of the falx cerebri, causing hemorrhagic cortical contusions. Note axonal "shearing" injury to the fornix
and midbrain
.*
+
+
+*Sagittal T1 MR in a patient with head trauma was obtained several days after the injury. Note subacute hemorrhage in the vermis
. The corpus callosum
appears slightly swollen, but no foci of T1 shortening are seen.*
+
+
+*Axial T2* GRE in the same patient shows confluent blooming
in the dorsolateral surface of the corpus callosum.*
+
+
+*Axial T2* SWI in the same patient shows the dorsolateral corpus callosum contusion
. Note punctate hemorrhages in the splenium
, most likely caused by axonal injury, not impaction against the falx cerebri.*
+
+
+*Axial NECT shows a bifrontal mixture of hemorrhage
and edema
. Severe cortical contusions with macerated frontal lobes are seen. Note mass effect with posterior displacement of the frontal horns
.*
+
+
+*Axial NECT in the same patient shows a right frontal hemorrhagic contusion
and edema
at 4 hours.*
+
+
+*Axial NECT in the same patient shows progressive enlargement and coalescence of the hemorrhagic contusion
at 24 hours. A new contusion is present in the left frontal lobe
.*
+
+
+*Axial NECT in the same patient shows subtle hypodense edema
in the frontal lobe.*
+
+
+*Axial T2 FS MR in the same patient shows bifrontal edema
and mild petechial hemorrhage
.*
+
+
+*Axial NECT shows a left frontal contusion tear. Homogeneous hematoma extends from the surface of the brain
into the right lateral ventricle
.*
+
+
+*Sagittal T1 MR shows an inferior frontal
and frontal polar
hyperintense hemorrhagic contusion. Note the convexity
and paratentorial
subdural hematoma.*
+
+
+*Axial FLAIR MR shows cerebral contusion hyperintensity involving the temporal cortices and frontal lobe rectus gyri
. The left uncus is beginning to herniate
.*
+
+
+*Axial T2 MR demonstrates cortical hyperintensity and early encephalomalacia of the anterior temporal lobe from an evolving cerebral contusion.*
+
+
+*Axial NECT shows a large cerebral contusion that rapidly enlarged over a short imaging interval with worsening midline shift. As seen here, hyperdense hemorrhage has progressed to frank hematoma.*
+
+
+*Axial NECT of a newly admitted patient with a closed head injury shows a small frontal cerebral hypodense contusion with foci of hyperdense hemorrhage
.*
+
+
+*Axial NECT in the same patient obtained 24 hours later shows the evolution and clear demarcation of the hypodense contusion with interspersed foci of hyperdense hemorrhage
.*
+
+
+*Axial NECT shows multifocal contusions involving the inferior frontal and temporal lobes. Some are nonhemorrhagic
, and others have hyperdense foci of hemorrhage
.*
+
+
+*Axial NECT demonstrates a contrecoup hemorrhagic cerebral contusion within a lacerated temporal lobe. Tentorial subdural
and coup scalp injury
are also seen.*
+
+
+*Axial NECT reveals extensive, bifrontal hemorrhagic contusions
.*
+
+
+*Axial NECT scan shows extensive frontotemporal contusions
and traumatic subarachnoid hemorrhage
in a patient with severe brain injury.*
+
+
+*Axial T2* GRE MR of the same patient best shows the hypointense hemorrhagic components as foci of parenchymal susceptibility or "blooming"
. Note that the subarachnoid hemorrhage is invisible by GRE.*
+
+
+*Axial NECT reveals a coup hemorrhagic contusion of the right cerebellar hemisphere. The contusion has coalesced into a focal hematoma
.*
+
+
+*Axial NECT in the same patient reveals a larger contrecoup hemorrhagic contusion involving the left inferior and periorbital frontal brain
. Note that morphology may be linear along gyral crests or focal; both are seen in this example. There is also an associated subdural hematoma of the posterior falx
.*
+
+
+*Axial NECT in a 33-year-old woman with head trauma shows a solitary hemorrhagic contusion
immediately adjacent to the greater sphenoid wing
.*
+
+
+*Coronal NECT in the same patient reformatted from the axial source data shows the classic location of a focal cortical contusion
, namely, inferior frontal gyrus adjacent to the skull base.*
+
+
+*Sagittal NECT reformatted from the axial source data shows the focal cortical contusion
was caused when the brain was forcibly impacted against the bony sphenoid wing
. Cortical contusions are gyral crest injuries caused by impact against unyielding bone or dura.*
+
+
+*Initial axial NECT scan in a 24-year-old man with a severe closed head injury and a GCS of 8 on admission shows only a focal contusion in the left inferior frontal lobe
and a small amount of subarachnoid hemorrhage in the inferior interhemispheric fissure
.*
+
+
+*Repeat NECT scan 6 hours later shows the interval appearance of cortical contusions along the surface of the right temporal lobe
. Cortical contusions commonly bloom (i.e., become more conspicuous) on follow-up imaging studies.*
+
diff --git a/docs_md/articles/chanter-syndrome_f14c06ed-2452-49b2-b82d-a3572040da08.md b/docs_md/articles/chanter-syndrome_f14c06ed-2452-49b2-b82d-a3572040da08.md
new file mode 100644
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--- /dev/null
+++ b/docs_md/articles/chanter-syndrome_f14c06ed-2452-49b2-b82d-a3572040da08.md
@@ -0,0 +1,276 @@
+---
+title: "CHANTER Syndrome"
+docid: "f14c06ed-2452-49b2-b82d-a3572040da08"
+authors:
+ - key: "a25c450b-3d34-4f64-bba3-cc0834813df6"
+ value: "Miral D. Jhaveri, MD, MBA"
+breadcrumbs:
+ -
+ name: "Brain"
+ slug: "brain"
+ treeNodeId: "0361042c-9c90-48e4-864e-a2e6428ee9c9"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "948c6f1e-8e18-45f3-bd9d-7a04ab0b8708"
+ -
+ name: "Pathology-Based Diagnoses"
+ slug: "pathology-based-diagnoses"
+ treeNodeId: "d60fe914-7897-46a0-9cd2-f88ffb322f16"
+ -
+ name: "Acquired Toxic/Metabolic/Degenerative Disorders"
+ slug: "acquired-toxicmetabolicdegenerativ-"
+ treeNodeId: "80461866-16fe-4ccf-b9b8-034117c0a1e6"
+ -
+ name: "Toxic, Metabolic, Nutritional, Systemic Diseases With CNS Manifestations"
+ slug: "toxic-metabolic-nutritional-system-"
+ treeNodeId: "9f567059-7d25-4aca-9920-93171423ef5e"
+ -
+ name: "CHANTER Syndrome"
+ slug: "chanter-syndrome"
+ treeNodeId: null
+category: "Brain"
+documentVersionId: "c3d4715f-918c-4ec5-ae5c-2cc82f9d22eb"
+imageCount: 4
+lastUpdated: "09/26/25"
+pageDescription: "CHANTER Syndrome"
+pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Acquired Toxic/Metabolic/Degenerative Disorders, Toxic, Metabolic, Nutritional, Systemic Diseases With CNS Manifestations, CHANTER Syndrome"
+pageTitle: "CHANTER Syndrome | STATdx"
+enhancedTitle: "CHANTER Syndrome"
+type: "DX"
+references: true
+breadcrumbs:
+ - "Brain"
+ - "Diagnosis"
+ - "Pathology-Based Diagnoses"
+ - "Acquired Toxic/Metabolic/Degenerative Disorders"
+ - "Toxic, Metabolic, Nutritional, Systemic Diseases With CNS Manifestations"
+ - "CHANTER Syndrome"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - **C**erebellar, **h**ippocampal, **a**nd Basal **n**uclei **t**ransient **e**dema with Restricted **d**iffusion (CHANTER)
+ - Clinicoradiographic pattern of neurologic injury occurring most commonly following polysubstance or opioid abuse
+- ### Imaging
+
+
+ - Bilateral, symmetric restricted diffusion in gray matter of cerebellum and hippocampi
+ - Variable diffusion restriction in basal ganglia
+ - Occasional occipital cortical restricted diffusion
+ - Cerebellar edema on CT
+ - May see associated obstructive hydrocephalus
+ - Hemorrhage uncommon
+ - No enhancement
+- ### Top Differential Diagnoses
+
+
+ - Hypoxic ischemic injury
+ - Toxic leukoencephalopathy
+ - Opioid-associated amnesic syndrome (OAA)
+ - Cerebellar and basal ganglia involvement uncommon
+ - Acute cerebral ischemia-infarction
+ - Pediatric opioid use‐associated neurotoxicity with cerebellar edema (POUNCE) syndrome
+- ### Pathology
+
+
+ - Acute intoxication with opioids, particularly fentanyl
+ - Theories suggest opioid exposure, impaired mitochondrial function, cellular damage, and edema
+- ### Clinical Issues
+
+
+ - Altered mental status
+ - Decreased level of consciousness
+ - Most patients near-complete recovery or mild-to-moderate residual impairment
+ - Some severe disability, progressive cerebellar edema, herniation, death
+ - Early diagnosis and treatment crucial
+
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - **C**erebellar, **h**ippocampal, **a**nd Basal **n**uclei **t**ransient **e**dema with Restricted **d**iffusion (CHANTER)
+- ### Definitions
+
+
+ - Clinicoradiographic pattern of neurologic injury occurring most commonly following polysubstance or opioid abuse
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Bilateral, symmetric restricted diffusion in gray matter of cerebellum and hippocampi and variable diffusion restriction in basal ganglia
+ - #### Location
+
+
+ - Cerebellum, hippocampus, basal ganglia
+ - Occasional cerebral cortical involvement
+- ### CT Findings
+
+
+ - #### NECT
+
+
+ - Cerebellar edema
+ - May see associated obstructive hydrocephalus
+ - #### CECT
+
+
+ - No enhancement
+- ### MR Findings
+
+
+ - #### FLAIR
+
+
+ - Symmetric hyperintensity in cerebellar cortex and hippocampi
+ - Variable hyperintensity in basal ganglia
+ - Occasional cortical hyperintensities, particularly occipital poles
+ - #### T2* GRE
+
+
+ - Hemorrhage uncommon
+ - #### DWI
+
+
+ - Bilateral, symmetric restricted diffusion in gray matter of cerebellum and hippocampi
+ - Variable diffusion restriction in basal ganglia
+ - Occasional occipital cortical restricted diffusion
+ - #### T1WI C+
+
+
+ - No enhancement
+ - #### MRA
+
+
+ - No vascular abnormality
+- ### Imaging Recommendations
+
+
+ - #### Best imaging tool
+
+
+ - MR with DWI
+
+## DIFFERENTIAL DIAGNOSIS
+
+- [Hypoxic Ischemic Injury](/document/adult-hypoxic-ischemic-injury/91ac293f-161c-4b3b-81e5-740f831eaa5d)
+ - Similar clinical and radiologic features
+ - Typically also involves cerebral cortex in addition to deep nuclei and cerebellum
+- [Acute Cerebral Ischemia-Infarction](/document/acute-cerebral-ischemiainfarction/7a3ed4a9-ae05-4d64-ae8e-6a30105501e1)
+ - Typically involves specific vascular distributions
+ - Cortical involvement common
+- [Toxic Leukoencephalopathy](/document/drug-abuse/48859403-0b26-44d8-ba74-e0919e4c3147)
+ - Heroin toxicity: "Chasing the dragon"
+ - Characteristically involves posterior limb internal capsule and posterior cerebral white matter
+ - May present with hydrocephalus and cerebellar involvement like CHANTER syndrome
+- [Opioid-Associated Amnesic Syndrome](/document/drug-abuse/48859403-0b26-44d8-ba74-e0919e4c3147)
+ - Commonly seen in setting of fentanyl overdose
+ - Similar imaging findings
+ - Restricted diffusion in hippocampi
+ - Predominant symptom of amnesia
+ - Cerebellar and basal ganglia involvement uncommon
+- [Pediatric Opioid Use‐Associated Neurotoxicity With Cerebellar Edema Syndrome](/document/drug-abuse/48859403-0b26-44d8-ba74-e0919e4c3147)
+ - Similar clinical presentation in pediatric patients
+ - Hippocampal and basal ganglia involvement is rare
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - Acute intoxication with opioids, particularly fentanyl
+ - Theories suggest opioid exposure, impaired mitochondrial function, cellular damage, and edema
+ - Cerebellum, hippocampus, and basal ganglia
+ - Predominantly involved due to their high concentration of opioid receptors
+ - Higher sensitivity to hypoxic injury
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - Altered mental status
+ - Decreased level of consciousness
+ - #### Other signs/symptoms
+
+
+ - Signs of increased intracranial pressure due to cerebellar edema, hydrocephalus
+ - Strong clinical correlation exists between CHANTER syndrome and recent opioid intoxication
+- ### Natural History & Prognosis
+
+
+ - Most patients near-complete recovery or mild-to-moderate residual impairment
+ - Some severe disability, progressive cerebellar edema, herniation, death
+ - Early diagnosis and treatment crucial
+- ### Treatment
+
+
+ - Opioid reversal with naloxone and control of cerebral edema with mannitol or hypertonic saline
+ - Control of cerebral edema with mannitol/hypertonic saline
+ - Management of obstructive hydrocephalus
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - **CHANTER**syndrome in setting of opioid overdose with DWI changes in cerebellum, hippocampus, and basal ganglia
+
+ 55f055a3-230b-43dd-a5a4-63716043ab8a
+
+## References
+
+## Selected References
+
+1. [Pandit R et al: Cerebellar, hippocampal, and basal nuclei transient edema with restricted diffusion (CHANTER) syndrome: radiologic features and findings. Korean J Radiol. 25(3):314-8, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38413115%5Bpmid%5D)
+1. [Suthar PP et al: Case 324: CHANTER syndrome. Radiology. 311(1):e222748, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38687220%5Bpmid%5D)
+1. [Wahlstrom BA et al: Radiographic findings of evolving sequelae of cerebellar, hippocampal, and basal nuclei transient edema with restricted diffusion (CHANTER) syndrome in a 37-year-old patient. Cureus. 16(11):e73467, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=39664119%5Bpmid%5D)
+1. [Rizkallah Alves B et al: Teaching neuroImage: CHANTER syndrome. Neurology. 101(22):e2338-9, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37813578%5Bpmid%5D)
+1. [Sheehan J et al: Reversible amnesia following opiate overdose: CHANTER syndrome. Pract Neurol. 23(4):350-1, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37068933%5Bpmid%5D)
+1. [Mallikarjun KS et al: Neuroimaging findings in CHANTER syndrome: a case series. AJNR Am J Neuroradiol. 43(8):1136-41, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35798385%5Bpmid%5D)
+1. [Jasne AS et al: Cerebellar hippocampal and basal nuclei transient edema with restricted diffusion (CHANTER) syndrome. Neurocrit Care. 31(2):288-96, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30788708%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+*Axial NECT in a 59-year old with substance use disorder presenting with altered mental status shows symmetric edema
in both cerebellar hemispheres with mass effect on the 4th ventricle
.*
+
+
+*Axial NECT in a 59-year old with substance use disorder presenting with altered mental status shows symmetric edema
in both cerebellar hemispheres with mass effect on the 4th ventricle
.*
+
+
+*Axial NECT in a 59-year old with substance use disorder presenting with altered mental status shows symmetric edema
in both cerebellar hemispheres with mass effect on the 4th ventricle
.*
+
+
+*Axial DWI images in the same patient show restricted diffusion in the basal ganglia, hippocampi, and both cerebellar hemispheres. Toxicology was positive for opiates, and the clinicoradiologic findings are consistent with CHANTER syndrome.*
+
+
+*Axial DWI images in the same patient show restricted diffusion in the basal ganglia, hippocampi, and both cerebellar hemispheres. Toxicology was positive for opiates, and the clinicoradiologic findings are consistent with CHANTER syndrome.*
+
+
+*Axial DWI (top) and FLAIR (bottom) images in a patient with fentanyl overdose show restricted diffusion
with FLAIR hyperintensity
in both cerebellar hemispheres.*
+
+
+*Axial DWI (top) and FLAIR (bottom) images in a patient with fentanyl overdose show restricted diffusion
with FLAIR hyperintensity
in both cerebellar hemispheres.*
+
+
+*Axial DWI in the same patient shows foci of restricted diffusion
in the basal ganglia bilaterally. The hippocampi also showed restricted diffusion (not shown). CHANTER syndrome is a pattern of neurologic injury occurring most commonly following polysubstance or opioid abuse.*
+
+
+*Axial DWI in the same patient shows foci of restricted diffusion
in the basal ganglia bilaterally. The hippocampi also showed restricted diffusion (not shown). CHANTER syndrome is a pattern of neurologic injury occurring most commonly following polysubstance or opioid abuse.*
+
diff --git a/docs_md/articles/complex-cranial-nerve-9-12-neuropathy_5f1443a4-b34c-4075-a8d1-d5aa3718348d.md b/docs_md/articles/complex-cranial-nerve-9-12-neuropathy_5f1443a4-b34c-4075-a8d1-d5aa3718348d.md
new file mode 100644
index 0000000..fc7b558
--- /dev/null
+++ b/docs_md/articles/complex-cranial-nerve-9-12-neuropathy_5f1443a4-b34c-4075-a8d1-d5aa3718348d.md
@@ -0,0 +1,447 @@
+---
+title: "Complex Cranial Nerve 9-12 Neuropathy"
+docid: "5f1443a4-b34c-4075-a8d1-d5aa3718348d"
+authors:
+ - key: "eef2f839-5706-47b9-89c3-60d8315b2b3a"
+ value: "Nicholas A. Koontz, MD"
+breadcrumbs:
+ -
+ name: "Head and Neck"
+ slug: "head-and-neck"
+ treeNodeId: "5c1f8e17-7acd-48d8-9d55-f9f8c2cad850"
+ -
+ name: "Differential Diagnosis"
+ slug: "differential-diagnosis"
+ treeNodeId: "deb55065-e1d6-4b6f-b3e3-181fafb4e218"
+ -
+ name: "Cranial Nerves and Brainstem"
+ slug: "cranial-nerves-and-brainstem"
+ treeNodeId: "385449a2-5859-451c-bed3-584babc08f0c"
+ -
+ name: "Clinically Based Differentials"
+ slug: "clinically-based-differentials"
+ treeNodeId: "f0574381-a384-4486-8fb9-e90591e4f9be"
+ -
+ name: "Complex Cranial Nerve 9-12 Neuropathy"
+ slug: "complex-cranial-nerve-9-12-neuropa-"
+ treeNodeId: null
+category: "Head and Neck"
+documentVersionId: "228824a3-c1e6-4a12-a71d-f3bdcba958d3"
+imageCount: 58
+lastUpdated: "04/13/26"
+pageDescription: "Complex Cranial Nerve 9-12 Neuropathy"
+pageKeywords: "Head and Neck, Differential Diagnosis, Cranial Nerves and Brainstem, Clinically Based Differentials, Complex Cranial Nerve 9-12 Neuropathy"
+pageTitle: "Complex Cranial Nerve 9-12 Neuropathy | STATdx"
+enhancedTitle: "Complex Cranial Nerve 9-12 Neuropathy"
+type: "DDX"
+references: true
+breadcrumbs:
+ - "Head and Neck"
+ - "Differential Diagnosis"
+ - "Cranial Nerves and Brainstem"
+ - "Clinically Based Differentials"
+ - "Complex Cranial Nerve 9-12 Neuropathy"
+---
+## ESSENTIAL INFORMATION
+
+- ### Key Differential Diagnosis Issues
+
+
+ - Lesions causing injury to CNIX-XII are found anywhere along nerve's course from medulla to end organ
+ - CNIX & CNXII remain suprahyoid
+ - CNX & CNXI course infrahyoid
+ - CNX in carotid space (CS)
+ - CNXI in posterior cervical space
+ - Proximal CNIX-XII course together
+ - Intracranial course: Medullary nuclei, basal cisterns
+ - Skull base: Jugular foramen (JF) (CNIX-XI), hypoglossal canal (CNXII)
+ - Extracranial: Naso- & oropharyngeal CS
+ - Imaging approach
+ - MR & CT are often complementary
+ - Field of view must include medulla & extend to hyoid bone (CNIX or XII neuropathy), entire neck (CNX neuropathy), or entire neck + upper mediastinum through AP window (CNX neuropathy)
+ - CECT: Often 1st-line modality to screen for causes
+ - Bone CT: Aides in differentiation of JF paraganglioma vs. schwannoma vs. meningioma
+ - Evaluate for calcified tumor matrix in chondrosarcoma
+ - Contrast-enhanced MR: Superior tissue contrast resolution
+ - Identification of causative mass
+- ### Helpful Clues for Common Diagnoses
+
+
+ - **Paraganglioma (Jugular)**
+ - Benign tumor arises from paraganglia cells at JF margin
+ - Centered along superolateral margin of JF; frequent superolateral vector of spread into middle ear
+ - CT: Permeative-destructive appearing bone changes; middle ear floor not seen
+ - MR: Flow voids; avidly enhancing
+ - Classic **s****alt & pepper**appearance with T1 bright "salt" (foci of hemorrhage or slow flow) & T2 dark "pepper" (high-velocity flow voids, often peripheral)
+ - Nuclear medicine: Increased role of 68-Ga DOTANOC PET/CT or PET/MR for detecting synchronous disease & rare metastases; higher sensitivity than CT or MR
+ - Paragangliomas may be multicentric &/or syndromic
+ - **Nasopharyngeal Carcinoma**
+ - Tumor usually invasive at presentation
+ - CT/MR: Invasion from nasopharynx → CS
+ - **Squamous Cell Carcinoma, Nodes, Internal Jugular**
+ - Internal jugular chain metastases
+ - Primary tumor often from nasopharynx, oropharynx, or oral cavity
+ - CT/MR: Extranodal extension affects CS
+ - **Cerebral Ischemia-Infarction, Acute,****Posterior Inferior Cerebellar Artery**
+ - Posterior inferior cerebellar artery infarct affects lateral medulla & inferior cerebellar hemisphere
+ - MR: DWI ↑ signal, ADC ↓ signal
+ - **Metastasis, Skull Base**
+ - CT: Invasive/destructive bony margins
+ - MR: T1 ↓ signal in fatty skull base; enhances; may have reduced diffusivity on DWI
+ - **Brainstem Tumors, Pediatric**
+ - MR: High T2 & FLAIR signal in brainstem
+ - Variable, often subtle enhancement
+- ### Helpful Clues for Less Common Diagnoses
+
+
+ - **Squamous Cell Carcinoma****, Palatine Tonsil**
+ - Deeply invasive squamous cell carcinoma (SCCa)
+ - Large palatine tonsil with posterolateral invasive margin reaching CS
+ - **Schwannoma, Jugular Foramen**
+ - Bone CT: Smooth, expansion of JF
+ - MR: Tubular enhancing mass centered in JF; "points" toward lateral medulla
+ - **Cavernous Malformation, Medulla**
+ - CT: Punctate Ca⁺⁺; high-density blood
+ - MR: GRE/SWI blooming ± developmental venous anomaly (mixed vascular malformation)
+ - **Metastases, Meningeal**
+ - Synonym: Meningeal carcinomatosis
+ - MR: Enhancing, thickened meninges
+ - Sometimes nodular in appearance
+ - ± reduced diffusivity
+ - FLAIR C+ imaging may increase sensitivity for identifying meningeal metastases
+ - **Dissection, Carotid Artery, Neck**
+ - Ipsilateral Horner syndrome; history of previous trauma or vasculopathy
+ - CTA/MRA: Carotid lumen narrowing
+ - Suprabifurcation location; terminates at carotid canal of skull base
+ - Look for mural thrombus
+ - Hyperdense on CT
+ - Intrinsically T1 bright; best seen on dedicated fat-saturated T1 C- sequence
+ - **Paraganglioma (Vagal)**
+ - Arises in vagal nodose ganglion of CS
+ - CT/MR: Centered ~ 2 cm below skull base
+ - Intense enhancement
+ - High velocity flow voids (MR)
+ - Classic **s****alt & pepper** appearance
+ - **Schwannoma, Carotid Space**
+ - CT: Tubular, circumscribed enhancing CS mass; displaces carotid anteriorly
+ - MR: Enhancing mass ± intramural cysts
+- ### Helpful Clues for Rare Diagnoses
+
+
+ - **Ependymoma, Basal Cistern**
+ - Childhood tumor; 4th ventricle origin often; spreads to basal cisterns
+ - CT: ~ 50% shows some Ca⁺⁺
+ - MR: Complex signal 4th ventricle-basal cistern tumor
+ - Ca⁺⁺, hemorrhage, cystic changes all possible
+ - **Multiple Sclerosis, Medulla**
+ - MR: High T2/FLAIR signal lesions in suprasellar white matter
+ - Medullary lesions not always visible, often better seen on T2WI than FLAIR
+ - **Meningioma, Jugular Foramen**
+ - Bone CT: Permeative-sclerotic hyperostotic changes in bones around JF
+ - MR: Centrifugal spreading tumor along dural surfaces
+ - Enhancing lesion; dural tails
+ - **Chondrosarcoma, Skull Base**
+ - Malignant lesion of chondroid origin most commonly centered at petrooccipital fissure
+ - Bone CT: Lytic lesion ± calcified chondroid matrix
+ - Calcified matrix less common at petroccipital location (< 30%) compared to other H&N sites (50-70%)
+ - MR: ↑ T2 signal; enhances avidly; relatively bright ADC signal
+ - **Schwannoma, Hypoglossal Nerve**
+ - Bone CT: Benign expansion hypoglossal canal
+ - MR: Tubular enhancing lesion ± intramural cysts
+- ### Alternative Differential Approaches
+
+
+ - CNIX-XII neuropathy via **segmental anatomic approach**
+ - Anatomic segments
+ - Intramedullary: Nuclei tracts
+ - Cisternal: Nerves in basal cistern
+ - Skull base: JF (CNIX-XI); hypoglossal canal (CNXII)
+ - Naso- & oropharyngeal CS: CNIX-XII
+ - Segmental approach complex CNIX-XII neuropathy DDx
+ - Medulla
+ - [Cerebral ischemia-infarction, acute](/document/acute-cerebral-ischemiainfarction/7a3ed4a9-ae05-4d64-ae8e-6a30105501e1)
+ - [Brainstem tumors, pediatric](/document/diffuse-midline-glioma-h3-k27-alte-/fc296ded-e5f7-4bee-8999-fc51ec72a256)
+ - [Cavernous malformation, medulla](/document/cavernous-malformation/cc2415f4-75a6-458d-800e-be31b3cf50c3)
+ - [Multiple sclerosis, medulla](/document/multiple-sclerosis/abe95a5e-394f-411b-aca6-72ab160a1d0d)
+ - Basal cistern
+ - Ependymoma, basal cistern
+ - [Metastases, meningeal](/document/skull-and-meningeal-metastases/5e4d4a09-e04a-49c9-a4e0-d2849a992a82)
+ - Skull base around JF
+ - Paraganglioma (Jugular)
+ - [Metastasis, skull base](/document/skull-base-metastasis/9f46be56-65de-44f1-bf85-8aa3c6b7d078)
+ - [Schwannoma, JF](/document/jugular-foramen-schwannoma/39fe8b5f-828f-4529-a1a5-2e267c0ab63c)
+ - [Meningioma, JF](/document/jugular-foramen-meningioma/c90dd6ee-1fc2-4e93-b200-d15889bb7f10)
+ - [Chondrosarcoma, skull base](/document/skull-base-chondrosarcoma/092dd3bf-00db-4a10-b62a-99e4e4c59992)
+ - [Schwannoma, hypoglossal nerve](/document/hypoglossal-nerve-schwannoma/732380f4-6fb0-4037-8103-fd87f443169b)
+ - CS, naso- & oropharynx
+ - [Paraganglioma (Vagal)](/document/vagal-paraganglioma/55cc2103-a155-4281-ac39-b0b8ab02e98e)
+ - [SCCa, nodes](/document/nodal-squamous-cell-carcinoma/75d61354-5c63-426a-8a44-7fbf37fec2ee)
+ - [Nasopharyngeal carcinoma](/document/nasopharyngeal-carcinoma/3b6e5802-e8c2-461a-8efd-1b634b92c8c1)
+ - [Squamous cell carcinoma, palatine tonsil](/document/palatine-tonsil-squamous-cell-carc-/ca8fe680-a845-4ecc-8165-43dc8e809d40)
+ - [Dissection, carotid artery, neck](/document/carotid-artery-dissection-in-neck/95470e51-7671-447c-9c49-30d4e55d04aa)
+ - [Schwannoma, CS](/document/carotid-space-schwannoma/77e63e12-2bf6-4295-b73d-501c8ffbbde0)
+
+## References
+
+## Selected References
+
+1. [Ragittaran J et al: Imaging of hypoglossal palsy: a pictorial synopsis. Clin Radiol. 81:106754, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=39721320%5Bpmid%5D)
+1. [Carlstrom LP et al: Lower cranial nerve schwannomas: cohort study and systematic review. Neurosurgery. 94(4):745-55, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=37874134%5Bpmid%5D)
+1. [Castillo AL et al: Jugular foramen tumors: surgical strategies and representative cases. Brain Sci. 14(2), 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38391756%5Bpmid%5D)
+1. [Palade DO et al: Paragangliomas of the head and neck: a review of the latest diagnostic and treatment methods. Medicina (Kaunas). 60(6), 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38929531%5Bpmid%5D)
+1. [Bal J et al: Management of non-vestibular schwannomas in adult patients: a systematic review and consensus statement on behalf of the EANS skull base section part III: lower cranial nerve schwannomas, jugular foramen (CN IX, X, XI) and hypoglossal schwannoma (XII). Acta Neurochir (Wien). 164(2):321-9, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=34854994%5Bpmid%5D)
+1. [Traylor KS et al: Cranial nerve anatomy. Neuroimaging Clin N Am. 32(3):565-76, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35843663%5Bpmid%5D)
+1. [Gutierrez S et al: Lower cranial nerve syndromes: a review. Neurosurg Rev. 44(3):1345-55, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=32638140%5Bpmid%5D)
+1. [Jain V: The role of imaging in the evaluation of hoarseness: a review. J Neuroimaging. 31(4):665-85, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34018650%5Bpmid%5D)
+1. [Thelen J et al: Multimodality imaging of paragangliomas of the head and neck. Insights Imaging. 10(1):29, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30830483%5Bpmid%5D)
+1. [Edwards B et al: Cranial nerve foramina: part ii - a review of the anatomy and pathology of cranial nerve foramina of the posterior cranial fossa. Cureus. 10(4):e2500, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29928560%5Bpmid%5D)
+1. [Job J et al: Imaging of the posterior skull base. Radiol Clin North Am. 55(1):103-21, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27890180%5Bpmid%5D)
+1. [Patel VA et al: End-organ radiographic manifestations of cranial neuropathies: a concise review. Clin Imaging. 44:5-11, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28364580%5Bpmid%5D)
+1. [Lopci E et al: Gallium-68 DOTANOC imaging in paraganglioma/pheochromocytoma: presentation of sample cases and review of the literature. Q J Nucl Med Mol Imaging. 57(2):134-45, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23598686%5Bpmid%5D)
+1. [Sharma P et al: 68Ga-DOTANOC PET/CT for baseline evaluation of patients with head and neck paraganglioma. J Nucl Med. 54(6):841-7, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23520216%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+**Paraganglioma (Jugular)**
+*Axial bone CT shows characteristic permeative-destructive bone changes
and superolateral vector of spread into the middle ear
of a jugular paraganglioma.*
+
+
+**Paraganglioma (Jugular)**
+*Axial bone CT shows characteristic permeative-destructive bone changes
and superolateral vector of spread into the middle ear
of a jugular paraganglioma.*
+
+
+**Paraganglioma (Jugular)**
+*Axial T1 C+ FS MR of a jugular paraganglioma
shows typical avid contrast enhancement and high-velocity flow voids
seen with paragangliomas of the head and neck. The mass is centered in the jugular foramen, but demonstrates a superolateral vector of spread into the middle ear
.*
+
+
+**Nasopharyngeal Carcinoma**
+*Axial CECT of nasopharyngeal carcinoma (NPC) shows a bulky mass
centered in the lateral pharyngeal recess, extending anteriorly to the nasal cavity. Posterolaterally, the mass involves the carotid space (CS)
, which contains CNIX-XII at this level. Note bulky contralateral retropharyngeal adenopathy
.*
+
+
+**Nasopharyngeal Carcinoma**
+*Axial T2 FS MR better maps the extent of the NPC
, including involvement of the ipsilateral CS by primary tumor
and of the contralateral CS by retropharyngeal adenopathy
.*
+
+
+**Squamous Cell Carcinoma, Nodes, Internal Jugular**
+*Axial CECT reveals a right tonsillar mass extending anteriorly into the tongue base
. Internal jugular nodes
appear infiltrating and with extranodal extension invading the CS. Note the normal CS
on the left, deep to the posterior belly of the digastric muscle.*
+
+
+**Squamous Cell Carcinoma, Nodes, Internal Jugular**
+*Axial CECT in the same patient shows an extranodal tumor invading the deep parotid space
, sternocleidomastoid muscle
, and the CS
.*
+
+
+**Cerebral Ischemia-Infarction, Acute, Posterior Inferior Cerebellar Artery**
+*Axial DWI MR of an acute posterior inferior cerebellar artery (PICA) infarction shows reduced diffusivity in the right lateral medulla
corresponding to the region of lower cranial nerve nuclei. Note diffusion bright thrombus within the right V4 vertebral artery
. Additional infarction is present in the right cerebellum
.*
+
+
+**Cerebral Ischemia-Infarction, Acute, Posterior Inferior Cerebellar Artery**
+*Axial FLAIR FS MR shows multifocal acute PICA infarction with increased signal in the right lateral medulla
and inferomedial cerebellum
. Note abnormal increased signal within the tortuous right V4 vertebral artery
from thrombus.*
+
+
+**Metastasis, Skull Base**
+*Axial bone CT in a patient with metastatic poorly differentiated squamous cell carcinoma (SCCa) of the lung demonstrates a large lytic lesion at the left skull base involving the jugular foramen
, carotid canal
, and inner table of mastoid
.*
+
+
+**Metastasis, Skull Base**
+*Axial T1 SPGR C+ shows an enhancing skull base metastatic deposit
replacing much of the jugular foramen through which CNIX-XI transit. Symptoms involving a combination of these nerves warrants careful assessment for a mass in this location.*
+
+
+**Brainstem Tumors, Pediatric**
+*Axial T2 FS MR of a child with pilocytic astrocytoma of the medulla shows a heterogeneously T2 hyperintense, exophytic mass
with prominent internal cystic area
. Note marked effacement of the cerebellomedullary cistern
, which contains the cisternal segments of multiple lower cranial nerves.*
+
+
+**Brainstem Tumors, Pediatric**
+*Axial T1 SPGR C+ of a medullary pilocytic astrocytoma shows the mass to be heterogeneously enhancing, including areas of dominant nodular enhancement
bilaterally.*
+
+
+**Squamous Cell Carcinoma, Palatine Tonsil**
+*Axial CECT demonstrates an invasive right palatine tonsil SCCa primary
with extensive extranodal disease in the right CS
, parotid space
, and perivertebral space
.*
+
+
+**Squamous Cell Carcinoma, Palatine Tonsil**
+*Axial CECT shows a right palatine tonsil SCCa primary tumor
spreading posterolaterally into the oropharyngeal CS
where CNIX-XII reside. Note also the massive extranodal SCCa in the right posterior cervical space
.*
+
+
+**Schwannoma, Jugular Foramen**
+*Axial T1 C+ FS MR shows the typical appearance of a schwannoma
with a bilobed configuration and "waist" at the expanded jugular foramen
. Note mass effect upon the upper CS
.*
+
+
+**Schwannoma, Jugular Foramen**
+*Coronal T1 C+ FS MR of a jugular foramen schwannoma
shows the characteristic vector of spread along the CNIX-XI bundle into the expanded jugular foramen
. Note benign remodeling of the jugular tubercle
with maintained dark cortical margin due to the slow-growing mass.*
+
+
+**Cavernous Malformation, Medulla**
+*Axial SWI shows a left dorsal medullary cavernous malformation
with marked susceptibility artifact from prior hemorrhage.*
+
+
+**Dissection, Carotid Artery, Neck**
+*Axial TOF MRA demonstrates a right cervical internal carotid artery (ICA) dissecting pseudoaneurysm with flow-related enhancement extending into both the false lumen
and true lumen
but no flow limitation.*
+
+
+**Paraganglioma (Vagal)**
+*Axial T1 C+ FS MR of a vagal paraganglioma shows an enhancing CS mass
2 cm below skull base displacing the ICA anteromedially
. Note multiple high-velocity flow voids
at the periphery, typical of paraganglioma.*
+
+
+**Paraganglioma (Vagal)**
+*Coronal 68-Ga DOTANOC PET/CT shows marked uptake in a large vagal paraganglioma
and a smaller carotid body tumor
. 68-Ga DOTANOC PET/CT is more sensitive than CT or MR for detecting paragangliomas, especially for identifying synchronous disease.*
+
+
+**Schwannoma, Carotid Space**
+*Axial T1 C+ FS MR shows a large CS schwannoma
, which enhances heterogeneously with internal nonenhancing cysts
. The mass splays the ICA
away from the internal jugular vein
, suggestive of vagal schwannoma.*
+
+
+**Ependymoma, Basal Cistern**
+*Axial T2 MR shows a large ependymoma
extending from the 4th ventricle lateral aperture into the cerebellomedullary cistern. Note mass effect upon the cerebellum and medulla with cerebellar vasogenic edema
. Dark signal within the mass was due to hemorrhage.*
+
+
+**Multiple Sclerosis, Medulla**
+*Axial T2 MR shows a lateral medullary focus
of multiple sclerosis. The CNIX-XI bundle
emerging from the postolivary sulcus is just visible within the basal cistern high-signal CSF.*
+
+
+**Meningioma, Jugular Foramen**
+*Axial T1 C+ FS MR shows a large right posterior fossa meningioma
that extends through the jugular foramen
and into the upper CS
. Note the extraaxial location of this avidly enhancing mass, which demonstrates conspicuous dural tails of enhancement
.*
+
+
+**Chondrosarcoma, Skull Base**
+*Axial bone CT shows a chondrosarcoma
centered at the petrooccipital fissure with expansile, erosive bone changes, including erosion into the jugular foramen
. Scant matrix mineralization
is present, a finding seen in approximately half of head and neck chondrosarcomas.*
+
+
+**Schwannoma, Hypoglossal Nerve**
+*Coronal T1 C+ FS MR shows a hypoglossal schwannoma
within an enlarged hypoglossal canal. The tumor encroaches upon the superolateral jugular foramen
. Note multiple intramural cysts, commonly seen with larger schwannomas.*
+
+
+### Additional Images
+
+
+**Paraganglioma (Jugular)**
+*Axial bone CT reveals a jugular foramen permeative-destructive lesion
with soft tissue evident in the middle ear cavity
. Note opposite normal corticated jugular foramen bony margins
.*
+
+
+**Paraganglioma (Jugular)**
+*Coronal T1 C+ FS MR shows the inferior portion of a large jugular paraganglioma involving the basal cistern
, area of hypoglossal canal
, and nasopharyngeal CS
.*
+
+
+**Paraganglioma (Jugular)**
+*Coronal T1 C+ FS MR depicts enhancing jugular paraganglioma filling the left jugular foramen, extending superolaterally into middle ear cavity
and medially into the hypoglossal canal
.*
+
+
+**Paraganglioma (Jugular)**
+*Axial bone CT shows characteristic permeative-destructive bone changes
and superolateral vector of spread into the middle ear
of a jugular paraganglioma. In contrast, note the crisp cortical margins of the normal right jugular foramen
.*
+
+
+**Paraganglioma (Jugular)**
+*Axial T1 C+ FS MR of a jugular paraganglioma
shows typical avid contrast enhancement and high-velocity flow voids
seen with paragangliomas in the head and neck. The mass is centered at the jugular foramen abutting the ICA
.*
+
+
+**Nasopharyngeal Carcinoma**
+*Axial CECT of nasopharyngeal carcinoma shows a bulky mass
centered in the lateral nasopharyngeal recess. This mass has invaded the nasopharyngeal carotid space
, which contains CNIX-XII at this level. Note the maintained CS tissue planes
on the contralateral side for comparison.*
+
+
+**Nasopharyngeal Carcinoma**
+*Axial T2 FS MR shows invasive nasopharyngeal carcinoma
and retropharyngeal adenopathy
that exert mass effect upon the effaced CS
. Note mastoid effusion due to obstructed eustachian tube.*
+
+
+**Nasopharyngeal Carcinoma**
+*Axial CECT shows an invasive carcinoma in the right lateral nasopharyngeal recess
. Notice that the CS (containing CNIX-XII at this level) has lost its distinct soft tissue planes
as a result of tumor invasion.*
+
+
+**Nasopharyngeal Carcinoma**
+*Axial CECT demonstrates the inferior aspect of a nasopharyngeal carcinoma
with invasion of the CS
deep to the styloid process
.*
+
+
+**Cerebral Ischemia-Infarction, Acute, Posterior Inferior Cerebellar Artery**
+*Axial DWI MR shows high signal (reduced diffusivity)
of the left lateral medulla in the area of the lower cranial nerve nuclei. An ADC map image (not shown) revealed a matching low signal, indicating that this brainstem stroke is acute (likely < 7-10 days in age).*
+
+
+**Metastasis, Skull Base**
+*Coronal bone CT demonstrates abnormal lucent areas
along the margin of the jugular foramen, indicating a widely invasive lesion. Metastatic tumor was the final tissue diagnosis.*
+
+
+**Metastasis, Skull Base**
+*Axial T1 C+ MR shows the left jugular foramen is filled with enhancing tumor, which in this case is metastatic carcinoma
. Notice the metastatic tumor also involves the meninges along the adjacent posterior fossa
.*
+
+
+**Brainstem Tumors, Pediatric**
+*Axial T2 FS MR of a pilocytic astrocytoma (WHO 1) shows a heterogeneously hyperintense mass
centered in the left posterolateral medulla with cystic component
. Note obliteration of the left cerebellomedullary cistern
through which the lower cranial nerves transit.*
+
+
+**Brainstem Tumors, Pediatric**
+*Axial FLAIR MR in a patient with posterolateral medulla glioma reveals a high signal intensity brainstem mass
. Notice the thickened high signal proximal CNIX-XI cranial nerve bundle
.*
+
+
+**Brainstem Tumors, Pediatric**
+*Axial T2WI MR reveals a small high-signal posterolateral medullary glioma
affecting the area of cranial nerve nuclei XI-XII.*
+
+
+**Schwannoma, Jugular Foramen**
+*Axial T1 C+ MR shows the typical appearance of a schwannoma
. Areas of nonenhancement correspond with intramural cysts
. Note the vector of spread along the CNIX-XI bundle into the expanded jugular foramen
.*
+
+
+**Schwannoma, Jugular Foramen**
+*Axial bone CT shows typical bone changes of a schwannoma. The smooth jugular foramen enlargement with thin, sclerotic margins
can be very helpful for differentiating schwannoma from meningioma or paraganglioma.*
+
+
+**Schwannoma, Jugular Foramen**
+*Axial T1 C+ FS MR in a patient with left vocal cord paralysis shows a well-circumscribed enhancing schwannoma
emerging from the left jugular foramen
.*
+
+
+**Schwannoma, Jugular Foramen**
+*Axial T2 MR reveals a jugular foramen schwannoma
. Notice that the vector of spread of the lesion is along the CNXI-XI bundle
toward the lateral medulla. It is impossible to predict which nerve this lesion arises from based on imaging alone.*
+
+
+**Cavernous Malformation, Medulla**
+*Axial T2 FS MR shows a left medullary cavernous malformation
with a dark rim of hemosiderin from a remote bleed and fluid-fluid level
from a more recent hemorrhage.*
+
+
+**Cavernous Malformation, Medulla**
+*Axial T1 C+ FS MR demonstrates a partially enhancing left medullary cavernous malformation
with associated developmental venous anomaly
, a so-called mixed vascular malformation. A GRE image (not shown) revealed significant blooming of the lesion, indicating previous hemorrhage. Enhancement in a cavernous malformation is uncommon but can be seen.*
+
+
+**Metastases, Meningeal**
+*Coronal T1 C+ MR shows extensive carcinomatosis of the posterior fossa meninges
. Note the metastatic tumor has entered the ICAs
.*
+
+
+**Dissection, Carotid Artery, Neck**
+*Axial 3D TOF MRA shows a left cervical ICA dissecting pseudoaneurysm
. There is maintained flow-related enhancement in the true ICA lumen
without flow-limiting stenosis.*
+
+
+**Dissection, Carotid Artery, Neck**
+*Axial MRA source image shows residual lumen of ICA dissection
in association with a pseudoaneurysm
.*
+
+
+**Paraganglioma (Vagal)**
+*Coronal T1 C+ FS MR reveals an ovoid, avidly enhancing CS mass
with a few sporadic high-velocity flow voids
visible. Note the lesion is centered above the carotid bifurcation and does not reach the skull base (jugular foramen), as is characteristic of vagal paraganglioma.*
+
+
+**Schwannoma, Carotid Space**
+*Axial T1 C+ FS MR of a CS schwannoma
shows a circumscribed, avidly enhancing mass with internal nonenhancing cysts
and typical vascular displacement pattern. Lack of tumor-associated flow voids helps differentiate from paraganglioma.*
+
+
+**Schwannoma, Carotid Space**
+*Sagittal CECT demonstrates a lenticular-shaped schwannoma with multiple intramural cysts
. The superior margin "points"
toward the jugular foramen.*
+
+
+**Meningioma, Jugular Foramen**
+*Coronal SPGR C+ shows enhancing meningioma
with extension through jugular foramen
and hypoglossal canal
into the nasopharyngeal CS
(where CNIX-XII reside). Note intraosseous tumor and hyperostosis of the jugular tubercle
, which can help differentiate meningioma from paraganglioma or schwannoma.*
+
+
+**Meningioma, Jugular Foramen**
+*Axial T1 C+ MR reveals a jugular foramen meningioma projecting into the basal cistern
, involving both the lateral clival bone marrow
and the nasopharyngeal CS
.*
+
+
+**Chondrosarcoma, Skull Base**
+*Axial T2WI FS MR demonstrates the inferior portion of a high-signal intensity petrooccipital fissure chondrosarcoma. Note the tumor invasion of the clival marrow space
and the jugular foramen
.*
+
+
+**Cerebral Ischemia-Infarction, Acute, Posterior Inferior Cerebellar Artery**
+*Axial graphic shows the vagal nuclei in the dorsolateral medulla
. Note the proximity of the glossopharyngeal
, vagal
, and spinal accessory
cranial nerves in the basal cistern. Lateral medullary infarction may cause complex cranial neuropathy in addition to long tract signs.*
+
+
+**Nasopharyngeal Carcinoma**
+*Axial graphic shows the nasopharyngeal CS, which contains the hypoglossal
, vagus
, spinal accessory
, and glossopharyngeal
nerves. Nasopharyngeal carcinoma commonly invades the CS, causing complex cranial neuropathy involving these nerves.*
+
diff --git a/docs_md/articles/creutzfeldt-jakob-disease-cjd_30a88a01-b24d-476d-a933-48aabcdb6f95.md b/docs_md/articles/creutzfeldt-jakob-disease-cjd_30a88a01-b24d-476d-a933-48aabcdb6f95.md
new file mode 100644
index 0000000..a5b7f93
--- /dev/null
+++ b/docs_md/articles/creutzfeldt-jakob-disease-cjd_30a88a01-b24d-476d-a933-48aabcdb6f95.md
@@ -0,0 +1,575 @@
+---
+title: "Creutzfeldt-Jakob Disease (CJD)"
+docid: "30a88a01-b24d-476d-a933-48aabcdb6f95"
+authors:
+ - key: "1fa14dfd-71ea-4960-908e-e720313bc63a"
+ value: "Santhosh Gaddikeri, MD"
+ - key: "a25c450b-3d34-4f64-bba3-cc0834813df6"
+ value: "Miral D. Jhaveri, MD, MBA"
+breadcrumbs:
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+ name: "Brain"
+ slug: "brain"
+ treeNodeId: "0361042c-9c90-48e4-864e-a2e6428ee9c9"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
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+ slug: "dementias-and-degenerative-disorde-"
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+ name: "Creutzfeldt-Jakob Disease (CJD)"
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+lastUpdated: "08/19/25"
+pageDescription: "Creutzfeldt-Jakob Disease (CJD)"
+pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Acquired Toxic/Metabolic/Degenerative Disorders, Dementias and Degenerative Disorders, Creutzfeldt-Jakob Disease (CJD)"
+pageTitle: "Creutzfeldt-Jakob Disease (CJD) | STATdx"
+enhancedTitle: "Creutzfeldt-Jakob Disease (CJD)"
+type: "DX"
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+breadcrumbs:
+ - "Brain"
+ - "Diagnosis"
+ - "Pathology-Based Diagnoses"
+ - "Acquired Toxic/Metabolic/Degenerative Disorders"
+ - "Dementias and Degenerative Disorders"
+ - "Creutzfeldt-Jakob Disease (CJD)"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Creutzfeldt-Jakob disease (CJD): Rapidly progressing, fatal, potentially transmissible dementia caused by prion
+- ### Imaging
+
+
+ - Best imaging clue: Progressive DWI/FLAIR hyperintensity of basal ganglia (BG), thalamus, and cerebral cortex
+ - Predominantly gray matter (GM): Caudate and putamen > globus pallidus (GP)
+ - Thalamus: Common in variant CJD (vCJD)
+ - Cerebral cortex: Frontal, parietal, and temporal
+ - Heidenhain variant: Occipital lobe
+ - 2 signs seen in 90% of vCJD but can also occur in sporadic CJD (sCJD)
+ - Pulvinar sign: Symmetric T2 hyperintensity of pulvinar of thalamus
+ - Hockey stick sign: Symmetric pulvinar and dorsomedial thalamic nuclear hyperintensity
+ - Best imaging tool: MR with DWI
+- ### Top Differential Diagnoses
+
+
+ - Hypoxic-ischemic injury
+ - Osmotic demyelination syndrome
+ - Other causes of dementia
+ - Alzheimer, frontotemporal, and multiinfarct dementia; dementia in motor neuron disease
+ - Leigh syndrome
+ - Corticobasal degeneration
+- ### Clinical Issues
+
+
+ - Definite CJD diagnosed by neuropathology
+ - Progressive dementia associated with myoclonic jerks and akinetic mutism; variable constellation of pyramidal, extrapyramidal, and cerebellar signs
+ - CSF protein biomarkers: 14-3-3 protein, total tau (t-tau), S100, and neuron-specific enolase (NSE)
+ - DWI MR has higher diagnostic accuracy, 97% more than any or all of these CSF biomarkers
+ - Incidence 1 per 1,000,000 (USA and internationally)
+ - sCJD (85%), familial (15%), infectious/iatrogenic (< 1%) (includes vCJD)
+ - Death usually ensues within months of onset
+
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - Creutzfeldt-Jakob disease (CJD)
+ - Sporadic Creutzfeldt-Jakob disease (sCJD)
+ - Variant Creutzfeldt-Jakob disease (vCJD)
+- ### Definitions
+
+
+ - Rapidly progressing, fatal, neurodegenerative disorder caused by prion (proteinaceous infectious particle devoid of DNA and RNA)
+ - Transmissible spongiform encephalopathy
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Progressive T2 hyperintensity of basal ganglia (BG), thalamus, and cerebral cortex
+ - #### Location
+
+
+ - Predominantly gray matter (GM)
+ - BG: Caudate and putamen > globus pallidus (GP)
+ - Thalamus (common in vCJD)
+ - Cerebral cortex (most commonly frontal, parietal, and temporal lobes)
+ - Cortical involvement often asymmetric
+ - Heidenhain variant: Occipital lobe
+ - Brownell-Oppenheimer: Cerebellum
+ - May involve only peripheral cortex
+ - Cortical involvement often asymmetric
+ - Primary sensorimotor cortex relatively spared
+ - White matter (WM) usually not involved
+ - Size: Slight decrease (atrophy)
+ - Morphology: Hyperintense T2 signal conforms to outline of BG and gyriform pattern in cortex
+- ### CT Findings
+
+
+ - NECT: Usually normal
+ - May show rapidly progressive atrophy and ventricular dilatation on serial CT
+ - Serial CT illustrates atrophy progression
+- ### MR Findings
+
+
+ - #### T1WI
+
+
+ - Normal
+ - GP hyperintensity reported in sCJD
+ - #### T2WI
+
+
+ - Hyperintense signal in BG, thalami, cortex
+ - Cerebral atrophy
+ - With time, hyperintense foci may develop in WM
+ - #### FLAIR
+
+
+ - 2 signs seen in 90% of vCJD but can also occur in sCJD
+ - **Pulvinar**sign: Bilateral symmetric hyperintensity of **pulvinar** (posterior) nuclei of **thalamus**
+ - **Hockey stick** sign: Symmetric **pulvinar and dorsomedial thalamic** nuclear hyperintensity
+ - Periaqueductal GM hyperintensity
+ - Cortical hyperintensity (common in sCJD)
+ - #### DWI
+
+
+ - Progressive hyperintensity in striatum and cortex
+ - Gyriform hyperintense areas in cerebral cortex (**cortical ribbon sign**)
+ - Correspond to localization of periodic sharp wave complexes on EEG
+ - DWI hyperintensity may disappear late in disease
+ - T1WI C+: No abnormal enhancement
+- ### Nuclear Medicine Findings
+
+
+ - F-18 FDG PET: Regional glucose hypometabolism correlates with sites of neuropathologic lesions
+ - SPECT with N-isopropyl-p-(I-123) iodoamphetamine (DaTSCAN)
+ - ↓ uptake of tracer in BG reported
+ - Sometimes in asymmetrical pattern
+- ### Imaging Recommendations
+
+
+ - Best imaging tool: MR with DWI and FLAIR
+
+## DIFFERENTIAL DIAGNOSIS
+
+- [Hypoxic-Ischemic Injury](/document/adult-hypoxic-ischemic-injury/91ac293f-161c-4b3b-81e5-740f831eaa5d)
+ - BG and parasagittal cortical areas involved
+ - Hyperintense BG lesions on T1WI and T2WI
+ - DWI + symmetric GM involvement
+- [Osmotic Demyelination Syndrome](/document/osmotic-demyelination-syndrome/2f646ac5-9994-4bc0-a7ff-f0103334a366)
+ - Extrapontine: T2-hyperintense putamen and caudate
+ - DWI positive acutely
+- ### Leigh Syndrome
+
+
+ - Primarily seen in pediatric patients
+ - T2 hyperintensity in putamen and GP
+- [Other Causes of Dementia](/document/alzheimer-disease/aa75d198-88b8-45c2-8a35-750631009166)
+ - [Alzheimer disease](/document/alzheimer-disease/aa75d198-88b8-45c2-8a35-750631009166)
+ - [Dementia in motor neuron disease](/document/amyotrophic-lateral-sclerosis-als/cef8f538-52a0-4590-b14d-3578e9214330)
+ - [Frontotemporal dementia](/document/frontotemporal-lobar-degeneration/596be24d-885f-40ed-832e-2de041f974f5)
+ - [Multiinfarct dementia](/document/vascular-dementia/20864adf-5e46-4fbf-ba27-e8e30fa6506f)
+- [Corticobasal Degeneration](/document/corticobasal-degeneration/99473ccd-173f-4ed5-9b92-e52d5630a8a9)
+ - Neuronal loss in substantia nigra, frontoparietal cortex, and striatum (BG atrophy may be subtle)
+ - Symmetric/asymmetric atrophy of pre- and postcentral gyri; prominent parasagittal involvement
+ - Subcortical gliosis: High intensity on T2WI
+- [Wilson Disease](/document/wilson-disease/b89eef10-ea47-4ca9-a3b7-b8aeeca86802)
+ - WM and deep GM lesions (BG, dentate nucleus, brainstem); variably T2 hyperintense
+ - T1-hypointense (rarely hyperintense) lesions
+- [Arteriolosclerosis](/document/arteriolosclerosis/07e561a5-0554-4867-b811-448c36890ee3)
+ - BG involvement: Typically asymmetric and multifocal (rather than diffuse as in CJD)
+ - Focal hyperintensities in deep WM
+ - DWI negative, unless acute
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Etiology
+
+
+ - Prion protein is misfolded isoform (PrPSc) of normal host-encoded protein (PrPc)
+ - PrPSc = conformationally isomer of PrPc
+ - PrPSc introduced into healthy cells → initiates self-perpetuating vicious cycle: PrPc → PrPSc → neurotoxicity
+ - sCJD: Spontaneous PrPc → PrPSc or somatic mutation
+ - Familial CJD (fCJD): Mutations in *PRNP*gene
+ - Iatrogenic CJD: Infection from prion-containing material
+ - Surgical instruments, dura mater grafts, stereotactic electrodes
+ - Cadaveric corneal transplants, human pituitary hormones (growth hormone and gonadotropins)
+ - vCJD: Bovine spongiform encephalopathy in cattle is transmitted to humans through infected beef
+ - Primarily present in UK
+ - a.k.a. new variant CJD (nvCJD)
+ - Risk for health care workers
+ - Physical contact with patients is no risk for transmission
+ - Special precautions in handling brain tissue
+ - All used materials and instruments decontaminated as per established protocols
+ - #### Genetics
+
+
+ - Can be inherited, sporadic, or acquired (infectious)
+ - 10-15% of human prion disease cases associated with dominant mutations in autosomal prion protein (PrPc) gene (*PRNP*) on chromosome 20
+ - PrPc is normal host protein on surface of many cells, particularly neurons
+ - #### Associated abnormalities
+
+
+ - EEG: Periodic (high-voltage) sharp wave complexes (PSWCs) on background of low-voltage activity
+ - 67-95% patients with sCJD show PSWCs at some point during course of illness
+ - False-positive EEG findings in Alzheimer dementia and vascular dementia patients
+ - PSWCs helpful in differentiating sCJD from other prion disease
+- ### Staging, Grading, & Classification
+
+
+ - Sporadic CJD
+ - Definite
+ - Characteristic neuropathology (biopsy or autopsy)
+ - Protease-resistant PrPSc (PrPres) by Western blot
+ - Probable
+ - Neuropsychiatric disorder with positive RT-QuIC in CSF or other tissues
+ - OR
+ - Rapidly progressive dementia and at least 2 out of 4 clinical features listed in table 1
+ - AND positive result on at least 1 of 3 lab tests listed in table 1
+ - AND without routine investigations indicating alternative diagnosis
+ - Possible
+ - Progressive dementia and at least 2 out of 4 clinical features listed in table 1
+ - AND absence of positive lab tests that would classify case as "probable"
+ - AND duration of illness < 2 years
+ - AND without routine investigations indicating alternative diagnosis
+ - Iatrogenic CJD: Progressive cerebellar syndrome in recipient of human cadaveric-derived pituitary hormone; or sporadic CJD with recognized exposure risk, e.g., antecedent neurosurgery with dura mater implantation
+ - Familial CJD: Definite or probable CJD **with** definite or probable CJD in 1st-degree relative; &/or neuropsychiatric disorder **with** disease-specific PrP gene mutation
+- ### Gross Pathologic & Surgical Features
+
+
+ - Mild cortical atrophy
+ - Diffuse or confined to affected structures
+ - Ventricular enlargement
+- ### Microscopic Features
+
+
+ - Spongiform encephalopathy: GM most affected
+ - Marked neuronal loss with reactive astrocytosis
+ - Replacement gliosis
+ - Neuronal vacuolation with spongiform changes
+ - Spongiform panencephalopathy (very rare)
+ - Primary extensive involvement of WM
+ - Loss of myelin and axons associated with generalized spongiform change in WM
+ - ± diffuse cerebral atrophy, loss of neurons, and proliferation of astrocytes in cerebral cortex
+ - 10% of patients with CJD have amyloid plaques in cerebellum or cerebral hemispheres
+ - Apple-green birefringence using Congo red staining when viewed under polarized light
+ - Variable accumulation of PrPSc in brain tissue
+ - PrPSc = abnormal, insoluble, protease-resistant amyloid form of PrPc
+ - Diffuse (common in sCJD) or discrete plaques
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - Rapidly progressive dementia associated with myoclonic jerks and akinetic mutism
+ - Variable constellation of pyramidal, extrapyramidal, and cerebellar signs
+ - #### Clinical profile
+
+
+ - **sCJD**: Cerebellar dysfunction, rapidly progressive cognitive impairment, both
+ - 6 molecular subtypes: MM1, MM2 (thalamic and cortical), MV1, MV2, VV1, and VV2
+ - Vary with respect to age at onset, disease duration, early symptoms, and neuropathology
+ - **vCJD**: Psychiatric and sensory symptoms
+ - **Heidenhain variant** of CJD
+ - Isolated visual signs/symptoms (initially)
+ - Predominantly occipital lobe degeneration
+ - Normal conventional T1 and T2WI of brain
+ - DWI/FLAIR may detect early cortical abnormalities
+ - **Brownell-Oppenheimer**: Cerebellar signs/symptoms
+ - Extrapyramidal type of CJD
+ - May show ↑ signal intensity in BG
+ - Pyramidal involvement with disease progression
+ - BG dysfunction
+ - Spinal cord involvement → muscle atrophy and fasciculations
+ - **CSF studies**
+ - CSF protein biomarkers: 14-3-3 protein, total tau (t-tau), S100, neuron-specific enolase (NSE), and thymosin β4
+ - 14-3-3 protein detection is adjunctive rather than diagnostic for prior disease
+ - t-tau > 1,150 picogram/mL has superior accuracy and specificity than 14-3-3 protein for CJD
+ - Significant false-positives and negatives with 14-3-3 and t-tau protein test results
+ - DWI MR has higher diagnostic accuracy than any or all CSF biomarkers
+ - Real-time quaking-induced conversion (RT-QUIC) testing of CSF to detected PrPsc
+ - More sensitive using olfactory epithelium (nasal brushing) than CSF
+ - Nasal brushing not performed in USA
+- ### Demographics
+
+
+ - #### Age
+
+
+ - Younger in vCJD, older in sCJD (6th-7th decades)
+ - #### Sex
+
+
+ - No sex preponderance
+ - #### Ethnicity
+
+
+ - sCJD occurs throughout world, in all races
+ - In USA, CJD ↓ in Black, American Indian, and Alaskan native populations than White populations
+ - vCJD limited to Europe (nearly all cases in UK)
+ - #### Epidemiology
+
+
+ - Incidence 1.0-1.5 per million in USA
+ - sCJD (85-95%), familial (5-15%), infectious/iatrogenic (< 1%)
+- ### Natural History & Prognosis
+
+
+ - Long incubation period but rapidly progressive once clinical symptoms begin
+ - Rapidly progressing dementia with death usually ensuing within months of onset
+ - Median survival from time of onset of symptoms to death is 4.5 months
+ - 90% live < 1 year
+- ### Treatment
+
+
+ - No effective treatment
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - Heidenhain variant of CJD in patients with visual disorders of unclear origin and dementia
+- ### Image Interpretation Pearls
+
+
+ - Conventional radiologic criteria for diagnosis of sCJD: FLAIR or DWI high signal changes in ≥ 2 cerebral cortical region (excluding frontal lobes) or in both caudate and putamen
+
+ 7435e050-edc1-415d-8aec-fdbbe9708ca6
+
+## References
+
+## Selected References
+
+1. [Barber D et al: Assessing the newly proposed MRI criteria for diagnosing sporadic Creutzfeldt-Jakob disease. Neuroradiology. 66(11):1907-15, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=39136713%5Bpmid%5D)
+1. [Hermann P et al: [Clinical characteristics and diagnostics of human spongiform encephalopathies: an update.] Nervenarzt. 95(4):376-84, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38503894%5Bpmid%5D)
+1. [Huang B et al: Creutzfeldt-Jakob disease presenting as psychiatric disorder: case presentation and systematic review. Front Neurol. 15:1428021, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=39268068%5Bpmid%5D)
+1. [Mattoli MV et al: The role of PET imaging in patients with prion disease: a literature review. Mol Imaging Biol. 26(2):195-212, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38302686%5Bpmid%5D)
+1. [Llorens F et al: Plasma total prion protein as a potential biomarker for neurodegenerative dementia: diagnostic accuracy in the spectrum of prion diseases. Neuropathol Appl Neurobiol. 46(3):240-54, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31216593%5Bpmid%5D)
+1. [Alaoui A et al: [MRI role in Creutzfeldt-Jakob disease: about a case.] Pan Afr Med J. 32:95, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31223386%5Bpmid%5D)
+1. [Baldwin KJ et al: Prion disease. Semin Neurol. 39(4):428-39, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31533183%5Bpmid%5D)
+1. [Groveman BR et al: Sporadic Creutzfeldt-Jakob disease prion infection of human cerebral organoids. Acta Neuropathol Commun. 7(1):12, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31196223%5Bpmid%5D)
+1. [Hayashi Y et al: Clinicopathological findings of an MM2-cortical-type sporadic Creutzfeldt-Jakob disease patient with cortical blindness during a course of glaucoma and age-related macular degeneration. Prion. 13(1):124-31, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31219399%5Bpmid%5D)
+1. [Muniz BC et al: The Heidenhain variant of Creutzfeldt-Jakob disease. Radiol Bras. 52(3):199-200, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31210697%5Bpmid%5D)
+1. [Xu Y et al: Sporadic Creutzfeldt-Jakob disease presenting as dizziness and cognitive decline: a case report. Medicine (Baltimore). 98(24):e16002, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31192942%5Bpmid%5D)
+1. [Fragoso DC et al: Imaging of Creutzfeldt-Jakob disease: imaging patterns and their differential diagnosis. Radiographics. 37(1):234-57, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28076012%5Bpmid%5D)
+1. [Koeller KK et al: Viral and prion infections of the central nervous system: radiologic-pathologic correlation: from the radiologic pathology archives. Radiographics. 37(1):199-233, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28076019%5Bpmid%5D)
+1. [Caobelli F et al: The role of neuroimaging in evaluating patients affected by Creutzfeldt-Jakob disease: a systematic review of the literature. J Neuroimaging. 25(1):2-13, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=24593302%5Bpmid%5D)
+1. [Kim MO et al: Clinical update of Jakob-Creutzfeldt disease. Curr Opin Neurol. 28(3):302-10, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25923128%5Bpmid%5D)
+1. [Felix-Morais R et al: Creutzfeldt-Jakob disease: typical imaging findings. BMJ Case Rep. 2014:bcr2014203997, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24682142%5Bpmid%5D)
+1. [Risacher SL et al: Neuroimaging biomarkers of neurodegenerative diseases and dementia. Semin Neurol. 33(4):386-416, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24234359%5Bpmid%5D)
+1. [Appleby BS et al: Characteristics of established and proposed sporadic Creutzfeldt-Jakob disease variants. Arch Neurol. 66(2):208-15, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19204157%5Bpmid%5D)
+1. [Iwasaki Y et al: Clinical diagnosis of Creutzfeldt-Jakob disease: accuracy based on analysis of autopsy-confirmed cases. J Neurol Sci. 277(1-2):119-23, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19056094%5Bpmid%5D)
+1. [Josephs KA et al: Rapidly progressive neurodegenerative dementias. Arch Neurol. 66(2):201-7, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19204156%5Bpmid%5D)
+1. [Manners DN et al: Pathologic correlates of diffusion MRI changes in Creutzfeldt-Jakob disease. Neurology. 72(16):1425-31, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19380702%5Bpmid%5D)
+1. [Meissner B et al: MRI lesion profiles in sporadic Creutzfeldt-Jakob disease. Neurology. 72(23):1994-2001, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19506221%5Bpmid%5D)
+1. [Clarençon F et al: MRI and FDG PET/CT findings in a case of probable Heidenhain variant Creutzfeldt-Jakob disease. J Neuroradiol. 35(4):240-3, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18466976%5Bpmid%5D)
+1. [Fulbright RK et al: MR imaging of familial Creutzfeldt-Jakob disease: a blinded and controlled study. AJNR Am J Neuroradiol. 29(9):1638-43, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18635614%5Bpmid%5D)
+1. [Heinemann U et al: Brain biopsy in patients with suspected Creutzfeldt-Jakob disease. J Neurosurg. 109(4):735-41, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18826363%5Bpmid%5D)
+1. [Meissner B et al: Isolated cortical signal increase on MR imaging as a frequent lesion pattern in sporadic Creutzfeldt-Jakob disease. AJNR Am J Neuroradiol. 29(8):1519-24, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18599580%5Bpmid%5D)
+1. [Ward HJ et al: Risk factors for sporadic Creutzfeldt-Jakob disease. Ann Neurol. 63(3):347-54, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18074392%5Bpmid%5D)
+1. [Yi SH et al: Relationship between clinical course and diffusion-weighted MRI findings in sporadic Creutzfeldt-Jakob Disease. Neurol Sci. 29(4):251-5, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18810600%5Bpmid%5D)
+1. [Kallenberg K et al: Creutzfeldt-Jakob disease: comparative analysis of MR imaging sequences. AJNR Am J Neuroradiol. 27(7):1459-62, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16908558%5Bpmid%5D)
+1. [Lin YR et al: Creutzfeldt-jakob disease involvement of rolandic cortex: a quantitative apparent diffusion coefficient evaluation. AJNR Am J Neuroradiol. 27(8):1755-9, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16971630%5Bpmid%5D)
+1. [Tschampa HJ et al: MRI in the diagnosis of sporadic Creutzfeldt-Jakob disease: a study on inter-observer agreement. Brain. 128(Pt 9):2026-33, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15958503%5Bpmid%5D)
+1. [Young GS et al: Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt-Jakob disease: high sensitivity and specificity for diagnosis. AJNR Am J Neuroradiol. 26(6):1551-62, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15956529%5Bpmid%5D)
+1. [Collins SJ et al: Transmissible spongiform encephalopathies. Lancet. 363(9402):51-61, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14723996%5Bpmid%5D)
+1. [Summers DM et al: The pulvinar sign in variant Creutzfeldt-Jakob disease. Arch Neurol. 61(3):446-7, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15023827%5Bpmid%5D)
+
+## Differential diagnosis
+
+### Homonymous Hemianopsia
+DDX:1cdca8f4-95f8-4d19-b28a-19a433d1a624
+
+## Tables
+
+# CDC Diagnostic Criteria for Creutzfeldt-Jakob Disease 2018
+
+| Clinical Features | Lab Tests |
+| --- | --- |
+| Myoclonus | Typical EEG (PSWCs) |
+| Visual or cerebellar signs | 14-3-3 CSF protein positive |
+| Pyramidal/extrapyramidal signs | DWI/FLAIR ↑ signal in caudate/putamen or at least 2 cortical regions (temporal, parietal, occipital) |
+| Akinetic mutism | |
+
+## Anatomy
+
+### Default Mode Network
+Brain/ANATOMY:a29f7551-d39d-4deb-933e-b8d2816168c3
+
+### Basal Ganglia
+Brain/ANATOMY:a9de3815-ec59-4c78-adf0-94974065a7e3
+
+### Thalamus
+Brain/ANATOMY:b7f0cd6b-4ba2-4ed4-8dd0-5ca56d9ae92b
+
+### Language Overview
+Brain/ANATOMY:40f2ed79-0d31-4943-aaa2-7c3244a7e87b
+
+### Functional Network Overview
+Brain/ANATOMY:ef0be4c8-3d36-4ca9-b4c5-f22f66d2b367
+
+### Attention Control Network
+Brain/ANATOMY:a1bedda5-6478-40b2-98e7-6c5f5363b06f
+
+### Visual Network
+Brain/ANATOMY:404625d9-3125-4923-9f9d-53d0f81c3542
+
+### Limbic Network
+Brain/ANATOMY:e1a20b61-b2c1-44c5-ba04-59843855bfef
+
+### Social Brain Anatomy
+Brain/ANATOMY:0352d34a-5966-494e-b9c3-c26bde257bca
+
+### Gyral/Sulcal Anatomy
+Brain/ANATOMY:849da2a0-4a32-4a07-8f00-c69291e59434
+
+### Gyral/Sulcal Anatomy
+Brain/ANATOMY:299a5990-1805-4018-85b5-191d8416385b
+
+### Functional Network Overview
+Brain/ANATOMY:7b97f239-0f6f-4809-ac44-594cdf4842d5
+
+### Brain
+Ultrasound/ANATOMY:080771c2-02f3-408d-ad70-04a80d849500
+
+
+## Images
+
+
+### Selected Images
+
+
+*Axial graphic images show usual findings of sCJD on the right with focal or diffuse symmetric or asymmetric cortical & corpus striatum involvement. Unusual findings of sCJD on the left with involvement of perirolandic cortex, cerebellum & pulvinar/hockey stick sign.*
+
+
+*Axial graphic images show usual findings of sCJD on the right with focal or diffuse symmetric or asymmetric cortical & corpus striatum involvement. Unusual findings of sCJD on the left with involvement of perirolandic cortex, cerebellum & pulvinar/hockey stick sign.*
+
+
+*Axial DWI (top left), ADC (top right), FLAIR (bottom left), & T2 (bottom right) images show hyperintense signal in bilateral caudate & putamen
with signal drop on ADC
. Findings consistent with probable CJD.*
+
+
+*Axial DWI shows symmetric hyperintensity in bilateral caudate & putamen
. Symmetric hyperintense signal involves bilateral pulvinar & dorsomedial thalamic nuclei, indicating hockey stick sign
. Also note asymmetric cortical hyperintensity (cortical ribbon sign) involving bilateral frontal, parietal, occipital lobes
, & insular cortices.*
+
+
+*Axial FLAIR MR (same patient) shows hyperintense signal in corresponding areas as marked in the previous image. Patient was diagnosed with probable vCJD.*
+
+
+*Axial DWI MR shows hyperintense signal involving bilateral pulvinar & dorsomedial nuclei of thalami
.*
+
+
+*Axial FLAIR MR in the same patient shows hyperintense signal involving bilateral pulvinar & dorsomedial nuclei of thalami
. Hockey stick sign is seen in 90% of vCJD, although it is not pathognomonic, as it can be seen with sCJD.*
+
+
+*Axial DWI MR in a 67-year-old man with rapidly progressing dementia shows typical findings of sporadic Creutzfeldt-Jakob disease (sCJD).*
+
+
+*Axial FLAIR MR in the same patient shows corresponding high signal in the caudate nuclei
& putamina
. MR with DWI is the imaging procedure of choice. Conventional radiological criteria for diagnosis of sCJD include FLAIR or DWI high signal changes in ≥ 2 cerebral cortical regions (excluding frontal lobes) or in both the caudate & putamen.*
+
+
+*Axial DWI MR of a 58-year-old man with rapidly progressive dementia, myoclonus, & ataxia due to sCJD Brownell-Oppenheimer clinical phenotype demonstrates diffusion restriction involving the left cerebellum
.*
+
+
+*Axial DWI MR of a 61-year-old woman patient with sCJD Heidenhain clinical phenotype presenting with visual hallucination & optical distortion is shown. Image demonstrates cortical restricted diffusion in bilateral occipital lobes
.*
+
+
+### Additional Images
+
+
+*Axial DWI MR in a different patient shows hyperintense signal consistent with restricted diffusion in right posterior temporal lobe & occipital lobe cortex.*
+
+
+*Axial DWI MR shows hyperintense signal consistent with restricted diffusion within both amygdalae.*
+
+
+*Axial DWI MR demonstrates bright signal of restricted diffusion in bodies of both caudate nuclei.*
+
+
+*Axial FLAIR MR shows bilateral hyperintense signal in putamina & thalami from CJD.*
+
+
+*Coronal FLAIR MR in the same patient with CJD demonstrates hyperintense signal in both thalami.*
+
+
+*Coronal FLAIR MR shows hyperintense signal in caudate nuclei, lentiform nuclei, & within temporal lobe cortices & hippocampi.*
+
+
+*Axial DWI MR shows bilateral restricted diffusion in the putamen & caudate nuclei with small foci in thalami.*
+
+
+*Axial T2WI MR shows bilateral increased signal intensity in putamen & caudate nuclei in a patient with CJD.*
+
+
+*Axial FLAIR MR shows symmetric hyperintensity in the caudate & putamen, characteristic of sCJD. sCJD is the most common type of CJD, representing 85% of cases.*
+
+
+*Axial DWI MR shows asymmetric diffusion restriction in the caudate nuclei & putamen. Involvement of the anterior more than the posterior putamen is typical of CJD. There is also asymmetric hyperintensity in the frontal & temporal lobe cortical ribbons
, typical of sCJD. (Courtesy N. Fischbein, MD.)*
+
+
+*Axial DWI MR shows classic sCJD with diffusion restriction in the caudate & putamen as well as throughout the cortex. Frontal, temporal, & parietal cortical involvement is most common. Relative sparing of the pre- & postcentral gyri is typical of CJD.*
+
+
+*Axial FLAIR MR shows bilateral, symmetric hyperintensities in the posterior thalami representing the "pulvinar" sign, which is characteristic of vCJD. Another "pulvinar" sign is the T1 shortening seen in Fabry disease.*
+
+
+*Axial DWI MR shows symmetric hyperintensity in the BG & thalami bilaterally. The thalamic involvement shows the hockey stick sign, which is symmetric pulvinar & dorsomedial thalamus hyperintensity. This sign is most commonly seen in vCJD but may also be present in sCJD, as in this case.*
+
+
+*Axial DWI MR in a patient with variant CJD (vCJD) demonstrates diffusion restriction in bilateral posteromedial aspect of thalami
, representing hockey stick sign.*
+
+
+*Axial DWI MR in a different patient with vCJD shows diffusion restriction in bilateral posterior aspect of thalami in pulvinar region indicating pulvinar sign
. These signs are more common in vCJD but can occur in sporadic cases.*
+
+
+*A 53-year-old man with rapidly progressing cognitive decline due to sporadic Creutzfeldt-Jakob disease (sCJD) is shown. Axial DWI MR demonstrates symmetric bilateral basal ganglia (BG) diffusion restriction
& asymmetric cortical restricted diffusion (cortical ribbon sign) in bilateral (right > > left) hemispheres
.*
+
+
+*Axial FLAIR MR in the same patient demonstrates hyperintense signal in bilateral BG
& cortex
. CSF was positive for 14-3-3 protein indicating probable sCJD.*
+
diff --git a/docs_md/articles/dural-arteriovenous-fistula_99b59be6-df58-44a9-b14a-be194dfea1c7.md b/docs_md/articles/dural-arteriovenous-fistula_99b59be6-df58-44a9-b14a-be194dfea1c7.md
new file mode 100644
index 0000000..b000031
--- /dev/null
+++ b/docs_md/articles/dural-arteriovenous-fistula_99b59be6-df58-44a9-b14a-be194dfea1c7.md
@@ -0,0 +1,530 @@
+---
+title: "Dural Arteriovenous Fistula"
+docid: "99b59be6-df58-44a9-b14a-be194dfea1c7"
+authors:
+ - key: "de7872b8-cec7-4fd5-a536-c54825607d0f"
+ value: "Ram Vaidhyanath, DMRD, DNB, FRCR"
+ - key: "99f10fe6-7f91-4026-bc60-d769c5d7c7c4"
+ value: "C. Douglas Phillips, MD, FACR"
+breadcrumbs:
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+ name: "Head and Neck"
+ slug: "head-and-neck"
+ treeNodeId: "5c1f8e17-7acd-48d8-9d55-f9f8c2cad850"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "4adbe5f4-083b-4e3e-b7ea-c0ed68a73a6f"
+ -
+ name: "Skull Base Lesions"
+ slug: "skull-base-lesions"
+ treeNodeId: "911e8b09-0b3c-4828-a6b8-455ab653d0c5"
+ -
+ name: "Dural Sinuses"
+ slug: "dural-sinuses"
+ treeNodeId: "a3a077b2-c26a-40b9-9167-817a2eb8df33"
+ -
+ name: "Dural Arteriovenous Fistula"
+ slug: "dural-arteriovenous-fistula"
+ treeNodeId: null
+category: "Head and Neck"
+documentVersionId: "4ff74b5f-fce7-4367-9f49-1f22e4ee8128"
+imageCount: 14
+lastUpdated: "06/26/25"
+pageDescription: "Dural Arteriovenous Fistula"
+pageKeywords: "Head and Neck, Diagnosis, Skull Base Lesions, Dural Sinuses, Dural Arteriovenous Fistula"
+pageTitle: "Dural Arteriovenous Fistula | STATdx"
+enhancedTitle: "Dural Arteriovenous Fistula"
+type: "DX"
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+ - "{'authors': 'Miral D. Jhaveri, MD, MBA; Philip R. Chapman, MD', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/d5939b27-59e9-41a7-bb04-58bb30d22048', 'category': 'Head and Neck', 'compareUrl': '/compare/document/d5939b27-59e9-41a7-bb04-58bb30d22048/related-anatomy/treeNode?subContext=Skull Base Overview', 'documentId': 'd5939b27-59e9-41a7-bb04-58bb30d22048', 'documentType': 'ANATOMY', 'documentUrl': '/document/skull-base-overview/d5939b27-59e9-41a7-bb04-58bb30d22048', 'enhancedTitle': 'Skull Base Overview', 'entryDate': '02/19/24', 'imageCount': 22, 'imageUrl': '/image/thumbnail/7dbd7632-9f35-47a9-a1bf-3711f685d1b5?size=174&quality=85', 'inCompareCart': False, 'rank': 10, 'referenceCount': 0, 'showCompareButton': False, 'title': 'Skull Base Overview'}"
+ - "{'authors': 'Winnie C. W. Chu, MBChB, FRCR; Vivian Y. F. Leung, PhD, RDMS', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/25e08cb1-5308-4ce1-91c1-28cf309cd842', 'category': 'Ultrasound', 'compareUrl': '/compare/document/25e08cb1-5308-4ce1-91c1-28cf309cd842/related-anatomy/treeNode?subContext=Scalp and Calvarial Vault', 'documentId': '25e08cb1-5308-4ce1-91c1-28cf309cd842', 'documentType': 'ANATOMY', 'documentUrl': '/document/scalp-and-calvarial-vault/25e08cb1-5308-4ce1-91c1-28cf309cd842', 'enhancedTitle': 'Scalp and Calvarial Vault', 'entryDate': '10/20/20', 'imageCount': 9, 'imageUrl': '/image/thumbnail/ed3c4b0c-e076-4a7c-8116-3d661a2b2df4?size=174&quality=85', 'inCompareCart': False, 'rank': 11, 'referenceCount': 0, 'showCompareButton': False, 'title': 'Scalp and Calvarial Vault'}"
+ - "{'authors': 'Anne G. Osborn, MD, FACR', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/fa5537f5-50aa-4a7d-abd1-a469b8a8b55f', 'category': 'Brain', 'compareUrl': '/compare/document/fa5537f5-50aa-4a7d-abd1-a469b8a8b55f/related-anatomy/treeNode?subContext=Intracranial Venous System Overview', 'documentId': 'fa5537f5-50aa-4a7d-abd1-a469b8a8b55f', 'documentType': 'ANATOMY', 'documentUrl': '/document/intracranial-venous-system-overview/fa5537f5-50aa-4a7d-abd1-a469b8a8b55f', 'enhancedTitle': 'Intracranial Venous System Overview', 'entryDate': '10/20/20', 'imageCount': 21, 'imageUrl': '/image/thumbnail/b6147a49-e975-4468-947c-61a0a3cd695d?size=174&quality=85', 'inCompareCart': False, 'rank': 12, 'referenceCount': 1, 'showCompareButton': False, 'title': 'Intracranial Venous System Overview'}"
+ - "{'authors': 'Anne G. Osborn, MD, FACR', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/7b5caf92-e4eb-4a23-ba29-45d1dbb1af65', 'category': 'Brain', 'compareUrl': '/compare/document/7b5caf92-e4eb-4a23-ba29-45d1dbb1af65/related-anatomy/treeNode?subContext=Posterior Fossa Veins', 'documentId': '7b5caf92-e4eb-4a23-ba29-45d1dbb1af65', 'documentType': 'ANATOMY', 'documentUrl': '/document/posterior-fossa-veins/7b5caf92-e4eb-4a23-ba29-45d1dbb1af65', 'enhancedTitle': 'Posterior Fossa Veins', 'entryDate': '10/20/20', 'imageCount': 14, 'imageUrl': '/image/thumbnail/014fad61-8985-4877-91c2-7202ddb77553?size=174&quality=85', 'inCompareCart': False, 'rank': 13, 'referenceCount': 2, 'showCompareButton': False, 'title': 'Posterior Fossa Veins'}"
+ - "{'authors': 'Stella Sin Yee Ho, RDMS, RVT, PhD; Deyond Y. W. Siu, MBChB, FRCR; Paula J. Woodward, MD, FSRU', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/914fd68b-ddab-4640-bcc4-883c425d13f8', 'category': 'Ultrasound', 'compareUrl': '/compare/document/914fd68b-ddab-4640-bcc4-883c425d13f8/related-anatomy/treeNode?subContext=Transcranial Doppler', 'documentId': '914fd68b-ddab-4640-bcc4-883c425d13f8', 'documentType': 'ANATOMY', 'documentUrl': '/document/transcranial-doppler/914fd68b-ddab-4640-bcc4-883c425d13f8', 'enhancedTitle': 'Transcranial Doppler', 'entryDate': '10/20/20', 'imageCount': 61, 'imageUrl': '/image/thumbnail/04e86bc1-0497-4d3f-8a6f-09a7e2f77575?size=174&quality=85', 'inCompareCart': False, 'rank': 14, 'referenceCount': 0, 'showCompareButton': False, 'title': 'Transcranial Doppler'}"
+cases: 2
+breadcrumbs:
+ - "Head and Neck"
+ - "Diagnosis"
+ - "Skull Base Lesions"
+ - "Dural Sinuses"
+ - "Dural Arteriovenous Fistula"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Dural arteriovenous fistula (DAVF)
+ - Acquired direct shunt between dural artery and dural venous sinus or cortical vein
+- ### Imaging
+
+
+ - Best imaging modality: DSA
+ - Most common site: Transverse sinus (TS)
+ - CECT findings in DAVF
+ - Tortuous enhancing dural feeders with enlarged dural sinus
+ - Enlarged cortical draining veins → aggressive DAVF
+ - ± flow-related aneurysms
+ - MR findings in DAVF
+ - Localized or generalized venous dilatation
+ - Focal T2 hyperintensity in adjacent white matter (venous congestion)
+ - Dynamic contrast-enhanced MRA and arterial spin-labeling sequences improves detection of intracranial DAVF
+- ### Top Differential Diagnoses
+
+
+ - Hypoplastic TS-sigmoid sinus (TS-SS)
+ - Jugular bulb pseudolesion
+ - Dural sinus thrombosis
+ - Pial arteriovenous malformation
+- ### Clinical Issues
+
+
+ - Accounts for 35% of infratentorial vascular malformations
+ - TS-SS DAVF presents with pulsatile tinnitus
+ - Usually present in middle-aged, older adult patients
+ - Prognosis depends on location, venous drainage pattern
+- ### Diagnostic Checklist
+
+
+ - If patient has objective pulsatile tinnitus and no other vascular lesion on cross-sectional imaging, angiography necessary to completely exclude DAVF
+ - Single pedicle or small DAVF may not be seen on MR or MRA
+ - Newer MRA sequences can help select patients for DSA
+
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - Dural arteriovenous fistula (DAVF)
+- ### Synonyms
+
+
+ - Dural arteriovenous (AV) shunt, dural fistula
+- ### Definitions
+
+
+ - Abnormal acquired direct shunt between dural artery and dural venous sinus or cortical vein
+ - Heterogeneous group of lesions with common angioarchitecture (AV shunts within dura)
+ - Distinct from true AV malformation because most DAVFs are acquired
+ - Exception is vein of Galen malformation
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Network of tiny vessels in wall of thrombosed dural venous sinus
+ - #### Location
+
+
+ - Skull base dural venous sinuses
+ - Most common site → transverse sinus (TS)
+ - 2nd most common site → cavernous sinus (CS)
+ - #### Size
+
+
+ - Variable size, but actual shunt nidus is usually < 2 cm
+ - #### Morphology
+
+
+ - Innumerable, serpiginous AV shunts in wall of dural sinus
+- ### CT Findings
+
+
+ - #### NECT
+
+
+ - Usually normal in cases presenting without hemorrhage
+ - Subarachnoid, subdural, or parenchymal hemorrhage may be seen in cases presenting acutely with hemorrhage
+ - Parenchymal hemorrhage not in typical location for hypertensive bleed
+ - #### CECT
+
+
+ - If small, CECT may be normal
+ - Larger DAVFs show tortuous dural feeders with enlarged dural sinus ± flow-related aneurysms
+ - Dilated vessels in proximity to parenchymal hemorrhage (if present); enlarged, tortuous cortical draining veins
+ - Enlarged superior ophthalmic veins (with carotid cavernous fistula, CCF)
+ - #### Bone CT
+
+
+ - Transosseous collateral channels may be seen in skull base, squamous temporal bone
+ - #### CTA
+
+
+ - May be quite useful in depiction of angioarchitecture
+- ### MR Findings
+
+
+ - #### T1WI
+
+
+ - May be normal
+ - Isointense thrombosed dural sinus ± flow voids
+ - #### T2WI
+
+
+ - Isointense thrombosed sinus ± flow voids
+ - Localized or generalized venous dilatation
+ - Focal T2 hyperintensity in adjacent white matter (venous congestion)
+ - #### FLAIR
+
+
+ - Isointense thrombosed sinus ± adjacent edema if venous congestion or ischemia present
+ - #### T2* GRE
+
+
+ - Usually normal in uncomplicated DAVF
+ - May show parenchymal hemorrhage in DAVF with cortical venous drainage
+ - Thrombosed dural sinus will bloom
+ - #### DWI
+
+
+ - Normal unless venous infarct or ischemia present
+ - #### T1WI C+
+
+
+ - Chronically thrombosed sinus enhances intensely
+ - Rare: Diffuse dural enhancement, parenchymal enhancement
+ - #### MRA
+
+
+ - Time-resolved contrast-enhanced MRA useful for depiction of angioarchitecture and dynamics
+ - TOF MRA positive in larger DAVF
+ - May be negative with small or slow-flow shunts
+ - May yield incomplete depiction of high-flow lesions
+ - Arterial spin-labeled imaging may be sensitive examination
+ - #### MRV
+
+
+ - Occluded involved sinus, collateral flow
+ - 3D phase-contrast MRA with low-velocity encoding can identify fistula, feeding arteries, flow reversal in draining veins
+- ### Angiographic Findings
+
+
+ - Conventional
+ - Most common site = wall of TS or sigmoid sinus (SS) (35-40%)
+ - Multiple arterial feeders are typical with dural/transosseous branches from external carotid artery (ECA), most commonly followed by internal carotid artery (ICA) and vertebral artery tentorial/dural branches
+ - Arterial inflow into parallel venous channel common
+ - Involved dural sinus often thrombosed
+ - Flow reversal in dural sinus/cortical veins correlates with ↑ symptoms, hemorrhage risk
+ - Tortuous engorged pial veins with venous congestion/hypertension (clinically aggressive)
+ - High flow may result in high-flow vasculopathy with progressive stenoses, outlet occlusion, bizarre vascular appearance
+ - CCF = 2nd most common site; classified on basis of arterial supply + venous drainage pattern, Barrow types
+ - Type A: Direct ICA-CS high-flow shunt (not true DAVF)
+ - Type B: Dural ICA branches-cavernous shunt
+ - Type C: Dural ECA-cavernous shunt
+ - Type D: ECA/ICA dural branches shunt to CS
+- ### Imaging Recommendations
+
+
+ - #### Best imaging tool
+
+
+ - DSA with superselective catheterization of involved dural supply
+ - Delineates vascular supply and venous drainage
+ - #### Protocol advice
+
+
+ - Screening MR and enhanced MRA
+ - Evaluate for other nonvascular causes of pulsatile tinnitus
+
+## DIFFERENTIAL DIAGNOSIS
+
+- ### Dural Sinus Hypoplasia-Aplasia
+
+
+ - Congenitally small TS-SS may have low flow on MRV, no enhancement on T1 C+ MR
+ - Sagittal T1WI shows no or very small sinus in normal anatomic location
+ - No signal abnormalities on T2, FLAIR, or GRE
+- [Sigmoid Sinus-Jugular Bulb Pseudolesion](/document/jugular-bulb-pseudolesion/2bf19ff5-bfee-4764-9e2d-cea04ccce4ea)
+ - Slow or asymmetric flow creates variable signal on MR sequences; use MRV to clarify
+- [Thrombosed Dural Sinus](/document/skull-base-dural-sinus-thrombosis/9eb7992b-1296-4a96-83b5-b6b96160ec0a)
+ - Collateral/congested venous drainage can mimic DAVF
+ - Can be spontaneous, traumatic, infectious (thrombophlebitis)
+- [Pial Arteriovenous Malformation](/document/arteriovenous-malformation/84792183-949d-4b25-a586-572dbe2e11a2)
+ - Congenital lesion with intraaxial nidus
+ - Pial arterial supply with possible parasitization of dural supply
+- ### Subdural Hematoma
+
+
+ - Clot beside dural sinus mimics clot in dural sinus
+ - False empty delta sign
+ - MRV shows dural sinus flow
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Etiology
+
+
+ - Adult DAVFs are usually **acquired**, not congenital
+ - May be idiopathic
+ - Can occur in response to **trauma**, **craniotomy**, **venous occlusion**, or **venous hypertension**
+ - Pathological activation of neoangiogenesis
+ - Proliferating capillaries within granulation tissue in dural sinus obliterated by organized thrombi
+ - Budding/proliferation of microvascular network in inner dura connects to plexus of thin-walled venous channels, creating microfistulae
+ - #### Genetics
+
+
+ - High FGF2, VEGFA expression in DAVFs
+ - #### Associated abnormalities
+
+
+ - Cortical drainage may lead to edema, encephalopathy, hemorrhage
+- ### Staging, Grading, & Classification
+
+
+ - **Cognard classification** of intracranial DAVFs correlates venous drainage pattern with clinical course
+ - Type I: Located in sinus wall; normal antegrade venous drainage; benign clinical course
+ - Type IIA: Located in main dural sinus; reflux into sinus but not cortical veins
+ - Type IIB: Reflux (retrograde drainage) into cortical veins; 10-20% hemorrhage
+ - Type III: Direct cortical drainage; no venous ectasia; 40% hemorrhage
+ - Type IV: Direct cortical drainage; venous ectasia; 65% hemorrhage
+ - Type V: Spinal perimedullary venous drainage; progressive myelopathy
+- ### Gross Pathologic & Surgical Features
+
+
+ - Multiple enlarged dural feeders converge on dural sinus
+- ### Microscopic Features
+
+
+ - Arterialized veins with irregular intimal thickening, variable loss of internal elastic lamina
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - 2 major modes of presentation
+ - Hemorrhage (parenchymal, multicompartmental)
+ - Hemorrhagic complications present acutely in stroke-like manner
+ - Venous hypertension/congestion (pulsatile tinnitus, dementia, seizures, encephalopathy)
+ - Nonhemorrhagic neurological presentations typically have subacute, slowly progressive onset
+ - Symptoms vary with site, type of shunt
+ - TS-SS → pulsatile tinnitus
+ - CS → pulsatile exophthalmos, chemosis, retroorbital pain
+ - Brainstem DAVF → quadriparesis, lower cranial nerve palsies
+ - #### Other signs/symptoms
+
+
+ - Uncommon: Encephalopathic symptoms (venous hypertension, ischemia/thrombosis)
+ - Progressive dementia
+ - More common with superior sagittal sinus DAVF
+ - Rare
+ - Life-threatening congestive heart failure
+ - Usually neonates, infants (vein of Galen malformation)
+ - #### Clinical profile
+
+
+ - Middle-aged patient with pulse-synchronous tinnitus
+- ### Demographics
+
+
+ - #### Age
+
+
+ - DAVFs usually present in middle-aged or older adult patients
+ - #### Epidemiology
+
+
+ - Rare, acquired lesions
+ - Account for 6% of supratentorial and 35% of infratentorial vascular malformations
+ - Account for 10-15% of all cerebrovascular malformations with AV shunting
+ - Patients presenting with hemorrhage may not have other risk factors for bleeding, such as HTN, anticoagulation, falls, alcohol excess
+- ### Natural History & Prognosis
+
+
+ - Prognosis, clinical course depends on location, venous drainage pattern
+ - 98% of DAVFs without retrograde venous drainage have benign course
+ - DAVFs draining into major dural sinus usually follow benign clinical course
+ - DAVFs with retrograde cortical venous drainage have aggressive clinical course
+ - Overall risk of hemorrhage from DAVF = 2% per year (depends on location and hemodynamics)
+ - Presence of secondary venous varix is strong predictor of future hemorrhage
+ - Spontaneous closure rare
+ - Acute deterioration has been reported after lumbar puncture
+- ### Treatment
+
+
+ - Observation in selected cases
+ - Treatment options if hemorrhage risk exists
+ - Endovascular → embolization
+ - Surgical resection → skeletonization of involved sinus
+ - Stereotactic radiosurgery
+ - Recurrences common
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - DAVFs are rare but treatable, so consider in patient with hemorrhage in atypical location for hypertensive bleed and no other cause
+ - If patient has objective pulsatile tinnitus and no other vascular lesion on cross-sectional imaging, DSA necessary to completely exclude DAVF
+- ### Image Interpretation Pearls
+
+
+ - Single pedicle or small DAVF may not be seen on MR or MRA
+ - Venous collateral flow in dural sinus thrombosis can become very prominent and mimic DAVF
+- ### Reporting Tips
+
+
+ - Evaluate both ICA/ECA and vertebral arteries when performing angiography in patient with spontaneous intracranial hemorrhage
+ - Identification of associated venous varix is important, as this finding signals ↑ risk of hemorrhage
+
+ ba94365c-71f6-4c20-925d-34c55f6eb15d
+
+## References
+
+## Selected References
+
+1. [Qedair J et al: Dural arteriovenous fistulas at the craniocervical junction: a systematic review and meta-analysis. Neurosurg Rev. 47(1):812, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=39441455%5Bpmid%5D)
+1. [Alkhaibary A et al: Intracranial dural arteriovenous fistula: a comprehensive review of the history, management, and future prospective. Acta Neurol Belg. 123(2):359-66, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36374476%5Bpmid%5D)
+1. [Chen X et al: Overview of multimodal MRI of intracranial dural arteriovenous fistulas. J Interv Med. 5(4):173-9, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36532312%5Bpmid%5D)
+1. [Dissaux B et al: Assessment of 4D MR angiography at 3T compared with DSA for the follow-up of embolized brain dural arteriovenous fistula: a dual-center study. AJNR Am J Neuroradiol. 42(2):340-6, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=33334853%5Bpmid%5D)
+1. [Grossberg JA et al: The use of contrast-enhanced, time-resolved magnetic resonance angiography in cerebrovascular pathology. Neurosurg Focus. 47(6):E3, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31786556%5Bpmid%5D)
+1. [Haller S et al: Arterial spin labeling perfusion of the brain: emerging clinical applications. Radiology. 281(2):337-56, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27755938%5Bpmid%5D)
+1. [Josephson CB et al: Computed tomography angiography or magnetic resonance angiography for detection of intracranial vascular malformations in patients with intracerebral haemorrhage. Cochrane Database Syst Rev. 9:CD009372, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25177839%5Bpmid%5D)
+1. [Kobayashi A et al: Prognosis and treatment of intracranial dural arteriovenous fistulae: a systematic review and meta-analysis. Int J Stroke. 9(6):670-7, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25042991%5Bpmid%5D)
+1. [Lin N et al: Non-galenic arteriovenous fistulas in adults: transarterial embolization and literature review. J Neurointerv Surg. 7(11):835-40, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25237068%5Bpmid%5D)
+1. [Morales H et al: Documented development of a dural arteriovenous fistula in an infant subsequent to sinus thrombosis: case report and review of the literature. Neuroradiology. 52(3):225-9, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=19823815%5Bpmid%5D)
+1. [Burrows PE et al: Venous variations of the brain and cranial vault. Neuroimaging Clin N Am. 13(1):13-26, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12802938%5Bpmid%5D)
+1. [Kai Y et al: Pre- and post-treatment MR imaging and single photon emission CT in patients with dural arteriovenous fistulas and retrograde leptomeningeal venous drainage. AJNR Am J Neuroradiol. 24(4):619-25, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12695191%5Bpmid%5D)
+1. [Klisch J et al: Transvenous treatment of carotid cavernous and dural arteriovenous fistulae: results for 31 patients and review of the literature. Neurosurgery. 53(4):836-56; discussion 856-7, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14519216%5Bpmid%5D)
+1. [Chung SJ et al: Intracranial dural arteriovenous fistulas: analysis of 60 patients. Cerebrovasc Dis. 13(2):79-88, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=11867880%5Bpmid%5D)
+1. [Coley SC et al: Dural arteriovenous fistulae: noninvasive diagnosis with dynamic MR digital subtraction angiography. AJNR Am J Neuroradiol. 23(3):404-7, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=11901008%5Bpmid%5D)
+1. [Nomura S et al: Subarachnoid hemorrhage caused by dural arteriovenous fistula of the sphenobasal sinus--case report. Neurol Med Chir (Tokyo). 42(6):255-8, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12116531%5Bpmid%5D)
+1. [Satomi J et al: Benign cranial dural arteriovenous fistulas: outcome of conservative management based on the natural history of the lesion. J Neurosurg. 97(4):767-70, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12405361%5Bpmid%5D)
+
+## Differential diagnosis
+
+### Posterior Skull Base Lesion
+DDX:8a29ab23-43c8-4534-8af8-fdf252b88642
+
+### Vascular Lesions of Orbit
+DDX:65543935-2c14-42c6-a420-b6f37135ff95
+
+### Jugular Foramen Lesion
+DDX:cf70b4e9-14e8-496e-8927-62fe1bb1c89c
+
+### Pulsatile Tinnitus
+DDX:e0a91ed6-f016-4ae9-8380-b74dee5ecd1a
+
+## Anatomy
+
+### Skull Base Overview
+Brain/ANATOMY:a63a24e0-d077-4a32-a355-2092de4f84f3
+
+### Central Skull Base
+Brain/ANATOMY:2a489198-8130-4d47-8226-6970858ed6ed
+
+### Posterior Skull Base
+Brain/ANATOMY:b363fd64-a5af-4719-a285-71cdbec60f69
+
+### Cranial Nerves Overview
+Brain/ANATOMY:1edc42e3-5c31-42b9-b8b8-44cc3bd38432
+
+### Dural Sinuses
+Brain/ANATOMY:deb22ea4-1ac5-4542-a85a-8945376ad725
+
+### Cranial Nerves Overview
+Head and Neck/ANATOMY:170ad135-ca16-497a-80de-5a24b9ca2f47
+
+### Central Skull Base
+Head and Neck/ANATOMY:8887bf04-6027-42cb-9a34-3e15c6cc27ff
+
+### Posterior Skull Base
+Head and Neck/ANATOMY:8a7bdc2c-475b-4ce8-a35c-3fad9242a8bc
+
+### Pterygopalatine Fossa
+Head and Neck/ANATOMY:8028d3aa-f348-48e4-b364-f793ca6aac88
+
+### Skull Base Overview
+Head and Neck/ANATOMY:d5939b27-59e9-41a7-bb04-58bb30d22048
+
+### Scalp and Calvarial Vault
+Ultrasound/ANATOMY:25e08cb1-5308-4ce1-91c1-28cf309cd842
+
+### Intracranial Venous System Overview
+Brain/ANATOMY:fa5537f5-50aa-4a7d-abd1-a469b8a8b55f
+
+### Posterior Fossa Veins
+Brain/ANATOMY:7b5caf92-e4eb-4a23-ba29-45d1dbb1af65
+
+### Transcranial Doppler
+Ultrasound/ANATOMY:914fd68b-ddab-4640-bcc4-883c425d13f8
+
+## Cases
+
+- {'cases': [{'authors': [{'key': '33151213-01b2-4542-9105-342e006b3915', 'value': 'H. Ric Harnsberger, MD'}], 'caseVersionId': '177460b5-b0e7-43c8-a869-6b4b61a098b5', 'description': 'Typical CT-Angiography case of transverse-sigmoid sinus dural arteriovenous fistula (dAVF).\n\nAxial bone CT images of left temporal bone (#1-4) presented from superior to inferior show the left posterior mastoid area is porous with multiple serpiginous canals (arrows) secondary to dAVF transosseous collaterals connecting the occipital artery to the recanalized transverse-sigmoid dural sinuses. Lateral left external carotid artery angiogram (#5-6) reveals an enlarged occipital artery (arrow) feeding a network of transosseous collaterals (curved arrow). Notice the sigmoid sinus (open arrow) is recanalized.', 'history': 'Patient presents with remote history of trauma and 6 month history of progressive left ear objective pulsatile tinnitus.', 'imagePoolId': '0dbc57a7-4073-4e96-91f4-a0b034e3649c', 'name': 'Transverse-sigmoid sinus; Type I', 'teachingPoint': None, 'demographics': '45 Years old male'}, {'authors': [{'key': '2f09c8c8-188d-4cc1-9cb1-63a0271101ef', 'value': 'Anthony J Scuderi, MD'}, {'key': '624acd80-0502-4325-be71-e68fec740eb3', 'value': 'Richard H. Wiggins, III, MD, CIIP, FSIIM, FAHSE, FACR'}], 'caseVersionId': '19d27fa7-1bd8-42fa-9ca6-58977eea14ea', 'description': 'Typical MR angiography and venography case of a prominent dural A-V fistula of the skull base. \n\nAxial MRA images (#1-3) demonstrate asymmetrically increased vascularity in the right skull base (open arrow) from a dural arteriovenous fistula. Source images (#4-9) from axial MRA show a network of tiny vessels in the inferior petrosal sinus (arrow, #4-7) and pars nervosa of the right jugular foramen (curved arrow, #6-9). \n\nCoronal MRV image (#10) confirms patency of the right transverse and sigmoid sinuses and ipsilateral internal jugular vein (open arrow). \n\nComment: Adult dural arteriovenous fistulas are usually acquired and represent 10-15% of all cerebrovascular malformations with arteriovenous shunting.', 'history': None, 'imagePoolId': 'd7282d75-3e8f-44e3-a30d-9bc7d7b55557', 'name': 'Prominent', 'teachingPoint': None, 'demographics': '68 Years old female'}, {'authors': [{'key': '33151213-01b2-4542-9105-342e006b3915', 'value': 'H. Ric Harnsberger, MD'}], 'caseVersionId': '44275edf-6837-4263-b898-8eaac653c547', 'description': 'Typical MR-MRA case of transverse sinus dural arteriovenous fistula (dAVF).\n\nSource images from MRA (#1-3) show the transosseous collateral vessels as high signal connecting vessels in the bone (arrow) with the enlarged distal occipital artery (curved arrow) and partially recanalized transverse sinus (open arrow) also visualized. Basal MRA view (#4) and magnified left side (#5) show the MIP (maximum intensity reprojection) reconstruction with both the distal occipital artery (curved arrow) and partially recanalized transverse sinus (open arrow). Coronal view of MRV (#6) reveals the left transverse and sigmoid sinus are smaller with the distal transverse sinus absent (arrow). \n\nComment: The distal transverse sinus-proximal sigmoid sinus is most common site for dAVF in the posterior skull base.', 'history': 'Patient with history of objective pulsatile tinnitus in left ear first noticed in the months following car accident with head injury.', 'imagePoolId': 'f1ef1345-a49f-4f6b-ac2a-0b1b80b9567a', 'name': 'Transverse sinus, MR', 'teachingPoint': None, 'demographics': '45 Years old male'}, {'authors': [{'key': '2f09c8c8-188d-4cc1-9cb1-63a0271101ef', 'value': 'Anthony J Scuderi, MD'}, {'key': '33151213-01b2-4542-9105-342e006b3915', 'value': 'H. Ric Harnsberger, MD'}], 'caseVersionId': '5953f596-8f77-4238-ac05-074977423973', 'description': 'Typical MR and cerebral angiogram case of skull base dural A-V fistula with retrograde venous drainage.\n\nAxial T2 MR (#1-3) and axial T1 post-contrast MR (#4-5) depict enlarged cerebral and cerebellar veins (arrows, #1-4) along the tentorium. Selective left internal carotid angiogram (#6) shows enlarged meningohypophyseal trunk (open arrow) connecting to the transverse and sigmoid sinus (curved arrow). Selective vertebral angiogram (#7) demonstrates enlarged dural feeders (arrow) to the transverse sinus. Selective left external carotid angiogram (#8) shows enlarged occipital artery (open arrow) with multiple fistulous connections to the transverse and sigmoid sinus. Note retrograde flow into the opposite transverse sinus (curved arrow).\n\nComment: The Cognard classification of intracranial dural A-V fistulas correlates venous drainage pattern with clinical course. Lesions that involve enlarged cerebral or cerebellar veins have an increased incidence of intracranial hemorrhage.', 'history': 'Patient presents with objective (both MD and patient can hear) pulsatile tinnitus on the left and headaches.', 'imagePoolId': '304e2600-7c61-492d-b5aa-7dc0e1762229', 'name': 'Retrograde venous drainage', 'teachingPoint': None, 'demographics': '60 Years old female'}, {'authors': [{'key': '2f09c8c8-188d-4cc1-9cb1-63a0271101ef', 'value': 'Anthony J Scuderi, MD'}, {'key': '6651ae1c-5f55-4d2e-9f68-46223037c90a', 'value': ' , '}], 'caseVersionId': '6e936550-3f96-429f-8187-963ada2822a6', 'description': 'Typical cerebral angiogram case of a high flow multi-hole dural A-V fistula of the skull base. \n\nRight external carotid angiogram (#1-4) in the AP, lateral, and oblique projections demonstrate multiple enlarged arterial feeders (arrows) with dural/transosseous branches from the ECA and tortuous, dilated suboccipital veins (open arrows). AP and lateral right vertebral angiogram (#5-9) shows additional vertebral artery supply (curved arrows) via tentorial/dural branches, again draining into the suboccipital veins (open arrow) and jugular vein (arrow). Selective right internal carotid angiogram (#10) demonstrates small cavernous segment vessels (arrow) supplying the fistula which also drains into the suboccipital veins (curved arrow).\n\nDigital subtraction angiography with superselective catheterization of dural and transosseous feeders is the best imaging tool for dural A-V fistulas. High flow through draining veins may result in a high flow vasculopathy, with progressive stenosis, outlet occlusion and hemorrhage.', 'history': None, 'imagePoolId': 'b68e88ee-224f-43cc-b65e-1c7ad5684003', 'name': 'High flow', 'teachingPoint': None, 'demographics': '68 Years old female'}, {'authors': [{'key': '2f09c8c8-188d-4cc1-9cb1-63a0271101ef', 'value': 'Anthony J Scuderi, MD'}, {'key': '624acd80-0502-4325-be71-e68fec740eb3', 'value': 'Richard H. Wiggins, III, MD, CIIP, FSIIM, FAHSE, FACR'}], 'caseVersionId': '761ba37a-3eea-4eaf-8cb7-8fd959558306', 'description': 'This case is a typical arteriovenous fistula involving the skull base.\n\nAxial post-contrast T1 MR (#1-3) demonstrate prominent flow voids in a dural/transosseous branch (open arrow, #1) of the external carotid artery, right retromandibular vein (arrow, #3), and jugular bulb (curved arrow, #2). A collapsed axial MRA (#4) and anterior-posterior view reformatted MRV (#5) show enlarged branches of the right external carotid artery and vascular congestion around the ipsilateral jugular foramen, with prominent arterialized flow in the ipsilateral transverse and sigmoid sinus (arrows, #4).\n\nComment: Adult dural A-V fistula usually presents in middle-aged or older patients. The vascular malformation is usually acquired, may be idiopathic and can occur in response to trauma, venous occlusion or venous hypertension.', 'history': 'Patient presents with right pulsatile tinnitus.', 'imagePoolId': 'e52a8059-9441-41d8-830b-64e1816a6c04', 'name': 'Skull base', 'teachingPoint': None, 'demographics': '68 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '420421e5-f7a1-4304-9c41-c6d28c92cde4', 'description': 'Routine MR imaging can be normal with small dAVFs. Larger, long-standing lesions can sometimes be identified in the wall of a thrombosed venous sinus.\n\nPre-contrast axial T1WIs (#1,2) show a thrombosed left transverse sinus that contains innumerable small "flow voids" (arrows). Axial T2WI MRs (#3,4), show the thrombosed left transverse sinus with multiple "flow voids" characteristic of a long-standing dAVF. Axial T1 C+ MRs (#5,6), show the chronically occluded left TS enhances strongly. Note presence of innumerable "flow voids" within the enlarged sinus wall. Images #7-10 are a series of coronal post-contrast T1WIs that demonstrate the enhancing sinus wall and "flow voids" surrounding the thrombosed lumen. #11 is a composite of the key images.', 'history': 'Patient with pulsatile tinnitus.', 'imagePoolId': 'ff7f8482-7b99-440b-97d3-529befc6803a', 'name': 'Classic', 'teachingPoint': None}], 'caseType': 'typical', 'name': 'TYPICAL'}
+- {'cases': [{'authors': [{'key': '2f09c8c8-188d-4cc1-9cb1-63a0271101ef', 'value': 'Anthony J Scuderi, MD'}, {'key': '624acd80-0502-4325-be71-e68fec740eb3', 'value': 'Richard H. Wiggins, III, MD, CIIP, FSIIM, FAHSE, FACR'}], 'caseVersionId': 'cbddb585-edf0-43cb-868d-c54336d79875', 'description': 'Variant CT, MRI and MR angiography case of dural A-V fistula in the cerebellopontine angle cistern. \n\nAxial T2 MR (#1-3) and T1 MR (#4) show prominent flow voids (arrows) in the right cerebellopontine angle cistern. Post-contrast axial CT (#5-6) and T1 MR (#7-8) demonstrate corresponding vessel enhancement (arrows). Axial MRA (#9-14) confirms a tangle of enlarged vessels, depicted by the flow-related enhancement (open arrows) arising from the posterior circulation. Axial bone CT (#15) shows normal appearance of the jugular foramina (open arrows).\n\nThe most sensitive imaging study for a suspected dural A-V fistula is digital subtraction angiography with superselective catheterization to delineate the vascular supply and venous drainage, although the diagnosis is often suspected from non-invasive studies.', 'history': None, 'imagePoolId': '89b6ea8a-c667-4591-97c2-012b169595cc', 'name': 'Cerebellopontine angle cistern', 'teachingPoint': None, 'demographics': '61 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '8226cc4e-d00c-4e05-9f9c-afe6d0001277', 'description': 'Axial T1WIs (#1,2) show mixed signal intensity left temporal lobe mass (arrows) that appears quite bizarre on T2WI (#3,4). T1C+ scans (#5-8) show no enhancement within the mass itself but several unusual tortuous vessels can be seen (open arrows) adjacent to the mass. Coronal scans show some adjacent dural enhancement (arrows, #7,8). Acute hemorrhage with significant inhomogeneity is seen on DWI (#9,10) and ADC (#11,12). Initial diagnosis was hemorrhage into an underlying neoplasm. \n\nAn MRA was obtained. Source image (#13) shows no evidence for vascularity within the hemorrhage. MRV (#14) is very revealing. The left transverse and sigmoid sinuses plus the internal jugular vein are occluded. A small patent segment (open arrow) is identified. A DSA was obtained. The lateral view of the left carotid arteriogram shows an enlarged meningohypophyseal trunk (curved arrow) and multiple transosseous perforating feeders from the occipital artery (arrows) draining into a short patent segment of the transverse sinus (black open arrow) that is occluded distally (white open arrow). \n\nAt surgery, a thrombosed dAVF that involved the occluded left transverse sinus was resected and the clot evacuated.\n\nAcutely thrombosed dAVFs may mimic hemorrhagic neoplasms.', 'history': 'History of stroke, on Coumadin. Sudden onset right-sided weakness. Outside NECT scan (not shown) disclosed left temporal lobe hematoma.', 'imagePoolId': '31c81bd7-f3d0-4c87-ab38-481121a4bc06', 'name': 'Thrombosed, mimics neoplasm', 'teachingPoint': None, 'demographics': '46 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '9b132788-ab0a-43bd-972f-c00f6bc00a76', 'description': 'Axial CECT scan shows an unusual vascular channel (arrow, #1) that parallels the right transverse-sigmoid sinus junction. Bone CT scan from the same study shows innumerable enlarged transcalvarial vascular channels (arrows, #2). Axial source image from MRA (#3) shows a large right transverse sinus (open arrow) with very prominent transosseous vessels (arrows). Submentovertex MIP view (#4) shows the large right transverse/sigmoid sinus (open arrow) with prominent vessels (arrows) that appear to arise from an enlarged right external carotid artery (ECA) and its branches (curved arrows). Coronal MIP of the MRA (#5) shows the enlarged ECA branches (curved arrows) and the innumerable transcalvarial perforating vessels (arrows). \n\nDSA was performed. Selective right ECA angiogram, lateral view, arterial phase (#6) shows a large occipital artery (curved arrow) and auricular artery with prominent transosseous perforating vessels (arrows). Venous reflux into a large right TS (open arrow) is present. Internal carotid angiogram (#7) shows several tentorial branches of an enlarged meningohypophyseal trunk (arrows) opacifying the transverse sinus (open arrow). Vertebral angiogram (#8-9) shows muscular branch and PICA contributions (arrows) to the dAVF.\n\nComment: This case was a Cognard Grade 2A lesion, with reflux into the dural sinuses but not the cortical veins. This grade generally carries a low risk of hemorrhage even though the patient had a subarachnoid hemorrhage three years prior to this study.', 'history': 'Patient with unexplained subarachnoid hemorrhage 3 years ago presents with increasing right-sided tinnitus.', 'imagePoolId': '7a16e240-2bd7-4b0d-8959-557d1b61c9ac', 'name': 'Amazing transcalvarial channels', 'teachingPoint': None, 'demographics': '77 Years old male'}], 'caseType': 'variant', 'name': 'VARIANT'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*Graphic of typical dural arteriovenous fistula (DAVF) with a short segment of a thrombosed transverse sinus (TS)
shows DAVF consisting of multiple dural vessels in the wall of thrombosed segment. Multiple dural & transosseous feeders arise from external
(ECA) & internal carotid arteries (ICA).*
+
+
+*MRA shows extensive, prominent vascularity in the right skull base
from a DAVF. Source images from MRA should be reviewed for correlation with MIP images. Dural sinuses
remain patent in this case. Thrombosis is often seen.*
+
+
+*Axial bone CT of the left temporal bone demonstrates the left posterior squamous temporal bone is permeated with multiple prominent serpiginous canals
secondary to DAVF transosseous collaterals.*
+
+
+*Midarterial lateral image from a left ECA DSA reveals an enlarged occipital artery
terminating in a network of transosseous collaterals
& shunting into the sigmoid sinus. Notice the sigmoid sinus
is partially & irregularly recanalized post thrombosis.*
+
+
+*Axial MRA source image in a patient with left pulsatile tinnitus demonstrates linear & punctate areas of flow-related signal in the left occipital bone
extending into a thrombosed left sigmoid sinus
.*
+
+
+*Axial MRA MIP in the same patient shows a prominent distal occipital artery
. Note the feeder from the superficial temporal artery
. The transverse sinus
is not recanalized. Flow-related MRA artifacts can mimic partial flow/recanalization.*
+
+
+*Axial T2WI MR in patient with DAVF shows enlargement of multiple prominent cerebellar veins
. Lesions with enlarged cortical veins have an increased incidence of intracranial hemorrhage. White matter edema from venous congestion may also be evident.*
+
+
+*Lateral ECA angiography in the same patient shows enlarged dural feeders
to DAVF. Deep cortical venous drainage
is well seen, & venous varix
is displayed. Tentorial DAVFs may have particularly complex anatomy.*
+
+
+*AP MRV reveals that the left TS and sigmoid sinus are smaller than the right with distal TS occlusion
. The distal TS-proximal sigmoid sinus confluence is the most common site for DAVFs of the posterior skull base.*
+
+
+*Composite MR shows an atypical DAVF with innumerable small flow voids
in the thrombosed segment of TS-sigmoid sinus junction. MRA may show flow-related enhancement of vessels, but angiography is often necessary to confirm.*
+
+
+### Additional Images
+
+
+*Lateral ECA shows an enlarged occipital artery
with multiple connections to the TS and sigmoid sinus. Retrograde flow into the opposite TS
is noted.*
+
+
+*Lateral ICA shows a type IV DAVF with enlarged tentorial branches from the meningohypophyseal trunk
and deep cortical venous drainage
.*
+
+
+*Coronal MIP of MRA in a patient with DAVF shows enlarged ECA branches
and innumerable transcalvarial perforating vessels
. This case was a Cognard grade 2A lesion with reflux into the dural sinuses but not the cortical veins.*
+
+
+*Axial bone CT shows multiple serpiginous canals
that represent DAVF transosseous collaterals connecting the occipital artery to the recanalized TS-sigmoid sinus dural sinuses. These prominent vascular channels may be easily overlooked.*
+
diff --git a/docs_md/articles/enhancing-cranial-nerves_6471fb1c-d46d-47cd-8322-1671e82335c3.md b/docs_md/articles/enhancing-cranial-nerves_6471fb1c-d46d-47cd-8322-1671e82335c3.md
new file mode 100644
index 0000000..05a523a
--- /dev/null
+++ b/docs_md/articles/enhancing-cranial-nerves_6471fb1c-d46d-47cd-8322-1671e82335c3.md
@@ -0,0 +1,302 @@
+---
+title: "Enhancing Cranial Nerve(s)"
+docid: "6471fb1c-d46d-47cd-8322-1671e82335c3"
+authors:
+ - key: "20008105-51da-4bbf-aba6-e8253b2d9c21"
+ value: "Daniel E. Meltzer, MD"
+ - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
+ value: "Anne G. Osborn, MD, FACR"
+breadcrumbs:
+ -
+ name: "Head and Neck"
+ slug: "head-and-neck"
+ treeNodeId: "5c1f8e17-7acd-48d8-9d55-f9f8c2cad850"
+ -
+ name: "Differential Diagnosis"
+ slug: "differential-diagnosis"
+ treeNodeId: "deb55065-e1d6-4b6f-b3e3-181fafb4e218"
+ -
+ name: "Cranial Nerves and Brainstem"
+ slug: "cranial-nerves-and-brainstem"
+ treeNodeId: "385449a2-5859-451c-bed3-584babc08f0c"
+ -
+ name: "Generic Imaging Patterns"
+ slug: "generic-imaging-patterns"
+ treeNodeId: "4240e9a5-d16b-4025-a36e-ce9e6110b24c"
+ -
+ name: "Enhancing Cranial Nerve(s)"
+ slug: "enhancing-cranial-nerves"
+ treeNodeId: null
+category: "Head and Neck"
+documentVersionId: "6cc8ec33-e091-4354-adc1-57d4990a566e"
+imageCount: 27
+lastUpdated: "08/06/18"
+pageDescription: "Enhancing Cranial Nerve(s)"
+pageKeywords: "Head and Neck, Differential Diagnosis, Cranial Nerves and Brainstem, Generic Imaging Patterns, Enhancing Cranial Nerve(s)"
+pageTitle: "Enhancing Cranial Nerve(s) | STATdx"
+enhancedTitle: "Enhancing Cranial Nerve(s)"
+type: "DDX"
+references: true
+breadcrumbs:
+ - "Head and Neck"
+ - "Differential Diagnosis"
+ - "Cranial Nerves and Brainstem"
+ - "Generic Imaging Patterns"
+ - "Enhancing Cranial Nerve(s)"
+---
+## ESSENTIAL INFORMATION
+
+- ### Key Differential Diagnosis Issues
+
+
+ - Enhancement of cisternal, cavernous sinus cranial nerve (CN) segments always abnormal
+ - Which CN(s) are affected
+ - Optic nerve: Multiple sclerosis (MS), neurofibromatosis type 1 (NF1) (optic glioma), viral/post viral
+ - CNIII & VI: Often ischemia (diabetes, arteriolosclerosis)
+ - CNVII: Bell palsy, Herpes zoster (Ramsay Hunt)
+ - CNVIII: Schwannoma (sporadic or NF2 associated), metastasis
+ - If multiple nerves involved, consider
+ - Metastases, lymphoma, leukemia
+ - NF2
+ - Lyme disease
+ - Chronic inflammatory demyelinating polyneuropathy (CIDP) (especially if nerves massively enlarged)
+ - History important
+ - Optic neuritis (majority have or develop MS)
+ - Known neoplasm
+ - Flu-like illness (ADEM, viral neuritis)
+- ### Helpful Clues for Common Diagnoses
+
+
+ - **Metastases**
+ - Most common: CSF spread
+ - Involves pia, CNs' may extend along perivascular spaces
+ - Multiple thickened nerves > solitary involvement
+ - Fundus of CPA/IAC most common site
+ - Less common: Perineural tumor extension from extracranial primary
+ - Extension into cisternal CN uncommon
+ - Squamous cell, adenoid cystic carcinoma (CNV & VII involvement most common)
+ - **Neurofibromatosis Type 2**
+ - Multiple inherited schwannomas, meningiomas, and ependymomas
+ - Bilateral vestibular schwannomas diagnostic
+ - Vestibular schwannoma plus schwannoma of 1 other CN highly suggestive
+ - Schwannoma of "small" CN (e.g., CNIII & IV) should raise consideration of NF2
+ - **Neurofibromatosis Type 1**
+ - **Plexiform Neurofibroma**
+ - Intracranial involvement less common than scalp, orbit, face (e.g., parotid gland)
+ - Plexiform neurofibromas of CNIII or CNV may extend intracranially, involve cavernous sinus
+ - **Optic Nerve Glioma**
+ - Most are typical pilocytic astrocytomas (PAs)
+ - 15-20% of NF1 patients develop pilocytic astrocytoma
+ - Most commonly in optic pathway
+ - Up to 1/3 of patients with optic pathway PA have NF1
+ - Enhancement varies from none to avid
+ - May be uni- or bilateral, extend to/from orbit, involve nerves/chiasm/hypothalamus
+ - **Multiple Sclerosis**
+ - Optic nerve most commonly affected
+ - 50-60% of patients with optic neuritis ultimately meet criteria for MS
+ - Imaging
+ - Mildly enlarged, enhancing optic nerve
+ - 40% extend to intracanalicular, prechiasmatic/chiasmatic segments
+ - Other CNs (e.g., trigeminal nerve) less commonly affected
+ - Non-MS associated optic neuropathy
+ - Infectious (viral)
+ - Anterior ischemic optic neuropathy
+- ### Helpful Clues for Less Common Diagnoses
+
+
+ - **Viral, Postviral Neuritis**
+ - **Bell Palsy**
+ - Herpetic peripheral facial nerve paralysis 2° to herpes simplex virus
+ - Enhancement of fundal tuft and labyrinthine segment CNVII
+ - Entire intratemporal CNVII may enhance
+ - **Herpes Zoster**
+ - Ramsay Hunt syndrome: Herpes zoster oticus secondary to varicella-zoster virus infection
+ - Vesicular rash external ear
+ - Contrast enhancement of entire intratemporal CNVII ± CNVIII in IAC fundus along with all or part of membranous labyrinth
+ - **ADEM**
+ - Autoimmune-mediated white matter demyelination of brain &/or spinal cord
+ - Days to weeks after upper respiratory infection or vaccination
+ - Multifocal white matter > gray matter lesions with variable contrast enhancement
+ - ± involvement of CN(s)
+ - Affected nerve minimally enlarged
+ - Transient enhancement
+ - Clinical and radiologic findings often improve rapidly
+ - **Lyme Disease**
+ - Most common: MS-like white matter lesions in patient with round, bull's-eye skin rash & flu-like illness following *Ixodes* tick bite
+ - Reservoirs: White tail deer/field mouse
+ - ± multiple enhancing CNs (CNVII most common)
+ - ± cauda equina, meningeal enhancement
+ - **Lymphoma/Leukemia**
+ - Secondary involvement of CNS in patients with systemic lymphoma or leukemia
+ - Diffuse pial tumor spread → multiple CNs
+ - **Neurosarcoid**
+ - Most common intracranial involvement = optic nerve/chiasm/hypothalamus
+ - Other CNs rare
+ - **Opportunistic Infection, AIDS**
+ - Tuberculous meningitis
+ - Cytomegalovirus neuritis (retina, optic nerve)
+- ### Helpful Clues for Rare Diagnoses
+
+
+ - **Ischemia**
+ - Diabetes, microvascular disease
+ - CNIII & VI most commonly affected
+ - Optic nerve (anterior ischemic optic neuropathy) less common
+ - Transient enhancement, then atrophy
+ - **Langerhans Cell Histiocytosis**
+ - Usually children
+ - Hypothalamus/infundibular stalk involvement most common
+ - Infiltrated, thickened structures enhance strongly, uniformly
+ - Lack of posterior pituitary bright spot in patients with diabetes insipidus
+ - Disseminated intracranial Langerhans cell histiocytosis rare
+ - Sulcal/cisternal enhancement
+ - Multiple enhancing CNs
+ - **Chronic Inflammatory Demyelinating Polyneuropathy**
+ - Typical setting: Chronic MS
+ - Serial demyelination, remyelination → "onion bulb" thickening of affected nerves
+ - Massive enlargement, enhancement of spinal > > CNs
+ - Hereditary sensory and motor neuropathies (e.g., Charcot-Marie-Tooth disease) may have similar imaging findings
+
+## References
+
+## Selected References
+
+1. [Badger D et al: Imaging of perineural spread in head and neck cancer. Radiol Clin North Am. 55(1):139-149, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27890182%5Bpmid%5D)
+1. [Lewis RA: Chronic inflammatory demyelinating polyneuropathy. Curr Opin Neurol. 30(5): 508-512, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28763304%5Bpmid%5D)
+1. [Drenckhahn A et al: Acute isolated partial oculomotor nerve palsy due to Lyme neuroborreliosis in a 5 year old girl. Eur J Paediatr Neurol. 20(6):977-979, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27323659%5Bpmid%5D)
+1. [Hyun JW et al: Leptomeningeal metastasis: Clinical experience of 519 cases. Eur J Cancer. 56:107-14, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26841095%5Bpmid%5D)
+1. [Stivaros SM et al: Multiple synchronous sites of origin of vestibular schwannomas in neurofibromatosis Type 2. J Med Genet. 52(8):557-62, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26104281%5Bpmid%5D)
+1. [Wang JJ et al: Ramsay Hunt syndrome in a patient with H7N9 influenza virus infection. Chin Med J (Engl). 128(3):417-8, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25635445%5Bpmid%5D)
+1. [Bester M et al: Neuroimaging of multiple sclerosis, acute disseminated encephalomyelitis, and other demyelinating diseases. Semin Roentgenol. 49(1):76-85, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24342677%5Bpmid%5D)
+1. [Brea Álvarez B et al: Perineural spread in head and neck tumors. Radiologia. 56(5):400-12, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25002354%5Bpmid%5D)
+1. [Glass LR et al: Optic nerve glioma: case series with review of clinical, radiologic, molecular, and histopathologic characteristics. Ophthal Plast Reconstr Surg. 30(5):372-6, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24762952%5Bpmid%5D)
+1. [Marin SE et al: The magnetic resonance imaging appearance of monophasic acute disseminated encephalomyelitis: an update post application of the 2007 consensus criteria. Neuroimaging Clin N Am. 23(2):245-66, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23608688%5Bpmid%5D)
+1. [Khanna S et al: Magnetic resonance imaging of optic neuritis in patients with neuromyelitis optica versus multiple sclerosis. J Neuroophthalmol. 32(3):216-20, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22659839%5Bpmid%5D)
+1. [Loor RG et al: Multiple cranial nerve dysfunction caused by neurosarcoidosis. Am J Otolaryngol. 33(4):484-6, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22154016%5Bpmid%5D)
+1. [Kale HA et al: Magnetic resonance imaging findings in chronic inflammatory demyelinating polyneuropathy with intracranial findings and enhancing, thickened cranial and spinal nerves. Australas Radiol. 51 Spec No, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17875147%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+**Metastases**
+*Axial T1 C+ MR in a patient with disseminated malignant glial neoplasm shows diffuse enhancing metastases covering the brain, CPA/IACs
, and both abducens nerves
.*
+
+
+**Metastases**
+*Axial T1 C+ MR in a patient with disseminated malignant glial neoplasm shows diffuse enhancing metastases covering the brain, CPA/IACs
, and both abducens nerves
.*
+
+
+**Metastases**
+*Axial T1 C+ MR in a patient with disseminated malignant glial neoplasm shows diffuse enhancing metastases covering the brain, CPA/IACs
, and both abducens nerves
.*
+
+
+**Neurofibromatosis Type 2**
+*Coronal T1 C+ FS MR of a patient with NF2 shows bilateral enhancing masses consistent with CNVIII schwannomas
. There is a schwannoma traversing the left jugular foramen
, most likely CNX. Superiorly, an additional mass
most likely represents a schwannoma of the left CNV. Note normal right CNV
.*
+
+
+**Plexiform Neurofibroma**
+*Axial T1 C+ FS MR in a patient with NF1 shows unusually extensive neurofibroma of CNIII branches, extending from orbit through markedly enlarged orbital fissure into expanded cavernous sinus
. Note scalp plexiform neurofibroma
. (Courtesy M. Martin, MD.)*
+
+
+**Optic Nerve Glioma**
+*Axial T1 C+ FS MR of the orbit in a 4-month-old child with sudden-onset right proptosis shows diffuse enlargement and abnormal enhancement of the left intraorbital optic nerve
, proptosis, and flattening of the dorsal globe.*
+
+
+**Multiple Sclerosis**
+*Axial T1 C+ FS MR shows enhancement of almost the entire length of the left optic nerve
, including the intracanalicular segment
.*
+
+
+**Multiple Sclerosis**
+*Coronal T1 C+ FS MR in a patient with multiple sclerosis and left trigeminal neuralgia shows enhancing left CNV
. Compare to normal nonenhancing right side
.*
+
+
+**Bell Palsy**
+*Axial T1 C+ FS MR with magnified view shows variant case with enhancing "fundal tuft" in IAC
and enhancing labyrinthine segment
leading to enhancing geniculate ganglion
.*
+
+
+**Herpes Zoster**
+*Axial T1 C+ FS MR shows enhancing CNVII in IAC
, tympanic segment
along with enhancement of the extracranial soft tissues involving the left ear
.*
+
+
+**ADEM**
+*Coronal T1 C+ FS MR in a child with intracranial parenchymal areas of signal abnormality consistent with ADEM (not shown) shows moderate enhancement and mild increase in the size of the left intraorbital optic nerve
.*
+
+
+**Lyme Disease**
+*Axial T1 C+ FS MR shows abnormal enhancement of the right facial nerve at the canalicular
, labyrinthine
, geniculate
, and tympanic
segments. There is also abnormal enhancement of the cisternal segments of the bilateral abducens nerves
.*
+
+
+**Lymphoma**
+*Axial T1 C+ FS MR in a patient with known systemic lymphoma and right 3rd nerve palsy shows thickened, enhancing right oculomotor nerve
and intraconal retrobulbar enhancing tumor
.*
+
+
+**Neurosarcoid**
+*Axial T1 C+ MR shows enhancement of both thickened optic nerves extending from the optic canal to the optic chiasm
.*
+
+
+**Opportunistic Infection, AIDS**
+*Axial T1 C+ MR in a patient with HIV/AIDS who presented with confusion and seizures shows tubercular meningitis
that thickens and encases the right trigeminal nerve
.*
+
+
+**Chronic Inflammatory Demyelinating Polyneuropathy**
+*Coronal T1 C+ FS MR in a 39-year-old woman with longstanding multiple sclerosis and left facial pain shows both trigeminal nerves thickened and enhancing
.*
+
+
+### Additional Images
+
+
+**Metastases**
+*Axial T1 C+ FS MR in a patient with known metastatic colon cancer shows IAC enhancement
extending through the cochlear aperture, across the modiolus and into the membranous labyrinth
.*
+
+
+**Optic Neuritis**
+*Axial T1 C+ FS MR shows diffusely enhancing intraorbital portion of left optic nerve
.*
+
+
+**Herpes Zoster**
+*Axial T1 C+ MR in a patient with Herpes zoster, right-sided trigeminal neuralgia, shows cisternal CNV enhancement
.*
+
+
+**ADEM**
+*Axial T1 C+ MR in a child presenting with right-sided vision loss 2 weeks following a flu-like episode is shown. Funduscopic examination showed swollen nerve head. Diffusely enhancing optic nerve
was initially diagnosed as optic nerve glioma. Lesion resolved following short course of steroids. Follow-up imaging showed no residual abnormality.*
+
+
+**Langerhans Cell Histiocytosis**
+*Sagittal T1 C+ MR in a child with known LCH shows enhancing mass infiltrating pituitary gland, stalk, hypothalamus and optic chiasm
. Note also dural thickening
.*
+
+
+**Neurofibromatosis Type 2**
+*Axial T1 C+ MR shows bilateral vestibular schwannomas with classic "ice cream on cone" appearance
. Note arachnoid cyst associated with left lesion
.*
+
+
+**Lyme Disease**
+*Axial T1 C+ FS MR shows enhancement in left IAC involving both CNVII and CNVIII
. Note pial enhancement along the pons
, extending along CNVI from its brainstem exit to Dorello canal
.*
+
+
+**Metastases**
+*Axial T1 C+ FS MR shows thickened, enhancing V2 in a patient with adenoid cystic carcinoma with perineural tumor spread in pterygopalatine fossa
, extending along the foramen rotundum
into the Meckel cave
.*
+
+
+**Neurofibromatosis Type 2**
+*Coronal T1 C+ FS MR in a patient with known NF2 shows trigeminal schwannomas in both Meckel caves
, as well as multiple schwannomas involving cervical spinal nerve roots
.*
+
+
+**ADEM**
+*Axial FLAIR shows a woman presenting with symptoms including left facial numbness shortly following vaccinations. There is abnormal signal in the left trigeminal nerve
and bilateral brachium pontis
. Signal abnormality in these areas was nearly resolved on 1 month follow-up imaging (not shown).*
+
+
+**Optic Nerve Glioma**
+*Axial CECT in a child with NF1 shows bilateral optic nerve gliomas extending through the optic canals to chiasm
. The right optic nerve is noticeably enlarged and enhancing
.*
+
+
+**Optic Nerve Glioma**
+*Axial CECT in a 4 month old with sudden onset proptosis demonstrates diffuse enlargement and enhancement of the left intraorbital optic nerve
, with extension posteriorly into the enlarged optic canal
, flattening of the dorsal globe, and moderate proptosis.*
+
+
+**Herpes Zoster**
+*Axial T1 C+ FS MR in a patient with 2-week history of rapid-onset sensorineural hearing loss shows linear enhancement of CNVIII in CPA/IAC
.*
+
diff --git a/docs_md/articles/group-b-streptococcal-meningitis_bafa10c7-e65b-4432-9959-b8e5e4af708c.md b/docs_md/articles/group-b-streptococcal-meningitis_bafa10c7-e65b-4432-9959-b8e5e4af708c.md
new file mode 100644
index 0000000..dac0771
--- /dev/null
+++ b/docs_md/articles/group-b-streptococcal-meningitis_bafa10c7-e65b-4432-9959-b8e5e4af708c.md
@@ -0,0 +1,482 @@
+---
+title: "Group B Streptococcal Meningitis"
+docid: "bafa10c7-e65b-4432-9959-b8e5e4af708c"
+authors:
+ - key: "b2e6dabb-ee1c-42a4-a332-9f0814c1c607"
+ value: "Surjith Vattoth, MD"
+breadcrumbs:
+ -
+ name: "Pediatrics"
+ slug: "pediatrics"
+ treeNodeId: "40c0ffd5-41a0-4a6f-99fd-74c72c2e0bd3"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "8c45dd6b-ac86-4be1-b770-4755c799ad00"
+ -
+ name: "Pediatric Neuroradiology"
+ slug: "pediatric-neuroradiology"
+ treeNodeId: "47bf4839-9cae-4078-8a93-b14004d9eef1"
+ -
+ name: "Brain"
+ slug: "brain"
+ treeNodeId: "64b09eb7-b76b-48be-9ef0-064fb3828f85"
+ -
+ name: "Pathology-Based Diagnoses"
+ slug: "pathology-based-diagnoses"
+ treeNodeId: "827f44e9-f4d6-4bf1-814e-08372b32333f"
+ -
+ name: "Infectious Disease"
+ slug: "infectious-disease"
+ treeNodeId: "d02631a2-c8a2-484e-870b-f0fac0240d90"
+ -
+ name: "Congenital/Neonatal Infections"
+ slug: "congenitalneonatal-infections"
+ treeNodeId: "dc8f69c6-7d3f-4061-90c6-e914c57c6fed"
+ -
+ name: "Group B Streptococcal Meningitis"
+ slug: "group-b-streptococcal-meningitis"
+ treeNodeId: null
+category: "Pediatrics"
+documentVersionId: "e07c4fe2-8689-4dfd-b0ed-401c245b33c1"
+imageCount: 22
+lastUpdated: "02/06/24"
+pageDescription: "Group B Streptococcal Meningitis"
+pageKeywords: "Pediatrics, Diagnosis, Pediatric Neuroradiology, Brain, Pathology-Based Diagnoses, Infectious Disease, Congenital/Neonatal Infections, Group B Streptococcal Meningitis"
+pageTitle: "Group B Streptococcal Meningitis | STATdx"
+enhancedTitle: "Group B Streptococcal Meningitis"
+type: "DX"
+references: true
+anatomy:
+ - "{'authors': 'Anne G. Osborn, MD, FACR', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/3210cf7d-5be6-4741-a11d-dd7f8c1bc286', 'category': 'Brain', 'compareUrl': '/compare/document/3210cf7d-5be6-4741-a11d-dd7f8c1bc286/related-anatomy/treeNode?subContext=Cranial Meninges', 'documentId': '3210cf7d-5be6-4741-a11d-dd7f8c1bc286', 'documentType': 'ANATOMY', 'documentUrl': '/document/cranial-meninges/3210cf7d-5be6-4741-a11d-dd7f8c1bc286', 'enhancedTitle': 'Cranial Meninges', 'entryDate': '10/20/20', 'imageCount': 19, 'imageUrl': '/image/thumbnail/dc8df822-4917-4d41-b48b-8b73821990a6?size=174&quality=85', 'inCompareCart': False, 'rank': 1, 'referenceCount': 1, 'showCompareButton': False, 'title': 'Cranial Meninges'}"
+ - "{'authors': 'Jeffrey S. Anderson, MD, PhD', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/e1a20b61-b2c1-44c5-ba04-59843855bfef', 'category': 'Brain', 'compareUrl': '/compare/document/e1a20b61-b2c1-44c5-ba04-59843855bfef/related-anatomy/treeNode?subContext=Limbic Network', 'documentId': 'e1a20b61-b2c1-44c5-ba04-59843855bfef', 'documentType': 'ANATOMY', 'documentUrl': '/document/limbic-network/e1a20b61-b2c1-44c5-ba04-59843855bfef', 'enhancedTitle': 'Limbic Network', 'entryDate': '10/20/20', 'imageCount': 3, 'imageUrl': '/image/thumbnail/17c31997-0c69-473f-ac55-0b912c1cef1a?size=174&quality=85', 'inCompareCart': False, 'rank': 2, 'referenceCount': 11, 'showCompareButton': False, 'title': 'Limbic Network'}"
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+cases: 2
+breadcrumbs:
+ - "Pediatrics"
+ - "Diagnosis"
+ - "Pediatric Neuroradiology"
+ - "Brain"
+ - "Pathology-Based Diagnoses"
+ - "Infectious Disease"
+ - "Congenital/Neonatal Infections"
+ - "Group B Streptococcal Meningitis"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Group B β-hemolytic streptococcal (GBS) meningitis
+ - Leading newborn meningitis cause in developed countries
+- ### Imaging
+
+
+ - Acute: Meningitis, cerebritis, vasculitis, ventriculitis, subdural effusion, empyema, arterial and venous infarction
+ - Arterial distributions often affected; variable dural, leptomeningeal, and parenchymal enhancement
+ - Ventriculitis: Ependymal enhancement, ventricular debris
+ - Missing neonatal T2 cortical ribbon (encephalitis or infarcts)
+ - Blurring/loss of gray matter-white matter junction → ± basal ganglia, thalamic, white matter hyperintensities
+ - ↑ FLAIR in cortex, subarachnoid space, and subdural, cisternal, and ventricular spaces
+ - Diffusion restriction within infarcts and empyema
+ - Rim enhancement with subdural effusion (thinner) and empyema (thicker)
+ - Arterial stenosis/occlusions; venous thrombosis
+- ### Top Differential Diagnoses
+
+
+ - Enteric, gram-negative meningitis
+ - *Escherichia coli*: Along with GBS meningitis, major cause of newborn meningitis in developed countries
+ - *Listeria monocytogenes*: Gram-positive rod
+ - *Enterobacter*: Most common cause of meningitis in 1st few months of life
+- ### Pathology
+
+
+ - Debris, exudates within subarachnoid spaces and ventricles
+ - Brain infarct/encephalomalacia; vasculitis &/or vasospasm
+- ### Clinical Issues
+
+
+ - Newborn with sepsis; typical signs/symptoms of meningitis subtle in neonate
+- ### Diagnostic Checklist
+
+
+ - No imaging features distinguish GBS meningitis from other neonatal meningitides
+ - Do MR with IV contrast, DWI, MRA, and postcontrast MRV
+
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - Group B streptococcal (GBS) meningitis
+- ### Synonyms
+
+
+ - Group B β-hemolytic streptococcal meningitis
+- ### Definitions
+
+
+ - Leading cause of newborn meningitis in developed countries
+ - Early-onset disease (EOD): GBS sepsis presenting in 1st week of life; 80% of GBS infections EOD
+ - Meningitis complicates in 10%
+ - Late-onset disease (LOD): GBS sepsis presenting between 1-4 weeks of life; 20% GBS infections LOD
+ - Meningitis complicates in 40-60%
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Meningoencephalitis in newborn
+ - #### Location
+
+
+ - Leptomeninges, cerebral hemispheres, and deep gray matter (GM)
+ - #### Size
+
+
+ - Extensive panlobular involvement typical
+ - #### Morphology
+
+
+ - Multifocal involvement
+ - Arterial distributions often affected, particularly basal ganglia (BG) and thalami
+ - Acute: Meningitis, cerebritis, vasculitis, ventriculitis, subdural effusion, empyema, arterial and venous infarction
+ - Occasional spinal cord involvement
+ - Chronic sequelae: Loculated hydrocephalus, cystic encephalomalacia
+- ### CT Findings
+
+
+ - #### NECT
+
+
+ - Hydrocephalus ± dependent debris in ventricles
+ - Hypodensities in arterial distributions, BG, thalami, white matter (WM)
+ - Occasional hyperdense foci = hemorrhagic venous infarcts, laminar necrosis
+ - Hypodense subdural collections (effusion vs. empyema)
+ - #### CECT
+
+
+ - Variable: Dural, leptomeningeal, and parenchymal enhancement
+ - Rim enhancement around subdural effusions and empyemas
+ - Ependymal enhancement/ventricular debris
+- ### MR Findings
+
+
+ - #### T1WI
+
+
+ - Hypo- and hyperintense foci common
+ - Multifocal hypointensities = edema, ischemia, infarction
+ - Hyperintense foci cortex, BG, and WM = laminar necrosis, hemorrhagic venous infarction
+ - #### T2WI
+
+
+ - Blurring/loss of GM-WM junction, ± BG, thalamic, and WM hyperintensity
+ - Focal cortical ↑ T2 in encephalitis (or infarcts) continuous with neonatal normally ↑ T2 subcortical WM looks like focally "missing cortical ribbon"
+ - #### FLAIR
+
+
+ - Hyperintensity in cortex and subarachnoid, subdural, cisternal, and ventricular spaces
+ - #### T2* GRE
+
+
+ - Blooming of hemorrhagic foci in brain or extraaxial spaces
+ - SWI better demonstrates blood products
+ - #### DWI
+
+
+ - Diffusion restriction in infarcts, pus collections
+ - Extremely rare for abscess and empyema to have no restricted diffusion
+ - Patterns of ischemic injury
+ - Multiple punctuate infarcts in BG
+ - Focal or diffuse cerebral infarcts
+ - Focal periventricular or subcortical WM infarcts
+ - #### T1WI C+
+
+
+ - Variable: Dural, leptomeningeal, parenchymal, and ependymal enhancement
+ - Rim enhancement with subdural effusion (thinner) and empyema (thicker)
+ - Postcontrast FLAIR better demonstrates abnormal meningeal enhancement
+ - #### MRA
+
+
+ - Arterial narrowing (vasculitis &/or vasospasm) ± occlusions
+ - #### MRV
+
+
+ - Dural venous sinus/cortical vein thrombosis (in up to 30%)
+ - #### MRS
+
+
+ - ↑ choline, ↓ NAA; ↑ lactate in areas of ischemia/infarction
+- ### Ultrasonographic Findings
+
+
+ - #### Grayscale ultrasound
+
+
+ - ↑ echogenicity of sulci and parenchyma → hydrocephalus → ventricular debris
+- ### Imaging Recommendations
+
+
+ - #### Best imaging tool
+
+
+ - MR with IV contrast, DWI, MRA, and MRV
+ - #### Protocol advice
+
+
+ - CECT for rapid initial assessment of hemodynamically unstable neonate
+
+## DIFFERENTIAL DIAGNOSIS
+
+- ### Other Neonatal Meningitides
+
+
+ - Enteric, gram-negative meningitis
+ - Account for majority of early-onset meningitis in developing countries
+ - Higher mortality than GBS meningitis
+ - Specific pathogens
+ - *Escherichia coli*: Major cause of newborn meningitis in developed countries (along with GBS meningitis)
+ - *Enterobacter*: Most common cause of meningitis in 1st few months of life
+ - *Citrobacter*: Rare; high morbidity/mortality secondary to frequent abscess (square morphology) formation
+ - Other meningitides
+ - *Listeria monocytogenes*: Gram-positive rod
+- [Congenital Infections (TORCH)](/document/torch-infections/3b509e1c-83cf-4793-8307-e0afa70781ce)
+ - CMV, toxoplasmosis, rubella: Infection occurs in utero with chronic sequelae present in neonate/infant
+ - CMV: Periventricular Ca⁺⁺, microcephaly, migrational abnormalities, encephalomalacia, cerebellar hypoplasia
+ - Toxoplasmosis: Parenchymal Ca⁺⁺, encephalomalacia, microphthalmia
+ - HSV-2: Infection acquired during vaginal birth; presents in first 2-4 weeks of life
+ - Meningoencephalitis with extensive edema, necrosis, late cystic encephalomalacia
+- [Hypoxic-Ischemic Encephalopathy](/document/hypoxic-ischemic-encephalopathy/92cff1ec-37d9-499c-b871-86d819568dab)
+ - Preterm: Injury to periventricular WM (mild) or thalami, BG, brainstem (severe)
+ - Term: Injury to mature vascular watershed (mild) or areas of early myelination/metabolic activity (severe)
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Etiology
+
+
+ - EOD: Aspiration of infected amniotic fluid or birth canal secretions
+ - LOD: Postnatal maternal contact, breast milk, nosocomial
+ - Bacteremia facilitated by immature neonatal immune system
+ - Development of meningitis related to magnitude/duration of bacteremia
+ - Production of β-hemolysin facilitates access of GBS across blood-brain barrier
+ - GBS agalactiae serotype 3 responsible for majority of GBS meningitis
+ - GBS potent activator of neonatal immune/inflammatory response
+- ### Gross Pathologic & Surgical Features
+
+
+ - Debris, exudates within subarachnoid spaces and ventricles
+ - Parenchymal infarction/encephalomalacia; luminal narrowing of vessels
+- ### Microscopic Features
+
+
+ - Inflammation of adventitia and vaso vasorum = vasculitis
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - Lethargy, poor feeding, irritability
+ - Seizures (40%) and bulging fontanelle typically late findings
+ - #### Clinical profile
+
+
+ - Newborn with sepsis
+ - Typical signs/symptoms of meningitis subtle or absent in neonate
+ - CSF analysis: ↑ WBCs, ↑ protein, ↓ glucose
+ - CSF/blood Gram stain: Gram-positive diplococci
+ - Maternal risk factors for EOD
+ - GBS colonization, GBS chorioamnionitis/bacteruria
+ - Membrane rupture > 18 hours; intrapartum fever ≥ 38°C
+ - Previous newborn with EOD; delivery at < 37 weeks
+- ### Demographics
+
+
+ - #### Age
+
+
+ - 90% of newborns with GBS EOD present within first 24 hours of life
+ - GBS LOD presents between 1-4 weeks after birth; occasionally up to 6 months
+ - #### Sex
+
+
+ - Male, preterm infants (< 37 weeks) most at risk for EOD
+ - #### Ethnicity
+
+
+ - Maternal GBS colonization rates highest in Black women
+ - #### Epidemiology
+
+
+ - 10-30% of pregnant women have asymptomatic GBS colonization of genital/gastrointestinal tract
+ - EOD in < 1% of newborns born to colonized women
+ - EOD incidence: 0.5/1,000 live births
+ - Incidence ↓ by > 50% as result of maternal screening and intrapartum chemoprophylaxis
+ - ↓ incidence of GBS EOD accompanied by ↑ incidence of neonatal gram-negative sepsis
+ - Term infants account for 50% of GBS EOD secondary to preterm intrapartum chemoprophylaxis
+- ### Natural History & Prognosis
+
+
+ - Prognosis
+ - Mortality of EOD: Full-term newborns (2%), 34-36-weeks gestation (10%), < 33-weeks gestation (30%)
+ - Morbidity of meningitis: Neurologic sequelae (12-30%) (cortical blindness, spasticity, global intellectual disability)
+ - Preterm neonatal LOD significantly ↑ risk of cerebellar hemorrhage and lower motor scores by 3 years of age
+- ### Treatment
+
+
+ - Maternal
+ - GBS screen: Rectovaginal swab at 35-37 weeks gestation
+ - Positive maternal GBS screen or presence of other risk factors: Intrapartum IV penicillin
+ - Future strategies
+ - GBS PCR assay and rapid *Streptococcus* screen at onset of labor
+ - GBS vaccine: Ideal prevention strategy; would prevent development of antibiotic-resistant pathogens
+ - Novel hexavalent capsular polysaccharide conjugate vaccine for maternal immunization being studied
+ - Neonatal meningitis
+ - High-dose IV penicillin → ± antiepileptics → CSF diversion may be required for complicated hydrocephalus
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Image Interpretation Pearls
+
+
+ - No imaging features distinguish GBS meningitis from other neonatal meningitides
+
+ 5014f9a9-4c6d-4294-ba35-789818efbbc3
+
+## References
+
+## Selected References
+
+1. [Mynarek M et al: Mortality and neurodevelopmental outcome after invasive group B streptococcal infection in infants. Dev Med Child Neurol. 66(1):125-33, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37306102%5Bpmid%5D)
+1. [Procter SR et al: Maternal immunisation against group B streptococcus: a global analysis of health impact and cost-effectiveness. PLoS Med. 20(3):e1004068, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36917564%5Bpmid%5D)
+1. [Kartam M et al: Late-onset sepsis in preterm neonates is associated with higher risks of cerebellar hemorrhage and lower motor scores at three years of age. Oman Med J. 37(2):e368, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35441041%5Bpmid%5D)
+1. [Buurman ET et al: A novel hexavalent capsular polysaccharide conjugate vaccine (GBS6) for the prevention of neonatal group B streptococcal infections by maternal immunization. J Infect Dis. 220(1):105-15, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30778554%5Bpmid%5D)
+1. [Martis JMS et al: Brain imaging can predict neurodevelopmental outcome of Group B streptococcal meningitis in neonates. Acta Paediatr. 108(5):855-64, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30256462%5Bpmid%5D)
+1. [Choi SY et al: Patterns of ischemic injury on brain images in neonatal group B Streptococcal meningitis. Korean J Pediatr. 61(8):245-52, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30130950%5Bpmid%5D)
+1. [Simonsen KA et al: Early-onset neonatal sepsis. Clin Microbiol Rev. 27(1):21-47, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24396135%5Bpmid%5D)
+1. [Tan YC et al: Treatment strategies for central nervous system infections: an update. Expert Opin Pharmacother. 1-17, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25328149%5Bpmid%5D)
+1. [Baker CJ: The spectrum of perinatal group B streptococcal disease. Vaccine. 31 Suppl 4:D3-6, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23973344%5Bpmid%5D)
+1. [Berardi A et al: Group B streptococcus late-onset disease: 2003-2010. Pediatrics. 131(2):e361-8, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23296441%5Bpmid%5D)
+1. [Hernández MI et al: Stroke patterns in neonatal group B streptococcal meningitis. Pediatr Neurol. 44(4):282-8, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21397170%5Bpmid%5D)
+1. [Thigpen MC et al: Bacterial meningitis in the United States, 1998-2007. N Engl J Med. 364(21):2016-25, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21612470%5Bpmid%5D)
+1. [McCourt EA et al: Isolated group B streptococcal endogenous endophthalmitis simulating retinoblastoma or persistent fetal vasculature in a healthy full-term infant. J AAPOS. 14(4):352-5, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20637664%5Bpmid%5D)
+1. [Centers for Disease Control and Prevention (CDC): Trends in perinatal group B streptococcal disease - United States, 2000-2006. MMWR Morb Mortal Wkly Rep. 58(5):109-12, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19214159%5Bpmid%5D)
+1. [Hamada S et al: Neonatal group B streptococcal disease: incidence, presentation, and mortality. J Matern Fetal Neonatal Med. 21(1):53-7, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18175244%5Bpmid%5D)
+1. [Yikilmaz A et al: Sonographic findings in bacterial meningitis in neonates and young infants. Pediatr Radiol. 38(2):129-37, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=17611750%5Bpmid%5D)
+1. [Heath PT et al: Perinatal group B streptococcal disease. Best Pract Res Clin Obstet Gynaecol. 21(3):411-24, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17336588%5Bpmid%5D)
+1. [Smirniotopoulos JG et al: Patterns of contrast enhancement in the brain and meninges. Radiographics. 27(2):525-51, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17374867%5Bpmid%5D)
+1. [Miyairi I et al: Group B streptococcal ventriculitis: a report of three cases and literature review. Pediatr Neurol. 34(5):395-9, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16648002%5Bpmid%5D)
+1. [American College of Nurse-Midwives. Related Articles et al: Early-onset group B strep infection in newborns: prevention and prophylaxis Number 2, April 2003 (replaces Clinical Bulletin number 2, January 1997). J Midwifery Womens Health. 48(5):375-81, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14526361%5Bpmid%5D)
+1. [Heath PT et al: Neonatal meningitis. Arch Dis Child Fetal Neonatal Ed. 88(3):F173-8, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12719388%5Bpmid%5D)
+1. [Stevens JP et al: Long term outcome of neonatal meningitis. Arch Dis Child Fetal Neonatal Ed. 88(3):F179-84, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12719389%5Bpmid%5D)
+1. [Doran KS et al: Late-onset group B streptococcal infection in identical twins: insight to disease pathogenesis. J Perinatol. 22(4):326-30, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12032798%5Bpmid%5D)
+1. [Gotoff SP: Group B streptococcal infections. Pediatr Rev. 23(11):381-6, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12415017%5Bpmid%5D)
+1. Barkovich AJ: Infections of the nervous system. In Pediatric Neuroimaging. Lippincott William & Wilkins. 715-70, 2000
+
+## Anatomy
+
+### Cranial Meninges
+Brain/ANATOMY:3210cf7d-5be6-4741-a11d-dd7f8c1bc286
+
+### Limbic Network
+Brain/ANATOMY:e1a20b61-b2c1-44c5-ba04-59843855bfef
+
+### Basal Ganglia
+Brain/ANATOMY:a9de3815-ec59-4c78-adf0-94974065a7e3
+
+## Cases
+
+- {'cases': [{'authors': [{'key': '196f55e2-14b6-4728-8de3-a59e54a37434', 'value': 'Anna Illner, MD'}, {'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '4eb3d230-92c1-4a77-b382-6214659d79a4', 'description': 'Subdural empyema complicating group B streptococcal meningitis. Axial T2W MR (#1) demonstrates small, bifrontal hyperintense subdural collections and focal area of hyperintensity in the left frontal subcortical white matter (open arrow). Axial T1W MR (#2) demonstrates hypointensity in the corresponding areas. Following contrast administration (#3, 4) thick, linear enhancement is seen over the surface of the left hemisphere (arrows) in addition to diffuse dural enhancement. There is diffusion restriction in the adjacent, peripheral left frontal and temporal lobe (#5, 6) with corresponding signal loss on ADC map (#7, 8). The findings are consistent with left frontal subdural empyema with cerebritis/ischemia in the adjacent brain. Tiny focus of restricted diffusion in the peripheral right frontal lobe (arrows, #5, 6) suggests infection of the overlying subdural effusion. Other complications of neonatal meningitis not seen in this patient include ventriculitis, and arterial and venous occlusions with infarctions.', 'history': None, 'imagePoolId': 'caeb122b-82a3-455b-bfe9-d7a988863c6e', 'name': 'Subdural empyema', 'teachingPoint': None, 'demographics': '4 Weeks old '}, {'authors': [{'key': '196f55e2-14b6-4728-8de3-a59e54a37434', 'value': 'Anna Illner, MD'}, {'key': 'e8af6d26-3aad-47c9-9083-5128aab09af2', 'value': 'Susan I. Blaser, MD, FRCPC'}], 'caseVersionId': '56a9cfda-0613-43e4-92e7-e7c81d39d19c', 'description': 'Axial T1W (#1), T2W (#2), and FLAIR (#3) MR images show focal, bilateral hyperintensity in the hypothalamus and basal ganglia. There is associated diffusion restriction (#4). Focal, small vessel infarctions occur in bacterial meningitis either secondary to direct inflammation of the vessels (vasculitis), or exudate-related vasospasm of the vessels in the basilar cisterns. \n\nFollow-up MR (#5, 6) two weeks later shows focal volume loss and gliosis of the involved basal ganglia and hypothalamus as well as generalized hemispheric volume loss. In addition, there is new diffuse left hemispheric T2 hyperintensity. MRA (#7) discloses focal attenuation of the left terminal internal carotid artery (arrow) indicating vasospasm related ischemia.', 'history': 'Seizures, encephalopathy.', 'imagePoolId': 'ae702590-4ea3-40d6-b5bb-7b6a2f2d4bf0', 'name': 'Vascular spasm', 'teachingPoint': None, 'demographics': '3 Months old male'}, {'authors': [{'key': '196f55e2-14b6-4728-8de3-a59e54a37434', 'value': 'Anna Illner, MD'}, {'key': 'e8af6d26-3aad-47c9-9083-5128aab09af2', 'value': 'Susan I. Blaser, MD, FRCPC'}], 'caseVersionId': 'b9a48d88-0410-4821-a0bb-e551810d9098', 'description': 'MRI performed at three months of age during acute presentation demonstrates T2 (#2, 3), and FLAIR (#1) hyperintensity and swelling in the peripheral left temporal and frontal lobes. Note few, ill-defined foci of hypointensity within cortex (arrows, #2, 3) likely representing laminar necrosis. The subarachnoid space over the convexities, left greater than right, and ventricular system are expanded (#3) with mild diffuse dural enhancement following contrast administration (#5). The combination of findings is compatible with meningitis complicated by communicating hydrocephalus and cerebritis. Note diffusion restriction within the areas of cerebritis in the left hemisphere (#4). \n\nMRI performed 5 months later (#6, 7) demonstrates expected sequelae with encephalomalacia, gliosis and ex-vacuo dilatation of the left lateral ventricle. Communicating hydrocephalus also likely contributes to ventricular enlargement.', 'history': 'Seizures, encephalopathy.', 'imagePoolId': '311d00f2-5304-40b1-9132-c58fe73c07ee', 'name': 'Early and follow-up', 'teachingPoint': None, 'demographics': '3 Months old male'}, {'authors': [{'key': 'e8af6d26-3aad-47c9-9083-5128aab09af2', 'value': 'Susan I. Blaser, MD, FRCPC'}], 'caseVersionId': '3d29ae83-dd7c-4007-8521-de20ad72be03', 'description': 'CECT reveals a large right MCA distribution infarction (arrows). Note the low attenuation defect of the vermis (curved arrow). MRI was performed several hours later and demonstrates an additional left posterior MCA distribution (open arrow) infarction. Note the extensive loss of the cortical ribbon on T2W image involving the right hemisphere and the left Perisylvian cortex.\n\nDWI shows diffuse, subtle right thalamic restriction and multiple tiny foci of restriction in addition to the bilateral larger MCA infarctions. MRA confirms loss of the right MCA artery (arrow). The left transverse sinus (arrow) also has a flow disturbance.\n\nLate onset group B streptococcal (GBS) meningitis presents within the first month of life, and occasionally as late as 6 months. Neurological sequelae are devastating. Vasculitis is common, with venous or arterial infarction occurring in 30%.', 'history': 'Acute deterioration in a previously well neonate.', 'imagePoolId': '645ec818-7e8e-4070-84f0-a9ca512f1c56', 'name': 'Vascular occlusions', 'teachingPoint': None, 'demographics': '3 Weeks old female'}], 'caseType': 'typical', 'name': 'TYPICAL'}
+- {'cases': [{'authors': [{'key': '196f55e2-14b6-4728-8de3-a59e54a37434', 'value': 'Anna Illner, MD'}, {'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '6a7ac439-53b2-42d4-9be4-4076fd5a0f84', 'description': 'Subacute Group B streptococcus infection. Axial NECT demonstrates bifrontal decreased white matter attenuation (arrows, #1). This corresponds to focal bifrontal T2 hyperintensity (arrow, #2) with several discrete cysts within the areas of hyperintensity, worse on the right (arrows, #3-6). The smaller cysts on the left have a hypointense rim (#3) suggestive of abscess. The large collection of cysts on the right appears retracted. The findings are consistent with the sequelae of cerebritis with evolution of white matter abscess cavities with cystic encephalomalacia on the right. Note punctate T2 hypointensities along septations (curved arrow, #5) and ventricular ependyma (curved arrows, #3-4) and T1 hyperintensity along the superior margin of frontal white matter signal abnormality (#9). These foci could either represent hemorrhage or dystrophic calcification; the latter is favored given the late-stage, encephalomalacic cavity in the right frontal white matter. FLAIR (#7) and DWI (#8) images confirm development of cystic encephalomalacia. Abscess formation is unusual in Group B streptococcal meningitis/cerebritis. The imaging findings are reminiscent of Citrobacter infection, where abscess formation is common.', 'history': None, 'imagePoolId': '4047708f-033c-454b-8455-87976a05d399', 'name': 'Abscess formation', 'teachingPoint': None, 'demographics': '24 Days old male'}], 'caseType': 'variant', 'name': 'VARIANT'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*Axial DWI MR in a 35-day-old infant with group B Streptococcal (GBS) meningoencephalitis shows bifrontal
, insular
, occipital
cortical and right thalamic
diffusion restricting encephalitis. Bifrontal hypointensities
were blood products on SWI (not shown).*
+
+
+*Axial DWI MR in a 35-day-old infant with group B Streptococcal (GBS) meningoencephalitis shows bifrontal
, insular
, occipital
cortical and right thalamic
diffusion restricting encephalitis. Bifrontal hypointensities
were blood products on SWI (not shown).*
+
+
+*Axial T2WI MR in the same patient at 55 days of age shows bifrontal
, right temporal
, insular
, and tiny focal right occipital
cortical cystic encephalomalacia with volume loss and dilated ventricles.*
+
+
+*Axial SWI MR at 55 days of age shows scattered, blooming, hypointense blood in cortical encephalomalacia
and extraaxial spaces
.*
+
+
+*Axial T1WI MR in a 9-day-old infant with GBS meningoencephalitis shows a solitary tiny speck
of right periventricular white matter (WM) T1 hyperintensity (prominently seen within unmyelinated T1-hypointense WM). This speck was hypointense on T2WI and diffusion restricting on DWI/ADC, suggesting periventricular WM injury due to ischemia.*
+
+
+*Axial T1WI C+ MR in an infant with GBS meningitis shows cortical
and leptomeningeal
enhancement. CSF Gram stain showed gram-positive diplococci.*
+
+
+*Axial FLAIR MR shows hyperintensity in the right frontal cortex
and within the right convexity subarachnoid spaces
. Note the early involvement of the left frontal subarachnoid space
. FLAIR MR is a sensitive tool to detect early complicated extraaxial fluid collections and FLAIR C+ for meningeal enhancement.*
+
+
+*Axial DWI MR in a patient with GBS meningitis shows restricted diffusion
in the subdural collections, consistent with empyemas. DWI helps to differentiate reactive subdural effusions (facilitated diffusion) from empyemas (restricted diffusion).*
+
+
+*Axial ADC with GBS meningoencephalitis shows multiple basal ganglia
and thalamic
infarctions. Note the scattered WM infarctions
.*
+
+
+*Axial T1WI C+ MR shows extensive leptomeningeal enhancement
. Note the ventricular ependymal enhancement
, dependent ventricular debris
(ventriculitis), and basal ganglia enhancement
due to perivascular space inflammation/arteritis. Ventricular dilation reflects early hydrocephalus.*
+
+
+*Sagittal MRV with contrast shows a hypointense clot
within the sagittal sinus, creating a partial occlusion. Small clots were also detected in the transverse sinuses.*
+
+
+### Additional Images
+
+
+*Axial T2WI MR in a child with GBS meningoencephalitis demonstrates focal basal ganglia
, thalamic
, and WM
hyperintensities, reflecting acute infarction secondary to perivascular space inflammatory involvement/arteritis. DWI showed diffusion restriction of the infarcts (not shown).*
+
+
+*Axial DWI MR in a 3-month-old infant with GBS meningoencephalitis shows bilateral frontotemporal cortical diffusion restriction
, which could be due to encephalitis or infarcts.*
+
+
+*Axial T2WI MR shows hydrocephalus and deep
and peripheral
arterial infarcts. Small bifrontal subdural empyemas
are barely visible.*
+
+
+*Axial CECT in the same patient shows hydrocephalus and hypodense deep and peripheral infarcts. Bifrontal, thick, rim-enhancing subdural empyemas
are easily seen.*
+
+
+*Axial T1WI MR shows ill-defined, dependent, hypointense debris within the posterior horns of the lateral ventricles
.*
+
+
+*Axial DWI MR in the same patient shows diffusion restriction
within the dependent intraventricular inflammatory exudate (pus).*
+
+
+*Axial T2WI MR shows multiple tiny, hyperintense foci within the basal ganglia
and internal capsules
. There is blurring of the bifrontal and bioccipital gray matter-WM junction with hyperintense cortex
. Focal cortical ↑ T2 in encephalitis (or infarcts) continuous with neonatal normally ↑ T2 subcortical WM looks like focally "missing cortical ribbon"
.*
+
+
+*Axial DWI MR shows diffusion restriction within multiple tiny basal ganglia
, internal capsules
, peripheral bioccipital cortical/subcortical
, and bifrontal lobe cortical
infarcts.*
+
+
+*Circle of Willis MRA shows occlusion of the right middle cerebral artery
. There is irregularity of the right distal internal carotid artery
and proximal right middle cerebral artery
.*
+
+
+*Axial DWI MR shows diffusion restriction within the right middle cerebral artery territory
and left perisylvian cortex
in a patient with right middle cerebral artery occlusion.*
+
+
+*Axial FLAIR MR with IV contrast in a neonate with group B streptococcal meningitis shows asymmetric hyperintensity in supratentorial subarachnoid spaces
and cortical hyperintensity
. Note the hyperintense subdural collection
that had restricted diffusion (not shown) on DWI (empyema).*
+
+
+*Coronal T1WI C+ MR shows bilateral frontal convexity subdural effusions
that had restricted diffusion on DWI (not shown). Effusions typically regress with appropriate IV therapy.*
+
diff --git a/docs_md/articles/hemifacial-spasm_5b8be233-f227-4365-9e58-df57a7950b29.md b/docs_md/articles/hemifacial-spasm_5b8be233-f227-4365-9e58-df57a7950b29.md
new file mode 100644
index 0000000..5e8ad74
--- /dev/null
+++ b/docs_md/articles/hemifacial-spasm_5b8be233-f227-4365-9e58-df57a7950b29.md
@@ -0,0 +1,364 @@
+---
+title: "Hemifacial Spasm"
+docid: "5b8be233-f227-4365-9e58-df57a7950b29"
+authors:
+ - key: "eef2f839-5706-47b9-89c3-60d8315b2b3a"
+ value: "Nicholas A. Koontz, MD"
+breadcrumbs:
+ -
+ name: "Head and Neck"
+ slug: "head-and-neck"
+ treeNodeId: "5c1f8e17-7acd-48d8-9d55-f9f8c2cad850"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "4adbe5f4-083b-4e3e-b7ea-c0ed68a73a6f"
+ -
+ name: "CPA-IAC"
+ slug: "cpa-iac"
+ treeNodeId: "8a2b8a8e-1cf0-4524-bc5f-925455c1538a"
+ -
+ name: "Vascular Lesions"
+ slug: "vascular-lesions"
+ treeNodeId: "0820e71d-ab9e-4713-9ebb-67b9d60671fa"
+ -
+ name: "Hemifacial Spasm"
+ slug: "hemifacial-spasm"
+ treeNodeId: null
+category: "Head and Neck"
+documentVersionId: "051ac1e7-c083-45b4-a01f-406d0e2486a4"
+imageCount: 36
+lastUpdated: "04/13/26"
+pageDescription: "Hemifacial Spasm"
+pageKeywords: "Head and Neck, Diagnosis, CPA-IAC, Vascular Lesions, Hemifacial Spasm"
+pageTitle: "Hemifacial Spasm | STATdx"
+enhancedTitle: "Hemifacial Spasm"
+type: "DDX"
+references: true
+breadcrumbs:
+ - "Head and Neck"
+ - "Diagnosis"
+ - "CPA-IAC"
+ - "Vascular Lesions"
+ - "Hemifacial Spasm"
+---
+## ESSENTIAL INFORMATION
+
+- ### Key Differential Diagnosis Issues
+
+
+ - Overall statistics
+ - In > 95% of cases, **arterial vascular loop** is cause of hemifacial spasm (HFS)
+ - All other causes listed account for < 5% of cases
+ - HFS
+ - Definition: Segmental myoclonus of muscles of face innervated by facial nerve
+ - Presentation: Patients 50-80 years old, unilateral
+ - Begins around eye, spreads gradually to other facial muscles
+ - Principal symptom: Rhythmic, involuntary, myoclonic facial muscle contractions
+ - Pathophysiology: Irritation of CNVII or facial nucleus
+ - Vascular loop syndrome affecting CNVII (a.k.a. primary HFS)
+ - By far most common cause of HFS
+ - Aberrant or ectatic vessels in cistern
+ - Anterior inferior cerebellar artery (AICA) most common offending artery (40-50%)
+ - Other less common causal vessels include posterior inferior cerebellar artery (PICA) (~ 30%), vertebral artery (VA) (~ 20%), or large vein (< 5%)
+ - Multivessel impingement is frequent (~ 40%)
+ - High-resolution MR-MRA routinely identifies compressive aberrant or ectatic arteries
+ - 3D-T2 SPACE, CISS, or FIESTA sequences most commonly employed
+ - Critical to recognize that vascular contact of facial nerve is very common (~ 50% of population) with only small minority (< 0.01%) manifesting HFS
+ - **Absent HFS symptoms, this is incidental finding that should be ignored and not reported!**
+- ### Helpful Clues for Common Diagnoses
+
+
+ - **Vascular Loop Syndrome Affecting CNVII**
+ - Negative high-resolution MR exam does not preclude surgery for smaller vascular loop causing HFS
+ - High-resolution MR makes this far less common
+ - Imaging findings
+ - MR-MRA: Asymmetric looping artery impinges on CNVII in CPA
+ - Root exit zone and attached segment (where CNVII is adherent to pons) are most sensitive to neurovascular compression
+ - AICA > PICA > VA > venous, though all are possible
+- ### Helpful Clues for Less Common Diagnoses
+
+
+ - **Epidermoid Cyst in CPA**
+ - Morphology: Assumes shape of cistern it occupies
+ - Insinuating margins, encasing cranial nerves and vessels
+ - Imaging findings
+ - Near CSF signal intensity of epidermoid cyst makes it difficult to see on T1, T2, and FLAIR sequences
+ - Light bulb bright on DWI
+ - **Meningioma in CPA**
+ - Morphology: Dural-based sessile mass
+ - Imaging findings
+ - Bone CT: Bony hyperostosis possible
+ - MR: Enhancing mass with dural tail(s)
+ - **Aneurysm in CPA****-IAC**
+ - Morphology: Ovoid or fusiform shape
+ - Imaging findings
+ - MR: Complex lesion signal from wall calcification, clot, and flow
+ - **Facial Nerve Schwannoma in CPA-IAC**
+ - Morphology: CPA-IAC "ice cream on cone" mass and labyrinthine segment tail
+ - Imaging findings
+ - Bone CT: Labyrinthine segment CNVII enlarged
+ - MR: Enhancing tubular mass; may have intramural cysts when large
+ - **Facial Nerve Schwannoma in T-Bone**
+ - Morphology: Tubular mass within enlarged facial nerve canal may pedunculate into middle ear cavity (tympanic segment CNVII) or mastoid air cells (mastoid segment CNVII)
+ - Imaging findings
+ - Bone CT: Smooth enlargement of CNVII canal; geniculate ganglion most commonly affected
+ - MR: Enhancing mass enlarges bony facial nerve canal
+ - **Facial Nerve Perineural Tumor**
+ - Morphology: Enlargement of intratemporal CNVII connected through stylomastoid foramen (usually from invasive parotid malignancy)
+ - Imaging findings
+ - Mastoid segment most common
+ - CT: Soft tissue replacement of fat at stylomastoid foramen ± enlargement of bony CNVII canal
+ - MR: Enhancing minimally enlarged intratemporal CNVII
+ - **Facial Nerve Venous Malformation ("Hemangioma") in T-Bone**
+ - True low-flow vascular malformation, **not**tumor
+ - Old hemangioma terminology should be avoided
+ - Morphology: Amorphous geniculate ganglion mass
+ - Imaging findings
+ - Bone CT: "Honeycomb" bone matrix (50%)
+ - MR: Avidly enhancing mass with foci of low signal intensity from calcifications
+- ### Helpful Clues for Rare Diagnoses
+
+
+ - **A****cute****Cerebral Ischemia-Infarction**
+ - Acute onset of brainstem-related symptoms
+ - Pontine cerebrovascular accident (CVA) secondary to basilar artery perforator injury
+ - Imaging findings
+ - MR: DWI shows reduced diffusivity in pons
+ - **Multiple Sclerosis**
+ - HFS is rare presentation of multiple sclerosis
+ - Imaging findings
+ - MR: T2-/FLAIR hyperintense white matter lesions; evaluate lateral pontine tegmentum (location of CNVII nuclei and intraaxial segments)
+ - Lesions with active or recent demyelination may show incomplete rim of enhancement
+ - **Arteriovenous Malformation**
+ - More commonly supratentorial
+ - Imaging findings
+ - MR: Large, ectatic arterial flow voids
+ - Enhancing nidus on T1 C+ FS sequence
+ - Large draining veins
+ - **Arachnoid Cyst in CPA**
+ - More common than epidermoid cyst in CPA, but epidermoid cyst in CPA more often associated with HFS
+ - Morphology
+ - Fills cistern with rounded or flat margins
+ - Imaging findings
+ - T1 C+: No enhancement
+ - FLAIR: Follows dark CSF signal intensity
+ - DWI: No reduced diffusivity
+ - **Venous Malformation ("Hemangioma") in IAC**
+ - Lesion of abnormal vascular morphogenesis; "hemangioma" is misnomer and should **not** be used
+ - Morphology: Distal intracanalicular (IAC) ovoid to round cystic mass
+ - Imaging findings
+ - Bone CT: Lesion with punctate calcifications
+ - MR: Avidly enhancing IAC lesion with foci of low signal intensity from calcifications
+- ### Alternative Differential Approaches
+
+
+ - Radiologist generally searches for cause of cranial neuropathy by following cranial nerve from origin to functional endplate
+ - Such anatomic approach permits segmentation of potential causes into anatomic groups
+ - Anatomic delineation of HFS causes
+ - 3 general anatomic sites where facial nerve may be injured causing HFS
+ - Intraaxial (nuclear)
+ - Cisternal (CPA or IAC cistern)
+ - Intratemporal (intratemporal facial nerve canal)
+ - Intraaxial (nuclear)
+ - [Acute cerebral ischemia-infarction](/document/acute-cerebral-ischemiainfarction/7a3ed4a9-ae05-4d64-ae8e-6a30105501e1)
+ - [Multiple sclerosis](/document/multiple-sclerosis/abe95a5e-394f-411b-aca6-72ab160a1d0d)
+ - [Arteriovenous malformation](/document/arteriovenous-malformation/84792183-949d-4b25-a586-572dbe2e11a2)
+ - Cisternal (CPA or IAC cistern)
+ - Vascular loop syndrome affecting CNVII
+ - [Epidermoid cyst in CPA](/document/cpa-iac-epidermoid-cyst/be3a0a82-4db6-49d9-a5f6-03c5bbe328d0)
+ - [Meningioma in CPA](/document/cpa-iac-meningioma/6b77ba6f-ceb0-43f7-bc36-ef3cbeddc7ae)
+ - [Aneurysm in CPA-IAC](/document/cpa-iac-aneurysm/6dbb0a1c-2124-4483-a5ee-143ef769817d)
+ - [Facial nerve schwannoma in CPA-IAC](/document/cpa-iac-facial-nerve-schwannoma/9e47ebb0-b89d-48d6-9ac9-54f946ac66a0)
+ - [Arachnoid cyst in CPA](/document/cpa-iac-arachnoid-cyst/d7ad00be-873e-4397-a8ed-78b5c7445b32)
+ - [Venous malformation ("hemangioma") in IAC](/document/iac-venous-malformation/3c6d2e04-88fa-412e-b8fc-dfb773a74f9d)
+ - Intratemporal (intratemporal CNVII canal)
+ - [Facial nerve schwannoma in T-bone](/document/temporal-bone-facial-nerve-schwann-/13c97224-d062-45b2-8ef9-df32b97a6f32)
+ - Facial nerve perineural tumor
+ - [Facial nerve venous malformation ("hemangioma") in T-bone](/document/temporal-bone-facial-nerve-venous--/86356905-9bc4-41af-9965-e0d48658c1f2)
+
+## References
+
+## Selected References
+
+1. [Bonomo R et al: Evaluating the diagnostic role of magnetic resonance imaging in trigeminal neuralgia, hemifacial spasm, and glossopharyngeal neuralgia: a controlled blinded study. Brain Spine. 5:105607, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=41141967%5Bpmid%5D)
+1. [Jesuthasan A et al: Hemifacial spasm: an update on pathophysiology, investigations and management. J Neurol. 272(8):502, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=40640398%5Bpmid%5D)
+1. [Kościołek D et al: The anterior inferior cerebral artery variability in the context of neurovascular compression syndromes: a narrative review. Biomedicines. 12(2), 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38398054%5Bpmid%5D)
+1. [Tanrikulu L: Clinical and educational aspects of neuroimaging in microsurgery of the posterior fossa: a comprehensive review. Cureus. 16(5):e60730, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38903320%5Bpmid%5D)
+1. [Malicki M et al: The superior cerebellar artery: variability and clinical significance. Biomedicines. 11(7), 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37509648%5Bpmid%5D)
+1. [Vilanilam GK et al: Compressive lesions of the head and neck: common and uncommon must-know entities. Neuroradiol J. 37(2):164-77, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37026517%5Bpmid%5D)
+1. [Hermier M: Imaging of hemifacial spasm. Neurochirurgie. 64(2):117-23, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29705020%5Bpmid%5D)
+1. [Donahue JH et al: Imaging of vascular compression syndromes. Radiol Clin North Am. 55(1):123-38, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27890181%5Bpmid%5D)
+1. [Deep NL et al: Magnetic resonance imaging assessment of vascular contact of the facial nerve in the asymptomatic patient. J Neurol Surg B Skull Base. 77(6):503-9, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27857878%5Bpmid%5D)
+1. [Haller S et al: Imaging of neurovascular compression syndromes: trigeminal neuralgia, hemifacial spasm, vestibular paroxysmia, and glossopharyngeal neuralgia. AJNR Am J Neuroradiol. 37(8):1384-92, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26892985%5Bpmid%5D)
+1. [Öcal R et al: Comparison of brain MRI angiography and brain MRI cisternography in patients with hemifacial spasm. Acta Neurol Belg. 116(4):593-8, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26908032%5Bpmid%5D)
+1. [Ray DK et al: Surgical outcome and improvement in quality of life after microvascular decompression for hemifacial spasms: a case series assessment using a validated disease-specific scale. Stereotact Funct Neurosurg. 88(6):383-9, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20948243%5Bpmid%5D)
+1. [Pyen JS et al: Tic convulsif caused by cerebellopontine angle schwannoma. Yonsei Med J. 2001 Apr;42(2):255-7. Retraction in: Yonsei Med J. 49(6):1060, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=19108035%5Bpmid%5D)
+1. [Desai K et al: Cerebellopontine angle epidermoid tumor presenting with hemifacial spasms. Neurol India. 51(2):288-9, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14571040%5Bpmid%5D)
+1. [Iwai Y et al: Hemifacial spasm due to cerebellopontine angle meningiomas--two case reports. Neurol Med Chir (Tokyo). 41(2):87-9, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11255633%5Bpmid%5D)
+1. [Takano S et al: Facial spasm and paroxysmal tinnitus associated with an arachnoid cyst of the cerebellopontine angle--case report. Neurol Med Chir (Tokyo). 38(2):100-3, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9557537%5Bpmid%5D)
+1. [Illingworth RD et al: Hemifacial spasm: a prospective long-term follow up of 83 cases treated by microvascular decompression at two neurosurgical centres in the United Kingdom. J Neurol Neurosurg Psychiatry. 60(1):72-7, 1996](http://www.ncbi.nlm.nih.gov/pubmed/?term=8558156%5Bpmid%5D)
+1. [Moriuchi S et al: Hemifacial spasm due to compression of the facial nerve by vertebral artery-posterior inferior cerebellar artery aneurysm and elongated vertebral artery--case report. Neurol Med Chir (Tokyo). 36(12):884-7, 1996](http://www.ncbi.nlm.nih.gov/pubmed/?term=9002718%5Bpmid%5D)
+1. [Nagata S et al: Hemifacial spasm caused by CP angle AVM associated with ruptured aneurysm in the feeding artery--case report. Neurol Med Chir (Tokyo). 31(7):406-9, 1991](http://www.ncbi.nlm.nih.gov/pubmed/?term=1720219%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+**Vascular Loop Syndrome Affecting CNVII**
+*Axial 3D T2 SPACE MR in a patient with left hemifacial spasm (HFS) shows focal neurovascular compression of the left facial nerve
by an anterior inferior cerebellar artery (AICA) loop
at the root exit zone (REZ), resulting in a bowstring appearance. AICA is the most common culprit vessel in HFS.*
+
+
+**Vascular Loop Syndrome Affecting CNVII**
+*Axial 3D T2 SPACE MR in a patient with left hemifacial spasm (HFS) shows focal neurovascular compression of the left facial nerve
by an anterior inferior cerebellar artery (AICA) loop
at the root exit zone (REZ), resulting in a bowstring appearance. AICA is the most common culprit vessel in HFS.*
+
+
+**Vascular Loop Syndrome Affecting CNVII**
+*Axial CISS MR in a patient with left HFS shows focal neurovascular compression of the left CNVII and CNVIII complex
at the REZ
by a tortuous loop of the vertebral artery
, a less common culprit vessel in HFS.*
+
+
+**Epidermoid Cyst in CPA**
+*Axial DWI trace MR in a patient with left HFS shows the characteristic appearance of a cerebellopontine angle (CPA) epidermoid cyst
, which is a light bulb bright, lobular mass on diffusion imaging. Epidermoid cysts are similar to CSF signal intensity on other pulse sequences.*
+
+
+**Meningioma in CPA**
+*Axial T1 C+ FS MR in a patient with left HFS shows a large, enhancing extraaxial mass
centered in the left CPA with extension into the internal auditory canal (IAC)
. Note a conspicuous dural tail of enhancement
associated with this large meningioma.*
+
+
+**Aneurysm in CPA-IAC**
+*Axial T1 C+ FS MR of a right posterior inferior cerebellar artery (PICA) saccular aneurysm
shows a round, complex signal mass centered in the right CPA. Note a PICA flow void
extending into the aneurysm with jet of contrast opacification
centrally and peripheral filling defects
of this partially thrombosed aneurysm.*
+
+
+**Facial Nerve Schwannoma in CPA-IAC**
+*Axial SPGR C+ MR of a left facial nerve schwannoma
shows an avidly enhancing CPA-IAC mass with a labyrinthine tail of enhancement
extending to the geniculate ganglion
, which differentiates it from a vestibular schwannoma.*
+
+
+**Facial Nerve Schwannoma in T-Bone**
+*Axial T1 C+ FS MR of a right facial nerve schwannoma shows a tubular, somewhat pedunculated enhancing mass centered at the anterior genu
, which extends through the widened labyrinthine segment facial nerve canal
into the IAC fundus
.*
+
+
+**Facial Nerve Perineural Tumor**
+*Coronal T1 MR shows invasive parotid space malignancy
with perineural tumor spread cephalad through the stylomastoid foramen to involve the mastoid segment of the facial nerve
.*
+
+
+**Facial Nerve Venous Malformation ("Hemangioma") in T-Bone**
+*Axial bone CT shows a CNVII venous malformation
centered at the geniculate fossa with characteristic "honeycomb" matrix. The lesion extends toward the labyrinthine segment
of CNVII.*
+
+
+**Acute Cerebral Ischemia-Infarction**
+*Axial DWI MR shows an acute left pontine infarction
with reduced diffusivity. Corresponding hypointensity was present on the ADC map (not shown). Patients with brainstem infarctions may develop hemifacial spasm in the subacute to chronic phase.*
+
+
+**Multiple Sclerosis**
+*Axial T1 SPGR C+ MR in a patient with multiple sclerosis shows incomplete ring enhancement
of a demyelinating plaque
in the lateral pons, indicating active or recent demyelination. This is in the expected location of the CNVII motor nucleus.*
+
+
+**Arteriovenous Malformation**
+*Axial CTA MIP of a ruptured arteriovenous malformation shows a tangle of vessels
in the left CPA with large intranidal aneurysm
. Note early opacification of the left transverse sinus
due to arterial-to-venous shunting as well as intraventricular hemorrhage
.*
+
+
+**Arachnoid Cyst in CPA**
+*Axial T2 FS MR shows a right CPA arachnoid cyst
, which exerts mild mass effect upon the cisternal segments of the vestibulocochlear and facial nerves
. The cyst also flattens the lateral cerebellum. This cyst follows CSF signal intensity on all sequences.*
+
+
+**Venous Malformation ("Hemangioma") in IAC**
+*Coronal T1 C+ MR of an lAC venous malformation shows a lateral IAC enhancing mass
with focus of internal low signal intensity from punctate intralesional calcification
.*
+
+
+### Additional Images
+
+
+**Vascular Loop Syndrome Affecting CNVII**
+*Axial T2 FS MR in a patient with left HFS shows neurovascular compression of the left facial nerve
by an ectatic vertebral artery
. Note the point of neurovascular impingement
against the adjacent cerebellar flocculus
.*
+
+
+**Vascular Loop Syndrome Affecting CNVII**
+*Coronal T2 MR shows an ectatic vertebral artery
"lifting" the posterior inferior cerebellar artery into the REZ of the facial nerve
. Note compression of the lateral pons.*
+
+
+**Vascular Loop Syndrome Affecting CNVII**
+*Axial T2 MR shows a markedly asymmetric left vertebral artery
lifting the PICA
into the medial aspect of the CPA cistern in the CNVII REZ vicinity.*
+
+
+**Epidermoid Cyst in CPA**
+*Axial DWI MR in a patient with chronic left HFS shows a left CPA mass
with reduced diffusivity and scalloped, insinuating margins, typical of an epidermoid cyst.*
+
+
+**Epidermoid Cyst in CPA**
+*Axial 3D-T2 SPACE MR in a patient with left HFS shows a large epidermoid cyst
that completely fills the left CPA, insinuating and distorting the normal anatomic contents of the cistern. Epidermoid cysts appear similar to CSF signal intensity on routine pulse sequences, but judicious windowing may help delineate the lesion, as in this case. Diffusion imaging offers definitive assessment, as epidermoid cysts are light bulb bright.*
+
+
+**Epidermoid Cyst in CPA**
+*Axial T2 MR demonstrates a right CPA cistern epidermoid cyst with penetration of the porus acusticus
and lobulated mass effect on the lateral margin of the brachium pontis
.*
+
+
+**Epidermoid Cyst in CPA**
+*Axial T2 MR shows a right CPA cistern epidermoid cyst
scalloping the cerebellar contour and bowing the cisternal facial nerve anteriorly
. The REZ
is also affected.*
+
+
+**Meningioma in CPA**
+*Axial T1 C+ FS MR in a patient with right HFS shows an avidly enhancing mass with the configuration of "ice cream" (CPA component)
"on cone" (IAC component)
. Note enhancing dural tails
, which help differentiate this histologically-proven CPA-IAC meningioma from a schwannoma.*
+
+
+**Meningioma in CPA**
+*Axial T2 MR reveals a dural-based CPA mass with IAC penetration
with a "CSF-vascular cleft"
between it and the adjacent brachium pontis-pons. Note the normal REZ of the contralateral left CNVII
.*
+
+
+**Aneurysm in CPA-IAC**
+*Axial T2 FS MR shows a giant right vetebral artery aneurysm
with complex signal intensity. The aneurysm obliterates the pontomedullary junction at the region of the right CNVII REZ
. The same tortuous right vertebral artery
also effaces the left CNVII REZ region.*
+
+
+**Aneurysm in CPA-IAC**
+*Axial T1 MR shows complex signal associated with a vertebral artery aneurysm
. The aneurysm is wedged into the medial CPA cistern in the immediate vicinity of the CNVII REZ.*
+
+
+**Facial Nerve Schwannoma in CPA-IAC**
+*Axial T1 C+ MR shows a small facial nerve schwannoma of the lateral IAC
, labyrinthine segment
, and geniculate ganglion
portions of the facial nerve.*
+
+
+**Facial Nerve Schwannoma in T-Bone**
+*Axial bone CT of a right CNVII schwannoma shows typical bone changes with benign segmental expansile changes of the geniculate fossa
and labyrinthine segment
facial nerve canal. Compared to the normal contralateral facial nerve canal
.*
+
+
+**Facial Nerve Schwannoma in T-Bone**
+*Axial T1 C+ MR shows an aggressive schwannoma involving the tympanic segment
, posterior genu
, and descending mastoid segment of the right facial nerve. Note several nonenhancing intramural cysts
.*
+
+
+**Facial Nerve Schwannoma in T-Bone**
+*Axial T1 C+ MR demonstrates a mastoid segment facial nerve schwannoma
. Notice that the enhancing tumor has dehisced the posterior wall of the external auditory canal
.*
+
+
+**Facial Nerve Perineural Tumor**
+*Axial T1 C+ FS MR in a patient with right parotid carcinoma and perineural tumor spread along the facial nerve shows abnormal thickening and enhancement of the descending mastoid segment of right CNVII
. Note the normal appearance of the contralateral CNVII descending mastoid segment
. While some enhancement of this segment of CNVII is considered normal, the degree of thickening and avidity of enhancement on the right is abnormal in this case.*
+
+
+**Facial Nerve Venous Malformation ("Hemangioma") in T-Bone**
+*Axial T1 C+ FS MR shows the typical appearance of a facial nerve venous malformation with an amorphous, enhancing mass
centered at the geniculate ganglion. Note a stippled appearance with tiny dark foci within the lesion, corresponding to the "honeycomb" matrix seen to better effect on bone CT.*
+
+
+**Facial Nerve Venous Malformation ("Hemangioma") in T-Bone**
+*Axial T1 C+ FS MR shows an enhancing lesion
within enlarged geniculate fossa. Note the black central dot of low signal intensity
corresponding with punctate calcification and suggesting the diagnosis of facial nerve venous malformation.*
+
+
+**Multiple Sclerosis**
+*Axial T2 FS MR in patient with multiple sclerosis shows a subtle demyelinating plaque
in the left lateral pons near the REZ of the facial nerve
. Demyelinating lesions accounting for HFS are often subtle and may not always be seen.*
+
+
+**Multiple Sclerosis**
+*Axial T2 MR shows a multiple sclerosis plaque
situated in the lateral right pons near the REZ of the facial nerve. Note a 2nd more subtle plaque
in the left cerebellum.*
+
+
+**Arteriovenous Malformation**
+*Axial T2 MR shows a left cerebellopontine cistern arteriovenous malformation nidus
with a large posterior draining vein
.*
+
+
+**Arachnoid Cyst in CPA**
+*Axial T2 MR through the CPA cistern reveals a CSF intensity arachnoid cyst on the left
. The arachnoid cyst flattens the cerebellar hemisphere and bows the facial and vestibulocochlear nerves
anteromedially.*
+
+
+**Arachnoid Cyst in CPA**
+*Axial T2 MR through the CPA cistern reveals a CSF intensity arachnoid cyst on the left
. The arachnoid cyst flattens the cerebellar hemisphere and bows the facial and vestibulocochlear nerves
anteromedially.*
+
diff --git a/docs_md/articles/herpes-encephalitis-type-1_556f5f76-c20b-44ca-a913-c53b11c93341.md b/docs_md/articles/herpes-encephalitis-type-1_556f5f76-c20b-44ca-a913-c53b11c93341.md
new file mode 100644
index 0000000..3508723
--- /dev/null
+++ b/docs_md/articles/herpes-encephalitis-type-1_556f5f76-c20b-44ca-a913-c53b11c93341.md
@@ -0,0 +1,536 @@
+---
+title: "Herpes Encephalitis Type 1"
+docid: "556f5f76-c20b-44ca-a913-c53b11c93341"
+authors:
+ - key: "b2e6dabb-ee1c-42a4-a332-9f0814c1c607"
+ value: "Surjith Vattoth, MD"
+breadcrumbs:
+ -
+ name: "Pediatrics"
+ slug: "pediatrics"
+ treeNodeId: "40c0ffd5-41a0-4a6f-99fd-74c72c2e0bd3"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "8c45dd6b-ac86-4be1-b770-4755c799ad00"
+ -
+ name: "Pediatric Neuroradiology"
+ slug: "pediatric-neuroradiology"
+ treeNodeId: "47bf4839-9cae-4078-8a93-b14004d9eef1"
+ -
+ name: "Brain"
+ slug: "brain"
+ treeNodeId: "64b09eb7-b76b-48be-9ef0-064fb3828f85"
+ -
+ name: "Pathology-Based Diagnoses"
+ slug: "pathology-based-diagnoses"
+ treeNodeId: "827f44e9-f4d6-4bf1-814e-08372b32333f"
+ -
+ name: "Infectious Disease"
+ slug: "infectious-disease"
+ treeNodeId: "d02631a2-c8a2-484e-870b-f0fac0240d90"
+ -
+ name: "Acquired Infections"
+ slug: "acquired-infections"
+ treeNodeId: "498c7a90-4551-45e8-b4ed-406d7b76ba3c"
+ -
+ name: "Herpes Encephalitis Type 1"
+ slug: "herpes-encephalitis-type-1"
+ treeNodeId: null
+category: "Pediatrics"
+documentVersionId: "0c936f46-0304-47b3-a87a-b1484b1beab1"
+imageCount: 24
+lastUpdated: "02/06/24"
+pageDescription: "Herpes Encephalitis Type 1"
+pageKeywords: "Pediatrics, Diagnosis, Pediatric Neuroradiology, Brain, Pathology-Based Diagnoses, Infectious Disease, Acquired Infections, Herpes Encephalitis Type 1"
+pageTitle: "Herpes Encephalitis Type 1 | STATdx"
+enhancedTitle: "Herpes Encephalitis Type 1"
+type: "DX"
+references: true
+breadcrumbs:
+ - "Pediatrics"
+ - "Diagnosis"
+ - "Pediatric Neuroradiology"
+ - "Brain"
+ - "Pathology-Based Diagnoses"
+ - "Infectious Disease"
+ - "Acquired Infections"
+ - "Herpes Encephalitis Type 1"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - **HSV1** brain infection
+ - Typically reactivation in immunocompetent patients
+ - **Different from neonatal (HSV2)**herpes encephalitis
+- ### Imaging
+
+
+ - T2/FLAIR hyperintensity in **mesial temporal, subfrontal, insular, and cingular cortex** with DWI restriction
+ - Typically **bilateral** disease **but asymmetric**
+ - **Hemorrhage** typically subacute/**late**
+ - Deep gray nuclei usually spared
+ - CT often normal early
+ - MR with DWI most sensitive for early diagnosis
+ - T2/FLAIR: Cortical, subcortical hyperintensity with relative white matter sparing
+ - GRE: If hemorrhagic, hypointensity blooms
+ - DWI: Restricted diffusion in limbic system
+ - T1WI C+: May see mild, patchy enhancement early
+ - **Gyriform enhancement** usually **late**, after 1 week
+- ### Top Differential Diagnoses
+
+
+ - Acute cerebral ischemia-infarction
+ - Status epilepticus
+ - Limbic encephalitis
+ - Infiltrating neoplasm
+- ### Pathology
+
+
+ - Hemorrhagic, necrotizing HSV1 encephalitis
+- ### Clinical Issues
+
+
+ - Common presentation: Fever, headache, seizures, ± viral prodrome
+ - Children often present with nonspecific symptoms
+ - PCR of CSF most accurate diagnosis
+ - HSV1 causes 95% of all herpetic encephalitis
+ - Start IV acyclovir immediately if herpes simplex encephalitis suspected
+- ### Diagnostic Checklist
+
+
+ - CT often normal early; MR with FLAIR/DWI most sensitive for early diagnosis
+
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - Herpes simplex encephalitis (HSE)
+- ### Definitions
+
+
+ - Older children and adults: Brain parenchyma infection caused by **HSV1**
+ - Typically reactivation in immunocompetent patients
+ - Different from neonatal herpes encephalitis
+ - **Neonates and infants: HSV2**
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Abnormal signal in **mesial temporal, subfrontal, insular, and cingular cortex** with DWI restriction
+ - **Bilateral but asymmetric** with **late hemorrhage**
+ - #### Location
+
+
+ - HSV1: Limbic system: Temporal lobes, insula, subfrontal area, cingulate gyri typical
+ - Cerebral convexity, posterior occipital cortex may become involved
+ - Typically bilateral disease but asymmetric
+ - Basal ganglia usually spared
+ - Rarely in midbrain and pons (mesenrhombencephalitis)
+ - Atypical patterns seen in infants and children (may be caused by HSV1 or HSV2)
+ - May primarily affect cerebral hemispheres
+- ### CT Findings
+
+
+ - #### NECT
+
+
+ - CT often normal early
+ - Low attenuation, mild mass effect in medial temporal lobes, insula; hemorrhage typically late feature
+ - Predilection for limbic system; basal ganglia spared
+ - Earliest CT findings at 3 days after symptom onset
+ - #### CECT
+
+
+ - Patchy or gyriform **enhancement** of temporal lobes (**late** acute/subacute feature)
+- ### MR Findings
+
+
+ - #### T1WI
+
+
+ - Cortical swelling with loss of gray matter-white matter (WM) differentiation
+ - May see subacute hemorrhage as ↑ signal within edematous brain
+ - Atrophy, encephalomalacia in late subacute/chronic cases
+ - #### T2WI
+
+
+ - Cortical and subcortical hyperintensity with relative WM sparing
+ - May see subacute hemorrhage as ↑ signal within edematous brain
+ - #### FLAIR
+
+
+ - Changes seen earlier than on T2WI
+ - #### T2* GRE
+
+
+ - If hemorrhagic, hypointensity blooms within edematous brain
+ - #### DWI
+
+
+ - **Restricted diffusion** in limbic system
+ - Look for bilateral disease
+ - DWI findings may precede T2/FLAIR changes
+ - #### T1WI C+
+
+
+ - May see mild, patchy enhancement early
+ - Gyriform enhancement usually seen 1 week after initial symptoms
+ - Meningeal enhancement occasionally seen
+ - Enhancement seen in temporal lobes, insular cortex, subfrontal area, cingulate gyrus
+ - #### MRS
+
+
+ - ↓ NAA, prominent lipid-lactate peak
+- ### Imaging Recommendations
+
+
+ - #### Best imaging tool
+
+
+ - MR (positive 24-48 hours earlier than CT)
+ - #### Protocol advice
+
+
+ - Multiplanar MR with coronal T2 &/or FLAIR, DWI, T2* GRE, contrast
+
+## DIFFERENTIAL DIAGNOSIS
+
+- ### HSV2 : Neonatal HSV encephalitis
+
+
+ - Neonates/infants with HSV2 have diffuse/scattered disease unlike HSV1 temporal lobe predilection
+ - Deep/periventricular WM &/or cortical early DWI restriction
+ - Early meningeal enhancement may be seen
+ - Congenital HSV2 may be transplacental or from exposure to maternal HSV2 genital lesions during vaginal birth
+ - Transplacental infection during 1st trimester: Cerebral and cerebellar necrosis, atrophy, microcephaly
+ - Intracranial Ca⁺⁺, hydranencephaly, chorioretinitis, and cutaneous and systemic involvement
+ - Birth canal infection: Presents within first 3 weeks of life
+- ### Neurosyphilis
+
+
+ - Can affect medial temporal lobes; mimic HSE
+ - Involve meninges, blood vessels (obliterative endarteritis)
+- [Other Encephalitides](/document/miscellaneous-encephalitis/c2e7e8a3-254a-48b4-8187-bfffaf95407a)
+ - **Human herpesvirus 6 (HHV-6)** in transplant patients, can mimic HSE
+ - West Nile can mimic HSE clinically, but typically involves basal ganglia &/or thalami
+- [Acute Cerebral Ischemia-Infarction](/document/acute-cerebral-ischemiainfarction/7a3ed4a9-ae05-4d64-ae8e-6a30105501e1)
+ - Typical vascular distribution [middle cerebral artery, anterior cerebral artery (ACA), posterior cerebral artery]
+ - Hyperacute symptoms vs. 2-3 day history of flu-like illness
+ - Reduced diffusivity in acute infarct
+ - ACA distribution ischemia may mimic HSE
+- [Status Epilepticus](/document/status-epilepticus/bb0430ba-9933-40b4-a95b-97ca85070486)
+ - Active seizures may disrupt blood-brain barrier, cause signal abnormalities and enhancement
+ - Temporal lobe epilepsy hyperperfusion may mimic HSE
+ - No hemorrhage in status epilepticus
+- [Limbic Encephalitis](/document/autoimmune-encephalitis/f94d90d8-17d1-40cf-abad-3fc39d18fad3)
+ - Rare autoimmune/paraneoplastic syndrome associated with primary tumor, often lung
+ - Predilection for limbic system, often bilateral
+ - Nonhemorrhagic; imaging may be indistinguishable
+ - Symptom onset weeks to months (vs. acute in HSE)
+- ### Infiltrating Neoplasm
+
+
+ - Low-grade gliomas may involve medial temporal lobe and cause epilepsy; onset usually indolent
+ - Gliomatosis cerebri may involve frontal and temporal lobes; may be bilateral
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Etiology
+
+
+ - Initial HSV1 infection usually occurs in oronasopharynx via contact with infected secretions
+ - Invades along cranial nerves (via lingual nerve, division of trigeminal nerve) to ganglia
+ - Remains dormant in trigeminal ganglion
+ - Reactivation may occur spontaneously or be precipitated by various factors
+ - Local trauma, immunosuppression, hormonal fluctuations, emotional stress
+- ### Staging, Grading, & Classification
+
+
+ - Herpesviruses include HSV1, HSV2, EBV, CMV, varicella-zoster virus (VZV), B virus, HHV-6, HHV-7, HHV-8
+ - HSV1 in adults, children
+ - HSV2 is more common in neonates
+ - HSV1 and HSV2 DNA viruses
+ - Viruses obligate intracellular pathogens
+- ### Gross Pathologic & Surgical Features
+
+
+ - Hemorrhagic, necrotizing encephalitis
+ - Severe edema, massive tissue necrosis with hemorrhage
+ - Involvement of temporal lobes, insular cortex, orbital surface of frontal lobes
+ - Less frequent involvement of cingulate gyrus and occipital cortex
+- ### Microscopic Features
+
+
+ - Intense perivascular cuffing, interstitial lymphocytic inflammation
+ - Intranuclear inclusion bodies in infected cells (neurons, glia, endothelial cells)
+ - Typically eosinophilic Cowdry A nuclear inclusions
+ - Immunohistochemistry shows viral antigens, HSV1 antibodies
+ - Chronic cases: Microglial nodules
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - Fever, headache, seizures, ± viral prodrome
+ - Children often present with nonspecific symptoms
+ - Behavioral changes, fever, headaches, seizures
+ - Patients typically immunocompetent
+ - HSV1 uncommon in AIDS patients
+ - #### Other signs/symptoms
+
+
+ - Altered mental status
+ - Focal or diffuse neurologic deficit (< 30%)
+ - #### Clinical profile
+
+
+ - CSF studies show lymphocytic pleocytosis, ↑ protein
+ - PCR of CSF most accurate
+ - Sensitivity/specificity nearly 95-100%
+ - False-negative PCR possible if CSF obtained < 72 hours after clinical onset
+ - EEG: Temporal high voltage discharges in background of generalized slowing
+ - Brain biopsy may be required for diagnosis
+- ### Demographics
+
+
+ - #### Age
+
+
+ - May occur at any age
+ - Highest incidence in adolescents and young adults
+ - ~ 1/3 of all patients < 20 years old
+ - #### Sex
+
+
+ - M = F
+ - #### Epidemiology
+
+
+ - HSV1 causes 95% of all HSE
+ - Most common cause of fatal sporadic encephalitis
+ - Most common nonepidemic cause of viral meningoencephalitis
+ - In adults, typically related to viral reactivation
+ - In neonates, related to maternal infection
+ - Incidence: 1-3/1 million
+- ### Natural History & Prognosis
+
+
+ - May progress to coma and death
+ - 50-70% mortality rate
+ - Rapid diagnosis, early treatment with antiviral agents can ↓ mortality, may improve outcome
+ - Nearly 2/3 of survivors have significant neurologic deficits despite acyclovir therapy
+ - Survival complicated by memory difficulties, hearing loss, intractable epilepsy, personality changes
+ - HSV encephalitis can initiate autoimmune reaction with antibodies to neuronal surface antigens
+ - → **secondary autoimmune encephalitis**
+ - Suspect if recurrence of neurologic symptoms after treated HSV encephalitis episode
+ - After ~ 24 days in children and 40 days in adults
+ - Mostly anti-N-methyl-d-aspartate receptor (**anti-NMDAR**)encephalitis
+ - More frequency of **movement disorders** than seizures, especially in children
+ - Psychiatric symptoms more in adults
+- ### Treatment
+
+
+ - Antiviral therapy with intravenous acyclovir
+ - Foscarnet may help in acyclovir unresponsive cases
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - Start IV acyclovir immediately if HSE suspected
+ - Unilateral disease may mimic stroke or tumor
+ - History often helpful
+ - Autoimmune limbic encephalitis if all clinical HSE tests negative and subacute onset of symptoms
+ - Acute onset of HSE helps differentiate from other etiologies
+- ### Image Interpretation Pearls
+
+
+ - CT often normal early; MR with FLAIR/DWI most sensitive for early diagnosis
+ - Imaging often key in diagnosis
+
+ 070df362-51d8-4a70-a718-eb0977985e80
+
+## References
+
+## Selected References
+
+1. [Ayvacioğlu Çağan C et al: Life after tetra hit: anti-NMDAR encephalitis after HSV encephalitis in a NMOSD coexistent with Sjögren's syndrome. Noro Psikiyatr Ars. 59(2):161-3, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35685056%5Bpmid%5D)
+1. [Gayretli Aydin ZG et al: Acyclovir unresponsive herpes simplex encephalitis in a child successfully treated with the addition of Foscarnet: case report. Arch Argent Pediatr. 117(1):e47-51, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30652455%5Bpmid%5D)
+1. [Nosadini M et al: Herpes simplex virus-induced anti-N-methyl-D-aspartate receptor encephalitis: a systematic literature review with analysis of 43 cases. Dev Med Child Neurol. 59(8):796-805, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28439890%5Bpmid%5D)
+1. [Rabinstein AA: Herpes virus encephalitis in adults: current knowledge and old myths. Neurol Clin. 35(4):695-705, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28962808%5Bpmid%5D)
+1. [Hatanpaa KJ et al: Neuropathology of viral infections. Handb Clin Neurol. 123:193-214, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25015486%5Bpmid%5D)
+1. [Mahan M et al: Neuroimaging of viral infections of the central nervous system. Handb Clin Neurol. 123:149-73, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25015484%5Bpmid%5D)
+1. [Steiner I et al: Update on herpes virus infections of the nervous system. Curr Neurol Neurosci Rep. 13(12):414, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24142852%5Bpmid%5D)
+1. [Studahl M et al: Acute viral infections of the central nervous system in immunocompetent adults: diagnosis and management. Drugs. 73(2):131-58, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23377760%5Bpmid%5D)
+1. [Ibitoye RT et al: Pitfalls in the management of herpes simplex virus encephalitis. BMJ Case Rep, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=23076702%5Bpmid%5D)
+1. [Sureka J et al: Clinico-radiological spectrum of bilateral temporal lobe hyperintensity: a retrospective review. Br J Radiol. 85(1017):e782-92, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22422381%5Bpmid%5D)
+1. [Akyldz BN et al: Diffusion-weighted magnetic resonance is better than polymerase chain reaction for early diagnosis of herpes simplex encephalitis: a case report. Pediatr Emerg Care. 24(6):377-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18562881%5Bpmid%5D)
+1. [Baringer JR: Herpes simplex infections of the nervous system. Neurol Clin. 26(3):657-74, viii, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18657720%5Bpmid%5D)
+1. [Bulakbasi N et al: Central nervous system infections of herpesvirus family. Neuroimaging Clin N Am. 18(1):53-84; viii, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18319155%5Bpmid%5D)
+1. [Hatipoglu HG et al: Magnetic resonance and diffusion-weighted imaging findings of herpes simplex encephalitis. Herpes. 15(1):13-7, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18983763%5Bpmid%5D)
+1. [Kataoka H et al: Early dynamic SPECT imaging in acute viral encephalitis. J Neuroimaging. 17(4):304-10, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17894618%5Bpmid%5D)
+1. [Obeid M et al: Diffusion-weighted imaging findings on MRI as the sole radiographic findings in a child with proven herpes simplex encephalitis. Pediatr Radiol. 37(11):1159-62, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17874320%5Bpmid%5D)
+1. [Rimon A et al: West Nile encephalitis mimicking herpes encephalitis. Pediatr Neurol. 35(1):62-4, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16814089%5Bpmid%5D)
+1. [Whitley RJ: Herpes simplex encephalitis: adolescents and adults. Antiviral Res. 71(2-3):141-8, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16675036%5Bpmid%5D)
+1. [Duckworth JL et al: Magnetic resonance restricted diffusion resolution correlates with clinical improvement and response to treatment in herpes simplex encephalitis. Neurocrit Care. 3(3):251-3, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16377839%5Bpmid%5D)
+1. [Kuker W et al: Diffusion-weighted MRI in herpes simplex encephalitis: a report of three cases. Neuroradiology. 46(2):122-5, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14747879%5Bpmid%5D)
+1. [Kaga K et al: Auditory agnosia in children after herpes encephalitis. Acta Otolaryngol. 123(2):232-5, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12701747%5Bpmid%5D)
+1. [Sämann PG et al: Serial proton MR spectroscopy and diffusion imaging findings in HIV-related herpes simplex encephalitis. AJNR Am J Neuroradiol. 24(10):2015-9, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14625226%5Bpmid%5D)
+1. [Cakirer S et al: MR imaging in epilepsy that is refractory to medical therapy. Eur Radiol. 12(3):549-58, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=11870469%5Bpmid%5D)
+1. [Bash S et al: Mesiotemporal T2-weighted hyperintensity: neurosyphilis mimicking herpes encephalitis. AJNR Am J Neuroradiol. 22(2):314-6, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11156776%5Bpmid%5D)
+1. [Kleinschmidt-DeMasters BK et al: The expanding spectrum of herpesvirus infections of the nervous system. Brain Pathol. 11(4):440-51, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11556690%5Bpmid%5D)
+1. [Teixeira J et al: Diffusion imaging in pediatric central nervous system infections. Neuroradiology. 43(12):1031-9, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11792040%5Bpmid%5D)
+1. [Leonard JR et al: MR imaging of herpes simplex type 1 encephalitis in infants and young children: a separate pattern of findings. AJR Am J Roentgenol. 174(6):1651-5, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10845501%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+*Coronal graphic shows the classic features of herpes encephalitis with bilateral but asymmetric involvement of the limbic system. There is inflammation involving the temporal lobes, cingulate gyri, and insular cortices.*
+
+
+*Coronal graphic shows the classic features of herpes encephalitis with bilateral but asymmetric involvement of the limbic system. There is inflammation involving the temporal lobes, cingulate gyri, and insular cortices.*
+
+
+*Coronal T1 C+ MR in a subacute case of HSV1 herpes encephalitis shows striking bilateral but asymmetric enhancement in the mesial
more than lateral temporal lobes
and insular cortex
. Subtle lesions are seen in the cingulate gyri
.*
+
+
+*Coronal T1 C+ MR in a subacute case of HSV1 herpes encephalitis shows striking bilateral but asymmetric enhancement in the mesial
more than lateral temporal lobes
and insular cortex
. Subtle lesions are seen in the cingulate gyri
.*
+
+
+*Axial DWI MR in a patient with acute herpes encephalitis shows diffusion restriction in the right mesial
and lateral temporal
lobes. Subtle diffusion restriction of the left hippocampus
is also seen.*
+
+
+*Axial DWI MR in a patient with acute herpes encephalitis shows diffusion restriction in the right mesial
and lateral temporal
lobes. Subtle diffusion restriction of the left hippocampus
is also seen.*
+
+
+*Axial FLAIR MR at a higher level in the same patient shows diffuse cortical swelling and hyperintensity in the right superior temporal lobe
, right insular cortex
, and bilateral cingulate gyri
. Note relative sparing of the underlying white matter.*
+
+
+*Axial FLAIR MR at a higher level in the same patient shows diffuse cortical swelling and hyperintensity in the right superior temporal lobe
, right insular cortex
, and bilateral cingulate gyri
. Note relative sparing of the underlying white matter.*
+
+
+*Axial FLAIR MR in child with HSV-1 herpes encephalitis shows typical left temporal lobe hyperintensity, swelling of amygdala
, hippocampus
, uncus
, and (unusually) anterior temporal pole
and lateral temporal lobe
. Note mild right amygdalar
, uncal
involvement.*
+
+
+*Axial FLAIR MR in child with HSV-1 herpes encephalitis shows typical left temporal lobe hyperintensity, swelling of amygdala
, hippocampus
, uncus
, and (unusually) anterior temporal pole
and lateral temporal lobe
. Note mild right amygdalar
, uncal
involvement.*
+
+
+*Sagittal T1 MR in patient 2 weeks after initial presentation of herpes encephalitis shows volume loss in temporal lobe
, linear gyriform T1 hyperintensity
, characteristic of subacute cortical hemorrhage.*
+
+
+*Sagittal T1 MR in patient 2 weeks after initial presentation of herpes encephalitis shows volume loss in temporal lobe
, linear gyriform T1 hyperintensity
, characteristic of subacute cortical hemorrhage.*
+
+
+*Axial T1 C+ MR in the subacute stage of herpes encephalitis shows bilateral but asymmetric hypointensities
in the insular regions. There is associated pial enhancement in the left insular region
. Pial, leptomeningeal, diffuse, ring-like enhancement patterns have been reported in herpes encephalitis.*
+
+
+*Axial SWI MR in the same patient shows patchy hypointense hemorrhage
in the left insular region. MR is superior to CT in detecting subacute or chronic hemorrhage.*
+
+
+*Axial NECT in a 45-year-old with fever and altered mental status shows questionable, subtle low-density changes
in the right medial temporal lobe.*
+
+
+*Axial T2 MR in the same patient shows signal abnormality
in the right medial temporal lobe. CSF PCR was positive for HSV-1. CT study may be normal in early stages of herpes encephalitis.*
+
+
+### Additional Images
+
+
+*Axial CECT shows a low-density right medial temporal lobe
in an older adult woman with fever, confusion, decreased consciousness, and seizure.*
+
+
+*Axial CECT in the same patient shows the involvement of insular cortex
. Herpes encephalitis was suggested on the basis of CT scan and acyclovir therapy was begun immediately.*
+
+
+*Axial FLAIR MR shows the typical findings of bilateral mesial temporal lobe hyperintensity
in herpes encephalitis. Note the gyral edema with relative sparing of underlying white matter.*
+
+
+*Axial FLAIR MR shows the typical findings of bilateral mesial temporal lobe hyperintensity
in herpes encephalitis. Note the gyral edema with relative sparing of underlying white matter.*
+
+
+*Axial DWI MR in the same patient shows restricted diffusion in both mesial temporal lobes
.*
+
+
+*Axial DWI MR in the same patient shows restricted diffusion in both mesial temporal lobes
.*
+
+
+*Axial T1 MR in a case of herpes encephalitis imaged several days after symptom onset shows gyral hyperintensity
, characteristic of petechial hemorrhage at this stage of the disease.*
+
+
+*Axial T1 MR in a case of herpes encephalitis imaged several days after symptom onset shows gyral hyperintensity
, characteristic of petechial hemorrhage at this stage of the disease.*
+
+
+*Axial T1 C+ MR in the same patient shows striking gyriform enhancement in the left temporal lobe
and subtle enhancement in the right temporal lobe cortex
.*
+
+
+*Axial T1 C+ MR in the same patient shows striking gyriform enhancement in the left temporal lobe
and subtle enhancement in the right temporal lobe cortex
.*
+
+
+*Coronal FLAIR MR shows classic bilateral, asymmetric involvement of the medial temporal lobes
and right insula
in this 46-year-old woman with herpes encephalitis. Basal ganglia sparing is typical.*
+
+
+*Coronal FLAIR MR shows classic bilateral, asymmetric involvement of the medial temporal lobes
and right insula
in this 46-year-old woman with herpes encephalitis. Basal ganglia sparing is typical.*
+
+
+*Axial T1 MR shows T1 hyperintensity representing subacute blood products in the right insula
on this follow-up study. Hemorrhage is typically a late feature of herpes encephalitis.*
+
+
+*Axial T1 MR shows T1 hyperintensity representing subacute blood products in the right insula
on this follow-up study. Hemorrhage is typically a late feature of herpes encephalitis.*
+
+
+*Axial NECT in a 25-year-old man with altered mental status and aphasia shows hemorrhage in the left temporal lobe with surrounding edema and mass effect. The patient had a poor prognosis despite early acyclovir therapy.*
+
+
+*Axial NECT in a 25-year-old man with altered mental status and aphasia shows hemorrhage in the left temporal lobe with surrounding edema and mass effect. The patient had a poor prognosis despite early acyclovir therapy.*
+
+
+*Axial T2 MR shows the classic appearance of herpes encephalitis with bilateral but asymmetric hyperintensity involving the insular cortex
and anterior cingulate gyrus
. Note the typical sparing of the deep gray nuclei. FLAIR MR may show subtle changes of herpes simplex encephalitis earlier than T2WI.*
+
+
+*Axial T2 MR shows the classic appearance of herpes encephalitis with bilateral but asymmetric hyperintensity involving the insular cortex
and anterior cingulate gyrus
. Note the typical sparing of the deep gray nuclei. FLAIR MR may show subtle changes of herpes simplex encephalitis earlier than T2WI.*
+
+
+*Axial T1 C+ MR in the same patient shows bilateral, asymmetric enhancement of the insular cortex
. Subtle enhancement of the cingulate gyri
is also noted. Also note some right temporal lobe abnormal cortical enhancement
.*
+
+
+*Axial T1 C+ MR in the same patient shows bilateral, asymmetric enhancement of the insular cortex
. Subtle enhancement of the cingulate gyri
is also noted. Also note some right temporal lobe abnormal cortical enhancement
.*
+
+
+*Axial T2 MR in an older adult with fever and confusion shows diffuse swelling and hyperintensity in the right mesial
and lateral
temporal lobe and orbitofrontal gyrus
.*
+
+
+*Axial T2 MR in an older adult with fever and confusion shows diffuse swelling and hyperintensity in the right mesial
and lateral
temporal lobe and orbitofrontal gyrus
.*
+
+
+*Axial FLAIR MR in the same patient shows marked edema and hyperintensity in the right mesial
and lateral
temporal lobe cortex with relative sparing of the subcortical white matter. This unilateral involvement by herpes encephalitis is atypical and may mimic a stroke. Clinical history is often helpful.*
+
+
+*Axial FLAIR MR in the same patient shows marked edema and hyperintensity in the right mesial
and lateral
temporal lobe cortex with relative sparing of the subcortical white matter. This unilateral involvement by herpes encephalitis is atypical and may mimic a stroke. Clinical history is often helpful.*
+
+
+*Axial NECT shows edema and hemorrhage
in the left temporal lobe in this young adult with altered mental status. Despite early acyclovir therapy, the patient succumbed to his disease. Mortality ranges from 50-70% in herpes simplex encephalitis patients.*
+
+
+*Axial NECT shows edema and hemorrhage
in the left temporal lobe in this young adult with altered mental status. Despite early acyclovir therapy, the patient succumbed to his disease. Mortality ranges from 50-70% in herpes simplex encephalitis patients.*
+
diff --git a/docs_md/articles/hypertensive-intracranial-hemorrhage_6f6d2768-06ba-4eae-93cf-3b639800522c.md b/docs_md/articles/hypertensive-intracranial-hemorrhage_6f6d2768-06ba-4eae-93cf-3b639800522c.md
new file mode 100644
index 0000000..7a8ecb4
--- /dev/null
+++ b/docs_md/articles/hypertensive-intracranial-hemorrhage_6f6d2768-06ba-4eae-93cf-3b639800522c.md
@@ -0,0 +1,526 @@
+---
+title: "Hypertensive Intracranial Hemorrhage"
+docid: "6f6d2768-06ba-4eae-93cf-3b639800522c"
+authors:
+ - key: "8d5254e9-8dda-478b-8f08-bdee97a32c79"
+ value: "Karen L. Salzman, MD, FACR"
+ - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
+ value: "Anne G. Osborn, MD, FACR"
+breadcrumbs:
+ -
+ name: "Brain"
+ slug: "brain"
+ treeNodeId: "0361042c-9c90-48e4-864e-a2e6428ee9c9"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "948c6f1e-8e18-45f3-bd9d-7a04ab0b8708"
+ -
+ name: "Pathology-Based Diagnoses"
+ slug: "pathology-based-diagnoses"
+ treeNodeId: "d60fe914-7897-46a0-9cd2-f88ffb322f16"
+ -
+ name: "Stroke"
+ slug: "stroke"
+ treeNodeId: "7a135176-0a69-4fc9-b200-59569fbf5166"
+ -
+ name: "Nontraumatic Intracranial Hemorrhage"
+ slug: "nontraumatic-intracranial-hemorrha-"
+ treeNodeId: "fec768e1-2e80-4b63-a846-579ea0a9ef90"
+ -
+ name: "Hypertensive Intracranial Hemorrhage"
+ slug: "hypertensive-intracranial-hemorrha-"
+ treeNodeId: null
+category: "Brain"
+documentVersionId: "e2b60a0e-0ef5-45df-898d-70cbd57d7f24"
+imageCount: 36
+lastUpdated: "08/21/25"
+pageDescription: "Hypertensive Intracranial Hemorrhage"
+pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Stroke, Nontraumatic Intracranial Hemorrhage, Hypertensive Intracranial Hemorrhage"
+pageTitle: "Hypertensive Intracranial Hemorrhage | STATdx"
+enhancedTitle: "Hypertensive Intracranial Hemorrhage"
+type: "DX"
+references: true
+ddx: true
+cases: 3
+breadcrumbs:
+ - "Brain"
+ - "Diagnosis"
+ - "Pathology-Based Diagnoses"
+ - "Stroke"
+ - "Nontraumatic Intracranial Hemorrhage"
+ - "Hypertensive Intracranial Hemorrhage"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Hypertensive intracranial hemorrhage (hICH)
+ - Acute intracerebral hemorrhage (ICH) 2° to systemic HTN
+ - 2nd most common cause of stroke
+- ### Imaging
+
+
+ - Initial screen = NECT in patients with HTN
+ - CT: Acute round or oval hyperdense mass
+ - Striatocapsular: Putamen/external capsule (60-65%)
+ - Thalamus (15-25%); pons, cerebellum (10%)
+ - Lobar 5-10%
+ - Multifocal microbleeds (1-5%)
+ - Heterogeneous density (coagulopathy, active bleed)
+ - Other findings: Intraventricular extension, mass effect, hydrocephalus, herniation
+ - MR signal intensity (varies with age of clot)
+ - **Hyperacute** (< 24 hours): T1 iso-hypo-/T2 hyperintense
+ - **Acute** (~ 1-3 days): T1 iso-hypo-/T2 hypointense
+ - **Subacute** (3-7 days): T1 hyper-/T2 hypohyperintense
+ - **Chronic** (weeks-months): T1 hyper-/T2 hypointense
+ - CTA spot sign = extravasation, indicates active bleeding
+- ### Top Differential Diagnoses
+
+
+ - Cerebral amyloid angiopathy (CAA)
+ - Hemorrhagic neoplasm, coagulopathy
+ - Deep cerebral venous thrombosis
+ - Drug abuse (especially in young patient)
+ - Vascular malformation (rare in older adults)
+- ### Clinical Issues
+
+
+ - HTN single most important risk factor for**all**types of stroke
+ - 10-15% of stroke patients have hICH
+ - 40-50% of nontraumatic ICHs caused by hICH
+ - HTN most common cause of spontaneous ICH in patients 45-70 years old
+ - 10-15% of hypertensive patients with spontaneous ICH have underlying aneurysm or AVM
+ - Consider CTA in lobar hemorrhages
+ - Subarachnoid extension of hematoma on CT is usually indicative of nonhypertensive etiology
+ - Consider lobar ICH caused by vascular abnormality
+
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - Hypertensive intracranial hemorrhage (hICH)
+- ### Synonyms
+
+
+ - Stroke, hypertensive hemorrhage (HH)
+- ### Definitions
+
+
+ - Acute nontraumatic intracerebral hemorrhage (ICH) secondary to systemic hypertension (HTN)
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Round or oval hyperdense mass in basal ganglia (BG) or thalamus in patients with HTN
+ - #### Location
+
+
+ - Striatocapsular: Putamen/external capsule (60-65%)
+ - Thalamus (15-25%)
+ - Pons, cerebellum (10%)
+ - Lobar (5-10%)
+ - #### Size
+
+
+ - Subcentimeter (microbleeds) to several centimeters
+ - #### Morphology
+
+
+ - Typically rounded or oval
+ - 2 distinct patterns seen with HTN
+ - Acute focal hematoma (hICH)
+ - Multiple subacute/chronic microbleeds (1-5%) (chronic HTN)
+ - BG and cerebellum > cortex subcortical white matter (WM)
+- ### CT Findings
+
+
+ - #### NECT
+
+
+ - Round or oval hyperdense parenchymal mass
+ - Heterogeneous density if coagulopathy or active bleeding
+ - Intraventricular extension of hemorrhage common
+ - Mass effect, hydrocephalus, herniation common
+ - #### CECT
+
+
+ - No enhancement in acute hICH
+ - #### CTA
+
+
+ - Avascular mass effect in acute hICH
+ - No underlying vascular lesion
+ - **Spot sign** showing contrast extravasation indicates active bleeding, suggests expanding hematoma
+- ### MR Findings
+
+
+ - #### T1WI
+
+
+ - Varies with age of clot
+ - Hyperacute hematoma (< 24 hours)
+ - Oxyhemoglobin (Hgb) (iso-/hypointense)
+ - Acute hematoma (~ 1-3 days)
+ - DeoxyHb (iso-/hypointense)
+ - Early subacute hematoma (~ 3-7 days)
+ - Intracellular metHb (hyperintense)
+ - Late subacute hematoma (week to months)
+ - Extracellular metHb (hyperintense)
+ - Chronic hematoma
+ - Hypointense (± hyperintense center)
+ - #### T2WI
+
+
+ - Appearance of hematoma varies with stage
+ - Hyperacute hematoma (< 24 hours)
+ - OxyHb (hyperintense)
+ - Acute hematoma (~ 1-3 days)
+ - DeoxyHb (hypointense)
+ - Subacute hematoma (3-7 days)
+ - Intracellular metHb (hypointense)
+ - Late subacute hematoma (week to months)
+ - Extracellular metHb (hyperintense)
+ - Chronic hematoma (months)
+ - Hemosiderin (hypointense)
+ - Remote hematoma (months to years)
+ - Hypointense hemosiderin scar ± central hyperintense cavity
+ - WM hyperintensities are hICH risk markers
+ - #### T2* GRE
+
+
+ - Multifocal hypointense lesions ("black dots") in deep gray nuclei, cerebellum, and peripheral WM
+ - Common with longstanding HTN
+ - Also seen with amyloid angiopathy (more peripheral)
+ - #### DWI
+
+
+ - Hypo- or mixed hypo-/hyperintense (early hematoma)
+ - #### T1WI C+
+
+
+ - Typically no enhancement with acute hematoma
+ - Helps exclude underlying mass
+ - Contrast extravasation = active hemorrhage
+ - #### MRA
+
+
+ - Negative
+ - SWI: Multifocal hypointense lesions ("black dots") in deep gray nuclei, cerebellum, and peripheral WM
+- ### Angiographic Findings
+
+
+ - Conventional
+ - DSA usually normal if history of HTN with deep ganglionic hemorrhage
+ - May show avascular mass effect
+ - Rare: "Bleeding globe" microaneurysm on lenticulostriate artery (LSA)
+ - Coexisting vascular abnormalities
+ - ↑ prevalence of unruptured intracranial aneurysms
+ - More common in females
+- ### Imaging Recommendations
+
+
+ - #### Best imaging tool
+
+
+ - Initial screen = NECT in patients with HTN
+ - If atypical age or history, consider MR with T2*/SWI/T1 C+ or CTA
+ - If hyperacute ischemic "stroke" suspected, MR with T2*/SWI and DWI
+ - If MR shows classic hematoma with coexisting multifocal "black dots," most likely amyloid angiopathy or chronic HTN, may coexist
+ - If MR shows atypical hematoma, MRA or CTA
+ - If MRA or CTA inconclusive, consider DSA
+ - #### Protocol advice
+
+
+ - Otherwise, MR (include T2* sequences, DWI, + MRA; T1WI C+ optional)
+
+## DIFFERENTIAL DIAGNOSIS
+
+- [Cerebral Amyloid Angiopathy](/document/cerebral-amyloid-disease/18edc9f3-9218-410c-8280-29c3e6df4c91)
+ - Lobar > > BG hemorrhage
+ - Usually older adults, often normotensive
+ - Only 5-10% of hICHs are lobar, but HTN is so common that it is always consideration
+- [Hemorrhagic Neoplasm](/document/parenchymal-metastases/2cf0bd40-4597-4c0b-83e4-74340304b98f)
+ - Metastases and primary (e.g., glioblastoma)
+ - Typically older adults
+- [Venous Thrombosis](/document/cortical-venous-thrombosis/8efaec3c-b3ac-46e2-9da8-6c68880f8236)
+ - May have history of dehydration, flu, pregnancy, birth control pills
+ - May present as lobar hematoma
+ - Look for hyperdense dural sinus
+- [Deep Cerebral Venous Thrombosis](/document/deep-cerebral-venous-thrombosis/056d86df-2bcb-4816-ace7-21f663d581f0)
+ - Less common than dural sinus or cortical vein thrombosis
+ - Look for hyperdense internal cerebral veins, intraventricular hemorrhage
+ - Look for hypodense bilateral thalami
+- ### Coagulopathy
+
+
+ - Older adults on anticoagulant therapy
+- [Drug Abuse](/document/drug-abuse/48859403-0b26-44d8-ba74-e0919e4c3147)
+ - Cocaine and other drugs may cause sudden ↑ HTN
+ - Be suspicious if unexplained BG bleed in young patient
+- [Vascular Malformation](/document/cavernous-malformation/cc2415f4-75a6-458d-800e-be31b3cf50c3)
+ - Patients usually normotensive, younger
+ - Most common is cavernous malformation
+ - Look for "black dots" (multiple lesions) on T2* (GRE, SWI) scans
+ - Less common = thrombosed hemorrhagic arteriovenous malformation (AVM), dural arteriovenous fistula (dAVF) or middle cerebral artery (MCA) aneurysm
+ - Look for stagnating vessels, early draining veins on DSA
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Etiology
+
+
+ - Chronic HTN with atherosclerosis, lipohyalinosis, fibrinoid necrosis, abrupt wall rupture ± pseudoaneurysm formation
+ - "Bleeding globe" (penetrating lenticulostriate pseudoaneurysm)
+ - Charcot-Bouchard aneurysm
+ - Diffuse microbleeds common
+ - Striatocapsular hematoma most common autopsy finding
+- ### Gross Pathologic & Surgical Features
+
+
+ - Large ganglionic hematoma ± IVH
+ - Subfalcine herniation, hydrocephalus (common)
+ - Coexisting small chronic hemorrhages, ischemic lesions (common)
+- ### Microscopic Features
+
+
+ - Severe arteriosclerosis with lipohyalinosis
+ - May see may see small fibrosed pseudoaneurysm or fibrous balls (fibrosed miliary aneurysm)
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - 10-15% of stroke patients have hICH
+ - Large ICHs present with sensorimotor deficits, impaired consciousness
+ - #### Clinical profile
+
+
+ - HTN single most important risk factor for**all**types of stroke
+ - Major risk factor = HTN (↑ risk of ICH 4x)
+- ### Demographics
+
+
+ - #### Age
+
+
+ - Adults, most common 45-70 years
+ - #### Sex
+
+
+ - M > F
+ - #### Ethnicity
+
+
+ - Higher incidence in Black patients
+ - #### Epidemiology
+
+
+ - 40-50% of primary nontraumatic ICHs caused by HH
+ - HTN most common cause of spontaneous ICH in patients 45-70 years
+ - 10-15% of all stroke cases; associated with highest mortality rate
+ - 10-15% of hypertensive patients with spontaneous ICH have underlying aneurysm or AVM
+- ### Natural History & Prognosis
+
+
+ - Presence of microbleeds on MR is strong predictor of future hICH
+ - Bleeding can persist for up to 6 hours following ictus
+ - Neurologic deterioration common within 48 hours
+ - ↑ hematoma, edema
+ - Hydrocephalus
+ - Herniation syndromes
+ - Recurrent hICH in 5-10% of cases, usually different location
+ - Prognosis related to location, size of hICH
+ - 80% mortality in massive hICH with IVH
+ - 1/3-1/2 of survivors are severely disabled
+- ### Treatment
+
+
+ - Control of intracranial pressure and hydrocephalus
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - Does patient have history of poorly controlled systemic HTN?
+ - Could there be underlying coagulopathy, hemorrhagic neoplasm, or vascular malformation?
+ - Consider substance abuse in young patients with unexplained hICH
+- ### Image Interpretation Pearls
+
+
+ - Underlying cause of lobar ICH is often difficult to determine
+ - Subarachnoid extension of hematoma on CT is usually indicative of nonhypertensive etiology; consider lobar ICH caused by vascular abnormality
+ - Definite diagnosis of cerebral amyloid angiopathy (CAA) vs. HTN-related hemorrhage requires histopathological confirmation and should not be based solely on hemorrhage pattern interpretation
+
+ fc7ea1fb-4b1b-4389-925b-ea500b5c14d7
+
+## References
+
+## Selected References
+
+1. [Horn M et al: Timing of spot sign appearance, spot sign volume, and leakage rate among phases of multiphase CTA predict intracerebral hemorrhage growth. AJNR Am J Neuroradiol. 45(6):693-700, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38782592%5Bpmid%5D)
+1. [Tartarin H et al: Uncommon causes of nontraumatic intracerebral hemorrhage. Stroke. 55(5):1416-27, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38572651%5Bpmid%5D)
+1. [Ducroux C et al: NCCT markers of intracerebral hemorrhage expansion using revised criteria: an external validation of their predictive accuracy. AJNR Am J Neuroradiol. 44(6):658-64, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37169542%5Bpmid%5D)
+1. [Nawabi J et al: Non-contrast computed tomography features predict intraventricular hemorrhage growth. Eur Radiol. 33(11):7807-17, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37212845%5Bpmid%5D)
+1. [Chen C et al: When less is more: non-contrast head CT alone to work-up hypertensive intracerebral hemorrhage. J Clin Neurosci. 100:108-12, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35447508%5Bpmid%5D)
+1. [Divani AA et al: The magnitude of blood pressure reduction predicts poor in-hospital outcome in acute intracerebral hemorrhage. Neurocrit Care. 33(2):389-98, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32524527%5Bpmid%5D)
+1. [Lioutas VA et al: Assessment of incidence and risk factors of intracerebral hemorrhage among participants in the Framingham Heart Study between 1948 and 2016. JAMA Neurol. 77(10):1252-60, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32511690%5Bpmid%5D)
+1. [Boulouis G et al: Association of key magnetic resonance imaging markers of cerebral small vessel disease with hematoma volume andeexpansion in patients with lobar and deep intracerebral hemorrhage. JAMA Neurol. 73(12):1440-7, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27723863%5Bpmid%5D)
+1. [Koivunen RJ et al: Incidence, risk factors, etiology, severity and short-term outcome of non-traumatic intracerebral hemorrhage in young adults. Eur J Neurol. 22(1):123-32, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25142530%5Bpmid%5D)
+1. [Marsh EB et al: Predicting symptomatic intracerebral hemorrhage versus lacunar disease in patients with longstanding hypertension. Stroke. 45(6):1679-83, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24811338%5Bpmid%5D)
+1. [Shams S et al: Cerebral microbleeds: different prevalence, topography, and risk factors depending on dementia diagnosis-the Karolinska imaging dementia study. AJNR Am J Neuroradiol. 36(4):661-6, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25523590%5Bpmid%5D)
+1. [Wilson D et al: Advances in understanding spontaneous intracerebral hemorrhage: insights from neuroimaging. Expert Rev Neurother. 14(6):661-78, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24852230%5Bpmid%5D)
+1. [Yang Z et al: Treatment of supratentorial spontaneous intracerebral hemorrhage using image-guided minimally invasive surgery: Initial experiences of a flat detector CT-based puncture planning and navigation system in the angiographic suite. AJNR Am J Neuroradiol. 35(11):2170-5, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24994826%5Bpmid%5D)
+1. [Mehndiratta P et al: Cerebral amyloid angiopathy-associated intracerebral hemorrhage: pathology and management. Neurosurg Focus. 32(4):E7, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22463117%5Bpmid%5D)
+1. [Zheng T et al: Vascular imaging adds value in investigation of basal ganglia hemorrhage. J Clin Neurosci. 19(2):277-80, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22118795%5Bpmid%5D)
+1. [Dubow J et al: Impact of hypertension on stroke. Curr Atheroscler Rep. 13(4):298-305, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21626308%5Bpmid%5D)
+1. [Shinohara Y et al: III. Intracerebral hemorrhage. J Stroke Cerebrovasc Dis. 20(4 Suppl):S74-99, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21835358%5Bpmid%5D)
+1. [Bogucki J et al: A new CT-based classification of spontaneous supratentorial intracerebral haematomas. Neurol Neurochir Pol. 43(3):236-44, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19618306%5Bpmid%5D)
+1. [Waran V et al: A new expandable cannula system for endoscopic evacuation of intraparenchymal hemorrhages. J Neurosurg. 111(6):1127-30, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19408977%5Bpmid%5D)
+1. [Shah QA et al: Acute hypertension in intracerebral hemorrhage: pathophysiology and treatment. J Neurol Sci. 261(1-2):74-9, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17550786%5Bpmid%5D)
+1. [Ferro JM: Update on intracerebral haemorrhage. J Neurol. 253(8):985-99, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16680558%5Bpmid%5D)
+1. [Ohtani R et al: Clinical and radiographic features of lobar cerebral hemorrhage: hypertensive versus non-hypertensive cases. Intern Med. 42(7):576-80, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12879949%5Bpmid%5D)
+
+## Differential diagnosis
+
+### Abnormal Shape/Configuration of Corpus Callosum
+DDX:238ca32d-6bc6-4f5a-81b1-6601dd605856
+
+## Cases
+
+- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '8eac95b8-8e45-48d0-a98d-432263728d5d', 'description': 'In this case, an axial NECT scan (#1) shows what appears to be a classic basal ganglionic hemorrhage that has dissected into the lateral ventricles. Because the patient was only mildly hypertensive, digital subtraction angiography (#2) was performed to look for an underlying vascular lesion. Selective injection of the right internal carotid artery shows a large avascular mass in the deep basal ganglia with a "round" shift of the anterior cerebral artery across the midline. Closer inspection shows a tangle of abnormal arteries (arrow) fed by a very large, medially displaced anterior choroidal artery. A mostly thrombosed arteriovenous malformation was confirmed at surgery.\n\nComment: When a middle-aged or younger patient has a spontaneous basal ganglia hemorrhage that looks like classic hypertensive striatocapsular hemorrhage, predisposing or underlying etiologies must be considered. Hypertensive hemorrhage from drug abuse is increasingly common.', 'history': 'Acute onset left hemiparesis, followed by coma.', 'imagePoolId': '0f3cbf2e-9b5c-48d5-8e6d-9bba1fcc870f', 'name': 'Mimic', 'teachingPoint': None, 'demographics': '46 Years old female'}], 'caseType': 'Other', 'name': 'OTHER'}
+- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '1b73f09a-1d13-4e32-a80f-d05e70aafdf9', 'description': 'NECT scan shows hypodense mass centered in the putamen/external capsule (arrows, #1). A punctate area of hyperdensity is seen within the mass (open arrow, #1). Sagittal (#3) and coronal (#4,5) images from the CTA obtained after the NECT scan show faint rim enhancement around the lesion (arrows) and a hyperdense linear mass within the lesion (open arrows) that appears to extend from a lenticulostriate artery (curved arrow).\n\nMR was obtained. Axial T1WI (#6) shows the mass (arrow) is a subacute hemorrhage. Note hypointensity within the mass (open arrow) that corresponds to the hyperdense lesion seen on CTA. The mass (arrows) remains mostly hyperintense on T2WI (#7) and FLAIR (#8) and is surrounded by a hypointense rim. The "dot" within the mass--a subacute hematoma (open arrow, #7,8) --remains hypointense. It "blooms" on T2*GRE (open arrow, #9), as does the hemosiderin rim surrounding the clot (arrow). DWI (#10) and ADC (#11) show typical changes of evolving hemorrhage. The enhanced scans (#12,13) show some rim enhancement around the subacute hematoma (arrows).', 'history': 'Hypertensive patient with several days of decreased right-sided motor function.', 'imagePoolId': '2fd06d58-0180-40df-852d-5584932259a0', 'name': 'Charcot-Bouchard aneurysms', 'teachingPoint': 'Findings on both CT and MR are those of a classic late subacute/early chronic hypertensive hemorrhage. What is most interesting is the identification of the small tubular structure within it, suggesting this is a thrombosed Charcot-Bouchard aneurysm, sometimes called a "bleeding globe." These are occasionally identified at autopsy but rarely seen on imaging studies.', 'demographics': '57 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '97233c6d-ca1c-49db-ad94-1f8ec91bef4b', 'description': 'Axial T2WI (#1) shows multiple discrete hyperintense lesions in both external capsules, putamina, thalami. The lesion in the external capsule/putamen has a hypointense rim surrounding it (open arrow). Axial FLAIR (#2) shows the classic confluent white matter lesions around the ventricular atria typical for chronic hypertension (curved arrows). T2*GRE (#3) shows the hypointense rim around the left external capsule/putamen very well (open arrow) and also discloses multiple hypointense blooming foci in the thalami, deep cerebral white matter (arrows). A small, healed old right external capsule/putamen hemorrhage (curved arrow) is present.', 'history': 'Longstanding, poorly treated hypertension.', 'imagePoolId': '6738ea5d-c587-47a3-850c-e10f3dc28ca6', 'name': 'Multiple chronic hemorrhages on GRE', 'teachingPoint': "Multiple gross as well as microhemorrhages and chronic-appearing white matter changes all attest to the patient's longstanding hypertension.", 'demographics': '69 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'f8def985-30af-4968-b302-307def2ff640', 'description': 'Axial T1WI MR (#1) shows a mostly isointense mass in the left basal ganglia (arrow). Note the possible old right basal ganglionic infarct (open arrow). Axial T2WI (#2) shows the left basal ganglionic mass is inhomogeneously hyperintense; the right basal ganglia lesion is hyperintense with a hypointense rim (open arrow). The large mass remains hyperintense on axial FLAIR (#3).\n\nThe axial T2* (GRE) scan (#4) shows that both lesions have a hypointense rim that blooms strongly.\n\nThe axial T1 C+ scans (#5-6) show a linear enhancing region within the hematoma (arrows). Note that the subfalcine herniation of the lateral ventricles has increased since the 1st sequence, and contrast is accumulating in the lateral aspect of the clot (curved arrows). This represents active bleeding.\n\nThis patient has both old (right) and very acute, actively bleeding (left) hypertensive hemorrhages.', 'history': 'Chronic untreated hypertensive patient with history of remote right basal ganglionic hemorrhage.', 'imagePoolId': 'f8322e36-0f5f-4a97-9b99-777235479018', 'name': 'Active bleed', 'teachingPoint': None, 'demographics': '38 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '47d2d1e5-4459-402b-b48e-be695bbf880b', 'description': 'A series of axial NECT scans demonstrate the high density mass (arrows) with surrounding low density edema (open arrows) in the most common location for hypertensive hemorrhage. Note compression of the right lateral ventricle by the mass.', 'history': 'Elderly hypertensive patient presented with acute onset left hemiparesis. Blood pressure was 190/110 in the emergency room.', 'imagePoolId': '5fd527e6-c5f0-4a88-8bc7-e93d55625d1e', 'name': 'Classic striatocapsular', 'teachingPoint': 'Spontaneous intracranial hemorrhage in elderly patients has many etiologies. When the hematoma is centered in the external capsule and putamen, as is illustrated by this case, hypertension is the most common cause.', 'demographics': '78 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'f3b1e164-e3df-468b-9f6b-b01cd0620069', 'description': 'The pons is a relatively uncommon site for hypertensive intracranial hemorrhage.\n\nThis series of axial NECT scans (images 1-4) illustrates the classic intrapontine high-density characteristic of hypertensive brainstem hemorrhage (arrows). Note blood has dissected into the 4th ventricle (images 1-2, curved arrows) and extends cephalad into the midbrain at the level of the cerebral peduncles (images 3-4, arrows).', 'history': 'Sudden-onset multiple lower cranial nerve palsies in a severely hypertensive patient.', 'imagePoolId': 'e65efa95-5ac8-4898-b021-87e117d89b18', 'name': 'Pontine', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '067d9a70-e50a-4308-bdb0-672a817f968c', 'description': 'A series of NECT scans (#1-8) show a hyperdense clot centered in the left external capsule and putamen (open arrows, #1-5, 8). Some intraventricular hemorrhage is present (arrows, #1-5). Moderate mass effect with left to right subfalcine herniation of the 3rd and lateral ventricles is seen. The mixed density of the clot, appreciated especially well on images #4-6, indicates some active bleeding in the clot. A fluid-fluid level is seen (curved arrow, #6). The site where the clot probably ruptured deep into the left lateral ventricle is seen on (arrow, #7).', 'history': 'Patient with untreated hypertension presented in the ER with "stroke," manifested as sudden onset of right-sided weakness.', 'imagePoolId': 'e872f238-ce30-4f42-b89b-a9ff159a05f6', 'name': 'With intraventricular blood', 'teachingPoint': None, 'demographics': '72 Years old female'}], 'caseType': 'typical', 'name': 'TYPICAL'}
+- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '38bb3bea-d02a-440a-9737-cdeec3a9589c', 'description': 'Series of axial NECT scans (#1-7) show an unusual hypertensive hemorrhage. There is a large pontine hematoma that extends cephalad into the midbrain and dissects along both internal capsules into the basal ganglia. Blood is present in the fourth, third, and right lateral ventricle. Because of the unusual pattern, a CTA was obtained. Coronal (#8,9) and sagittal (#10-12) reconstructions show no evidence for aneurysm or vascular malformation. The unusual shape of the hematoma is especially well appreciated in image (#8), where the main pontine component and bilateral cephalad extension are demonstrated (arrows).', 'history': 'History of hypertension, "found down" by neighbor and brought to ED.', 'imagePoolId': '3223923e-8406-47f1-8ac3-1772135794cd', 'name': 'Pontine with massive extension', 'teachingPoint': None, 'demographics': '68 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'a5f933b7-d498-4a94-b194-db6e789fb9c6', 'description': 'Only 15-25% of hypertensive intracranial hemorrhages occur in the posterior fossa. When they do, they can be clinically devastating events.\n\nAxial NECT scans (images 1-3) show a large high density mass in the left cerebellar hemisphere (arrows) with some adjacent areas of slightly lesser increased attenuation (curved arrows) indicating active hemorrhage. Image 4 shows upward herniation of the cerebellum through the tentorial incisura with compression of the brainstem (open arrow). The patient expired shortly after the scan was obtained.', 'history': 'Elderly hypertensive, anticoagulated patient "found down" at home.', 'imagePoolId': '9c7f3058-46da-4549-9931-f33256b646fc', 'name': 'Active bleeding', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'b460d7ee-3bbc-4601-8e63-3f8271f745a8', 'description': 'Chronic hypertension may cause tiny, microscopic cerebral hemorrhages known as microbleeds. These typically occur in the setting of diffuse, severe white matter abnormalities.\n\nAxial T2 weighted MR scan (#1) shows diffuse, confluent high signal intensity in the deep periventricular white matter, especially around the atria and occipital horns of the lateral ventricles. The thalami are also severely affected. Multifocal hyperintensities are also present in the basal ganglia. Axial FLAIR scans (#2-3) show patchy increased signal intensity in the pons and major cerebellar peduncles as well as striking involvement of the deep white matter and thalami. Two definite foci of decreased signal intensity are seen in the thalami (#3, arrows).\n\nSelected images from the T2* (GRE) scan are shown in images 4-9. Innumerable microbleeds ("black dots") that show significant blooming artifact can be identified along with larger areas in the deep basal ganglia that represent residua from gross hemorrhage. \n\nWe recommend performing T2* scans in most patients over the age of 60 years.', 'history': 'Elderly demented patient with long-standing untreated hypertension.', 'imagePoolId': 'c7902657-316a-405f-b754-518da153f8bb', 'name': 'Microbleeds', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '895a7d07-9dad-433b-b497-294d7c4b732f', 'description': 'Axial NECT scan (#1) shows a slit-like low-density area in the external capsule (open arrow) with a residual focus of hematoma (arrow) in the lateral putamen. NECT scan (#2) obtained on second presentation shows new left parietal lobar hemorrhage (arrow) with surrounding edema. Third scan (#3), obtained one week later, shows resolving hematoma (arrow) with persisting edema. MR at the same time (#4) shows early subacute hemorrhage, with T1 shortening in the periphery of the lesion, while the center of the clot is isointense. T2WI (#5) shows most of the lesion is hyperintense. A dark hemosiderin rim is seen around the lesion (arrows).', 'history': 'Hypertensive patient who survived initial bleed then had another.', 'imagePoolId': '3439f3a1-8ebf-4949-abc0-604c0e297733', 'name': 'Two hemorrhages', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '5e734bec-79ac-4db9-a0cb-3651759b1ffe', 'description': 'Longstanding systemic hypertension with microvasculopathy is a common cause of multifocal hyperintense white matter lesions in older adult patients. Microbleeds are increasingly well-recognized as a less common but very important manifestation of longstanding hypertension.\n\nAxial T2-weighted scans (images 1-6) show multifocal scattered and confluent hyperintense foci in the basal ganglia and cerebellar and hemispheric white matter, especially adjacent to the lateral ventricles. T2* sequence (images 7-9) shows numerous "black dots" (arrows) that were inapparent on the T2WIs. Centered mostly in the basal ganglia, these represent the microbleeds of chronic hypertension. Microbleeds caused by amyloid angiopathy, another cause of "black dots" in the brain parenchyma of elderly patients, tend to primarily involve the cortex.', 'history': 'Older adult hypertensive, demented patient.', 'imagePoolId': '5b04bccb-f17f-43bc-b48d-70f50a1dd7c1', 'name': 'Microbleeds', 'teachingPoint': None}], 'caseType': 'variant', 'name': 'VARIANT'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*Axial graphic shows a classic acute hypertensive basal ganglia (BG)/external capsule hemorrhage
with dissection into the lateral ventricle. Surrounding edema is also present. Hemorrhage extends through the foramen of Monro to the 3rd ventricle.*
+
+
+*Axial CT in a hypertensive patient shows a classic appearance of a left BG hemorrhage
involving the anterior putamen. The putamen and external capsule are the most common location for hICH. Note the extensive intraventricular hemorrhage
.*
+
+
+*Axial CT in a 42-year-old woman with a history of hypertension shows the classic appearance of a large BG hemorrhage
with mass effect, surrounding edema, and intraventricular extension
.*
+
+
+*Axial CTA in the same patient shows a spot sign
indicating active bleeding. The spot sign suggests hematoma expansion and is highly predictive of neurologic deterioration and is associated with a worse prognosis. Mortality approaches 80% in patients with large hematomas.*
+
+
+*Axial SWI MR shows a hypertensive BG hematoma
and multiple hypointense foci "blooming" related to microbleeds
in this chronic hypertension patient. A central location (deep gray nuclei) of microhemorrhages can help differentiate hypertension from amyloid angiopathy, which typically has more peripheral hemorrhage.*
+
+
+*Axial NECT in a 36-year-old hypertensive man acute headache shows a frontal lobar hemorrhage
. Lobar hemorrhages represent 5-10% of hICH. CTA revealed an AVM.*
+
+
+*Coronal CT in a 27-year-old pregnant woman with pre-eclampsia, hypertension shows a parenchymal hematoma
with intraventricular hemorrhage. Given young age, additional imaging ultimately revealed reversible cerebral vasoconstriction syndrome (RCVS).*
+
+
+*Axial CTA in a patient who presented with a "hypertensive hemorrhage" shows a large MCA aneurysm
within a parenchymal hemorrhage
. CTA is often key in evaluation of an acute hemorrhage in a patient with an atypical history or an unusual hemorrhage location.*
+
+
+*Axial SWI MR in a young adult shows a right BG hemorrhage
. No additional blooming is present. This "hypertensive hemorrhage" was related to amphetamine abuse. Drug abuse should be considered in young patients.*
+
+
+*Axial CT in a 71-year-old hypertensive patient found down shows a large right BG hemorrhage
with marked mass effect and midline shift. Large hematomas have a high mortality rate of ~ 80%.*
+
+
+### Additional Images
+
+
+*Axial NECT shows a classic acute putaminal/external capsule hemorrhage
in this patient with untreated hypertension. Note the blood in the lateral ventricles
.*
+
+
+*Axial NECT shows a large, hypertensive bleed in the left cerebellum
with an adjacent area of lesser hyperdensity
indicating active bleeding. Blood is also in the lower 4th ventricle
.*
+
+
+*Axial NECT illustrates classic intrapontine high density, characteristic of hypertensive brainstem hemorrhage
. Note the blood that has dissected into the 4th ventricle
.*
+
+
+*Axial T1WI MR in a 38-year-old man with uncontrolled hypertension shows a mostly isointense mass in the left BG
. Note the possible old right basal ganglionic infarct
.*
+
+
+*Axial T2WI MR in the same patient shows acute, inhomogeneously hyperintense hemorrhage in the left BG
and a chronic right BG infarct surrounded by hemosiderin
.*
+
+
+*Axial T1WI C+ MR in the same patient shows a linear enhancing focus
with contrast accumulating in the outside margin of the clot
. The lesion is actively bleeding.*
+
+
+*Axial T2* GRE MR shows old left thalamic/internal capsule hemorrhage
. Innumerable "black dots"
represent residua from hypertensive microbleeds.*
+
+
+*Axial NECT shows classic acute intraparenchymal hematoma in the left BG/external capsule in this older woman with longstanding, poorly controlled systemic hypertension.*
+
+
+*Axial FLAIR MR shows late subacute hemorrhage in the BG extending into the insula. Signal is from extracellular methemoglobin, showing hyperintensity on T2WI.*
+
+
+*Axial NECT shows acute right BG/thalamic hemorrhage with intraventricular rupture. There is very little surrounding edema, in contrast to hemorrhage associated with tumor (spontaneous intracerebral hemorrhage).*
+
+
+*Axial T2WI MR shows a different pattern of chronic hypertension, with numerous scattered microbleeds
that show magnetic susceptibility. No acute hemorrhage is present.*
+
+
+*Axial T2WI MR shows numerous deeper microbleeds. These findings can be seen with cerebral amyloid angiopathy , chronic hypertension, and numerous small vascular malformations.*
+
+
+*Axial NECT in a patient with cocaine-induced hypertension shows a less typical location for a hypertensive hemorrhage (i.e., within the pons). Note the marked mass effect upon the 4th ventricle
.*
+
+
+*Axial T1WI MR shows a less typical location for a hICH (in the anterior right temporal lobe, lateral to the BG). The clot is ~ 1 week old (hyperintensity is due to extracellular metHb).*
+
+
+*Axial NECT scan shows a classic hypertensive BG hemorrhage
. Intraventricular rupture
is common with large bleeds.*
+
+
+*Axial T2* GRE MR shows numerous "black dots"
that were inapparent on T2W images. Most are in the deep gray matter of the thalami. These represent the microbleeds of chronic hypertension, which are a less common, but important, manifestation of chronic systemic hypertension.*
+
+
+*Axial T2* GRE MR in a patient with a remote history of hypertensive hemorrhage in the left putamen and external capsule
shows multifocal "black dots" in the BG and thalami
with only a few lesions in the cortex
.*
+
+
+*Axial CT in a 55-year-old man who presented to the ER with an acute stroke shows a hemorrhage in the right temporal lobe and external capsule with surrounding edema and mass effect.*
+
+
+*Coronal CTA in the same patient shows a large MCA aneurysm
as the etiology for this patient's acute hemorrhage. CTA is often key in evaluation of an acute hemorrhage in a patient with an atypical history or an unusual hemorrhage location.*
+
+
+*Coronal CTA in a young patient with a right BG hemorrhage
shows displacement of the lenticulostriate arteries
medially, compared to the normal left side
by the hematoma. There is no spot sign that would indicate active bleeding. No underlying vascular lesion is present.*
+
+
+*Axial NECT in an older hypertensive man shows a right cerebellar hemorrhage. The posterior fossa (pons, cerebellum) is a relatively uncommon location (~ 10%) for hypertensive hemorrhages, yet it is the 3rd most common overall site (after the BG and thalami).*
+
+
+*Axial CT in a 61-year-old woman with a history of hypertension shows the classic appearance of a left BG hemorrhage involving the putamen and external capsule (striatocapsular). Note the additional areas of periventricular hypodensity
likely related to chronic small vessel ischemia.*
+
+
+*CTA in an 80-year-old with acute occipital hemorrhage shows contrast extravasation
into the hematoma; a spot sign indicates active bleeding. Surgery disclosed an actively bleeding hemorrhagic metastasis. Lobar hemorrhages account for just 5-10% of hypertensive bleeds.*
+
+
+*Axial CTA in a patient who presented with a "hypertensive hemorrhage" shows a large MCA aneurysm
. CTA is often key in evaluation of an acute hemorrhage in a patient with an atypical history or an unusual hemorrhage location. If subarachnoid hemorrhage is present, consider lobar ICH caused by an underlying vascular abnormality.*
+
+
+*Axial NECT in a 52-year-old hypertensive man with sudden onset of multiple cranial neuropathies shows a pontine hemorrhage
. The posterior fossa (pons, cerebellum) is a relatively uncommon location (~ 10%) for hypertensive hemorrhages.*
+
+
+*Coronal CT in a 65-year-old man who presented to the ER with an acute stroke shows a large hemorrhage
in the right BG with surrounding edema and significant mass effect with midline shift. Intraventricular hemorrhage is also noted, a common feature of hypertensive hemorrhage.*
+
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+++ b/docs_md/articles/intracranial-atherosclerosis_c5ef0315-edbd-4ace-8930-484a116610b9.md
@@ -0,0 +1,515 @@
+---
+title: "Intracranial Atherosclerosis"
+docid: "c5ef0315-edbd-4ace-8930-484a116610b9"
+authors:
+ - key: "2bca6b86-1eca-4e93-b997-4e18913686a7"
+ value: "Hediyeh Baradaran, MD, MS"
+ - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
+ value: "Anne G. Osborn, MD, FACR"
+breadcrumbs:
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+ name: "Brain"
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+ -
+ name: "Atherosclerosis and Carotid Stenosis"
+ slug: "atherosclerosis-and-carotid-stenos-"
+ treeNodeId: "c38e4a24-1b9d-4aeb-9f0c-10bff9fdb11d"
+ -
+ name: "Intracranial Atherosclerosis"
+ slug: "intracranial-atherosclerosis"
+ treeNodeId: null
+category: "Brain"
+documentVersionId: "4af5b6a7-d2b8-4775-b0de-b6b37cc8d3a4"
+imageCount: 27
+lastUpdated: "10/01/25"
+pageDescription: "Intracranial Atherosclerosis"
+pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Stroke, Atherosclerosis and Carotid Stenosis, Intracranial Atherosclerosis"
+pageTitle: "Intracranial Atherosclerosis | STATdx"
+enhancedTitle: "Intracranial Atherosclerosis"
+type: "DX"
+references: true
+anatomy:
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+ - "{'authors': 'Stella Sin Yee Ho, RDMS, RVT, PhD; Deyond Y. W. Siu, MBChB, FRCR; Paula J. Woodward, MD, FSRU', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/914fd68b-ddab-4640-bcc4-883c425d13f8', 'category': 'Ultrasound', 'compareUrl': '/compare/document/914fd68b-ddab-4640-bcc4-883c425d13f8/related-anatomy/treeNode?subContext=Transcranial Doppler', 'documentId': '914fd68b-ddab-4640-bcc4-883c425d13f8', 'documentType': 'ANATOMY', 'documentUrl': '/document/transcranial-doppler/914fd68b-ddab-4640-bcc4-883c425d13f8', 'enhancedTitle': 'Transcranial Doppler', 'entryDate': '10/20/20', 'imageCount': 61, 'imageUrl': '/image/thumbnail/04e86bc1-0497-4d3f-8a6f-09a7e2f77575?size=174&quality=85', 'inCompareCart': False, 'rank': 2, 'referenceCount': 0, 'showCompareButton': False, 'title': 'Transcranial Doppler'}"
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+ - "{'authors': 'Anne G. Osborn, MD, FACR; Edward P. Quigley, III, MD, PhD; Adriene C. Eastaway, MD, MS', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/0ff112d1-0e58-4694-9b49-0a0327ea1310', 'category': 'Brain', 'compareUrl': '/compare/document/0ff112d1-0e58-4694-9b49-0a0327ea1310/related-anatomy/treeNode?subContext=Vertebrobasilar System', 'documentId': '0ff112d1-0e58-4694-9b49-0a0327ea1310', 'documentType': 'ANATOMY', 'documentUrl': '/document/vertebrobasilar-system/0ff112d1-0e58-4694-9b49-0a0327ea1310', 'enhancedTitle': 'Vertebrobasilar System', 'entryDate': '10/20/20', 'imageCount': 23, 'imageUrl': '/image/thumbnail/abd9ea97-2366-40d5-8be0-a908b2b9e655?size=174&quality=85', 'inCompareCart': False, 'rank': 5, 'referenceCount': 0, 'showCompareButton': False, 'title': 'Vertebrobasilar System'}"
+cases: 2
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+ - "Brain"
+ - "Diagnosis"
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+ - "Stroke"
+ - "Atherosclerosis and Carotid Stenosis"
+ - "Intracranial Atherosclerosis"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Intracranial atherosclerotic vascular disease (ASVD)
+ - Intracranial atherosclerotic disease (ICAD)
+ - Intracranial atherosclerotic stenosis (ICAS)
+- ### Imaging
+
+
+ - Vessel wall imaging (VWI) now gold standard for evaluating ICAS; identifies high-risk features
+ - Crescent-shaped or eccentric thickening
+ - High-risk features
+ - Plaque enhancement (typically crescentic/incomplete, short segment)
+ - Vessel remodeling
+ - Plaque surface irregularity/ulceration
+ - Intraplaque hemorrhage (much less common than in extracranial ASVD)
+ - CTA/MRA/DSA: Visualizes lumen, not wall
+ - Focal stenosis, luminal irregularities in cortical vessels can mimic vasculitis
+ - Most common cause of vasculitic-like appearance at angiography in middle-aged/older patient is intracranial ASVD
+ - Prevalence of high-risk plaque on VWI with zero or mild stenosis on DSA ~ 50%
+ - Associated with ischemic stroke, poor outcome
+- ### Top Differential Diagnoses
+
+
+ - Vasculitis/arteritis
+ - Vasospasm, dissection
+ - Nonocclusive thrombus or embolus
+- ### Clinical Issues
+
+
+ - Disease burden of intracranial ASVD greatly underestimated
+ - "Cryptogenic" stroke/embolic stroke of undetermined source = 25-40%
+ - No cardiac cause or extracranial ASVD stenosis
+ - ICAD now recognized as important stroke etiology
+ - Most common intracranial vascular stenosis in adults
+ - Independent correlation with T2/FLAIR hyperintensities
+ - Poor prognosis unless treated aggressively
+
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - Intracranial atherosclerotic vascular disease (ASVD)
+ - Intracranial atherosclerotic stenosis (ICAS)
+ - Vessel wall imaging (VWI)
+- ### Definitions
+
+
+ - Narrowing or ectasia of intracranial arteries secondary to ASVD
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - ASVD plaque in vessel wall directly visualized with high-resolution VWI
+ - VWI > > modalities depicting lumen (DSA, CTA, MRA)
+ - Crescent-shaped or eccentric thickening
+ - "High-risk" features
+ - Plaque enhancement (classically crescentic/incomplete, short segment)
+ - Vessel remodeling
+ - Plaque surface irregularity/ulceration
+ - Intraplaque hemorrhage (much less common than in extracranial ASVD)
+ - Stenotic intracranial artery on CTA/MRA/DSA
+ - Visualizes lumen, not vessel wall
+ - Prevalence of high-risk plaque on VWI with zero or mild stenosis on DSA ~ 50%
+ - Strongly associated with ischemic stroke, unfavorable outcome
+ - #### Location
+
+
+ - Distal basilar artery (BA), cavernous/supraclinoid ICA most common
+ - Less common sites
+ - Circle of Willis (COW)
+ - MCA less common **but** high stroke risk
+ - #### Morphology
+
+
+ - Most common: Eccentric, irregular thickening of vessel wall ± ulceration, hemorrhage
+ - Less common: Dolichoectasia
+ - Enlargement/tortuosity without stenosis
+- ### CT Findings
+
+
+ - #### NECT
+
+
+ - Mural Ca⁺⁺
+ - #### CTA
+
+
+ - CTA has high sensitivity/specificity
+ - In patients with > 50% stenosis or occlusion of large arteries
+ - Caveat: Mural Ca⁺⁺ may ↓ specificity
+- ### MR Findings
+
+
+ - #### T1WI
+
+
+ - ↓/absent flow void
+ - Also seen in slow flow
+ - Proximal (extracranial) stenosis, dissection
+ - VWI may show crescentic, asymmetric hyperintensity of intraplaque hemorrhage
+ - #### T2WI
+
+
+ - ↓/absent flow void on standard T2WI
+ - T2-hyperintense plaque on high-resolution VWI
+ - #### FLAIR
+
+
+ - Slow flow or occlusion may appear hyperintense
+ - Dot sign (slow flow in distal branches)
+ - Intracranial ASVD independently associated with progressively greater white matter hyperintensity (WMH) burden
+ - #### T1WI C+ FS
+
+
+ - VWI may demonstrate short segment, irregular enhancement
+ - Degree of enhancement similar to or greater than pituitary infundibulum
+ - May demonstrate contrast filling mural ulcerations
+ - #### MRA
+
+
+ - 3D TOF contrast-enhanced MRA
+ - Focal stenosis, ectasia, or irregularity
+ - 3D TOF may overestimate stenosis
+ - Secondary to spin saturation
+ - Poor evaluation for slow, in-plane flow
+ - Enhanced MRA less affected by spin saturation (also faster)
+ - Combined with CTA, sensitivity/specificity ~ DSA
+ - CTA > MRA for evaluation of in-stent restenosis
+ - Dolichoectasia may also cause reduced flow
+- ### Ultrasonographic Findings
+
+
+ - Transcranial Doppler (TCD): ↑ velocities
+- ### Angiographic Findings
+
+
+ - DSA may show
+ - Focal stenosis, luminal irregularities
+ - Thrombosis, occlusion
+ - Ectasia/elongation
+ - "Giant" serpentine/fusiform aneurysms (less common)
+ - DSA goals include
+ - Grade extracranial stenosis, criteria standardized by NASCET (North American Symptomatic Carotid Endarterectomy Trial)
+ - Identify "tandem" lesions
+ - Assess collateral status
+ - Potential assessment of plaque ulceration
+ - Potential intervention (angioplasty ± stenting)
+ - Best for poststenting evaluation
+ - Measuring ICAS
+ - Warfarin-aspirin symptomatic intracranial disease (WASID) method
+ - % stenosis = [1- (diameter stenosis/diameter normal)] x 100
+ - For MCA, intracranial vertebral and BAs use most severe degree of stenosis vs. diameter of proximal artery at its widest, nontortuous normal segment
+- ### Imaging Recommendations
+
+
+ - #### Best imaging tool
+
+
+ - Gold standard is now high-resolution MR with VWI
+ - DSA/CTA/MRA depict lumen, not wall
+ - #### Protocol advice
+
+
+ - High-resolution thin-section 3T
+ - Black blood VWI
+ - CTA or MRA for proximal intracranial stenoses
+
+## DIFFERENTIAL DIAGNOSIS
+
+- [Vasculitis/Arteritis](/document/primary-arteritis-of-cns/490b3aed-37e2-4ec6-95dd-76efc734490f)
+ - Usually involves smaller (tertiary) branches, more frequently circumferential enhancement, lack of T2-hyperintense plaque
+ - More likely associated with parenchymal hemorrhage, subarachnoid hemorrhage
+ - Can be primary or secondary
+ - Often associated with systemic disease
+ - Elevated ESR, autoimmune parameters
+- [Vasospasm](/document/vasospasm/397752a7-6998-4375-8090-e097d775d180)
+ - Subarachnoid hemorrhage related, maximal 7 days post bleed
+ - Drug related (sympathomimetics)
+- [Moyamoya](/document/moyamoya/a8059f6b-95e2-4fb2-82aa-5b51ee9dcd1a)
+ - Usually involves distal ICA and proximal COW with relative sparing of BA
+ - Frequently bilateral
+- ### Dissection
+
+
+ - Smooth tapering
+ - T1-hyperintense crescent = thrombus, best seen with fat-sat sequences
+ - Younger patients
+ - Can have minimal or no history of trauma
+- ### Nonocclusive Thrombus or Embolus
+
+
+ - Appearance of rounded, central nonopacification with peripheral enhancing rim on contrast study
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Etiology
+
+
+ - Atherosclerosis is chronic inflammatory disease that is systemic, multifactorial
+ - Intracranial arteries are muscular, with few elastic fibers
+ - Adventitia less abundant, media thinner
+ - Compared with extracranial vessels, proliferative fibrosis more prevalent than lipid infiltration
+ - "Unstable" atherosclerotic plaques → stenosis, artery-to-artery embolism, downstream hypoperfusion
+ - Probably multiple etiologies of ASVD
+ - Lipid hypothesis
+ - High plasma LDL leads to LDL-cholesterol deposition in intima
+ - Response to injury hypothesis
+ - Focal endothelial change or intimal injury leads to platelet aggregation and plaque formation
+ - Unifying hypothesis
+ - Endothelial injury leads to ↑ permeability of LDL; plaques grow by thrombus formation on plaque surface and transendothelial leakage of plasma lipids
+ - Smoking associated with intracranial atherosclerosis
+ - Intracranial atherosclerosis associated with atherosclerosis of carotids, coronaries, aorta, renal arteries, iliofemoral system
+ - #### Associated abnormalities
+
+
+ - Anatomy
+ - Most often involves arterial bifurcations, e.g., ICA and BA
+ - May involve distal arterioles leading to vasculitis pattern of alternating stenosis and dilatation
+- ### Staging, Grading, & Classification
+
+
+ - WASID is standardized method for measuring ICAS
+- ### Gross Pathologic & Surgical Features
+
+
+ - Earliest macroscopic finding: Intimal fatty streaks
+ - Fibrous atheromatous plaques contain
+ - Smooth muscle cells, monocytes, other leukocytes
+ - Connective tissue: Collagen, elastic fibers, proteoglycans
+ - Intra- and extracellular lipid deposits
+ - Angiogenesis produces new capillaries at plaque periphery
+ - Leads to intraplaque hemorrhage and ulceration
+ - Hemorrhage leads to dystrophic ferrocalcinosis (seen as calcification on CT, iron on MR)
+ - Arterial narrowing due to plaque
+ - Flow limiting beyond 50% ICAS
+ - Ischemic symptoms depend on collaterals
+ - Slow occlusion leads to more collaterals, fewer symptoms
+ - Rapid occlusion (from thrombosis or emboli) does not permit time for collaterals to develop, infarct likely
+ - Arterial irregularity from disrupted endothelium may form thrombogenic surface leading to thrombosis or emboli
+- ### Microscopic Features
+
+
+ - Intracranial plaque components
+ - Lipid, fibrous tissue, calcium
+ - Chronic inflammatory markers (e.g., IL-6)
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - Transient ischemic attack, due to emboli, severe stenosis, progressive occlusion
+ - Plaque rupture usually leads to stroke
+ - Vascular stenosis leads to stuttering ischemia from intermittent thrombosis
+- ### Demographics
+
+
+ - #### Age
+
+
+ - Older age
+ - #### Sex
+
+
+ - M = F
+ - #### Epidemiology
+
+
+ - Disease burden of intracranial ASVD underestimated
+ - 3rd most common cause of thromboembolic stroke after carotid and cardiac sources
+ - Basis for cerebral thromboembolism in > 90%
+ - Most common cause of intracranial vascular stenosis in adults
+ - ↑ prevalence in Black, Asian, Hispanic, Indian populations
+ - 15-30% of strokes in Blacks, 30-50% in Asians
+- ### Natural History & Prognosis
+
+
+ - Poor; progressive disease unless treated aggressively
+ - High risk of recurrent stroke
+- ### Treatment
+
+
+ - Low saturated fat and cholesterol diet and exercise
+ - Cholesterol-lowering drugs ("statins") if lifestyle interventions are insufficient
+ - Plaque stabilization ("statins") may ↓ stroke
+ - Angioplasty &/or stenting in some cases
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - CTA &/or MRA as excellent screening tool
+ - High-resolution VWI
+- ### Image Interpretation Pearls
+
+
+ - Status of collaterals important; patients with developed collaterals tolerate stenosis/occlusion better
+
+ dc196abf-8592-4d1d-a583-871e741b227d
+
+## References
+
+## Selected References
+
+1. [Sanchez S et al: Comprehensive imaging analysis of intracranial atherosclerosis. J Neurointerv Surg. 17(3):311-20, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=38719445%5Bpmid%5D)
+1. [Chen LH et al: Epidemiology, pathophysiology, and imaging of atherosclerotic intracranial disease. Stroke. 55(2):311-23, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38252756%5Bpmid%5D)
+1. [Song JW et al: Vessel wall magnetic resonance imaging biomarkers of symptomatic intracranial atherosclerosis: a meta-analysis. Stroke. 52(1):193-202, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33370193%5Bpmid%5D)
+1. [Vranic JE et al: High-resolution magnetic resonance vessel wall imaging for the evaluation of intracranial vascular pathology. Neuroimaging Clin N Am. 31(2):223-33, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33902876%5Bpmid%5D)
+1. [Leao DJ et al: Intracranial vessel wall imaging: applications, interpretation, and pitfalls. Clin Radiol. 75(10):730-9, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32197916%5Bpmid%5D)
+1. [Arenillas JF et al: Intracranial arterial wall imaging: techniques, clinical applicability, and future perspectives. Int J Stroke. 14(6):564-73, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30982434%5Bpmid%5D)
+1. [Baek JH et al: Angiographical identification of intracranial, atherosclerosis-related, large vessel occlusion in endovascular treatment. Front Neurol. 10:298, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31040811%5Bpmid%5D)
+1. [Kern KC et al: Vessel wall imaging of cerebrovascular disorders. Curr Treat Options Cardiovasc Med. 21(11):65, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31728661%5Bpmid%5D)
+1. [Wang Y et al: Culprit intracranial plaque without substantial stenosis in acute ischemic stroke on vessel wall MRI: a systematic review. Atherosclerosis. 287:112-21, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31254918%5Bpmid%5D)
+1. [Wang Y et al: Intracranial atherosclerotic disease. Neurobiol Dis. 124:118-32, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30439443%5Bpmid%5D)
+1. [Xu W: High-resolution MRI of intracranial large artery diseases: how to use it in clinical practice? Stroke Vasc Neurol. 4(2):102-4, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31338221%5Bpmid%5D)
+1. [Dakay K et al: Symptomatic intracranial atherosclerosis with impaired distal perfusion: a case study. Stroke. 49(1):e10-13, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29167389%5Bpmid%5D)
+1. [Tan HW et al: Intracranial vessel wall imaging with magnetic resonance imaging: current techniques and applications. World Neurosurg. 112:186-98, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29360586%5Bpmid%5D)
+1. [de Havenon A et al: High-resolution vessel wall MRI for the evaluation of intracranial atherosclerotic disease. Neuroradiology. 59(12):1193-202, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28942481%5Bpmid%5D)
+1. [Mandell DM et al: Intracranial vessel wall MRI: principles and expert consensus recommendations of the American Society of Neuroradiology. AJNR Am J Neuroradiol. 38(2):218-29, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27469212%5Bpmid%5D)
+1. [Pu Y et al: Intracranial atherosclerosis: from anatomy to pathophysiology. Int J Stroke. 12(3):236-45, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28067615%5Bpmid%5D)
+1. [Gupta A et al: Gadolinium enhancement in intracranial atherosclerotic plaque and ischemic stroke: a systematic review and meta-analysis. J Am Heart Assoc. 5(8), 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27528408%5Bpmid%5D)
+1. [Lehman VT et al: Clinical interpretation of high-resolution vessel wall MRI of intracranial arterial diseases. Br J Radiol. 89(1067):20160496, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27585640%5Bpmid%5D)
+1. [Gounis MJ et al: Imaging inflammation in cerebrovascular disease. Stroke. 46(10):2991-7, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26351362%5Bpmid%5D)
+1. [Holmstedt CA et al: Atherosclerotic intracranial arterial stenosis: risk factors, diagnosis, and treatment. Lancet Neurol. 12(11):1106-14, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24135208%5Bpmid%5D)
+1. [Samuels OB et al: A standardized method for measuring intracranial arterial stenosis. AJNR Am J Neuroradiol. 21(4):643-6, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10782772%5Bpmid%5D)
+
+## Anatomy
+
+### Carotid Space
+Head and Neck/ANATOMY:627bdee1-4bde-46f2-b93d-958882586337
+
+### Transcranial Doppler
+Ultrasound/ANATOMY:914fd68b-ddab-4640-bcc4-883c425d13f8
+
+### Aortic Arch and Great Vessels
+Brain/ANATOMY:a7a252f0-2ac6-402a-8c87-cfce8adc799b
+
+### Intracranial Internal Carotid Artery
+Brain/ANATOMY:7e9c58f7-6ba0-41e2-a363-43299fac430e
+
+### Vertebrobasilar System
+Brain/ANATOMY:0ff112d1-0e58-4694-9b49-0a0327ea1310
+
+## Cases
+
+- {'cases': [{'authors': [{'key': 'a46404bc-5d2a-4aa8-82da-5ab48b0df998', 'value': 'Juan F. Gomez, MD'}], 'caseVersionId': '7993d687-553e-46a4-8207-2f885d7761c6', 'description': 'Typical angiographic images of intracranial atherosclerotic disease.\n\nAP (#1) and lateral (#2) right vertebral artery angiogram shows a severe stenosis of the proximal basilar artery (curved arrows, #1-2) from atherosclerosis. In addition, other areas of disease involvement are noted in the right posterior cerebral artery (arrows, #1-2) and distal right vertebral artery (open arrows, #1-2).\n\nAP (#3) left common carotid artery angiogram shows stenosis involving the supraclinoid internal carotid artery (open arrow, #3) and distal middle cerebral artery M1 and M2 segments (arrows, #3). Diffuse subtle luminal irregularity of the visualized arteries from atherosclerotic disease is also visualized. Incidentally noted is a hypoplastic left anterior cerebral artery A1 segment.\n\nComment: The signs and symptoms of atherosclerotic disease depend on its location, the severity of stenosis that it induces, and the development of collateral circulation. Digital subtraction angiography remains the gold standard for the evaluation of intracranial atherosclerotic disease. However, differential diagnoses such as vasculitis still represent a diagnostic challenge. Care should be taken to differentiate congenital hypoplasia from narrowing induced by atherosclerotic disease.', 'history': 'History of transient ischemic attacks.', 'imagePoolId': '58452e0b-3df8-4e48-bb28-1e2f0ce018c2', 'name': 'Intracranial arterial occlusive disease', 'teachingPoint': None, 'demographics': '75 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '18b13b69-4922-4430-be3e-a0bc90bd67cf', 'description': 'Axial NECT scans (#1, 3) with corresponding bone CT (#2,4) show calcification in the supraclinoid internal carotid arteries (arrows). Note that the patient was poorly positioned when initially scanned (#1, 2) and the symmetric nature of the unusually extensive vascular calcification was not apparent until the patient was repositioned and rescanned (#3, 4).', 'history': 'Elderly patient with trauma. ', 'imagePoolId': '79ace041-1dc8-4353-ac45-90125973a649', 'name': 'Calcified suprasellar ICA', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'e5e68b66-9075-403c-bad3-7366195b9806', 'description': 'Sagittal T1WI (#1) shows an elongated basilar artery with a thickened wall (arrows). Note mass effect on the hypothalamus and anterior third ventricle caused by the ectatic artery (curved arrow). The T2WI (#2) shows the "flow void" of the ectatic artery (arrow) compresses the pons. Axial (#3) and coronal (#4) T1 C+ scans show the ectatic basilar artery enhances strongly. \n\nVertebrobasilar dolichoectasia is one of the most common manifestations of intracranial atherosclerosis.', 'history': 'Asymptomatic elderly patient.', 'imagePoolId': '166bae87-98ea-49cc-b3d0-85b2670a4e72', 'name': 'Vertebrobasilar dolichoectasia', 'teachingPoint': None}], 'caseType': 'typical', 'name': 'TYPICAL'}
+- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'fb0d9f36-c8a2-4b2a-aed6-dd0c07536a2c', 'description': 'Sagittal (#1), axial (#2) and coronal (#3,4) images from CTA are shown. They were initially called normal. However, there is a very high grade stenosis at the left distal supraclinoid ICA just proximal to its terminal bifurcation (arrows), compared to the normal right side (open arrows). \n\nMR was obtained. The left supraclinoid ICA tapers down to a thread-like "flow void" as shown on the axial T1WI (arrow, #5). FLAIR scan through the superficial sulci (#6) shows hyperintense CSF signal on the left side compared to the normally suppressed, hypointense CSF on the right. Post-contrast axial (#7,8) and coronal (#9) images show contrast-enhancing sulci on the left, actually representing slow flow in pial branches of the MCA caused by the high-grade ICA stenosis. \n\nDSA was performed and confirms 95% stenosis of the distal left ICA (arrows, #10-13). \n\nSlow antegrade or retrograde flow through pial cortical branches may cause the appearance of hyperintense sulci on FLAIR and enhancement on T1C+ scans.', 'history': 'TIAs. NECT scan in ER was negative, so CTA with CT perfusion was obtained. Symptoms slowly resolved.', 'imagePoolId': 'aa1e3c61-f0c1-4272-a53b-a2b30db330e1', 'name': 'Pial collaterals enhance', 'teachingPoint': None, 'demographics': '51 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'fd3bc5a0-030d-4b19-a714-66970251be91', 'description': 'A series of axial T2WIs (#1-3) show a somewhat unusual pattern of intracranial white matter hyperintensities, sometimes called "UBOs" (unknown bright objects). The lesions are confined to the right hemisphere and form almost a distinct line in the deep cerebral white matter (#2, 3, arrows). Note the normal "flow void" in the left cavernous internal carotid artery (#1, open arrow). No such flow void is seen in the right cavernous sinus. Instead, there is hyperintensity within the lumen of the right cavernous internal carotid artery (#1, curved arrow). \n\nAngiography in this patient (not shown) disclosed an occluded right cervical internal carotid artery with no flow in the cavernous segment. The right hemisphere was largely supplied from the left ICA via a patent anterior communicating artery.\n\nLow-flow vascular hemodynamics may cause infarcts along watershed zones. The deep cerebral white matter has its own watershed zone (as of course does the cortex). The white matter watershed is at the junction of the deep penetrating arteries from the cortex with penetrating branches from the circle of Willis (like the lenticulostriate and thalamoperforating arteries). Infarcts along this zone may cause the appearance of so-called "rosary-like" white matter lesions that form a line along the affected deep white matter watershed. This patient probably had a high grade carotid stenosis for many years before the cervical ICA occluded.', 'history': 'Right hemisphere TIAs.', 'imagePoolId': '66aba136-b1b8-4494-867d-725fd8d9acda', 'name': 'Deep white matter watershed', 'teachingPoint': None}, {'authors': [{'key': '07a2c087-6202-49e7-870b-7aa162d18f06', 'value': 'Bronwyn E. Hamilton, MD'}], 'caseVersionId': '7c31b16d-633b-4be0-90f8-08e8fbc82563', 'description': 'Digital subtraction angiography (#1-2) demonstrates multifocal areas of irregular narrowing in the vertebrobasilar system (curved arrows). Similarly, internal carotid artery injection (#3-4) demonstrates multifocal arterial stenoses (curved arrows). \n\nIntracranial atherosclerosis most commonly involves arterial bifurcations, and may appear as alternating foci of stenosis and dilatation in distal arterioles. It is the most common cause of intracranial vascular narrowing in adults. The appearance can be difficult to differentiate from vasculitis however, particularly when more distal branches are involved. Correlating with clinical history is helpful. Ischemic symptoms may depend on the status of collateral vessels.', 'history': 'Patient with a long history of smoking presents with recent ischemic symptoms.', 'imagePoolId': '5d20da28-8749-45c2-aa71-21063c937368', 'name': 'Vasculitis mimic', 'teachingPoint': None, 'demographics': '51 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '8cfd1920-a6a0-42f8-b8ea-7a025e7efcc1', 'description': 'Anteroposterior view (#1), digital subtraction angiogram, left common carotid artery shows about a 70% stenosis of the internal carotid artery just distal to its origin (arrow). Note subtraction artifact caused by calcified plaque (open arrows). Lateral view (#2) of the intracranial circulation shows a second, irregular stenosis in the cavernous segment of the internal carotid artery (arrows). \n\nWhen two flow-limiting lesions are present in the same vessel, the hemodynamic effect is additive. In this case, the presence of a so-called "tandem" lesion means stenting the cervical ICA would still leave a distal flow-limiting lesion. EC-IC bypass was considered but not performed in this patient, who was treated medically.', 'history': 'Transient ischemic attacks.', 'imagePoolId': '7d1afced-cff8-456e-8b30-a3b6783b6f0c', 'name': '"Tandem" stenoses', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '1106366a-229d-4b4c-acec-29b997e4b228', 'description': 'Axial T2WIs (#1-3) show multifocal white matter hyperintensities in the right centrum semiovale (#3, arrows). Note that the lesions form almost a straight line from anterior to posterior. Refer back to images 1 and 2. There is a prominent "flow void" in the left cavernous carotid artery. The expected "flow void" of the right ICA is missing. The cavernous ICA is thrombosed. The "rosary-like" lesions seen on image #3 are caused by low hemodynamics at the deep white matter watershed zone, which is at the junction of the deep penetrating branches of the cortical (pial) vessels and the long, unpaired penetrating branches from the circle of Willis (e.g., thalamoperforating and lenticulostriate vessels).\n\nThe lesson of this case? Look for the "flow voids" in the cavernous sinus. The carotid occlusion was initially overlooked in this patient.', 'history': 'Right hemisphere TIAs.', 'imagePoolId': '00db0082-211b-4ac4-ab7c-98a1d9fa9bf7', 'name': 'Occluded cavernous ICA', 'teachingPoint': None}], 'caseType': 'variant', 'name': 'VARIANT'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*Coronal graphic shows atherosclerotic plaques (ASVD)
involving the major intracranial arteries and their branches. Inset shows penetrating (lenticulostriate) arteries
and lacunar infarcts
. ASVD plaques are typically short segment, irregular, noncircumferential, and may enhance.*
+
+
+*Coronal graphic shows atherosclerotic plaques (ASVD)
involving the major intracranial arteries and their branches. Inset shows penetrating (lenticulostriate) arteries
and lacunar infarcts
. ASVD plaques are typically short segment, irregular, noncircumferential, and may enhance.*
+
+
+*AP DSA of left internal carotid angiogram in a 72-year-old woman with stroke shows a high-grade stenosis
of the left M1 middle cerebral artery (MCA) segment just before the origin of the anterior temporal artery.*
+
+
+*Axial high-resolution black blood VWI shows thick, circumferential, short-segment enhancement of the distal M1 MCA
. Note high-grade luminal stenosis
. VWI is the now gold standard for evaluating ICAS.*
+
+
+*Coronal high-resolution black blood VWI shows the vessel wall enhancement
is asymmetric. Note parenchymal enhancement
in the basal ganglia caused by the lateral lenticulostriate artery infarct. Intracranial atherosclerotic stenoocclusive disease was documented later at autopsy.*
+
+
+*3D TOF MRA demonstrates severe stenosis of the right cavernous internal carotid artery (ICA)
in a 63-year-old man with several vascular risk factors.*
+
+
+*Axial high-resolution T2 VWI shows eccentric T2 hyperintensity in the cavernous ICA in the area of severe stenosis
, compatible with atherosclerotic plaque. VWI is the gold standard, as it identifies high-risk features, including plaque enhancement, vessel remodeling, plaque surface irregularity/ulceration, and plaque hemorrhage.*
+
+
+*Axial high-resolution black blood VWI postcontrast demonstrates enhancement associated with the eccentric atherosclerotic plaque in the right ICA
. Enhancing plaques are strongly associated with ischemic stroke risk.*
+
+
+*Sagittal high-resolution black blood VWI shows enhancing atherosclerotic plaque in the left M1 segment
. This patient had an infarct
but had a "normal" CTA due to the nonstenotic nature of the plaque, highlighting the value of VWI in cases of cryptogenic stroke.*
+
+
+*Axial CTA MIP with multifocal stenoses throughout the MCAs and posterior cerebral arteries (PCAs)
related to intracranial atherosclerosis. Intracranial atherosclerosis typically involves vessels more proximally.*
+
+
+*Sagittal CTA MIP in a patient with vascular disease shows with multifocal stenosis within the visualized basilar artery
related to intracranial atherosclerosis.*
+
+
+### Additional Images
+
+
+*Autopsy case of vertebrobasilar dolichoectasia shows yellow atheromatous plaques
in an extremely tortuous basilar artery. Note the mild ectasia of both middle cerebral arteries
. (Courtesy R. Hewlett, MD.)*
+
+
+*Sagittal (L) and coronal (R) CTAs reformatted from the axial source data show an enlarged, elongated, moderately tortuous vertebrobasilar artery
. Note ectasia of the proximal PCAs
.*
+
+
+*Axial T2 MR shows a typical pontine perforating artery infarct
. The basilar artery "flow void"
appears normal.*
+
+
+*Axial thin-section 3T T1 FS MR in the same patient with subacute right pontine infarct
shows a crescent of hyperintensity
in the wall of the basilar artery suggesting intraplaque hemorrhage.*
+
+
+*Axial thin-section T2 MR in the same patient shows that the subacute mural hematoma is hyperintense
. Note narrowed residual lumen ("flow void")
of the atherosclerotic basilar artery.*
+
+
+*Axial T2* GRE MR in the same patient shows "blooming" in the wall of the basilar artery
suggesting intramural hemorrhage.*
+
+
+*Axial thin-section 3T T1 C+ FS MR in the same patient shows enhancement of the subacute infarct
. Partial enhancement of the basilar artery wall is also present
, consistent with inflammation within an ASVD plaque.*
+
+
+*Lateral DSA of a vertebrobasilar angiogram shows multifocal areas of irregularity and stenosis
secondary to atherosclerotic plaques in the basilar artery. Note "beading" of the anterior inferior
and posterior
inferior cerebellar arteries.*
+
+
+*Lateral angiography during arterial phase shows severe focal stenosis in the proximal basilar artery
. Note the tandem stenosis
in the distal basilar artery.*
+
+
+*Lateral angiography during arterial phase in the same patient shows nonocclusive severe atheromatous narrowing of the basilar artery proximally
and distally
. It is important to confirm stenoses using at least 2 projections.*
+
+
+*A 68-year-old man in the ER with acute stroke symptoms had a negative NECT (not shown). MR shows extensive focal and confluent hyperintensities
in the coronal radiata and deep periventricular white matter (WM) of both hemispheres.*
+
+
+*Axial DWI MR in the same patient shows multiple foci of restricted diffusion
in the left deep hemispheric WM. This represents ischemia in the internal "watershed" zone between the perforating arteries and major territorial vessels.*
+
+
+*CT perfusion in the same patient in the ER with "stroke" shows significantly prolonged mean transit time (MTT) in the deep WM of the left cerebral hemisphere
with relative sparing of the overlying cortex
. This suggests abnormal perfusion in the deep internal watershed zone of the left hemisphere.*
+
+
+*Because the MR perfusion scan suggested deep watershed zone ischemia, a DSA was performed. While there are some minor irregularities of both proximal internal carotid arteries
, no flow-limiting stenoses were identified. The most severe disease affects the left cavernous ICA
.*
+
+
+*Anterior oblique view of the left internal carotid DSA in the same patient shows atherosclerotic irregularities in the distal ICA
with high-grade stenoses in the M2/M3
and A2 segments
. This is intracranial ASVD with acute multiple lacunar infarcts in the deep watershed zone.*
+
+
+*Oblique view of the right intracranial ICA DSA injection shows a high-grade stenosis of the cavernous segment
as well as multifocal areas of irregularity and narrowing of more distal vessels
. The findings in this case are those of severe intracranial ASVD.*
+
+
+*AP vertebral artery injection DSA shows multifocal stenoses in the vertebral artery
and basilar artery
in a patient with intracranial atherosclerosis.*
+
diff --git a/docs_md/articles/intracranial-herniation-syndromes_e85ea6f9-0ff8-43ee-a79f-8b2698f5c7fd.md b/docs_md/articles/intracranial-herniation-syndromes_e85ea6f9-0ff8-43ee-a79f-8b2698f5c7fd.md
new file mode 100644
index 0000000..66dc1f9
--- /dev/null
+++ b/docs_md/articles/intracranial-herniation-syndromes_e85ea6f9-0ff8-43ee-a79f-8b2698f5c7fd.md
@@ -0,0 +1,463 @@
+---
+title: "Intracranial Herniation Syndromes"
+docid: "e85ea6f9-0ff8-43ee-a79f-8b2698f5c7fd"
+authors:
+ - key: "b2e6dabb-ee1c-42a4-a332-9f0814c1c607"
+ value: "Surjith Vattoth, MD"
+ - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
+ value: "Anne G. Osborn, MD, FACR"
+breadcrumbs:
+ -
+ name: "Brain"
+ slug: "brain"
+ treeNodeId: "0361042c-9c90-48e4-864e-a2e6428ee9c9"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "948c6f1e-8e18-45f3-bd9d-7a04ab0b8708"
+ -
+ name: "Pathology-Based Diagnoses"
+ slug: "pathology-based-diagnoses"
+ treeNodeId: "d60fe914-7897-46a0-9cd2-f88ffb322f16"
+ -
+ name: "Trauma"
+ slug: "trauma"
+ treeNodeId: "5ba86a0e-d2d2-4c84-b223-f6f31a3b90e9"
+ -
+ name: "Secondary Effects of CNS Trauma"
+ slug: "secondary-effects-of-cns-trauma"
+ treeNodeId: "a8eb9c3c-8751-4d76-bb9a-702ca66f895d"
+ -
+ name: "Intracranial Herniation Syndromes"
+ slug: "intracranial-herniation-syndromes"
+ treeNodeId: null
+category: "Brain"
+documentVersionId: "310a771a-d562-4d5f-8df3-d1a7b6496ec6"
+imageCount: 28
+lastUpdated: "09/04/25"
+pageDescription: "Intracranial Herniation Syndromes"
+pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Trauma, Secondary Effects of CNS Trauma, Intracranial Herniation Syndromes"
+pageTitle: "Intracranial Herniation Syndromes | STATdx"
+enhancedTitle: "Intracranial Herniation Syndromes"
+type: "DX"
+references: true
+breadcrumbs:
+ - "Brain"
+ - "Diagnosis"
+ - "Pathology-Based Diagnoses"
+ - "Trauma"
+ - "Secondary Effects of CNS Trauma"
+ - "Intracranial Herniation Syndromes"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Brain displaced from one compartment into another
+- ### Imaging
+
+
+ - **Subfalcine herniation (SFH)**
+ - **Cingulate** **gyrus** displaced under falx
+ - Ipsilateral ventricle compressed, contralateral dilated
+ - **Unilateral descending transtentorial herniation (DTH)**
+ - **Temporal lobe** displaced medially into incisura
+ - Effaced suprasellar, perimesencephalic cisterns
+ - **Bilateral DTH ("central herniation")**
+ - Both temporal lobes herniated into tentorial hiatus
+ - Diencephalon crushed against skull base
+ - CSF spaces effaced, midbrain/pons displaced inferiorly
+ - **Ascending transtentorial herniation**
+ - **Cerebellum** displaced up through incisura
+ - Quadrigeminal cistern, tectum flattened
+ - **Tonsillar herniation**
+ - Tonsils impacted into foramen magnum
+ - Cisterna magna obliterated
+ - **Transalar (transsphenoidal)** **herniation**
+ - **Ascending transalar** (middle fossa mass)
+ - **Descending transalar**(frontal mass)
+ - Brain, MCA herniated across sphenoid wing
+ - **Transdural/transcranial herniation**
+ - Brain extruded through dural/skull defect
+ - **Paradoxical herniation after decompressive craniectomy**
+- ### Top Differential Diagnoses
+
+
+ - Intracranial hypotension
+- ### Pathology
+
+
+ - Herniations, ↑ ICP, vascular compression, infarction
+- ### Clinical Issues
+
+
+ - **Infarcts**: SFH: **Anterior cerebral artery (****ACA)**; DTH: **PCA**, **basilar perforators**; tonsillar herniation: **PICA**
+ - Transalar herniation: Ascending: **ICA**; descending: **MCA**
+ - Features of ↑ ICP & hydrocephalus
+ - **Hemiparesis**: Contralateral; ipsilateral in **Kernohan notch**
+- ### Diagnostic Checklist
+
+
+ - DWI: Ischemia; GRE, SWI: Hemorrhage
+
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - Subfalcine herniation (SFH)
+ - Descending transtentorial herniation (DTH)
+- ### Definitions
+
+
+ - Herniation of brain from one compartment (normally separated by calvarial &/or dural boundaries) to another
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Several herniation types (findings vary)
+ - SFH: Cingulate gyrus displaced across midline
+ - DTH: Temporal lobe displaced over & into tentorial incisura
+ - **SFH**
+ - Most common herniation
+ - **Cingulate gyrus** displaced under falx
+ - Ipsilateral cingulate gyrus pushed down & under falx with compression of contralateral cingulate gyrus
+ - Along with depression of ipsilateral corpus callosum & elevation/compression of contralateral corpus callosum
+ - Ipsilateral lateral ventricle compressed & anterior falx deviated in mild SFH
+ - Ipsilateral lateral & 3rd ventricles pushed to contralateral side in severe SFH
+ - Complications
+ - Early: Contralateral ventricle enlarged secondary to obstruction at foramen of Monro
+ - Late: Anterior cerebral arteries (ACAs) displaced → compressed against free edge of falx → infarct
+ - **Unilateral DTH**
+ - 2nd most common herniation
+ - **Earliest** sign: **Widening**of ipsilateral with compression of contralateral perimesencephalic cistern
+ - Due to brainstem rotation & contralateral/caudal shift
+ - Later both perimesencephalic cisterns compressed
+ - Contralateral temporal horn dilation due to compression of its neck
+ - **Medial temporal lobe** displaced medially into incisura
+ - Early/mild DTH: **Uncus** effaces ipsilateral suprasellar cistern **anteriorly**
+ - Moderate DTH: **Hippocampus** effaces ipsilateral suprasellar/perimesencephalic cistern **posteriorly**
+ - Displaces, mildly compresses midbrain
+ - Severe DTH: Medial temporal lobe, temporal horn displaced inferiorly into upper cerebellopontine angle cistern
+ - Suprasellar cistern obliterated
+ - Lateral ventricle trigone calcification may be rarely pushed below tentorial incisura, if posterior temporoparietooccipital mass
+ - Complications
+ - **Ipsilateral 3rd nerve palsy** due to nerve compression against tentorial edge by uncus
+ - Contralateral midbrain compressed against tentorium; may cause **Kernohan notch**
+ - Ipsilateral hemiplegia (false localizing sign)
+ - Supratentorial mass compress**contralateral**cerebral peduncle against tentorial edge
+ - Midbrain **Duret hemorrhages (DHs)**
+ - May also involve pons & cerebellar peduncles
+ - DH due to stretching/tearing of basilar artery perforators or draining veins, grave prognosis
+ - Posterior cerebral artery (PCA) displaced inferiorly over free edge of tentorium
+ - PCA kinking/occlusion leads to secondary occipital infarct; usually calcarine branch compression
+ - Aqueduct of Sylvius compression with hydrocephalus
+ - **Bilateral DTH ("central herniation")**
+ - Less common with severe supratentorial mass effects
+ - Both temporal lobes herniated into tentorial hiatus
+ - Optic chiasm/diencephalon crushed against skull base
+ - Midbrain/pons displaced inferiorly
+ - Anterior inferior 3rd ventricle displaced posteriorly behind dorsum sella
+ - Angle between midbrain & pons more acute
+ - Complications
+ - Penetrating basal arteries occlusion → basal infarcts
+ - **Ascending transtentorial herniation**
+ - Less common than descending herniation
+ - Cerebellum displaced up through incisura
+ - Quadrigeminal cistern compressed, tectum flattened
+ - Complications: Aqueduct obstruction → hydrocephalus
+ - **Tonsillar herniation**
+ - Most common herniation seen with posterior fossa mass
+ - Sometimes due to severe supratentorial mass effect
+ - Tonsils pushed inferiorly, impacted below foramen magnum > 5 mm
+ - Tonsil folia become vertically oriented
+ - Cisterna magna obliterated
+ - Complications
+ - 4th ventricle obstruction → hydrocephalus
+ - Posterior inferior cerebellar artery (PICA) compression → infarcts
+ - **Transalar (transsphenoidal) herniation**
+ - Rare; ascending or descending
+ - Brain, middle cranial artery (MCA) herniated across sphenoid wing
+ - **Ascending**: Middle cranial fossa/temporal lobe mass displaces sylvian fissure, MCA, up/over sphenoid wing
+ - **Descending**: Anterior fossa/frontal lobe mass displaces orbital gyri (smaller herniation) & gyrus rectus (larger herniations) posteroinferiorly over sphenoid wing, displaces sylvian fissure/MCA backward
+ - Complication
+ - Ascending: Supraclinoid internal cerebral artery (ICA) compressed against anterior clinoid process → ACA & MCA infarct
+ - Descending: MCA compressed against sphenoid → infarct
+ - **Transdural/transcranial herniation**
+ - Rare; sometimes called brain fungus
+ - Brain, vessels herniated through dural &/or skull defect
+ - Trauma (skull fracture lacerates dura), craniotomy
+ - ↑ intracranial pressure (ICP) forces brain through dura ± subgaleal extension; can be life threatening
+ - **Growing skull fracture** a.k.a. **leptomeningeal cyst** (misnomer) or **craniocerebral erosion**
+ - Rare complication of head trauma in children < 3 years old; extremely rare in adults
+ - Piaarachnoid membranes herniate out through dural/bony defects with gradual fracture gap enlargement due to CSF pulsation
+ - 3 categories based on etiology, CT imaging, & surgical management required
+ - Type I: Leptomeningeal cyst in skull defect
+ - Type II: Underlying gliotic damaged brain in defect
+ - Type III: Extension of porencephalic cyst through skull defect into subgaleal location
+ - **Paradoxical herniation (PH) after unilateral decompressive craniectomy (DC)**
+ - Brain midline shift in direction opposite to DC skull defect & brainstem compression
+ - DC disrupts Monro-Kellie doctrine & introduces new variable, atmospheric pressure
+ - 14.7 lbs/in² or 1033 cm H₂O
+ - Atmospheric pressure, altered cerebral blood flow & CSF circulation may cause intracranial hypotension (IH)
+ - **Syndrome of trephined** (**sinking skin flap syndrome**)
+ - Sinking skin flap on CT/MR, usually between 2 weeks to 2 months after DC
+ - Rarely, tense skin flap without sinking at site of DC during first 2 weeks; MR features of IH
+- ### CT Findings
+
+
+ - #### NECT
+
+
+ - Ventricles displaced; sulci/cisterns obliterated
+- ### MR Findings
+
+
+ - #### T1WI
+
+
+ - Best anatomic definition
+ - #### T2WI
+
+
+ - Best for complications (e.g., edema, infarcts, hydrocephalus)
+ - #### T2* GRE
+
+
+ - Hemorrhagic foci (e.g., DH), SWI best
+ - #### DWI
+
+
+ - Secondary ischemia/infarcts
+ - DTI
+ - ± corticospinal tract disruption
+ - Kernohan notch → loss of fractional anisotropy
+- ### Imaging Recommendations
+
+
+ - #### Best imaging tool
+
+
+ - NECT best rapid screen; multiplanar MR for complications
+ - #### Protocol advice
+
+
+ - DWI: Ischemia; GRE, SWI: Hemorrhage
+
+## DIFFERENTIAL DIAGNOSIS
+
+- [Intracranial Hypotension](/document/intracranial-hypotension/6765f123-04be-4634-beb5-216838f5ca6b)
+ - Brain "pulled," not "pushed" down
+ - Pituitary gland often engorged, subdural collections/hematomas
+ - Dural thickening, enhancement often present
+- [Chiari 1](/document/chiari-1/835a2ce4-4ac1-4ba6-a2b9-72427f112a3a)
+ - Congenital anomaly with low-lying tonsils
+ - Pointed, peg-shaped cerebellar tonsils with effaced CSF spaces around foramen magnum
+ - Look for spinal cord syringohydromyelia
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Etiology
+
+
+ - Trauma most common clinical setting
+ - Mass lesions, large infarcts, & inflammatory lesions
+ - Hemorrhage, extracellular fluid, or added tissue accumulate within closed space
+ - CSF spaces (cisterns, ventricles) initially compressed
+ - Intracranial volume cannot be accommodated
+ - Displacement of brain, vessels → herniation
+ - Secondary effects exacerbate severity of primary injuries
+ - Herniations, ↑ ICP, altered cerebral hemodynamics → ischemia & infarction
+ - PCA occlusion → occipital infarct most common
+ - ACA occlusion → distal (cingulate gyrus) infarcts
+ - Perforating vessels → basal ganglia, capsule infarcts
+ - Stretching/tearing of basilar artery perforators or draining veins → brainstem DH
+ - #### Associated abnormalities
+
+
+ - Secondary obstructive hydrocephalus
+ - Ischemia, hemorrhage, necrosis
+- ### Gross Pathologic & Surgical Features
+
+
+ - Grossly swollen edematous brain; effaced sulci
+ - Gyri compressed & flattened against calvarium
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - Contralateral hemiparesis, ipsilateral pupil-involving CNIII palsy
+ - Ipsilateral hemiparesis: False localizing signs
+ - Kernohan notch → compression of opposite cerebral peduncle against tentorium
+ - ↓ mental status or obtundation; DH usually seen with severe herniation, 12-24 hours before death
+ - Syndrome of trephined (sinking skin flap syndrome)
+ - Persistent/orthostatic headaches, dizziness, memory & mood disturbances weeks to months after DC
+- ### Natural History & Prognosis
+
+
+ - Brain death if ICP & mass effect ↑ unabated
+ - Traumatic brain herniation with lesser midbrain compression preoperatively → improved clinical outcomes postsurgery
+- ### Treatment
+
+
+ - Mitigate secondary effects; remove mass or DC
+ - Prolonged posttraumatic brain hypersensitivity
+ - May offer potential "therapeutic window"
+ - Possible use of neuroprotective agents
+ - PH after DC: Eliminate pressure gradient across DC
+ - Place patient in Trendelenburg position, IV hydration
+ - Stop all ICP reduction, such as ventriculostomy drainage
+ - Seal CSF leaks; early cranioplasty
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - IH syndrome
+ - Can mimic some features of herniations caused by supratentorial mass
+ - Common features
+ - "Slumping" midbrain
+ - "Closed" midbrain-pontine angle
+ - Tonsillar herniation
+ - ± subdural hematomas
+ - Distinguishing features in IH
+ - Brain appears "pulled" down, not "pushed" down
+ - Dural thickening, enhancement
+ - Pituitary engorgement
+- ### Image Interpretation Pearls
+
+
+ - Use DWI, GRE/SWI in brain trauma & suspected herniation
+
+ e81e7aff-b627-48a9-9744-769c503c2145
+
+## References
+
+## Selected References
+
+1. [Lara-Reyna J et al: Midbrain volume in brain herniation: a volumetric analysis in operative traumatic brain injury. Surg Neurol Int. 15:437, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=39640336%5Bpmid%5D)
+1. [Park HY et al: Sinking skin flap syndrome or syndrome of the trephined: a report of two cases. Ann Rehabil Med. 43(1):111-4, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30852878%5Bpmid%5D)
+1. [Saran S et al: Neglected type III growing skull fracture in a 65-year-old female. Ann Afr Med. 17(1):46-7, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29363637%5Bpmid%5D)
+1. [Ji H et al: Paradoxical herniation after unilateral decompressive craniectomy: a retrospective analysis of clinical characteristics and effectiveness of therapeutic measures. Turk Neurosurg. 27(2):192-200, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27593769%5Bpmid%5D)
+1. [Wu H et al: The diagnosis and surgical treatment of central brain herniations caused by traumatic bifrontal contusions. J Craniofac Surg. 25(6):2105-8, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25304144%5Bpmid%5D)
+1. [Arbour RB: Early metabolic/cellular-level resuscitation following terminal brain stem herniation: implications for organ transplantation. AACN Adv Crit Care. 24(1):59-78, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23343814%5Bpmid%5D)
+1. [Bor-Seng-Shu E et al: Posttraumatic refractory intracranial hypertension and brain herniation syndrome: cerebral hemodynamic assessment before decompressive craniectomy. Biomed Res Int. 2013:750809, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24377095%5Bpmid%5D)
+1. [Young GB: Impaired consciousness and herniation syndromes. Neurol Clin. 29(4):765-72, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=22032659%5Bpmid%5D)
+1. [Kalita J et al: Brain herniations in patients with intracerebral hemorrhage. Acta Neurol Scand. 119(4):254-60, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19053952%5Bpmid%5D)
+1. [Hussain SI et al: Brainstem ischemia in acute herniation syndrome. J Neurol Sci. 268(1-2):190-2, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18164729%5Bpmid%5D)
+1. [Marupaka SK et al: Atypical Duret haemorrhages seen on computed tomography. Emerg Med Australas. 20(2):180-2, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18377408%5Bpmid%5D)
+1. [Timms C et al: Brainstem distortion from postoperative cerebellar herniation through a dural and bony defect. J Clin Neurosci. 15(9):1050-1, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18501610%5Bpmid%5D)
+1. [Yoo WK et al: Kernohan's notch phenomenon demonstrated by diffusion tensor imaging and transcranial magnetic stimulation. J Neurol Neurosurg Psychiatry. 79(11):1295-7, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18940992%5Bpmid%5D)
+1. [Laine FJ et al: Acquired intracranial herniations: MR imaging findings. AJR Am J Roentgenol. 165:967-73, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7677003%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+*Submentovertex gross pathology shows findings of unilateral descending transtentorial herniation (DTH). Undersurface of herniated temporal lobe shows "grooving"
from impaction of uncus/amygdala anteriorly and hippocampus posteriorly against the tentorium. Note the 3rd nerve compression
and midbrain displacement
. (Courtesy R. Hewlett, MD.)*
+
+
+*Submentovertex gross pathology shows findings of unilateral descending transtentorial herniation (DTH). Undersurface of herniated temporal lobe shows "grooving"
from impaction of uncus/amygdala anteriorly and hippocampus posteriorly against the tentorium. Note the 3rd nerve compression
and midbrain displacement
. (Courtesy R. Hewlett, MD.)*
+
+
+*Axial MP RAGE T1 C+ MR 5 days after hematoma evacuation
shows DTH
with multiple contrast-enhancing subacute perforator infarcts
.*
+
+
+*Axial NECT in a patient with left posterior cerebral hemorrhage
shows severe brain edema, subfalcine herniation (SFH)
, rightward midline shift, and DTH with obliteration of suprasellar and perimesencephalic cisterns
. Note that the left lateral ventricle trigone with its calcified choroid plexus is displaced inferiorly into tentorial incisura
.*
+
+
+*Axial NECT in the same patient shows Duret hemorrhages (DH) in midbrain
. DH is due to stretching/tearing of basilar artery perforators or draining veins (carries grave prognosis).*
+
+
+*Sagittal reformatted NECT in bilateral descending transtentorial (central) herniation shows effaced basal cisterns with brainstem descent/compression
, pineal calcification descent
, & tonsillar descent
.*
+
+
+*Sagittal MP RAGE T1 C+ MR in a patient with posterior fossa tumor
shows ascending transtentorial herniation with compression of quadrigeminal cistern
by the cerebellum
displaced up through incisura & tectal flattening
. Also note tonsillar herniation below foramen magnum
.*
+
+
+*Right parasagittal CTA (top) shows ascending transalar herniation due to large right temporal lobe hematoma
. Right sylvian fissure and MCA branches
are displaced anterosuperomedially over the sphenoid wing
. Note normal counterparts on left parasagittal image (bottom).*
+
+
+*Axial T2 MR (top) shows descending transalar herniation due to left anterior cranial fossa tumor
. Displaced orbital gyri
and gyrus rectus
compress left MCA cistern. Note resolution after surgery (bottom).*
+
+
+*Axial T2 MR of an infant with a left parietal skull fracture shows transcalvarial herniation of the brain and accompanying vessels
through torn dura
. Note bilateral chronic SDHs
.*
+
+
+*Axial NECT 1 month after right-sided decompressive craniectomy (DC) shows sinking skin flap
with paradoxical brain herniation. Note midline shift toward the opposite side
. Brainstem compression and MR findings of intracranial hypotension may be seen in severe cases with "syndrome of the trephined."*
+
+
+### Additional Images
+
+
+*Axial T2 FS MR shows SFH with displacement across the midline
and compression of the left lateral ventricle
caused by an SDH
.*
+
+
+*Axial T1 MR shows a unilateral DTH. The uncus is medially displaced
, narrowing the suprasellar cistern
, compressing the brainstem
, and widening the ambient cistern
.*
+
+
+*Axial T2 MR in the same patient shows the temporal lobe herniated into the posterior fossa
, where it compresses the cerebral peduncle
.*
+
+
+*Axial NECT shows central herniation and obliteration of the suprasellar cistern
. The inferior 3rd ventricle is displaced posteriorly and inferiorly
behind the dorsum sellae
. No other cisterns are visible.*
+
+
+*Sagittal T1 MR shows central transtentorial and tonsillar herniation
below the foramen magnum. The chiasm
and 3rd ventricle
are inferiorly displaced, and the CSF spaces are narrowed.*
+
+
+*Axial T2 MR shows an upward herniation. The mass obliterates the quadrigeminal plate cistern
, the brainstem is compressed
, and the prepontine cistern is narrowed. Hydrocephalus is secondary to obstructed aqueduct
.*
+
+
+*Sagittal T1 MR shows a superior transtentorial herniation
and inferior tonsillar herniation
due to a large mass in the cerebellum (Lhermitte-Duclos). Note the hydrocephalus.*
+
+
+*Axial T2 MR shows ascending transtentorial herniation due to a cerebellar mass
. Note the anterior displacement of the brainstem
and obstructive hydrocephalus.*
+
+
+*Axial T2 MR shows unilateral DTH secondary to a left temporal lobe mass with effacement of the left suprasellar cistern
and midbrain compression
evident.*
+
+
+*Axial NECT shows bilateral DTH through the tentorial incisura
. The midbrain is compressed and deformed.*
+
+
+*Axial NECT shows a parenchymal hematoma in the right temporal lobe with resultant transalar herniation of the anterior temporal lobe across the greater sphenoid wing
.*
+
+
+*Axial CECT shows multiple enhancing metastases. A right frontal mass effect results in an SFH with left displacement of the cingulate gyrus and ACAs under the falx
.*
+
+
+*Axial gross pathology section through the ventricles in a patient who died of multiple traumatic injuries shows findings of severe SFH. Ventricles are displaced across the midline, and the cingulate gyrus
is herniated under the falx. Left posterior cerebral artery (PCA) infarct occurred secondary to DTH. (Courtesy R. Hewlett, MD.)*
+
+
+*Axial NECT in severe trauma shows SFH
with severely compressed left
and slightly dilated right lateral ventricle
.*
+
+
+*Sagittal autopsy demonstrates findings of complete bilateral DTH with the optic chiasm and the 3rd ventricle
draped over the sella. The midbrain is displaced inferiorly, and the angle with pons is decreased
. (Courtesy R. Hewlett, MD.)*
+
+
+*Axial NECT shows findings of ascending transtentorial herniation. The vermis is displaced superiorly through the tentorial incisura
, flattening and compressing the tectal plate
. The aqueduct is obstructed, causing acute hydrocephalus with transependymal CSF flow
.*
+
+
+*Gross pathology of an autopsied brain seen posteriorly shows bilateral tonsillar herniation. Note the grooving of tonsils
from impaction against the foramen magnum as the tonsils
are forced inferiorly into the upper cervical spine. (Courtesy R. Hewlett, MD.)*
+
+
+*Axial T2 MR shows severe tonsillar herniation with both tonsils
displaced inferiorly, filling the cisterna magna, and displacing the upper cervical spinal cord anteriorly.*
+
diff --git a/docs_md/articles/intrinsic-skull-base-lesion_563bc74a-c052-4fdb-a5f2-5a318578bb62.md b/docs_md/articles/intrinsic-skull-base-lesion_563bc74a-c052-4fdb-a5f2-5a318578bb62.md
new file mode 100644
index 0000000..d93ed6f
--- /dev/null
+++ b/docs_md/articles/intrinsic-skull-base-lesion_563bc74a-c052-4fdb-a5f2-5a318578bb62.md
@@ -0,0 +1,351 @@
+---
+title: "Intrinsic Skull Base Lesion"
+docid: "563bc74a-c052-4fdb-a5f2-5a318578bb62"
+authors:
+ - key: "99f10fe6-7f91-4026-bc60-d769c5d7c7c4"
+ value: "C. Douglas Phillips, MD, FACR"
+breadcrumbs:
+ -
+ name: "Head and Neck"
+ slug: "head-and-neck"
+ treeNodeId: "5c1f8e17-7acd-48d8-9d55-f9f8c2cad850"
+ -
+ name: "Differential Diagnosis"
+ slug: "differential-diagnosis"
+ treeNodeId: "deb55065-e1d6-4b6f-b3e3-181fafb4e218"
+ -
+ name: "Skull Base"
+ slug: "skull-base"
+ treeNodeId: "c64c7693-b8db-4b9f-aab5-d26fb08455f5"
+ -
+ name: "Anatomically Based Differentials"
+ slug: "anatomically-based-differentials"
+ treeNodeId: "1646983c-a8e8-4a00-8155-b1de0fc2ca45"
+ -
+ name: "Intrinsic Skull Base Lesion"
+ slug: "intrinsic-skull-base-lesion"
+ treeNodeId: null
+category: "Head and Neck"
+documentVersionId: "586480e5-e067-4991-8c4d-19a82e0c60aa"
+imageCount: 32
+lastUpdated: "07/10/18"
+pageDescription: "Intrinsic Skull Base Lesion"
+pageKeywords: "Head and Neck, Differential Diagnosis, Skull Base, Anatomically Based Differentials, Intrinsic Skull Base Lesion"
+pageTitle: "Intrinsic Skull Base Lesion | STATdx"
+enhancedTitle: "Intrinsic Skull Base Lesion"
+type: "DDX"
+references: true
+breadcrumbs:
+ - "Head and Neck"
+ - "Differential Diagnosis"
+ - "Skull Base"
+ - "Anatomically Based Differentials"
+ - "Intrinsic Skull Base Lesion"
+---
+## ESSENTIAL INFORMATION
+
+- ### Key Differential Diagnosis Issues
+
+
+ - Lesion may be focal, diffuse, localized, or part of systemic disease
+ - Variable presentation of skull base lesion
+ - Headache, cranial neuropathy
+ - On occasion, may be incidental imaging finding
+ - Imaging strategy
+ - CT & MR often complementary
+ - CT best shows aggressive or benign bone features
+ - MR may show characteristic signal or enhancement
+- ### Helpful Clues for Common Diagnoses
+
+
+ - **Metastasis, Skull Base**
+ - Key facts
+ - Central skull base most frequent site
+ - Most often prostate, breast, & lung carcinoma
+ - Imaging
+ - CT: Lytic, destructive, or sclerotic
+ - MR: Variable signal, usually enhance
+ - **Fibrous Dysplasia, Skull Base**
+ - Key facts
+ - Benign expansile bone anomaly
+ - Prone to enlarge during childhood
+ - Imaging
+ - CT: Characteristic **ground-glass** density
+ - May narrow foramina & fissures
+ - MR: Heterogeneous, mass-like lesion
+ - T2: Ground-glass hypointense, lucent hyperintense, variable enhancement
+ - Monostotic 6x more common than polyostotic fibrous dyplasia (FD)
+ - Unusual or odd skull base lesion in asymptomatic or minimally symptomatic patient consider FD
+ - Cystic variants can present confusing imaging appearance on MR; CT can confirm diagnosis
+ - **Paget Disease, Skull Base**
+ - Key facts
+ - Chronic bone disorder with abnormal bone breakdown & formation
+ - Polyostotic form is more common
+ - Skull involved in 25-65% of patients
+ - Imaging
+ - CT: Expansion of bone with mixed "**cotton wool**" sclerotic ± lytic areas
+ - Widening of diploic space
+ - Inner & outer tables of involved
+ - MR: Mainly T2 hypointense, cystic areas hyperintense
+ - **Chordoma, Clivus**
+ - Key facts
+ - Benign but locally aggressive primary tumor of notochord remnants
+ - Arise near sphenooccipital synchondrosis
+ - Midline, expansile, multilobulated, well-defined mass
+ - Imaging
+ - CT: Lytic destructive midline sphenoid mass ± irregular bone spicules
+ - MR: Characteristic high T2 signal, heterogeneous enhancement
+ - Tumor "thumb" indents anterior pons
+ - **Multiple Myeloma, Skull Base**
+ - Key facts
+ - Focal mass of malignant plasma cells
+ - More frequently seen in calvarium
+ - Imaging
+ - CT: Multiple well-defined lytic lesions
+ - MR: Focal lesions replace normal high signal of fat on T1WI in marrow containing spaces of skull base
+ - DWI also sensitive to marrow malignancy (low ADC)
+ - Often little or no uptake of Tc-99m pertechnetate (cold lesion)
+ - **Plasmacytoma, Skull Base**
+ - Key facts
+ - Isolated tumor of malignant plasma cells
+ - Imaging
+ - CT: Solitary lesion, bony lysis
+ - MR: T2 intermediate signal; moderate enhancement
+ - **Chondrosarcoma, Skull Base**
+ - Key facts
+ - Malignant cartilaginous neoplasm
+ - Arises from petroclival synchondrosis
+ - Imaging
+ - CT: Expansile, destructive mass at petrooccipital fissure producing erosive or destructive bone changes in clivus & petrous apex
+ - 50% have chondroid matrix with "rings & arcs" calcification
+ - MR: Variable, usually high signal on T2
+ - Degree of T2 hyperintensity is inversely related to degree of calcified matrix
+ - Usually has nodular or focal enhancement within tumor on T1 C+
+ - **Pneumatization Arrest, Sphenoid**
+ - Key facts
+ - Incidental lesion of basisphenoid
+ - Often accompanied by hypodevelopment of ipsilateral sphenoid sinus
+ - Imaging
+ - CT: Nonexpansile with sclerotic margin
+ - Contains fat & curvilinear calcification
+ - MR: Heterogeneous, often focal T1 fat
+- ### Helpful Clues for Less Common Diagnoses
+
+
+ - **Langerhans Histiocytosis, Skull Base**
+ - Key facts
+ - Proliferation of bone marrow-derived Langerhans cells & eosinophils
+ - Skull base involvement more often with multifocal or acute disseminated forms
+ - Imaging
+ - Nonspecific destructive soft tissue mass
+ - **Arachnoid Granulations, Dural Sinuses**
+ - Key facts
+ - Usually incidental imaging finding
+ - Imaging
+ - More numerous around dural sinuses
+ - CT: Small, well-defined "pits" in skull base
+ - MR: Often subtle, focal T2 hyperintensity
+ - **Osteomyelitis, Skull Base**
+ - Key facts
+ - Primary bone infection, acute or chronic
+ - Imaging
+ - CT: Permeative lytic when acute
+ - Chronic may be lytic or lytic-sclerotic
+ - MR: Marrow replacement, enhancement
+ - Often extensive involvement of dura
+ - **Meningioma, Skull Base**
+ - Key facts
+ - Dural-based, benign extraaxial tumor
+ - May occur as intraosseous lesion
+ - Imaging
+ - CT: Bony changes may be hyperostosis, erosion, or permeative destruction
+ - MR: Extension along dural surfaces with low T2 signal
+- ### Helpful Clues for Rare Diagnoses
+
+
+ - **Giant Cell Tumor, Skull Base**
+ - Key facts
+ - Benign long bone tumor
+ - Skull base: Sphenoid & temporal bones
+ - Can be locally aggressive &/or recur
+ - Imaging
+ - CT: Destructive mass with focally interrupted, thinned cortical shell
+ - MR: Scant matrix, larger lesions more heterogeneous, marked enhancement
+ - **Cephalocele, Skull Base**
+ - Key facts
+ - Skull base defect with variable protrusion of dura, CSF, &/or neural tissue
+ - Imaging
+ - CT: Focal bone defect
+ - MR: Tissue components within cephalocele best depicted on MR
+ - **Ecchordosis Physaliphora**
+ - Key facts
+ - Considered to be notochordal remnant
+ - Well-defined lesion of clivus with prepontine intradural cystic mass connected by stalk or pedicle to clival lesion
+ - Imaging
+ - CT: Soft tissue density lesion
+ - MR: T1 low, T2 high, no enhancement
+ - **Pseudotumor, Skull Base**
+ - Key facts
+ - Idiopathic inflammatory lesion
+ - This disease is likely IgG4-related disease
+ - Imaging
+ - CT: Soft tissue mass, permeative bone change
+ - MR: Enhancing infiltrative process, T2 hypointense, T1 iso- to hypointense
+ - Preservation of normal architecture despite infiltrative mass can be important hint
+
+## References
+
+## Selected References
+
+1. [Conley LM et al: Imaging of the central skull base. Radiol Clin North Am. 55(1):53-67, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27890188%5Bpmid%5D)
+1. [Folbe AJ et al: Endoscopic resection of clival malignancies. Otolaryngol Clin North Am. 50(2):315-329, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28314400%5Bpmid%5D)
+1. [Go JL et al: Imaging of the sella and parasellar region. Radiol Clin North Am. 55(1):83-101, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27890190%5Bpmid%5D)
+1. [Iida E et al: Imaging of paranasal sinuses and anterior skull base and relevant anatomic variations. Radiol Clin North Am. 55(1):31-52, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27890187%5Bpmid%5D)
+1. [Kunimatsu A et al: Skull base tumors and tumor-like lesions: A Pictorial Review. Pol J Radiol. 82:398-409, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28811848%5Bpmid%5D)
+1. [Schmalfuss IM: Imaging of endoscopic approaches to the anterior and central skull base. Clin Radiol. 73(1):94-105, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28716213%5Bpmid%5D)
+1. [Zamora C et al: Sellar and parasellar imaging. Neurosurgery. 80(1):17-38, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28362892%5Bpmid%5D)
+1. [Chevallier KM et al: Differentiating pediatric rhabdomyosarcoma and Langerhans cell histiocytosis of the temporal bone by imaging appearance. AJNR Am J Neuroradiol. 37(6):1185-9, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26869468%5Bpmid%5D)
+1. [Chang PC et al: Central skull base osteomyelitis in patients without otitis externa: imaging findings. AJNR Am J Neuroradiol. 24(7):1310-6, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12917118%5Bpmid%5D)
+1. [Tehranzadeh J et al: Computed tomography of Paget disease of the skull versus fibrous dysplasia. Skeletal Radiol. 27:664-72, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9921927%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+**Metastasis, Skull Base**
+*Axial T1 MR obtained in a patient with a history of lung cancer and new right CNXII palsy shows focal loss of bright marrow signal at the right skull base
with abnormal tissue around the hypoglossal canal
.*
+
+
+**Metastasis, Skull Base**
+*Axial T1 MR obtained in a patient with a history of lung cancer and new right CNXII palsy shows focal loss of bright marrow signal at the right skull base
with abnormal tissue around the hypoglossal canal
.*
+
+
+**Metastasis, Skull Base**
+*Coronal fused PET/CT of metastasis to skull base shows markedly intense FDG uptake in the right medial skull base
, corresponding to the lesion depicted on MR study. Multiple other bone and visceral metastases were also present.*
+
+
+**Fibrous Dysplasia, Skull Base**
+*Axial T1 C+ FS MR reveals expansion of left greater sphenoid wing
, which is also heterogeneous but predominantly low in signal intensity. Note the focal, ill-defined pools of enhancement demonstrated within substance of lesion
.*
+
+
+**Fibrous Dysplasia, Skull Base**
+*Axial bone CT of fibrous dysplasia can clarify the etiology. Most of the greater wing
has characteristic ground-glass with areas of MR enhancement corresponding to regions of greater lucency
. Note the narrowed vidian canal
.*
+
+
+**Paget Disease, Skull Base**
+*Axial T1 C+ FS MR at the level of the skull base reveals intense heterogeneous enhancement of the skull base
and "fuzzy" heterogeneous expanded bones of the skull base and calvarium
.*
+
+
+**Paget Disease, Skull Base**
+*Axial bone CT shows a diffuse cotton wool appearance of almost the entire skull base with expansion of the squamous temporal bone
, petrous apex
, and occipital bone
. Also note stapes prosthesis on the right
, placed for prior diagnosis of conductive hearing loss. This CT is characteristic of Paget disease.*
+
+
+**Chordoma, Clivus**
+*Sagittal T2 MR shows a markedly hyperintense mass arising in the lower clivus
, extending into the prepontine cistern and indenting the pontomedullary junction
. Tumor extends into the prevertebral soft tissues
and upper spinal canal
. The mass is similar in signal to CSF, which can be a characteristic finding of chordoma.*
+
+
+**Chordoma, Clivus**
+*Axial bone CT of chordoma reveals the lytic destructive nature of the mass centered in the clivus
. Only tiny spicules of residual bone
are identified within the soft tissue component.*
+
+
+**Multiple Myeloma, Skull Base**
+*Axial bone CT demonstrates a well-circumscribed, dominant lytic lesion involving the clivus
. Additional lytic foci of multiple myeloma are seen in the left sphenoid
, left mandible
, and calvarium elsewhere
.*
+
+
+**Multiple Myeloma, Skull Base**
+*Coronal bone CT demonstrates sharply contoured lytic lesions in the central skull base
with a characteristic punched-out appearance. Small right calvarial lesions
are also noted as well as multiple lesions in the facial bones, including the mandible
.*
+
+
+**Plasmacytoma, Skull Base**
+*Axial T2 FS MR demonstrates a large central skull base mass
, which expands bone and appears to push laterally to the cavernous sinuses. The mass is very homogeneous and has intermediate signal intensity.*
+
+
+**Plasmacytoma, Skull Base**
+*Axial bone CT demonstrates the sharply circumscribed nature of the lytic plasmacytoma with narrow transition zone to normal bone. Note the complete loss of clival marrow
and erosion of the left petrous apex
. The lesion has a bland matrix, without evidence of calcifications.*
+
+
+**Chondrosarcoma, Skull Base**
+*Axial T2 MR shows a heterogeneous hyperintense mass centered over the right petro-occipital fissure with extension into the petrous temporal bone
and sphenoid sinus
. The internal carotid artery is displaced laterally by the mass
.*
+
+
+**Chondrosarcoma, Skull Base**
+*Axial bone CT of chondrosarcoma shows a large paramedian lytic lesion of the right basiocciput and petrous bone centered at the petroclival fissure with characteristic "arcs and whorls"
of calcification.*
+
+
+**Pneumatization Arrest, Sphenoid**
+*Sagittal T1 MR in an asymptomatic patient demonstrates a classic, modestly expansile lesion of the sphenoid bone
with internal high signal intensity, confirming the fatty nature of the contents of the lesion.*
+
+
+**Pneumatization Arrest, Sphenoid**
+*Axial CT delineates a well-defined lesion
with sclerotic margins and predominantly low density (fat) centrally. Sphenoid sinus ipsilateral to lesion is characteristically smaller. Normal trabeculae traverse the lesion. MR can confirm fatty contents when unclear on CT.*
+
+
+**Langerhans Histiocytosis, Skull Base**
+*Axial CECT shows a nonspecific but destructive lesion
of the central and anterior skull base, filling the sella and invading the orbits
bilaterally. The tumor surrounds carotid arteries
, indicating involvement of cavernous sinuses bilaterally. A key differential point is that the lesion is found in a pediatric patient.*
+
+
+**Arachnoid Granulations, Dural Sinuses**
+*Coronal bone CT shows a well-defined lytic lesion
in the floor of the left middle fossa. Subtle lobulation of these well-defined lesions is typical, as is the location near dural sinuses.*
+
+
+**Osteomyelitis, Skull Base**
+*Axial T1 C+ FS MR shows heterogeneous left skull base enhancement
with soft tissue extension into the high left nasopharyngeal carotid space, encasing and narrowing the left internal carotid artery
. This pseudomonas osteomyelitis developed as a delayed complication of external otitis.*
+
+
+**Meningioma, Skull Base**
+*Axial T1 C+ MR demonstrates a uniformly enhancing lesion uniformly involving the retroclival dura and extending into prepontine cistern
to nearly encase the basilar artery
and extend into the left internal auditory canal
.*
+
+
+**Giant Cell Tumor, Skull Base**
+*Axial T2 MR demonstrates a heterogeneous but predominantly low signal intensity expansile mass of the central skull base
. Profound T2 hypointensity is thought to reflect hemorrhage with hemosiderin deposition or calcification within lesion.*
+
+
+**Giant Cell Tumor, Skull Base**
+*Axial bone CT reveals a thin irregularly sclerotic "eggshell" of cortex. Expansile margins suggest a benign process. Focal areas of bone dehiscence
and matrix calcifications are evident
. Heterogeneous density on soft tissue images was present.*
+
+
+**Cephalocele, Skull Base**
+*Axial CT cisternography reveals lobulated contour of large defect
in left greater sphenoid wing, which has filled with contrast media
, indicating communication with subarachnoid space. Note moderate expansion of the sphenoid wing in comparison to the right side.*
+
+
+**Ecchordosis Physaliphora**
+*Axial T2 MR demonstrates a subtle well-defined bilobed lesion
arising from dorsal clivus and extending into the prepontine cistern
, although not causing any deformity of the pons.*
+
+
+**Pseudotumor, Skull Base**
+*Axial T1 MR shows symmetric low signal intensity of the basiocciput
but also infiltrating prevertebral muscles
and hypoglossal canal
. This nonspecific process could represent lymphoma, nasopharyngeal carcinoma, or metastases; steroid response is typical of pseudotumor.*
+
+
+**Pseudotumor, Skull Base**
+*Sagittal PET in a pseudotumor reveals intense uptake at the base of the skull
in the site of soft tissue process identified on MR. This finding is again nonspecific; imaging may help to alert pathologist of potential diagnosis.*
+
+
+### Additional Images
+
+
+**Fibrous Dysplasia, Skull Base**
+*Axial T2 FS MR shows a markedly hypointense lesion
of the greater sphenoid wing with small foci of hyperintensity
. Areas of more cystic change in fibrous dysplasia are often hyperintense.*
+
+
+**Chordoma, Clivus**
+*Axial T2 MR demonstrates an expansile, predominantly hyperintense mass
arising in the clivus and eroding the posterior clival cortex
. The mass otherwise has more benign, rounded, well-defined contours as it extends anteriorly to displace the longus capitis muscles and deform the pharynx.*
+
+
+**Multiple Myeloma, Skull Base**
+*Axial bone CT shows multiple tiny lytic lesions
in the skull base with sharply demarcated borders. Note the additional lesion in the occipital bone
. Multiplicity of disease narrows the differential diagnosis to metastatic disease or multiple myeloma.*
+
+
+**Chondrosarcoma, Skull Base**
+*Axial T2 MR through the skull base demonstrates a markedly high signal intensity tumor
involving the right petrous apex and extending into the cerebellopontine angle cistern. Location suggests chondrosarcoma arising from petroclival synchondrosis. Such a tumor often has rounded contours, though aggressive malignancy.*
+
+
+**Osteomyelitis, Skull Base**
+*Axial T1 C+ FS MR in a patient with Gradenigo syndrome shows extensive enhancement of the petrous apex
but also adjacent dural surfaces. There is involvement of the middle fossa dura
, dura of the internal auditory canal
, and mild spasm of the adjacent internal carotid artery
.*
+
+
+**Cephalocele, Skull Base**
+*Coronal CT cisternography shows subarachnoid contrast
and pooling of additional contrast in an expanded greater sphenoid wing
, indicating communication with subarachnoid space.*
+
diff --git a/docs_md/articles/jugular-bulb-pseudolesion_2bf19ff5-bfee-4764-9e2d-cea04ccce4ea.md b/docs_md/articles/jugular-bulb-pseudolesion_2bf19ff5-bfee-4764-9e2d-cea04ccce4ea.md
new file mode 100644
index 0000000..d445716
--- /dev/null
+++ b/docs_md/articles/jugular-bulb-pseudolesion_2bf19ff5-bfee-4764-9e2d-cea04ccce4ea.md
@@ -0,0 +1,432 @@
+---
+title: "Jugular Bulb Pseudolesion"
+docid: "2bf19ff5-bfee-4764-9e2d-cea04ccce4ea"
+authors:
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+ - "{'authors': 'Thomas Jose Eluvathingal Muttikkal, MD; H. Ric Harnsberger, MD', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/18e60151-70bc-40a1-9b4f-4b86f8fd65c2', 'category': 'Head and Neck', 'compareUrl': '/compare/document/18e60151-70bc-40a1-9b4f-4b86f8fd65c2/related-anatomy/treeNode?subContext=CNXI (Accessory Nerve)', 'documentId': '18e60151-70bc-40a1-9b4f-4b86f8fd65c2', 'documentType': 'ANATOMY', 'documentUrl': '/document/cnxi-accessory-nerve/18e60151-70bc-40a1-9b4f-4b86f8fd65c2', 'enhancedTitle': 'CNXI (Accessory Nerve)', 'entryDate': '11/28/23', 'imageCount': 10, 'imageUrl': '/image/thumbnail/f43628f4-42a9-4331-8508-079cb8abf634?size=174&quality=85', 'inCompareCart': False, 'rank': 8, 'referenceCount': 0, 'showCompareButton': False, 'title': 'CNXI (Accessory Nerve)'}"
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+cases: 1
+breadcrumbs:
+ - "Head and Neck"
+ - "Diagnosis"
+ - "Skull Base Lesions"
+ - "Jugular Foramen"
+ - "Jugular Bulb Pseudolesion"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Asymmetric, large jugular bulb (JB) flow phenomenon simulates neoplasm or thrombosis on MR sequences
+- ### Imaging
+
+
+ - Best diagnostic clue: Complex MR signal in JB with normal jugular foramen (JF) cortex & jugular spine
+ - Complex MR signal does not persist on all MR sequences
+ - Normal bony margins of JB on temporal bone CT
+- ### Top Differential Diagnoses
+
+
+ - High JB
+ - JB diverticulum
+ - Dehiscent JB
+ - Sigmoid sinus-JB thrombosis
+ - Jugular paraganglioma
+ - JF schwannoma
+ - JF meningioma
+- ### Clinical Issues
+
+
+ - Found incidentally on brain MR during work-up for unrelated symptoms
+ - Surgical exploration must be avoided by radiologist making correct diagnosis
+ - No treatment or follow-up required
+- ### Diagnostic Checklist
+
+
+ - JB pseudolesion is most common JB "lesion"
+ - Once abnormality is seen in JF on MR, first question to ask is, "Am I looking at JB pseudolesion?"
+ - Do not mistake JB pseudolesion for schwannoma or venous sinus thrombosis
+ - If JB pseudolesion is observed while patient is in imaging center, add MRV to protocol to clarify
+ - Use bone CT or CTA/CTV to evaluate bony margins of JF if MR diagnosis uncertain
+
+## TERMINOLOGY
+
+- ### Synonyms
+
+
+ - Jugular bulb (JB) pseudomass; "leave alone" lesion of jugular foramen (JF)
+- ### Definitions
+
+
+ - Asymmetric, large JB flow phenomenon simulates neoplasm or thrombosis on MR sequences
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - **Complex MR signal** intensity in **JB**with normal JF cortex & jugular spine
+ - #### Location
+
+
+ - JF bulb
+ - Prominent JB more commonly **right-sided**
+ - #### Size
+
+
+ - Typical JB measures 1.0-1.5 cm
+ - #### Morphology
+
+
+ - Rounded area of heterogeneous signal intensity centered on JF
+- ### CT Findings
+
+
+ - #### CECT
+
+
+ - Normal enhancing sigmoid sinus (SS) & JB
+ - No filling defect to suggest thrombosis
+ - #### Bone CT
+
+
+ - Asymmetric JB with **intact cortical margins** & jugular spine
+ - CTV: Asymmetric JB shows same enhancement as internal jugular vein (IJV) & SS
+- ### MR Findings
+
+
+ - #### T1WI
+
+
+ - Variable signal; may have soft tissue intensity or heterogeneous signal
+ - #### T2WI
+
+
+ - Heterogeneous signal intensity
+ - Usually conspicuous when iso- to hyperintense
+ - #### FLAIR
+
+
+ - Heterogeneous signal intensity, often hyperintense
+ - #### T2* GRE
+
+
+ - No significant blooming or susceptibility artifact
+ - #### T1WI C+
+
+
+ - Avid enhancement of JB
+ - Identical enhancement to adjacent IJV & SS
+ - #### MRV
+
+
+ - JB shows asymmetric enlargement without evidence of thrombosis
+ - Phase-contrast MRV: Shows normal flow in JB & SS
+ - SWI
+ - No significant hypointense signal
+- ### Angiographic Findings
+
+
+ - Catheter venography: Normal, asymmetrically large SS & JB fill with contrast
+ - JB often "high-riding"
+- ### Imaging Recommendations
+
+
+ - #### Protocol advice
+
+
+ - T1 C+ MR & MRV add to certainty of diagnosis
+ - Temporal bone CT or CTA/CTV helpful to show intact surrounding JF cortical margins & spine if MR diagnosis uncertain
+
+## DIFFERENTIAL DIAGNOSIS
+
+- [High Jugular Bulb](/document/high-jugular-bulb/5f469f6f-9fa8-41cf-ac6f-7387e7950de3)
+ - Most cephalad portion of JB extends superior to floor of internal auditory canal ± basal turn of cochlea
+ - Bone CT: JB cortical margins intact; no middle ear extension
+ - Increased signal from complex flow may be present on some MR sequences
+- [Jugular Bulb Diverticulum](/document/jugular-bulb-diverticulum/0177d04f-dd01-4f39-b81e-6fcf4208c4d0)
+ - Focal polypoid mass extending from cephalad JB into middle ear
+ - Smooth bone margins, intact sigmoid plate
+- [Dehiscent Jugular Bulb](/document/dehiscent-jugular-bulb/e1822e40-ed20-4b65-be48-c82b59042b89)
+ - Usually present with vascular "mass" behind intact tympanic membrane
+ - Sigmoid plate dehiscence on CT
+ - Increased signal from complex flow may be present on some MR sequences
+- [Sigmoid Sinus-Jugular Bulb Thrombosis](/document/skull-base-dural-sinus-thrombosis/9eb7992b-1296-4a96-83b5-b6b96160ec0a)
+ - NECT: Hyperdense SS/JB, normal bony margins
+ - CECT/CTA/CTV: Look for intraluminal thrombus
+ - Vasa vasorum of vein wall may enhance as thin white rim (empty delta sign)
+ - Subacute phase: Increased signal on T1WI MR secondary to methemoglobin
+ - MR signal depends on stage of clot
+ - MRV: Filling defect or lack of flow
+- [Jugular Paraganglioma](/document/jugular-paraganglioma/ff5672b9-8cab-4a5d-9848-bb6cc0e23c21)
+ - Permeative bony changes along JF
+ - T1 MR: JF mass with high-velocity flow voids
+ - Vector of spread: Superolateral from JB to middle ear
+- [Jugular Foramen Schwannoma](/document/jugular-foramen-schwannoma/39fe8b5f-828f-4529-a1a5-2e267c0ab63c)
+ - Smoothly scalloped, enlarged JF
+ - T1 C+ MR: Dumbbell-shaped, enhancing mass in JF
+ - Vector of spread: Superomedial along CNIX-XI
+- [Jugular Foramen Meningioma](/document/jugular-foramen-meningioma/c90dd6ee-1fc2-4e93-b200-d15889bb7f10)
+ - Permeative-sclerotic or hyperostotic bony change around JF
+ - T1 C+ MR: Enhancing dural tails along margins
+ - Vector of spread: Centrifugal along dural surfaces
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Etiology
+
+
+ - Normal developmental variant
+ - Large, asymmetric JB provides setting where **MR signal** from slow, complex flow may **mimic pathology**
+ - Embryology-anatomy
+ - JB formation is postnatal event
+ - Begins when infant changes from fetal to postnatal circulatory type (from lying down to erect posture)
+ - JB 1st seen on angiograms of children ≥ 2 years
+ - JB thought to result from "pounding effects" of ascending negative pulse waves originating in right atrium
+ - Hammering effects of negative pulse waves hitting roof of jugular sinus enlarges surrounding osseus structures
+ - Direct line from right atrium to right "jugular sinus" vs. indirect course on left via left brachiocephalic vein explains why **right JB** is larger than left
+- ### Gross Pathologic & Surgical Features
+
+
+ - Normal variant, not surgical lesion
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - **Asymptomatic**
+ - Found incidentally on brain MR during work-up for unrelated symptoms
+- ### Demographics
+
+
+ - #### Age
+
+
+ - May be discovered at any age
+ - #### Epidemiology
+
+
+ - Most common "lesion" of JF found on MR imaging
+- ### Natural History & Prognosis
+
+
+ - Following sequence of events commonly associated with JB pseudolesion
+ - Patient undergoes brain MR for unrelated symptoms
+ - JB pseudolesion reported on MR interpretation as **"possible JB thrombosis or JF schwannoma"**
+ - Patient is referred to neurootologist for therapy
+ - Temporal bone CT done to clarify extent of lesion
+ - Temporal bone CT reveals **normal**, asymmetric JB
+ - Further MR work-up shows evidence of flowing blood on 1 or more sequences; normal MRV
+ - Patient returns to regular life, "cured" by follow-up imaging
+ - Normal anatomic structure
+ - Asymmetrically large JB does not enlarge with time
+ - Making correct diagnosis at imaging prevents unnecessary surgical exploration
+- ### Treatment
+
+
+ - No treatment or follow-up required
+ - Important radiologic diagnosis to prevent surgical exploration
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - JB pseudolesion is most common JB "lesion"
+ - Once abnormality is seen in JF on MR, 1st question that must be asked is, "Am I looking at JB pseudolesion?"
+ - Do not mistake JB pseudolesion for JF schwannoma or venous sinus thrombosis
+- ### Image Interpretation Pearls
+
+
+ - If JB pseudolesion is observed while patient is in imaging center, add MRV to protocol to clarify
+ - If still uncertain as to etiology of JF lesion, follow-up with temporal bone CT to evaluate bony margins of JF
+ - JB pseudolesion is diagnosed when MR & CT show following
+ - MR: Large JB mixed high signal does not hold up on all MR sequences
+ - Temporal bone CT: Shows normal bony margins to asymmetrically large ± high JB
+
+ a4d43a35-a318-43d5-ad6c-e80a589867f8
+
+## References
+
+## Selected References
+
+1. [Alves IS et al: Imaging of vertigo and dizziness: a site-based approach, part 1 (middle ear, bony labyrinth, and temporomandibular joint). Semin Ultrasound CT MR. 45(5):360-71, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=39374861%5Bpmid%5D)
+1. [Expert Panel on Neurological Imaging et al: ACR Appropriateness Criteria® tinnitus: 2023 update. J Am Coll Radiol. 20(11S):S574-91, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=38040471%5Bpmid%5D)
+1. [Salman R et al: Pediatric tinnitus: the role of neuroimaging. J Neuroimaging. 32(3):400-11, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35307901%5Bpmid%5D)
+1. [Totten DJ et al: Persistent conductive hearing loss after tympanostomy tube placement due to high-riding jugular bulb. Laryngoscope. 131(4):E1272-4, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33512006%5Bpmid%5D)
+1. [Liu GS et al: Systematic review of temporal bone-resurfacing techniques for pulsatile tinnitus associated with vascular wall anomalies. Otolaryngol Head Neck Surg. 160(5):749-61, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30667295%5Bpmid%5D)
+1. [Sarioglu FC et al: Variations of the vascular canals in the cochlear implant candidates. Int J Pediatr Otorhinolaryngol. 123:123-7, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31100707%5Bpmid%5D)
+1. [Manjila S et al: Jugular bulb and skull base pathologies: proposal for a novel classification system for jugular bulb positions and microsurgical implications. Neurosurg Focus. 45(1):E5, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29961385%5Bpmid%5D)
+1. [Yeo WX et al: Surgical management of pulsatile tinnitus secondary to jugular bulb or sigmoid sinus diverticulum with review of literature. Am J Otolaryngol. 39(2):247-52, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29336902%5Bpmid%5D)
+1. [Kizildag B et al: The relationship between tinnitus and vascular anomalies on temporal bone CT scan: a retrospective case control study. Surg Radiol Anat. 38(7):835-41, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26831324%5Bpmid%5D)
+1. [Mortimer AM et al: Endovascular treatment of jugular bulb diverticula causing debilitating pulsatile tinnitus. J Neurointerv Surg. 8(3):e11, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=25691694%5Bpmid%5D)
+1. [Bae SC et al: Single-center 10-year experience in treating patients with vascular tinnitus: diagnostic approaches and treatment outcomes. Clin Exp Otorhinolaryngol. 8(1):7-12, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25729489%5Bpmid%5D)
+1. [Mundada P et al: CT arteriography and venography in the evaluation of pulsatile tinnitus with normal otoscopic examination. Laryngoscope. 125(4):979-84, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25379666%5Bpmid%5D)
+1. [Sakaida H et al: Dehiscent high jugular bulb attached to the tympanic membrane. Ear Nose Throat J. 94(6):210; 212, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26053975%5Bpmid%5D)
+1. [Atmaca S et al: High and dehiscent jugular bulb: clear and present danger during middle ear surgery. Surg Radiol Anat. 36(4):369-74, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24002578%5Bpmid%5D)
+1. [Shaikh MF et al: A novel approach for surgical repair of dehiscent high jugular bulb. Laryngoscope. 123(7):1803-5, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23172677%5Bpmid%5D)
+1. [Shweel M et al: Diagnostic utility of magnetic resonance imaging and magnetic resonance angiography in the radiological evaluation of pulsatile tinnitus. Am J Otolaryngol. 34(6):710-7, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24041839%5Bpmid%5D)
+1. [El-Begermy MA et al: A novel surgical technique for management of tinnitus due to high dehiscent jugular bulb. Otolaryngol Head Neck Surg. 142(4):576-81, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20304281%5Bpmid%5D)
+1. [Fayad JN et al: Jugular foramen tumors: clinical characteristics and treatment outcomes. Otol Neurotol. 31(2):299-305, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=19779386%5Bpmid%5D)
+1. [Friedmann DR et al: Clinical spectrum of patients with erosion of the inner ear by jugular bulb abnormalities. Laryngoscope. 120(2):365-72, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=19924772%5Bpmid%5D)
+1. [Vachata P et al: An anatomical and radiological study of the high jugular bulb on high-resolution CT scans and alcohol-fixed skulls of adults. J Clin Neurosci. 17(4):473-8, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20167495%5Bpmid%5D)
+1. [Meckel S et al: Display of dural sinuses with time-resolved, contrast-enhanced three-dimensional MR venography. Cerebrovasc Dis. 25(3):217-24, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18216463%5Bpmid%5D)
+1. [Dai PD et al: Morphological and positional relationships between the sigmoid sinus and the jugular bulb. Surg Radiol Anat. 29(8):643-51, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17962901%5Bpmid%5D)
+1. [Bilgen C et al: Jugular bulb diverticula: clinical and radiologic aspects. Otolaryngol Head Neck Surg. 128(3):382-6, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12646841%5Bpmid%5D)
+1. [Tsunoda A: Sensorineural hearing loss caused by a high jugular bulb. J Laryngol Otol. 114(11):867-9, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=11144839%5Bpmid%5D)
+1. [Palacios E et al: Jugular bulb appearing as a mass. Ear Nose Throat J. 78(8):536, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10485143%5Bpmid%5D)
+1. [Caldemeyer KS et al: The jugular foramen: a review of anatomy, masses, and imaging characteristics. Radiographics. 17(5):1123-39, 1997](http://www.ncbi.nlm.nih.gov/pubmed/?term=9308106%5Bpmid%5D)
+1. [Atilla S et al: Computed tomographic evaluation of surgically significant vascular variations related with the temporal bone. Eur J Radiol. 20(1):52-6, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7556255%5Bpmid%5D)
+1. [Tomura N et al: Normal variations of the temporal bone on high-resolution CT: their incidence and clinical significance. Clin Radiol. 50(3):144-8, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7889701%5Bpmid%5D)
+1. [Dietz RR et al: MR imaging and MR angiography in the evaluation of pulsatile tinnitus. AJNR Am J Neuroradiol. 15(5):879-89, 1994](http://www.ncbi.nlm.nih.gov/pubmed/?term=8059655%5Bpmid%5D)
+1. [Okudera T et al: Development of posterior fossa dural sinuses, emissary veins, and jugular bulb: morphological and radiologic study. AJNR Am J Neuroradiol. 15(10):1871-83, 1994](http://www.ncbi.nlm.nih.gov/pubmed/?term=7863937%5Bpmid%5D)
+1. [Ongre A: The jugular bulb once more. AJR Am J Roentgenol. 135(5):1117, 1980](http://www.ncbi.nlm.nih.gov/pubmed/?term=6778161%5Bpmid%5D)
+1. [Stern J et al: Jugular bulb diverticula in medial petrous bone. AJR Am J Roentgenol. 134(5):959-61, 1980](http://www.ncbi.nlm.nih.gov/pubmed/?term=6768272%5Bpmid%5D)
+
+## Differential diagnosis
+
+### Jugular Foramen Lesion
+DDX:cf70b4e9-14e8-496e-8927-62fe1bb1c89c
+
+### Normal Skull Base Venous Variants
+DDX:1e250956-d4d8-45f5-b228-92a8282f51ac
+
+## Anatomy
+
+### Carotid Space
+Head and Neck/ANATOMY:627bdee1-4bde-46f2-b93d-958882586337
+
+### CNX (Vagus Nerve)
+Head and Neck/ANATOMY:83868689-c995-4608-bed3-f59664cbd586
+
+### Accessory Nerve (CNXI)
+Brain/ANATOMY:9d50453e-c26a-46a3-826e-265736e43174
+
+### Glossopharyngeal Nerve (CNIX)
+Brain/ANATOMY:172680f5-d290-4a02-b07e-63c1da75e148
+
+### Hypoglossal Nerve (CNXII)
+Brain/ANATOMY:71012f02-fab7-42ed-bc60-e584dc229ccb
+
+### Vagus Nerve (CNX)
+Brain/ANATOMY:68b6ede4-c797-4d55-8d29-aed3df441741
+
+### CNIX (Glossopharyngeal Nerve)
+Head and Neck/ANATOMY:2e74a767-3f28-49be-a50f-1dbcc10ce90f
+
+### CNXI (Accessory Nerve)
+Head and Neck/ANATOMY:18e60151-70bc-40a1-9b4f-4b86f8fd65c2
+
+### CNXII (Hypoglossal Nerve)
+Head and Neck/ANATOMY:f734d678-561c-47fd-afb5-dab6afacc1a8
+
+## Cases
+
+- {'cases': [{'authors': [{'key': '624acd80-0502-4325-be71-e68fec740eb3', 'value': 'Richard H. Wiggins, III, MD, CIIP, FSIIM, FAHSE, FACR'}], 'caseVersionId': '4d0035c1-d210-4f39-9d5a-64475943b037', 'description': 'This is a typical case of a jugular foramen pseudolesion on MR.\n\nThe axial (#1-2) and the coronal (#3-4) T1 weighted pre-contrasted images show the heterogeneous signal intensity (arrow) within the right petrous apex. The post-contrasted axial (#5-8) and coronal (#9-10) images show the heterogeneous enhancement (arrow) within the jugular bulb, similar to the enhancement within the transverse sinuses (open arrow).', 'history': None, 'imagePoolId': '93f40956-03f9-44c1-9bf2-6c96c5796e25', 'name': 'Classic', 'teachingPoint': None}, {'authors': [{'key': '624acd80-0502-4325-be71-e68fec740eb3', 'value': 'Richard H. Wiggins, III, MD, CIIP, FSIIM, FAHSE, FACR'}], 'caseVersionId': '807b83b0-d2a0-46ab-9375-c307c4ddb4dc', 'description': 'This is a classic case of jugular foramen pseudolesion on MR.\n\nThe axial T1 weighted (#1-2), and T2 weighted (#3-5) images show the heterogeneous signal at the prominent right jugular foramen (arrow).\n\nThe post-contrasted axial (#6-7) and coronal (#8-9) images show the avid, heterogeneous signal correlating to this location (arrow), proving that this is actually the normal jugular foramen.\n\nComment: The can normal be asymmetry of the jugular foramen, and on MR, there can be confusing heterogeneous flow within the jugular bulbs. This is often clearly normal jugular foramen on CT.', 'history': None, 'imagePoolId': '5c71862a-4468-41e9-b3dc-ce7dc632a563', 'name': 'Classic', 'teachingPoint': None}, {'authors': [{'key': '624acd80-0502-4325-be71-e68fec740eb3', 'value': 'Richard H. Wiggins, III, MD, CIIP, FSIIM, FAHSE, FACR'}], 'caseVersionId': '98e2e3a0-b215-4711-b9d9-c55f31a0b0a9', 'description': 'This is a classic example of a jugular bulb pseudolesion on MR.\n\nThe axial T2 weighted images (#1-3) show the oval, bright signal intensity (arrow) within the left petrous apex, with signal intensity similar to that of CSF. The axial T1 weighted post-contrasted images (#4-7) show the avid, homogeneous enhancement of the same region (arrow).', 'history': 'Patient presented with a clinical history of headaches.', 'imagePoolId': '133d6658-e0c0-44a0-8107-f48ba8741fd5', 'name': 'Classic', 'teachingPoint': None, 'demographics': '33 Years old female'}, {'authors': [{'key': '624acd80-0502-4325-be71-e68fec740eb3', 'value': 'Richard H. Wiggins, III, MD, CIIP, FSIIM, FAHSE, FACR'}], 'caseVersionId': '9a36e383-702b-4ff9-ae64-28847eb03b2b', 'description': 'This is a classic case of a jugular bulb pseudolesion on MRI.\n\nThe axial T1 noncontrasted image (#1) shows the heterogeneous signal intensity within the left petrous apex (arrow). There is heterogeneous low signal intensity seen (arrow) on the axial T2 weighted image (#2). The axial T1 weighted post-contrasted image (#3) shows the avid, heterogeneous enhancement (arrow), similar to the transverse sinuses (open arrows). There is similar heterogeneous avid enhancement (arrow) seen on the coronal T1 weighted post-contrasted image (#4).', 'history': None, 'imagePoolId': '990b9b2a-d5a1-4139-88bc-56901f0670c8', 'name': 'Classic', 'teachingPoint': None, 'demographics': '27 Years old male'}, {'authors': [{'key': '624acd80-0502-4325-be71-e68fec740eb3', 'value': 'Richard H. Wiggins, III, MD, CIIP, FSIIM, FAHSE, FACR'}], 'caseVersionId': 'd63880cb-2b55-4bfd-a7cb-081b0dffde0b', 'description': 'This is a typical case of a jugular bulb pseudolesion on MRI.\n\nThe axial T1 weighted (#1-2), T2 weighted (#3-4) and FLAIR (#5-6) images show the heterogeneous signal intensity within the right petrous apex, near the jugular foramen (arrow), with relatively low signal intensity on T1 (#1-2) and increased signal intensity on T2 and FLAIR (#3-6). The axial T1 weighted post-contrasted images (#7-8) show the heterogeneous enhancement of this lesion (arrow), and the contiguity with the right transverse sinus (open arrow). The coronal T1 weighted post-contrasted images (#9-10) confirm the avid enhancement within the right jugular bulb (arrow). The 2 MRV images (#11-12) demonstrate the prominent and asymmetric right jugular bulb (arrow), consistent with a jugular bulb pseudolesion.', 'history': 'Patient presented with a history recent Horner’s syndrome on right.', 'imagePoolId': 'f255879f-f94c-4d45-9d32-e0e2a7c3f6cf', 'name': 'Classic', 'teachingPoint': None, 'demographics': '65 Years old male'}], 'caseType': 'typical', 'name': 'TYPICAL'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*Axial T1WI MR shows heterogeneous signal in the jugular foramen (JF)
, concerning for pathology in a patient with vertigo. Pseudolesions of the JF are common, often related to an asymmetric, large jugular bulb and turbulent flow or a high-riding jugular bulb.*
+
+
+*Axial CTA in the same patient shows normal enhancement of the jugular bulbs
. The enhancement is similar to the enhancement of the normal sigmoid sinuses
. Other sequences, such as MRA/MRV, can confirm this as a jugular bulb pseudolesion.*
+
+
+*Axial T1WI MR shows heterogeneous signal intensity within the right JF
, concerning for pathology. Other MR sequences proved this to be a jugular bulb pseudolesion, related to an asymmetric, large jugular bulb and turbulent flow.*
+
+
+*Coronal T1WI C+ MR shows a jugular bulb pseudolesion
related to turbulent flow in a mildly asymmetric right jugular bulb. This pseudolesion may be mistaken for a schwannoma or venous thrombosis. Other MR sequences confirmed this pseudolesion.*
+
+
+### Additional Images
+
+
+*Axial T1WI MR shows a rounded area of soft tissue signal intensity in the JF
, suspicious for pathology. Other MR sequences revealed it to be a jugular bulb pseudolesion.*
+
+
+*Axial T2WI MR reveals increased signal intensity at the right JF
, suspicious for JF schwannoma. Bone CT showed a normal asymmetrically large jugular bulb.*
+
+
+*Coronal T1WI C+ MR shows intense enhancement of a jugular bulb "lesion"
. Additional MR sequences and T-bone CT confirmed a jugular bulb pseudolesion.*
+
+
+*Coronal T1WI MR shows an ovoid area of heterogeneous signal intensity within the right jugular bulb
. Turbulent flow within the jugular bulb excludes the diagnosis of a jugular bulb pseudolesion.*
+
+
+*Axial T1WI C+ MR shows heterogeneous enhancement within the right sigmoid sinus
and jugular bulb
that could be mistaken for thrombosis. Subsequent MRV in this patient confirmed diagnosis of a jugular bulb pseudolesion rather than thrombosis.*
+
+
+*Axial T1WI C+ MR reveals an "enhancing mass" in the JF
. The presence of irregular margins suggests paraganglioma, but additional MR sequences and T-bone CT confirmed a jugular bulb pseudolesion as the diagnosis.*
+
+
+*Axial T1WI MR shows heterogeneous signal intensity within the left JF
, concerning for pathology. Other MR sequences proved this to be a jugular bulb pseudolesion. These pseudolesions are most commonly related to an asymmetrically large jugular bulb.*
+
+
+*Axial T1WI C+ FS MR shows heterogeneous enhancement of the left JF
, concerning for thrombosis. Other MR sequences confirmed this as a jugular bulb pseudolesion. Right mastoid surgical changes
are present.*
+
+
+*Axial T1WI C+ MR shows a jugular bulb pseudolesion
related to turbulent flow in a mildly asymmetric right jugular bulb. This pseudolesion may be mistaken for a schwannoma or venous thrombosis. Other MR sequences confirmed this a pseudolesion.*
+
+
+*Axial T2WI MR shows a hyperintense right JF "lesion"
, concerning for a JF schwannoma in this older adult patient with new-onset right-sided numbness.*
+
+
+*Axial T1WI C+ FS MR in the same patient shows normal enhancement of a mildly prominent right jugular bulb
. The enhancement is similar to the enhancement of the normal sigmoid sinuses
. Other sequences, including an MRA/MRV, confirmed this as a jugular bulb pseudolesion.*
+
diff --git a/docs_md/articles/langerhans-cell-histiocytosis-skull-and-brain_d4ceb50c-b678-490e-b948-6e939ac9b3b3.md b/docs_md/articles/langerhans-cell-histiocytosis-skull-and-brain_d4ceb50c-b678-490e-b948-6e939ac9b3b3.md
new file mode 100644
index 0000000..b231d52
--- /dev/null
+++ b/docs_md/articles/langerhans-cell-histiocytosis-skull-and-brain_d4ceb50c-b678-490e-b948-6e939ac9b3b3.md
@@ -0,0 +1,589 @@
+---
+title: "Langerhans Cell Histiocytosis, Skull and Brain"
+docid: "d4ceb50c-b678-490e-b948-6e939ac9b3b3"
+authors:
+ - key: "a25c450b-3d34-4f64-bba3-cc0834813df6"
+ value: "Miral D. Jhaveri, MD, MBA"
+ - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
+ value: "Anne G. Osborn, MD, FACR"
+breadcrumbs:
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+ slug: "brain"
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+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "948c6f1e-8e18-45f3-bd9d-7a04ab0b8708"
+ -
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+ slug: "pathology-based-diagnoses"
+ treeNodeId: "d60fe914-7897-46a0-9cd2-f88ffb322f16"
+ -
+ name: "Neoplasms"
+ slug: "neoplasms"
+ treeNodeId: "b9969ac8-c6b2-417c-aff8-85e2a3071075"
+ -
+ name: "Hematolymphoid Tumors Involving CNS"
+ slug: "hematolymphoid-tumors-involving-cns"
+ treeNodeId: "24b3933d-63e0-4802-80f7-6bacc6787fb5"
+ -
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+ -
+ name: "Langerhans Cell Histiocytosis, Skull and Brain"
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+lastUpdated: "08/20/25"
+pageDescription: "Langerhans Cell Histiocytosis, Skull and Brain"
+pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Neoplasms, Hematolymphoid Tumors Involving CNS, Histiocytic Tumors, Langerhans Cell Histiocytosis, Skull and Brain"
+pageTitle: "Langerhans Cell Histiocytosis, Skull and Brain | STATdx"
+enhancedTitle: "Langerhans Cell Histiocytosis, Skull and Brain"
+type: "DX"
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+ddx: true
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+ - "{'authors': 'Paula J. Woodward, MD, FSRU; Stella Sin Yee Ho, RDMS, RVT, PhD; Deyond Y. W. Siu, MBChB, FRCR', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/5c505fa3-4683-42db-9bd3-3f0e6a686938', 'category': 'Ultrasound', 'compareUrl': '/compare/document/5c505fa3-4683-42db-9bd3-3f0e6a686938/related-anatomy/treeNode?subContext=Orbit', 'documentId': '5c505fa3-4683-42db-9bd3-3f0e6a686938', 'documentType': 'ANATOMY', 'documentUrl': '/document/orbit/5c505fa3-4683-42db-9bd3-3f0e6a686938', 'enhancedTitle': 'Orbit', 'entryDate': '10/20/20', 'imageCount': 32, 'imageUrl': '/image/thumbnail/4c142c98-d86b-4f33-a1ab-cee1defe625a?size=174&quality=85', 'inCompareCart': False, 'rank': 21, 'referenceCount': 0, 'showCompareButton': False, 'title': 'Orbit'}"
+cases: 2
+breadcrumbs:
+ - "Brain"
+ - "Diagnosis"
+ - "Pathology-Based Diagnoses"
+ - "Neoplasms"
+ - "Hematolymphoid Tumors Involving CNS"
+ - "Histiocytic Tumors"
+ - "Langerhans Cell Histiocytosis, Skull and Brain"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Langerhans cell histiocytosis (LCH): Myeloid neoplastic disorder
+ - Activating mutations of MAPK pathway with *BRAF*V600E mutations
+ - Divided into 2 groups
+ - Single-system LCH
+ - Usually skin, bones, or lymph nodes
+ - Multisystem LCH
+ - Multiple bones, reticuloendothelial system, pituitary/hypothalamus
+- ### Imaging
+
+
+ - NECT
+ - Sharp lytic skull defect with beveled edges
+ - Mastoid: Geographic destruction, soft tissue mass
+ - MR
+ - Absent posterior pituitary "bright spot"
+ - Thick, enhancing infundibulum
+ - Enhancing masses in choroid plexus, leptomeninges, basal ganglia
+ - Neurodegenerative LCH occurs with cerebellar white matter (WM) disease
+- ### Top Differential Diagnoses
+
+
+ - Lytic calvarial lesions (surgical, epidermoid, dermoid)
+ - Pituitary infundibular/hypothalamic thickening, enhancement
+ - Tumor (germinoma, metastasis, pituicytoma)
+ - Neurosarcoid (especially in adult)
+- ### Clinical Issues
+
+
+ - Multisystem LCH typically presents at < 5 years
+ - Single system between 5-15 years
+ - Adult-onset accounts for ~ 1/3 of total LCH cases
+ - Prognosis variable
+ - Single-site LCH (favorable prognosis)
+ - Multifocal = unpredictable especially *BRAF*-mutant LCH
+ - Long-term morbidity with risk organ involvement (e.g., liver, spleen, bone marrow)
+- ### Diagnostic Checklist
+
+
+ - Calvarium = most frequent bony site involved by LCH
+ - Thick, enhancing pituitary stalk is most common CNS manifestation of LCH
+ - If initially "normal" MR in patient with DI, repeat in 2-3 months
+ - Consider LCH for ataxic patient with choroid plexus masses, cerebellar WM demyelination
+
+## TERMINOLOGY
+
+- ### Synonyms
+
+
+ - Langerhans cell histiocytosis (LCH)
+ - Several entities (eosinophilic granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease, and "histiocytosis X" now under single designation of LCH
+- ### Definitions
+
+
+ - LCH: Myeloid neoplastic disorder characterized by lesions with CD1a (+)/Langerin (CD207) (+) histiocytes and inflammatory infiltrate
+ - Activating mutations of MAPK pathway with *BRAF*V600E mutations being most recurrent mutations
+ - Activating somatic *BRAF*V600E mutations
+ - Associated with more severe disease
+ - Divided into 2 groups (based on number of lesions, systems involved)
+ - Single-system LCH
+ - 70-80% of cases (usually skin, bones, or lymph nodes)
+ - Single site (e.g., 1 bone lesion)
+ - Multisite (e.g., multiple bone lesions)
+ - Multisystem
+ - 20% of cases
+ - Chronic, recurring
+ - Multiple bones, reticuloendothelial system, pituitary/hypothalamus
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Calvarium: Sharply marginated lytic skull defect with beveled margins
+ - Skull base (mastoid most common): Geographic destruction ± soft tissue mass
+ - Brain: Thick, enhancing infundibulum, pituitary with absent posterior pituitary bright spot on T1WI
+ - #### Location
+
+
+ - Calvarium
+ - Most common bony site
+ - Frontal, parietal bones > temporal, occipital
+ - Also mastoid portion of temporal bone, mandible, orbit, facial bones
+ - Brain: Pituitary gland/infundibulum, hypothalamus
+ - Rare: Choroid plexus, leptomeninges, basal ganglia, cerebellar white matter (WM), and brain parenchyma
+ - #### Size
+
+
+ - Skull and facial bones: May grow, coalesce
+ - Pituitary infundibulum: Small lesions due to early endocrine dysfunction (central DI)
+ - #### Morphology
+
+
+ - Variable patterns of bony lysis ("geographic" skull)
+ - Soft tissue masses vary from discrete ↔ infiltrative
+- ### Radiographic Findings
+
+
+ - #### Radiography
+
+
+ - Calvarium: Well-defined lytic lesion, beveled edge, lack of marginal sclerosis
+ - ± button sequestra or sclerotic margins when healing
+ - Mastoid: Geographic destruction, often bilateral, little regional adenopathy
+ - Facial/orbital: More variable patterns of bony lysis, discrete ↔ permeative
+- ### CT Findings
+
+
+ - #### NECT
+
+
+ - Calvarium
+ - Lytic defect, "beveled" edge (inner table > outer table)
+ - Small soft tissue mass, no periosteal reaction
+ - Mastoid
+ - Bone destruction, often bilateral, soft tissue mass
+ - #### CECT
+
+
+ - Calvarium/mastoid: Enhancing soft tissue in lytic defect
+ - Brain: Enhancing, thick pituitary stalk, ± hypothalamic mass or enhancement
+- ### MR Findings
+
+
+ - #### T1WI
+
+
+ - Soft tissue mass at site of bony lysis
+ - ± T1 shortening due to lipid-laden histiocytes
+ - Brain
+ - Pituitary/infundibulum: Absent posterior pituitary bright spot, thick stalk, ± soft tissue mass
+ - **Neurodegenerative LCH**can have symmetric T1 hyperintensity in globi pallidi, dentate nuclei
+ - #### T2WI
+
+
+ - Skull, mastoid, orbital/facial lesions: Soft tissue masses show slight T2 hyperintensity
+ - Neurodegenerative LCH
+ - T2 hyperintense areas in pontine tegmentum
+ - ± cerebellar WM hyperintensity (autoimmune-mediated demyelination)
+ - #### FLAIR
+
+
+ - **Neurodegenerative LCH**
+ - Symmetric hyperintense cerebellar WM (butterfly wings appearance)
+ - Nonenhancing T2/FLAIR hyperintensity in hemispheric WM
+ - #### T1WI C+
+
+
+ - Skull, mastoid, orbital/facial: Enhancing soft tissue masses (defined or infiltrating)
+ - Brain
+ - Infundibulum: Thick, strongly enhancing stalk
+ - Enhancing masses in choroid plexus, leptomeninges, and basal ganglia
+- ### Nuclear Medicine Findings
+
+
+ - #### Bone scan
+
+
+ - Tc-99m bone scan: Variable (cold ↔ warm)
+ - #### PET
+
+
+ - F-18 FDG: ↑ uptake in proliferating lesions, ↓ uptake for burned-out lesions
+- ### Imaging Recommendations
+
+
+ - #### Best imaging tool
+
+
+ - Skull: NECT (with CECT for mastoid disease)
+ - Brain: MR with contrast
+ - #### Protocol advice
+
+
+ - Skull: CT using bone algorithm; include coronal and sagittal reconstructions
+ - Brain MR
+ - Pituitary MR: Small field of view, thin section, no gap, sagittal and coronal T1WI with contrast
+ - If initially "normal" in patient with DI, repeat in 2-3 months
+
+## DIFFERENTIAL DIAGNOSIS
+
+- ### Lytic Calvarial Lesions
+
+
+ - Surgical (burr hole, shunt, surgical defect)
+ - [Epidermoid](/document/epidermoid-cyst/e8b8ccaa-d29f-4257-9a03-364553efaccf)
+ - [Dermoid](/document/dermoid-cyst/23e1981b-6cad-42b3-883a-ded7357b8752)
+ - [Leptomeningeal cyst](/document/leptomeningeal-cyst-growing-fractu-/6cc4e026-56db-40c1-aab4-83db704c9539)
+ - [Tuberculosis](/document/tuberculosis/02e003d4-80b1-408d-9685-61f60637993e)
+ - Syphilis
+ - [Neurosarcoid](/document/neurosarcoid/0f637e19-adcd-4bf6-b2b9-8272613cb9e9)
+ - [Metastases](/document/skull-and-meningeal-metastases/5e4d4a09-e04a-49c9-a4e0-d2849a992a82)
+ - [Multiple myeloma](/document/multiple-myeloma/af7515c0-c479-496f-b834-3dcb7cf43348)
+- ### Temporal Bone Destructive Processes
+
+
+ - Severe mastoiditis: Infection usually spares bony labyrinth
+ - [Fibrous dysplasia: Skull base lesions may be lytic](/document/fibrous-dysplasia/c7ea8845-f92e-4df8-8e48-a2e1369717ca)
+ - [Rhabdomyosarcoma: Often with large ipsilateral cervical nodes](/document/rhabdomyosarcoma/7b9e59f4-389f-4b66-90de-8a1c2c35609e)
+- ### Pituitary Infundibular/Hypothalamic Thickening or Masses
+
+
+ - [Germinoma (child with DI)](/document/germinoma/69ff4834-1d53-4e17-8fa2-2134b9db2802)
+ - [Neurosarcoid (adult)](/document/neurosarcoid/0f637e19-adcd-4bf6-b2b9-8272613cb9e9)
+ - [Lymphocytic hypophysitis](/document/lymphocytic-hypophysitis/747903f7-8aa7-424c-8c60-89f12e19aba2)
+ - [Less common = pituicytoma, lymphoma, metastasis](/document/pituicytoma/fe593d2b-aab5-4f7e-95b5-0f20e8df96c5)
+- ### Other Histiocytoses
+
+
+ - Rosai-Dorfman disease
+ - Cervical lymphadenopathy; intracranial 5% (dural-based masses)
+ - Erdheim-Chester disease
+ - Also commonly associated with *BRAF* V600E mutation
+ - 12-19% of ECD cases have concurrent, clonally-related LCH
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Genetics
+
+
+ - Pathologic LCH cells are clonal proliferation of mature dendritic cells
+ - Clonal monocytosis, monocyte release into circulation, circulating LCH
+ - *BRAF* V600E mutations in 50-60%; *MAP2K1* mutations in 25%
+ - *BRAF*V600E mutation associated with more severe clinical course, resistance to chemotherapy, increased risk of relapse
+- ### Staging, Grading, & Classification
+
+
+ - Single-system unifocal: Single lesion involving 1 organ
+ - Single-system pulmonary: Isolated involvement of lung(s)
+ - Single-system multifocal: > 1 lesion involving 1 organ
+ - Multisystem: ≥ 2 involved organs
+- ### Gross Pathologic & Surgical Features
+
+
+ - Yellow, gray, or brown tumor mass
+- ### Microscopic Features
+
+
+ - Large epithelioid LCH cells (histiocytes) + abundant reactive eosinophils
+ - Monoclonality of Langerhans cells
+ - Positive CD1a, S100, &/or CD207 (Langerin)
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - Broad disease spectrum
+ - Ranges from asymptomatic isolated skin/bone lesion to multisystem, life-threatening disease
+ - Calvarial: Pain, subscalp mass, bony defect
+ - Mastoid destruction: Pain, chronic otitis externa, retroauricular subscalp mass
+ - Retroorbital mass: Exophthalmos, ± painful ophthalmoplegia
+ - Pituitary infundibular involvement: Central DI, ± visual disturbance, ± hypothalamic dysfunction
+ - Neurodegenerative LCH: Chronic, slowly progressing cerebellar syndrome, pyramidal tract signs, &/or neuropsychiatric symptoms
+ - #### Clinical profile
+
+
+ - Child < 2 years with diabetes insipidus, ± lytic calvarial lesion
+- ### Demographics
+
+
+ - #### Age
+
+
+ - Multisystem LCH typically presents at < 5 years
+ - Isolated bone lesions typically between 5-15 years
+ - Adult-onset accounts for ~ 1/3 of total LCH cases
+ - Mean age at onset, diagnosis = 33 years
+ - #### Sex
+
+
+ - M:F = 1:2
+- ### Natural History & Prognosis
+
+
+ - Variable depending on age of onset and extent of involvement
+ - Single-site LCH
+ - Favorable prognosis nearing 100% with < 20% rate of recurrence at 5 years
+ - Multifocal = unpredictable, especially *BRAF*-mutant LCH
+ - Long-term disease with high morbidity, especially with risk organ (e.g., liver, spleen, bone marrow)
+ - 5-year overall survival rate ≤ 77%
+- ### Treatment
+
+
+ - Therapeutic options depend on symptoms, location, and extent of disease
+ - Single-system unifocal LCH, surgery with goal of completion resection
+ - Multifocal or multisystem disease
+ - Options: Chemotherapy, surgery, radiation therapy, targeted therapy (MAPK signaling pathway inhibitors)
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - CNS LCH for ataxic patient with choroid plexus masses and cerebellar WM demyelination
+- ### Image Interpretation Pearls
+
+
+ - Skull is most frequent bony site involved by LCH
+ - Thick, enhancing pituitary stalk is most common CNS manifestation of LCH
+
+ 052a42aa-b75b-4af9-a281-b74a38934ab2
+
+## References
+
+## Selected References
+
+1. [Bielamowicz K et al: Langerhans cell histiocytosis: NACHO update on progress, chaos, and opportunity on the path to rational cures. Cancer. 130(14):2416-39, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38687639%5Bpmid%5D)
+1. [Orr T et al: Langerhans cell histiocytosis of the sella in a pediatric patient: case report with review of the literature. Childs Nerv Syst. 40(9):2947-52, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38775956%5Bpmid%5D)
+1. [Abla O: Langerhans cell histiocytosis: promises and caveats of targeted therapies in high-risk and CNS disease. Hematology Am Soc Hematol Educ Program. 2023(1):386-95, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=38066856%5Bpmid%5D)
+1. [Sconocchia T et al: Langerhans cell histiocytosis: current advances in molecular pathogenesis. Front Immunol. 14:1275085, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37965340%5Bpmid%5D)
+1. [Gulati N et al: Langerhans cell histiocytosis: Version 2021. Hematol Oncol. 39 Suppl 1:15-23, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34105821%5Bpmid%5D)
+1. [Tillotson CV et al: Langerhans cell histiocytosis. StatPearls, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=28613635%5Bpmid%5D)
+1. [Durham BH: Molecular characterization of the histiocytoses: neoplasia of dendritic cells and macrophages. Semin Cell Dev Biol. 86:62-76, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=29526544%5Bpmid%5D)
+1. [Héritier S et al: Progress towards molecular-based management of childhood Langerhans cell histiocytosis. Arch Pediatr. 26(5):301-7, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31281037%5Bpmid%5D)
+1. [Leung AKC et al: Childhood Langerhans cell histiocytosis: a disease with many faces. World J Pediatr. 15(6):536-45, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31456157%5Bpmid%5D)
+1. [Pan Z et al: Histiocytic and dendritic cell neoplasms. Surg Pathol Clin. 12(3):805-29, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31352989%5Bpmid%5D)
+1. [Papo M et al: Systemic histiocytosis (langerhans cell histiocytosis, Erdheim-Chester disease, Destombes-Rosai-Dorfman disease): from oncogenic mutations to inflammatory disorders. Curr Oncol Rep. 21(7):62, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31115724%5Bpmid%5D)
+1. [Park H et al: Imaging of histiocytosis in the era of genomic medicine. Radiographics. 39(1):95-114, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30500304%5Bpmid%5D)
+1. [Thacker NH et al: Pediatric Langerhans cell histiocytosis: state of the science and future directions. Clin Adv Hematol Oncol. 17(2):122-31, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30845115%5Bpmid%5D)
+1. [Wang Y et al: Neuroimaging features of CNS histiocytosis syndromes. Clin Imaging. 60(1):131-40, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31874337%5Bpmid%5D)
+1. [Kim JH et al: Magnetic resonance imaging features in solitary cerebral Langerhans cell histiocytosis: case report and review of literature. World Neurosurg. 116:333-6, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29929024%5Bpmid%5D)
+1. [Kobayashi M et al: Langerhans cell histiocytosis in adults: advances in pathophysiology and treatment. Cancer Sci. 109(12):3707-13, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30281871%5Bpmid%5D)
+1. [Krooks J et al: Langerhans cell histiocytosis in children: diagnosis, differential diagnosis, treatment, sequelae, and standardized follow-up. J Am Acad Dermatol. 78(6):1047-56, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29754886%5Bpmid%5D)
+1. [Tran G et al: Langerhans cell histiocytosis: a neoplastic disorder driven by Ras-ERK pathway mutations. J Am Acad Dermatol. 78(3):579-90.e4, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29107340%5Bpmid%5D)
+1. [Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25384298%5Bpmid%5D)
+1. [Badalian-Very G et al: Pathogenesis of Langerhans cell histiocytosis. Annu Rev Pathol. 8:1-20, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=22906202%5Bpmid%5D)
+
+## Differential diagnosis
+
+### Bitemporal Heteronymous Hemianopsia
+DDX:d241797d-f01f-4430-97dd-932ac761e5ac
+
+### Enhancing Cranial Nerve(s)
+DDX:6471fb1c-d46d-47cd-8322-1671e82335c3
+
+### Suprasellar Mass
+DDX:a675a04d-11c1-4192-80b2-899850a30416
+
+### Lateral Ventricular Mass
+DDX:ed97f84d-f861-46b9-ad19-f56d8ee04ba6
+
+## Anatomy
+
+### Scalp and Calvarial Vault
+Brain/ANATOMY:0783a6ca-5f20-48a8-802b-66c4578542f5
+
+### Cranial Meninges
+Brain/ANATOMY:3210cf7d-5be6-4741-a11d-dd7f8c1bc286
+
+### Pia and Perivascular Spaces
+Brain/ANATOMY:aade2e69-4af2-432e-8324-b4f0121d66d7
+
+### Ventricles and Choroid Plexus
+Brain/ANATOMY:e00c86e1-f0b9-4b88-9184-c22d8dd927d8
+
+### Bony Orbit and Foramina
+Head and Neck/ANATOMY:2a360457-05ee-41aa-ab5d-965b07b2fe13
+
+### Abducens Nerve (CNVI)
+Brain/ANATOMY:655e63da-d744-4267-9d90-07f52493bbc0
+
+### Oculomotor Nerve (CNIII)
+Brain/ANATOMY:495aba11-106f-439d-8516-6a445b085919
+
+### Optic Nerve (CNII)
+Brain/ANATOMY:54712d62-1940-4028-8161-4cfd3c4dbb20
+
+### Posterior Skull Base
+Brain/ANATOMY:b363fd64-a5af-4719-a285-71cdbec60f69
+
+### Trigeminal Nerve (CNV)
+Brain/ANATOMY:06e99da6-48fc-45f4-a571-97d12a15f365
+
+### Trochlear Nerve (CNIV)
+Brain/ANATOMY:cc69e654-b9cd-465b-8437-fba44f3c034b
+
+### CNIII (Oculomotor Nerve)
+Head and Neck/ANATOMY:ad21daaf-1f28-4f9c-bc74-3d0142c167ab
+
+### CNII (Optic Nerve)
+Head and Neck/ANATOMY:1b5322bb-bdca-4605-9ab5-43598b5c322f
+
+### CNIV (Trochlear Nerve)
+Head and Neck/ANATOMY:52486223-9cb5-43f5-b9af-c46431ae6637
+
+### CNVI (Abducens Nerve)
+Head and Neck/ANATOMY:923d7c4a-93a3-42ba-b8db-c7b15348e473
+
+### CNV (Trigeminal Nerve)
+Head and Neck/ANATOMY:9db87cfa-58ff-45fd-a7fd-fe8c73eb9770
+
+### Globe
+Head and Neck/ANATOMY:1808ae07-2bbe-40fe-9921-df2edd4277f7
+
+### Optic Nerve/Sheath Complex
+Head and Neck/ANATOMY:fdb933e1-ce0c-4f99-9ddb-cbe5f3b0f015
+
+### Orbit Overview
+Head and Neck/ANATOMY:1aaca035-b803-48cb-87ba-78c9004482b7
+
+### Posterior Skull Base
+Head and Neck/ANATOMY:8a7bdc2c-475b-4ce8-a35c-3fad9242a8bc
+
+### Orbit
+Ultrasound/ANATOMY:5c505fa3-4683-42db-9bd3-3f0e6a686938
+
+## Cases
+
+- {'cases': [{'authors': [{'key': '8d0c0f3b-13c2-45ac-8116-3725810235ec', 'value': 'Gary L. Hedlund, DO'}, {'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}], 'caseVersionId': '00022d2b-00ad-4d2e-8dd7-3ae5958c42c7', 'description': 'Coronal T1WI MR image (#1) demonstrates a thickened pituitary infundibulum (arrows).\n\nSagittal (#2) and coronal (#3) postenhanced T1WI MR images show thickening and robust enhancement of the pituitary infundibulum (arrows).', 'history': 'Onset of central diabetes insipidus.', 'imagePoolId': 'f6377ecf-ffa5-4578-b09b-fbe60eb0a3e2', 'name': 'Classic, hypothalamic', 'teachingPoint': 'Absence of the posterior pituitary bright spot, infundibular thickening, and diabetes insipidus are collectively strongly suggestive of Langerhans cell histiocytosis. Absence of the posterior pituitary bright spot, infundibular/suprasellar mass, and CSF tumor spread is suggestive of germinoma.', 'demographics': '8 Years old female'}, {'authors': [{'key': 'e8af6d26-3aad-47c9-9083-5128aab09af2', 'value': 'Susan I. Blaser, MD, FRCPC'}], 'caseVersionId': '56cfa96b-7111-493c-8693-f07fc98cfa57', 'description': 'Frontal (#1) and lateral (#2) radiographs reveal mastoid lytic lesion (curved arrow) subjacent to soft tissue mass (arrow). Mastoid destruction is identified on NECT (#3) bone algorithm image. Note clean bony margins. The mass is intermediate in signal intensity on T1WI (#4), heterogeneously decreased in signal on T2WI (#5) and vividly enhancing on enhanced, fat-saturated axial (#6) and coronal (#7) T1WIs. Temporal bone involvement is variable, reported in approximately 10-60% of patients. Mastoid swelling, middle ear polyps, and otorrhea are typical presenting complaints.', 'history': 'Patient presented with retroauricular swelling, query mastoid abscess.', 'imagePoolId': 'f9f28047-1cc1-4973-a507-059ba8f59742', 'name': 'Unilateral', 'teachingPoint': None, 'demographics': '1 Years old female'}, {'authors': [{'key': 'e8af6d26-3aad-47c9-9083-5128aab09af2', 'value': 'Susan I. Blaser, MD, FRCPC'}, {'key': '8d0c0f3b-13c2-45ac-8116-3725810235ec', 'value': 'Gary L. Hedlund, DO'}], 'caseVersionId': 'b5dccfcb-9242-4c08-93e8-174eb5e87bea', 'description': 'Axial NECT with bone algorithm (#1) reveals bilateral mastoid destruction (arrows). There is opacification of bilateral middle ear compartments and the walls of the mastoid antra have been broached. NECT with soft tissue windows (#2, 3). Bone destruction is accompanied by bilateral large hyperattenuated soft tissue masses (arrows). Poor filling of the sigmoid sinuses is seen following contrast administration on CECT (#4). Enhancement pattern may be variable, here there is rim enhancement. Typically the soft tissue component enhances strongly.', 'history': None, 'imagePoolId': '0c832d65-b4f0-4ff6-9b11-e8bc34b7036c', 'name': 'Bilateral', 'teachingPoint': None}, {'authors': [{'key': 'e8af6d26-3aad-47c9-9083-5128aab09af2', 'value': 'Susan I. Blaser, MD, FRCPC'}, {'key': '8d0c0f3b-13c2-45ac-8116-3725810235ec', 'value': 'Gary L. Hedlund, DO'}], 'caseVersionId': 'c8ea9df6-0f04-4c50-904e-191ceacec0d2', 'description': 'Sagittal (#1) and coronal (#2) unenhanced T1W images reveal thickening of the pituitary stalk (arrow). There is avid enhancement (arrow) of the thickened stalk on enhanced sagittal (#3) and coronal (#4) T1W images. The infundibular stalk is the most common site of intracranial involvement by histiocytosis and the most common clinical presentation of pituitary infundibular involvement is diabetes insipidus (DI). There may also be visual disturbance or hypothalamic dysfunction.', 'history': None, 'imagePoolId': '791cf4e6-b18a-48da-9b96-ac1f73372ef8', 'name': 'Infundibular thickening', 'teachingPoint': None}, {'authors': [{'key': 'e8af6d26-3aad-47c9-9083-5128aab09af2', 'value': 'Susan I. Blaser, MD, FRCPC'}, {'key': '999a14fb-b7a4-463e-8883-939a5029ac25', 'value': 'Lane F. Donnelly, MD'}], 'caseVersionId': 'd237b5d3-ce08-4060-94fa-b56e641c870a', 'description': 'Townes view (#1) shows shows typical sharply circumscribed lytic occipital bone defect. Unequal involvement of inner and outer cortex give rise to a "beveled edge" (curved arrow). Solitary lesions have the best prognosis. They may undergo spontaneous remission or may require treatment by curettage if painful.', 'history': None, 'imagePoolId': 'df19977c-782d-47bf-a9b9-ba82df954521', 'name': 'Beveled edges', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'aafec080-e264-47a7-b4d7-0406081d126d', 'description': 'Coronal T1C+ (#1) scan shows a diffusely enlarged, enhancing pituitary gland that bows the optic chiasm upwards (arrow). Sagittal T1C+ scan shows the mass is mostly in the infundibular stalk and hypothalamus but is contiguous through the diaphragma sellae with the pituitary gland below (arrow).', 'history': 'Child with histiocytosis and diabetes insipidus.', 'imagePoolId': '0fb3da9c-34f5-4f75-ad50-91cf6c59ba8c', 'name': 'Gland, infundibulum involved', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '90b12fa0-cb24-4110-a923-a1cc2df07634', 'description': 'Sagittal and coronal T1WIs (#1,2) show thickened infundibular stalk (arrows). The thick stalk appears isointense with brain on both T1 and T2WIs (arrow, #3) and enhances moderately with contrast (#4,5).', 'history': 'Central diabetes insipidus.', 'imagePoolId': 'c7b69782-793c-43c4-a824-8e3085eb4b07', 'name': 'Stalk only', 'teachingPoint': None}], 'caseType': 'typical', 'name': 'TYPICAL'}
+- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}, {'key': '6e26637f-9cb3-4b8a-8e59-79c6c8bfe097', 'value': 'Hilda E. Stambuk, MD'}], 'caseVersionId': '34aea66f-ab51-4603-a099-ab00476118e5', 'description': 'Axial T2WI (#1) shows multiple very hypointense masses in the suprasellar cistern (curved arrow), choroid plexus of both lateral ventricles (arrows) and tentorial apex (open arrow). Sagittal (#2,3) and axial (#4,5) T1C+ scans show the suprasellar mass enhances strongly, uniformly, and diffusely infiltrates the pituitary gland, stalk, optic chiasm and hypothalamus (curved arrows). The dura along the tentorium is thickened, nodular and enhancing (open arrows). The markedly enlarged, infiltrated choroid plexus in both temporal horns also enhances strongly (arrows, #5).', 'history': 'Child with known Langerhans cell histiocytosis.', 'imagePoolId': '71773bf9-577d-4905-bbb2-307a9237ac07', 'name': 'Also dural, pituitary, choroid plexus', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '92ce082c-3a8f-48ec-b413-ffc63cfae008', 'description': 'The precontrast sagittal (#1) and coronal (#2) T1WIs show a thickened infundibulum (arrow) with absence of the normal superior to inferior tapering of the stalk on the coronal study. Note coexisting sphenoid sinusitis (curved arrow, #1) and absence of the posterior pituitary "bright spot" on the sagittal scan. The post-contrast T1WI (#3) shows the thickened stalk enhances strongly and uniformly (arrow).', 'history': 'Diabetes insipidus, sinusitis. ', 'imagePoolId': '497d1660-6278-4406-ae56-30bd4c627f55', 'name': 'Thick infundibulum', 'teachingPoint': 'The most likely diagnosis in this case is sarcoidosis. However, the sinus involvement raises the possibility of other entities, such as lymphoma and histiocytosis. Further evaluation in this patient disclosed Langerhans cell histiocytosis (LCH). This case is unusual because LCH is rare in adults. But it does occur and should be considered in the differential diagnosis of thickened infundibulum.', 'demographics': '39 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'a22fc7e6-116c-4c97-90e5-7f8c86d6478f', 'description': 'Axial T2-weighted MR scans (#1-3) show hyperintense signal within the cerebellar white matter (#1), and masses within the lateral ventricles that are isointense with white matter (arrows, #2, 3). T1C+ axial (#4-8) and coronal (#9, 10) scans show the cerebellar abnormalities do not enhance but there are multiple parenchymal and intraventricular foci of contrast enhancement. The punctate and linear enhancing areas in the parenchyma (arrows, #5, 8-10) represent involvement of the perivascular spaces while the enhancing intraventricular masses (open arrows, #6, 7, 9) are in the choroid plexi. \n\nBiopsy-proven LCH in the brain.', 'history': 'Child with known systemic Langerhans cell histiocytosis (LCH).', 'imagePoolId': 'bb0290c1-5276-440c-bc5b-17b57e6e80bf', 'name': 'Choroid plexus, secondary parenchymal changes', 'teachingPoint': None}], 'caseType': 'variant', 'name': 'VARIANT'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*Lateral graphic demonstrates 3 sharply defined lytic lesions
of the membranous calvarium with geographic destruction. Note the beveled margins of the bony lysis
.*
+
+
+*Axial Bone CT in a 7-year-old with scalp mass demonstrates a lytic lesion of the right frontal bone
with an associated scalp mass
. There is a characteristic pattern of sharply defined geographic bony lysis with unequal inner and outer table involvement creating a beveled edge appearance. Surgical resection confirmed LCH.*
+
+
+*Sagittal T1 MR in a 17-year-old boy with central diabetes insipidus demonstrates nodular thickening of the pituitary stalk
. There is absence of the normal posterior pituitary focus of T1 shortening
, a common finding with diabetes insipidus.*
+
+
+*Sagittal T1 C+ FS MR in the same patient shows nodular thickening and enhancement of the pituitary stalk
. Pituitary stalk thickening is the most common finding with CNS involvement in LCH.*
+
+
+*Sagittal T1 C+ MR in a patient with LCH shows a suprasellar mass involving the hypothalamus and infundibular stalk infiltrating the pituitary gland
. Note dural thickening along the straight sinus and tentorium
.*
+
+
+*Axial T1 C+ FS MR in the same patient shows the enhancing mass in the hypothalamus-pituitary axis
. Intensely enhancing masses are seen in both choroid plexi
. Note thickening along the tentorial apex
.*
+
+
+*Axial bone CT in a 51-year-old man with dizziness, hearing loss, and ringing in his ears shows a destructive mass in the right temporal bone that has partially eroded the ossicles and sigmoid sinus plate.*
+
+
+*(L) Axial T2 MR in the same patient shows a hyperintense lobulated mass in the eroded temporal bone
. (R) T1 C+ FS MR shows the mass enhances strongly but somewhat heterogeneously
. Surgery disclosed LCH.*
+
+
+*Axial T1 C+ MR in a child with multisystem LCH shows some T1 shortening in the globi pallidi and an enhancing mass in the right choroid plexus.*
+
+
+*Axial FLAIR MR in the same patient shows confluent regions of demyelination with FLAIR signal hyperintensity involving the cerebellar white matter
. Note the involvement of the abducens nuclei
. This is neurodegenerative LCH.*
+
+
+### Additional Images
+
+
+*Axial bone CT shows the classic appearance of LCH as a sharply marginated lytic calvarial lesion
. Note the "beveled" edges
around the lesion and the adjacent soft tissue mass
. This is a classic single-system, single-site lesion of LCH.*
+
+
+*Axial CECT in a 15-month girl with mastoid region swelling, central DI, and ataxia shows bilateral destructive temporal bone masses
.*
+
+
+*T1 C+ FS MR in the same patient shows a thickened, enhancing infundibular stalk
.*
+
+
+*T2 MR in the same patient shows patchy hyperintensities in the dentate nuclei
representing autoimmune-mediated demyelination.*
+
+
+*Coronal T1 C+ MR shows a robustly enhancing destructive mass
of the right lateral orbital wall. Note the mass extending into the right suprazygomatic space
in this case of LCH.*
+
+
+*Axial T1 C+ MR shows enhancing choroid plexus masses
within the ventricular trigones. Note the mottled enhancement (perivascular infiltration) within the basal ganglia
.*
+
+
+*Axial T2 MR in a patient with systemic LCH shows hyperintense signal within the cerebellar white matter, findings consistent with autoimmune mediated demyelination
.*
+
+
+*Sagittal T1 MR shows thickening of the pituitary infundibulum
and absence of the normal posterior pituitary bright spot
.*
+
+
+*Sagittal T1 C+ MR shows a thickened, vividly enhancing pituitary stalk
in this patient with LCH.*
+
+
+*Coronal bone CT in a child with LCH shows a central destructive lesion in the skull base extending into the anterior cranial fossa
, left orbit
, and upper nasopharynx
.*
+
+
+*Coronal T1 C+ FS MR in the same patient shows the mass
enhances intensely.*
+
+
+*Sagittal T1 C+ FS MR in a 5-year-old boy with chronic headaches and diabetes insipidus demonstrates a heterogeneously enhancing lesion
of the central skull base. Note the thickening and displacement of the pituitary infundibulum
.*
+
+
+*Axial NECT in a 5-year-old girl with multiple scalp masses demonstrates 2 lytic lesions
of the left frontal bone. Both lesions show the characteristic pattern of sharply defined geographic bony lysis. Note the unequal inner and outer table involvement creating a beveled edge appearance.*
+
+
+*Sagittal T1 MR in a 7-year-old girl with central diabetes insipidus demonstrates a soft tissue mass
involving the hypothalamus. Note the absence of the normal posterior pituitary focus of T1 shortening
, a common finding in children with diabetes insipidus.*
+
+
+*Coronal T1 C+ FS MR in the same patient shows enhancement of the hypothalamic nodule
. Pituitary stalk thickening is the most common finding with CNS involvement.*
+
diff --git a/docs_md/articles/lower-extremity-vasculature-anatomy_f5620b22-4f6a-46f7-9848-9d05fb0ff20e.md b/docs_md/articles/lower-extremity-vasculature-anatomy_f5620b22-4f6a-46f7-9848-9d05fb0ff20e.md
new file mode 100644
index 0000000..599419b
--- /dev/null
+++ b/docs_md/articles/lower-extremity-vasculature-anatomy_f5620b22-4f6a-46f7-9848-9d05fb0ff20e.md
@@ -0,0 +1,195 @@
+---
+title: "Lower Extremity Vasculature Anatomy"
+docid: "f5620b22-4f6a-46f7-9848-9d05fb0ff20e"
+authors:
+ - key: "96cc692f-3ae1-4d10-bd6f-2e1d308c3fc3"
+ value: "Suvranu Ganguli, MD"
+breadcrumbs:
+ -
+ name: "Vasculature"
+ slug: "vasculature"
+ treeNodeId: "6de1ee4d-afe9-419c-a868-d4074ec0fb7e"
+ -
+ name: "Anatomy"
+ slug: "anatomy"
+ treeNodeId: "3bbfaa8b-2dbc-41eb-b3da-3bd83a9737c4"
+ -
+ name: "Lower Extremity Vasculature Anatomy"
+ slug: "lower-extremity-vasculature-anatomy"
+ treeNodeId: null
+category: "Vasculature"
+documentVersionId: "b6696083-ebe9-47e6-9784-d49eca3448c7"
+imageCount: 9
+lastUpdated: "01/31/23"
+pageDescription: "Lower Extremity Vasculature Anatomy"
+pageKeywords: "Vasculature, Anatomy, Lower Extremity Vasculature Anatomy"
+pageTitle: "Lower Extremity Vasculature Anatomy | STATdx"
+enhancedTitle: "Lower Extremity Vasculature Anatomy"
+type: "ANATOMY"
+breadcrumbs:
+ - "Vasculature"
+ - "Anatomy"
+ - "Lower Extremity Vasculature Anatomy"
+---
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - Common femoral artery (CFA)
+ - Superficial femoral artery (SFA)
+ - Profunda femoris artery (PFA)
+ - Greater saphenous vein (GSV)
+ - Small saphenous vein (SSV)
+- ### Synonyms
+
+
+ - Internal iliac artery = hypogastric artery
+ - Small saphenous vein = lesser saphenous vein
+
+## IMAGING ANATOMY
+
+- ### Anatomy Relationships
+
+
+ - Abdominal aorta bifurcates into common iliac arteries
+ - Common iliac arteries are 3-6 cm long, 8-10 mm in diameter
+ - Divide into internal/external iliac arteries
+ - Internal iliac arteries bifurcate into anterior and posterior divisions; supply pelvic muscles, viscera
+ - Anterior division yields inferior gluteal, obturator, internal pudendal, vesicle, uterine arteries
+ - Posterior division yields superior gluteal, iliolumbar, lateral sacral arteries
+ - External iliac arteries become CFA at inguinal ligament
+ - Ligament delineated by origins of inferior epigastric and deep circumflex iliac arteries
+ - CFA is usually 5-7 cm long, 5-9 mm in diameter
+ - Bifurcates into SFA and PFA
+ - CFA and vein are within femoral sheath, continuation of abdominal wall fascia; femoral nerve outside sheath
+ - PFA origin is posterolateral to SFA origin
+ - Main trunk has deep course adjacent to femur
+ - Anastomoses with SFA/popliteal branches
+ - Supplies proximal hip
+ - Medial and lateral femoral circumflex arteries
+ - Provides collaterals in proximal SFA occlusion/stenosis
+ - SFA provides dominant in-line arterial supply in thigh
+ - Courses beneath sartorius, anterior to femoral vein
+ - Exits Hunter canal at adductor hiatus as popliteal artery
+ - Multiple muscular branches; supreme geniculate (medial) branch is largest
+ - Popliteal artery extends from adductor hiatus to calf
+ - Knee joint delineates above-/below-knee segments
+ - Levels influence intervention choices, outcomes
+ - Yields superior medial, superior lateral, middle, inferior medial, and inferior lateral geniculate arteries
+ - Bifurcates into anterior tibial, tibioperoneal trunk
+ - Tibioperoneal trunk divides into posterior tibial, peroneal arteries
+ - 3 below-knee runoff arteries
+ - Anterior tibial artery
+ - Courses to foot in anterior compartment of calf
+ - Continues into foot as dorsalis pedis artery
+ - Posterior tibial artery
+ - Extends from upper calf to medial malleolus
+ - Contained in deep posterior calf compartment
+ - Terminates as medial and lateral plantar arteries
+ - Forms plantar arch of foot
+ - Peroneal artery
+ - Descends posteromedial to fibula in deep posterior calf compartment
+ - Ends in characteristic forked anterior and posterior perforating branches
+
+## ANATOMY IMAGING ISSUES
+
+- ### Lower Extremity Vascular Pathology
+
+
+ - Atherosclerotic occlusive disease
+ - Acute limb ischemia
+ - Embolic vs. thrombotic occlusion
+ - Endovascular or surgical treatment
+ - Chronic limb ischemia
+ - Symptoms relate to occlusion level, collaterals
+ - Medical, endovascular, and surgical management
+ - Aneurysms
+ - True or false (pseudoaneurysm)
+ - CFA aneurysm
+ - Most frequent site for pseudoaneurysm; secondary to catheterization, surgical anastomosis
+ - Popliteal artery aneurysm
+ - Most common lower extremity aneurysm
+ - Presents with limb ischemia in > 50%
+ - Thromboembolic complications common
+ - Arteriovenous malformations
+ - Lower extremities are most common location
+ - Congenital abnormalities
+ - Persistent sciatic artery
+ - Internal iliac artery continues as sciatic artery and then as popliteal artery
+ - May have hypoplastic SFA; sciatic artery dominates blood supply to lower extremity (complete form)
+ - Cystic adventitial disease
+ - Focal cystic mucin accumulation in adventitia
+ - Cysts cause vascular compression, claudication
+ - Popliteal artery is most often affected
+ - Ergotism
+ - Ergot alkaloids cause diffuse vasospasm
+ - Arterial constriction may progress to occlusion
+ - Fibromuscular dysplasia
+ - Common and external iliac arteries
+ - Popliteal artery entrapment
+ - Artery deviates around gastrocnemius muscle or is compressed between muscular structures
+ - Claudication; may progress to occlusion
+ - Trauma
+ - 1/3 of cases of vascular trauma involve extremities
+ - Penetrating or blunt trauma
+ - Arterial injury with 30-40% of knee dislocations
+ - Vasculitis
+ - Buerger disease (most common); others (uncommon)
+ - Venous diseases
+ - Deep vein thrombosis (DVT)
+ - Blood clots in deep veins of lower extremity
+ - Risk of embolism to pulmonary arteries
+ - May result in chronic venous insufficiency
+ - Chronic venous insufficiency
+ - Chronic swelling, pigmentation, venous stasis ulcers; secondary to DVT (postthrombotic syndrome)
+ - Varicose veins, incompetent perforators
+ - Klippel-Trenaunay syndrome: Congenital disorder
+ - Capillary malformations (port-wine stain), soft tissue or bone hypertrophy, varicose veins or venous malformations affecting extremity
+ - Venous malformations
+ - Congenital abnormality; deep or superficial malformations; sclerotherapy for treatment
+
+ 1d8c329b-4ef8-4226-9bea-c542554afd68
+
+
+## Images
+
+
+### Pelvic and Lower Extremity Arterial Anatomy
+
+
+*AP MIP from MR angiogram of the lower abdomen and pelvis displays the arteries of the pelvis.*
+
+
+*AP MIP from MR angiogram of the lower abdomen and pelvis displays the arteries of the pelvis.*
+
+
+*Single AP image from catheter angiography of the region of the right hip displays the arteries of the hip and thigh.*
+
+
+*Single AP image from catheter angiography of the region of the thigh displays the arterial anatomy.*
+
+
+### Lower Extremity Arterial Anatomy
+
+
+*Single AP image from catheter angiography of the region of the knee displays the arterial anatomy.*
+
+
+*AP MIP from MR angiogram of the left lower extremity displays the runoff arteries below the knee.*
+
+
+*Single lateral film from catheter angiography of the foot displays the arterial anatomy of the foot.*
+
+
+### Upper and Lower Extremity Venous Anatomy
+
+
+*AP MIP from MR venogram of the pelvis and thighs displays the veins of the lower pelvis and thighs.*
+
+
+*Single lateral radiograph from ascending venogram of the lower leg displays the veins below the knee.*
+
+
+*AP MIP from MR venogram of the upper extremity displays the upper arm and central thoracic veins.*
+
diff --git a/docs_md/articles/oculomotor-trochlear-or-abducens-neuropathy_01092f4c-ec92-43df-a667-b3ee93b8a268.md b/docs_md/articles/oculomotor-trochlear-or-abducens-neuropathy_01092f4c-ec92-43df-a667-b3ee93b8a268.md
new file mode 100644
index 0000000..bbafe90
--- /dev/null
+++ b/docs_md/articles/oculomotor-trochlear-or-abducens-neuropathy_01092f4c-ec92-43df-a667-b3ee93b8a268.md
@@ -0,0 +1,420 @@
+---
+title: "Oculomotor, Trochlear, or Abducens Neuropathy"
+docid: "01092f4c-ec92-43df-a667-b3ee93b8a268"
+authors:
+ - key: "eef2f839-5706-47b9-89c3-60d8315b2b3a"
+ value: "Nicholas A. Koontz, MD"
+breadcrumbs:
+ -
+ name: "Head and Neck"
+ slug: "head-and-neck"
+ treeNodeId: "5c1f8e17-7acd-48d8-9d55-f9f8c2cad850"
+ -
+ name: "Differential Diagnosis"
+ slug: "differential-diagnosis"
+ treeNodeId: "deb55065-e1d6-4b6f-b3e3-181fafb4e218"
+ -
+ name: "Cranial Nerves and Brainstem"
+ slug: "cranial-nerves-and-brainstem"
+ treeNodeId: "385449a2-5859-451c-bed3-584babc08f0c"
+ -
+ name: "Clinically Based Differentials"
+ slug: "clinically-based-differentials"
+ treeNodeId: "f0574381-a384-4486-8fb9-e90591e4f9be"
+ -
+ name: "Oculomotor, Trochlear, or Abducens Neuropathy"
+ slug: "oculomotor-trochlear-or-abducens-n-"
+ treeNodeId: null
+category: "Head and Neck"
+documentVersionId: "de8b3b02-ee8c-4569-a2b4-ff7bcf0cf651"
+imageCount: 55
+lastUpdated: "04/13/26"
+pageDescription: "Oculomotor, Trochlear, or Abducens Neuropathy"
+pageKeywords: "Head and Neck, Differential Diagnosis, Cranial Nerves and Brainstem, Clinically Based Differentials, Oculomotor, Trochlear, or Abducens Neuropathy"
+pageTitle: "Oculomotor, Trochlear, or Abducens Neuropathy | STATdx"
+enhancedTitle: "Oculomotor, Trochlear, or Abducens Neuropathy"
+type: "DDX"
+references: true
+breadcrumbs:
+ - "Head and Neck"
+ - "Differential Diagnosis"
+ - "Cranial Nerves and Brainstem"
+ - "Clinically Based Differentials"
+ - "Oculomotor, Trochlear, or Abducens Neuropathy"
+---
+## ESSENTIAL INFORMATION
+
+- ### Key Differential Diagnosis Issues
+
+
+ - Cranial nerves supply 6 extraocular muscles
+ - CNIII: All muscles but superior oblique (CNIV) & lateral rectus (CNVI)
+ - CNIII, IV, VI all have brainstem, cisternal, cavernous sinus (CS), & orbital components
+ - CNIII anatomy
+ - Nucleus in dorsal midbrain at level superior colliculus
+ - Cisternal CNIII parallels posterior communicating artery
+ - Midbrain → interpeduncular cistern → oculomotor cistern → CS → superior orbital fissure (SOF) → orbit
+ - Arterial supply to CNIII feeds nerve core
+ - Parasympathetic fibers (pupillary sphincter) in superficial CNIII
+ - CNIII neuropathy in patient > 40 years of age
+ - Patient old enough to suffer from vasculopathic oculomotor neuropathy
+ - Pupillary sparing often followed clinically
+ - CNIII neuropathy in patient < 40 years of age or any complete CNIII neuropathy
+ - Posterior communicating artery aneurysm must be excluded
+ - CTA or MR/MRA followed by conventional cerebral angiogram if unanswered questions
+ - CNIV anatomy
+ - Midbrain nucleus → dorsal decussation → free margin tentorium → CS → SOF → orbit
+ - CN6 anatomy
+ - Pontine tegmentum nucleus → exits at ventral pontomedullary junction → prepontine cistern → abducens (Dorello) canal → CS → SOF → orbit
+- ### Helpful Clues for Common Diagnoses
+
+
+ - **Microvascular Infarction (****CNIII, CNIV, CNVI****)**
+ - Key facts: Acute-onset CNIII, CNIV, CNVI neuropathy
+ - CNIII: Usually pupil sparing; diabetics ± hypertension
+ - Imaging: Usually normal; enhancement of affected CNIII, CNIV, or CNVI rare
+ - **Saccular Aneurysm, Posterior Communicating Artery (CNIII)**
+ - Key facts: Complete CNIII neuropathy
+ - Imaging: CTA, MRA, angiography
+ - CTA: Ovoid contrast collection; ± Ca⁺⁺ in wall
+ - MR/MRA: Complex signal from flow, clot, & Ca⁺⁺ in wall of aneurysm
+ - **Multiple Sclerosis**
+ - Key facts: CN symptoms in younger patient, suspect multiple sclerosis
+ - Imaging: White matter plaques
+ - Often subtle; lesions in midbrain & pons may or may not be visible
+ - **Cerebral Ischemia-Infarction, Acute**
+ - Key facts: Acute onset CNIII, CNIV, CNVI symptoms
+ - Imaging: DWI demonstrates reduced diffusivity
+ - **Meningioma, Cavernous Sinus**
+ - Key facts: From dural margin of CS
+ - Imaging: Extraaxial mass with enhancing dural tails
+ - **Metastases, Intracranial, Cavernous Sinus**
+ - Imaging: Enlarged, irregular enhancing CS ± reduced diffusivity from cellular tumor
+ - **Schwannoma, Cavernous Sinus (****CNIII, CNIV, CNVI****)**
+ - Key facts: May be asymptomatic
+ - Imaging: Smoothly enhancing fusiform mass in enlarged CS
+ - **Metastases, Parenchymal, Brainstem**
+ - Imaging: Enhancing brainstem lesion often with significant associated edema
+ - **Thrombosis, Cavernous Sinus**
+ - Key facts: Often septic patient; look for sinonasal source
+ - Imaging: Nonenhancing clot in enlarged CS ± large superior orbital veins; clot often diffusion bright
+- ### Helpful Clues for Less Common Diagnoses
+
+
+ - **Idiopathic Orbital Inflammation (Pseudotumor)**
+ - Key facts: Painful, limited eye movement
+ - Imaging: May involve any part of orbit, including optic nerve sheath complex, extraocular muscles, lacrimal glands, globes, intra- & extraconal spaces, & orbital apex
+ - Often low T1 & T2 signal due to fibrosis or high cellularity
+ - Moderate to avid enhancement
+ - **Intracranial Idiopathic Inflammatory Pseudotumor**
+ - Key facts: Idiopathic orbital inflammation can extend intracranially via SOF, optic canal, or transosseous spread
+ - Can involve CS, dura, trigeminal cave, & cranial nerves, mimicking perineural tumor spread
+ - Imaging: Thickened, enhancing meninges of CS wall, ± enhancing CNs
+ - Often low T1 & T2 signal due to fibrosis or high cellularity
+ - May have enhancing dural tails like meningioma or mimic perineural tumor spread of malignancy
+ - **Carotid-Cavernous Fistula, Traumatic**
+ - Key facts: Posttraumatic red eye; often weeks to months after initial trauma
+ - Imaging: Enlarge CS & superior ophthalmic vein
+ - **Saccular Aneurysm, Cavernous ICA**
+ - Imaging: Unilateral CS enlargement
+ - Complex signal from clot, flow, & Ca⁺⁺
+ - **Lymphoproliferative Lesions, Orbit**
+ - Imaging: Invasive mass anywhere in orbit; often marked low ADC signal intensity on DWI due to high cellularity
+- ### Helpful Clues for Rare Diagnoses
+
+
+ - **Metastasis, Orbit**
+ - Key facts: Breast, prostate, lung most common
+ - Imaging: May involve any part of orbit; variable appearance ranging from well-defined mass to diffuse enhancing soft tissue
+ - **Pituitary Macroadenoma**
+ - Key facts: > 10 mm; suprasellar ± CS
+ - Imaging: Large pituitary with suprasellar ± CS involvement
+ - Encasement of cavernous carotid artery has reported 100% specificity for predicting CS invasion
+ - **Cavernous Malformation**
+ - Key facts: Solitary or multiple (familial)
+ - MR: Lesion blood products & popcorn ball appearance; blooms on GRE
+ - 40-60% Ca⁺⁺ on NECT
+ - **Schwannoma, Cisternal (CNIII, CNIV, CNVI)**
+ - Key facts: May be incidental
+ - Imaging: Enhancing fusiform mass extending along CN course
+ - **Direct Cranial Nerve Involvement by Glioma**
+ - Key facts: Brainstem gliomas infiltrate along white matter tracts & may rarely extend directly into CN
+ - Imaging: Intraaxial brainstem mass directly associated with enlarged, enhancing overlying CN; usually signal abnormality within affected CN
+- ### Alternative Differential Approaches
+
+
+ - Segmental anatomic approach
+ - Search pattern for CNIII, CNIV, & CNVI palsy that includes brainstem, cistern, CS, & orbit
+ - Segment approach to oculomotor, trochlear, & abducens neuropathy differential diagnosis
+ - Midbrain (CNIII, CNIV) & pons (CNVI)
+ - [Multiple sclerosis](/document/multiple-sclerosis/abe95a5e-394f-411b-aca6-72ab160a1d0d)
+ - [Cerebral ischemia-infarction, acute](/document/acute-cerebral-ischemiainfarction/7a3ed4a9-ae05-4d64-ae8e-6a30105501e1)
+ - [Metastases, parenchymal, brainstem](/document/parenchymal-metastases/2cf0bd40-4597-4c0b-83e4-74340304b98f)
+ - [Cavernous malformation](/document/cavernous-malformation/cc2415f4-75a6-458d-800e-be31b3cf50c3)
+ - Direct CN involvement by glioma
+ - Cistern
+ - Microvascular infarction (CNIII, CNIV, & CNVI)
+ - [Saccular aneurysm, posterior communicating artery (CNIII)](/document/saccular-aneurysm/47a680d8-1417-440d-ada5-806c9a466f72)
+ - [Pituitary macroadenoma](/document/pituitary-microadenomapituitary-ne-/ff665ceb-8936-4259-84b0-7a568e53890f)
+ - Schwannoma, cisternal (CNIII, CNIV, & CNVI)
+ - CS
+ - [Saccular aneurysm, cavernous ICA](/document/saccular-aneurysm/47a680d8-1417-440d-ada5-806c9a466f72)
+ - [Thrombosis, CS](/document/cavernous-sinus-thrombosis/5aa5ff42-6af9-40a0-b3ef-830b23a1a3cd)
+ - [Meningioma, CS](/document/meningioma-cns-who-grade-1/ba6617a5-b897-418e-9010-e912b61697ed)
+ - [Metastases, intracranial, CS](/document/miscellaneous-intracranial-metasta-/96fa9a10-08a7-4be1-a10c-01196a6a6ba1)
+ - Carotid-cavernous fistula, traumatic
+ - Schwannoma, CS (CNIII, CNIV, & CNVI)
+ - Orbit
+ - [Idiopathic orbital inflammatory disease](/document/idiopathic-orbital-inflammation-ps-/4c2bf0cd-7441-4a40-b590-eeec906aa647)
+ - Pseudotumor, intracranial
+ - Lymphoproliferative lesions, orbit
+ - Metastasis, orbit
+
+## References
+
+## Selected References
+
+1. [Vellingiri S et al: Isolated third cranial nerve palsy in benign idiopathic intracranial hypertension. BMJ Case Rep. 18(1), 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=39753284%5Bpmid%5D)
+1. [Ertilav E et al: Evaluation of patients with painful ophthalmoplegia for benign and secondary etiologies. Neuroophthalmology. 48(5):338-47, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=39145318%5Bpmid%5D)
+1. [Son Y et al: Clinical predictors of causative radiographic findings in adults with acute onset diplopia. Front Neurol. 15:1470805, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=39664751%5Bpmid%5D)
+1. [Fang Y et al: Orbital inflammatory pseudotumor: new advances in diagnosis, pathogenesis, and treatment. Eur J Med Res. 28(1):395, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37794419%5Bpmid%5D)
+1. [Traylor KS et al: Cranial nerve anatomy. Neuroimaging Clin N Am. 32(3):565-76, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35843663%5Bpmid%5D)
+1. [Goyal P et al: Orbital apex disorders: imaging findings and management. Neuroradiol J. 31(2):104-25, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29415610%5Bpmid%5D)
+1. [Patel VA et al: End-organ radiographic manifestations of cranial neuropathies: a concise review. Clin Imaging. 44:5-11, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28364580%5Bpmid%5D)
+1. [Mabray MC et al: Direct cranial nerve involvement by gliomas: case series and review of the literature. AJNR Am J Neuroradiol. 36(7):1349-54, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25857757%5Bpmid%5D)
+1. [Micko AS et al: Invasion of the cavernous sinus space in pituitary adenomas: endoscopic verification and its correlation with an MRI-based classification. J Neurosurg. 122(4):803-11, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25658782%5Bpmid%5D)
+1. [Murchison AP et al: Neuroimaging and acute ocular motor mononeuropathies: a prospective study. Arch Ophthalmol. 129(3):301-5, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21402985%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+**Saccular Aneurysm, Posterior Communicating Artery (CNIII)**
+*Axial T2 MR in a patient with diplopia shows a large right posterior communicating artery aneurysm
with mass effect upon the right CNIII
, which is medially displaced. Note the normal left CNIII
, which has a more lateral cisternal course.*
+
+
+**Saccular Aneurysm, Posterior Communicating Artery (CNIII)**
+*Axial T2 MR in a patient with diplopia shows a large right posterior communicating artery aneurysm
with mass effect upon the right CNIII
, which is medially displaced. Note the normal left CNIII
, which has a more lateral cisternal course.*
+
+
+**Saccular Aneurysm, Posterior Communicating Artery (CNIII)**
+*Axial TOF MRA of a recently ruptured right posterior communicating artery aneurysm
shows persistent flow-related enhancement entering the aneurysm from a supraclinoid internal carotid artery (ICA) feeder
but otherwise low internal signal intensity
from partial thrombosis.*
+
+
+**Multiple Sclerosis**
+*Axial FLAIR MR shows demyelinating plaque
in the right central midbrain and periaqueductal gray corresponding to the region of the oculomotor nuclear complex. Note additional plaques
in the periventricular white matter.*
+
+
+**Multiple Sclerosis**
+*Axial 3D FLAIR MR in a multiple sclerosis patient with new diplopia shows a hyperintense demyelinating lesion
in the right dorsal pons in the region of the abducens nucleus.*
+
+
+**Cerebral Ischemia-Infarction, Acute**
+*Axial DTI trace MR in a patient with diplopia shows an acute lacunar infarction
with reduced diffusivity involving the right ventral midbrain extending to the periaqueductal gray matter, a location that includes the oculomotor and trochlear nuclei.*
+
+
+**Cerebral Ischemia-Infarction, Acute**
+*Axial DWI MR in a patient with diplopia shows an acute lacunar infarction
with reduced diffusivity involving the right dorsal pons at the level of the facial colliculus. An infarction in this location may injure the abducens nucleus and motor fibers of the facial nerve.*
+
+
+**Meningioma, Cavernous Sinus**
+*Axial T1 C+ FS MR shows the typical appearance of a right cavernous sinus (CS) meningioma
, which encases the ICA
and extends into the orbital apex
. Note a small enhancing dural tail
, typical of a meningioma.*
+
+
+**Meningioma, Cavernous Sinus**
+*Coronal T2 FS MR of a right CS meningioma
shows the typical findings of partial ICA encasement
, a CSF/vascular cleft
between tumor and brain, and thin, T2 dark overlying dura
. Note mass effect upon the temporal lobe without vasogenic edema, compatible with an indolent tumor.*
+
+
+**Metastases, Intracranial, Cavernous Sinus**
+*Axial T1 C+ FS MR in a patient with parotid carcinoma and new diplopia shows amorphous hyperenhancement in the expanded right cavernous sinus
extending into the trigeminal cave
.*
+
+
+**Metastases, Intracranial, Cavernous Sinus**
+*Axial DWI trace MR in the same patient shows increased signal filling the expanded right CS
and trigeminal cave
, which was dark on ADC map (not shown), indicating high cellularity tumor. DWI can be a helpful adjunct sequence for mapping extent of tumor and predicting benignity vs. malignancy.*
+
+
+**Schwannoma, Cavernous Sinus (CNIII, IV, VI))**
+*Axial NECT shows a bilobed, smooth remodeling mass in the orbital apex and anterior CS. This schwannoma has a "waist" formed by the enlarged superior orbital fissure
.*
+
+
+**Schwannoma, Cavernous Sinus (CNIII, IV, VI))**
+*Axial T1 C+ MR demonstrates an enhancing schwannoma with components in the orbital apex
and anterior CS
with a "waist" formed at the superior orbital fissure.*
+
+
+**Metastases, Parenchymal, Brainstem**
+*Axial T1 SPGR C+ in a patient with metastatic lung cancer and new diplopia shows an enhancing right midbrain metastasis
in the region of the oculomotor nuclear complex.*
+
+
+**Metastases, Parenchymal, Brainstem**
+*Axial FLAIR C+ FS MR in the same patient shows the right midbrain metastasis
to have bright signal
due to a combination of intrinsic T2 signal hyperintensity and T1 shortening from gadolinium. Note confluent vasogenic edema and obstructive hydrocephalus with periventricular interstitial edema
.*
+
+
+**Thrombosis, Cavernous Sinus**
+*Axial T1 C+ FS MR in an IV drug user with CS thrombosis shows an enlarged right CS with filling defects
. Note additional cortical vein thrombosis
, multiple thrombosed scalp veins
, and extensive inflammation.*
+
+
+**Thrombosis, Cavernous Sinus**
+*Axial DWI MR shows bright thrombus in the cavernous sinuses
, a cortical vein
, the superior ophthalmic vein
, and scalp veins
in an IV drug user with septic thrombophlebitis. Due to a high signal-to-noise ratio, DWI can be a helpful adjunct for identifying thrombus.*
+
+
+**Idiopathic Orbital Inflammation (Pseudotumor)**
+*Axial T1 C+ MR shows an apical subtype of idiopathic orbital inflammatory disease involving the orbital apex
and superior orbital fissure
with resultant optic and oculomotor nerve involvement.*
+
+
+**Intracranial Idiopathic Inflammatory Pseudotumor**
+*Axial T1 C+ MR reveals an intracranial pseudotumor involving the left CS
and tentorium cerebelli
. The cavernous ICA is narrowed
. Distinguishing this lesion from meningioma with imaging may be extremely difficult.*
+
+
+**Carotid-Cavernous Fistula, Traumatic**
+*Axial CTA in a patient with a direct carotid-cavernous fistula 6 months post blunt facial trauma shows a large pseudoaneurysm of the left ICA
has ruptured into the CS, which is engorged
. Note enlarged extraocular muscles
and proptosis.*
+
+
+**Carotid-Cavernous Fistula, Traumatic**
+*Coronal CTA of a traumatic carotid-cavernous fistula shows a large, cavernous ICA pseudoaneurysm
with rupture into CS
through a large vessel wall defect
. Note asymmetric enlargement of pterygoid venous plexus
.*
+
+
+**Saccular Aneurysm, Cavernous ICA**
+*Axial T2 FS MR shows a giant left cavernous ICA aneurysm
. Note the large internal flow void
and complex, lamellar wall signal intensity
in this partially thrombosed aneurysm with turbulent internal flow.*
+
+
+**Lymphoproliferative Lesions, Orbit**
+*Axial T1 C+ FS MR shows infiltrating, avidly enhancing lymphoma of the right orbit involving the extraconal, conal, intraconal, and preseptal areas.*
+
+
+**Metastasis, Orbit**
+*Coronal NECT shows right lateral orbital wall metastasis involving the suprazygomatic masticator space
, orbital apex
, and retromaxillary fat pad
.*
+
+
+**Pituitary Macroadenoma**
+*Coronal T1 C+ FS MR shows pituitary macroadenoma with right CS invasion. The macroadenoma
enhances less than the compressed pituitary parenchyma
, expands the right CS
, and encases the ICA without flow limitation. Carotid encasement has a 100% specificity for diagnosing CS invasion.*
+
+
+**Cavernous Malformation**
+*Axial SWI shows a large cavernous malformation
in a patient with diplopia. Note the characteristic gradient susceptibility, which involves the expected locations of the oculomotor nuclear complexes and trochlear nuclei.*
+
+
+**Schwannoma, Cisternal (CNIII, IV, VI)**
+*Axial T1 C+ FS MR in a patient with neurofibromatosis type 2 and diplopia shows enhancing schwannomas involving the cisternal segments of the bilateral oculomotor nerves
. Note additional tram-track enhancement of the optic nerves
from bilateral optic nerve sheath meningiomas.*
+
+
+### Additional Images
+
+
+**Saccular Aneurysm, Posterior Communicating Artery (CNIII)**
+*Axial CTA reveals a left posterior communicating artery aneurysm
in this patient presenting with a complete CNIII palsy, including absent pupillary reflex.*
+
+
+**Saccular Aneurysm, Posterior Communicating Artery (CNIII)**
+*Sagittal 3D CTA reconstruction shows a large posterior communicating artery aneurysm
with a complex shape. This patient presented with complete CNIII palsy on the side of the aneurysm.*
+
+
+**Multiple Sclerosis**
+*Axial FLAIR MR demonstrates a large, amorphous multiple sclerosis plaque
in the left midbrain. CNIII fibers pass through this lesion on their way ventrally to exit the midbrain in the interpeduncular cistern. The 2nd lesion is seen in the occipital lobe white matter
.*
+
+
+**Multiple Sclerosis**
+*Axial T2WI MR shows a left midbrain demyelinating lesion
in a patient with known multiple sclerosis and new-onset left CNIII palsy. Note 2nd occipital white matter lesion
.*
+
+
+**Cerebral Ischemia-Infarction, Acute**
+*Axial DWI MR shows reduced diffusivity
in the ventromedial midbrain in a patient with acute onset of right CNIII palsy. This midbrain infarction involves the area of CNIII fibers coursing from the dorsal CNIII nucleus to exit the midbrain in the interpeduncular cistern.*
+
+
+**Cerebral Ischemia-Infarction, Acute**
+*Axial T2WI MR demonstrates a subtle area of high signal in the right ventral midbrain
from an acute infarction in a patient with acute onset of right CNIII palsy.*
+
+
+**Meningioma, Cavernous Sinus**
+*Coronal T1 C+ FS MR of a right CS wall meningioma
shows subtotal encasement of the ICA
, which has a maintained flow void indicating patency.*
+
+
+**Meningioma, Cavernous Sinus**
+*Axial T1 C+ MR reveals an enhancing dural-based mass involving the right CS
. Intracranial pseudotumor and CS schwannoma were both considered along with CS meningioma in the differential diagnosis.*
+
+
+**Meningioma, Cavernous Sinus**
+*Coronal T1WI MR shows an expanded right CS due to meningioma involvement
along the lateral wall. The lesion extends to abut the right maxillary division of the trigeminal nerve in the foramen rotundum
.*
+
+
+**Metastases, Intracranial, Cavernous Sinus**
+*Axial SPGR C+ in a patient with lung carcinoma and diplopia shows a large, heterogeneously enhancing mass centered in the right CS
, which has invaded the sphenoid sinuses
.*
+
+
+**Metastases, Intracranial, Cavernous Sinus**
+*Coronal SPGR C+ in a patient with leukemic- phase diffuse large B-cell lymphoma and new-onset diplopia shows complete replacement of the CSs by mass-like enhancing leukemic infiltrate
.*
+
+
+**Metastases, Intracranial, Cavernous Sinus**
+*Axial T1 C+ MR demonstrates a large right CS metastasis
in this patient with known breast cancer and acute onset of right CNIII, CNIV, and CNVI palsies. Note perineural spread
along the preganglionic segment of the trigeminal nerve.*
+
+
+**Metastases, Intracranial, Cavernous Sinus**
+*Axial T1 C+ MR shows a large right CS metastatic deposit of breast carcinoma
. Note the tumor spreads laterally along the middle cranial fossa floor
and involves the petrous apex
.*
+
+
+**Metastases, Parenchymal, Brainstem**
+*Axial 3D FLAIR MR in a patient with metastatic melanoma shows a hemorrhagic left anterior midbrain metastasis
in the location of the oculomotor nuclear complex. Note marked edema
, which involves the region of the trochlear nucleus.*
+
+
+**Metastases, Parenchymal, Brainstem**
+*Axial FLAIR C+ MR in a patient with lung carcinoma shows a large metastatic deposit
in the left dorsal pons, involving the location of the abducens nucleus. Note multiple other parenchymal metastases
, which are very conspicuous on postcontrast FLAIR imaging.*
+
+
+**Metastases, Parenchymal, Brainstem**
+*Coronal T1 C+ MR reveals a necrotic right midbrain lung carcinoma metastasis
crossing the plane of the oculomotor nerve fibers as they track ventrally to exit the interpeduncular cistern.*
+
+
+**Metastases, Parenchymal, Brainstem**
+*Axial T2WI MR demonstrates midbrain edema
associated with a lung carcinoma metastasis. Left body weakness accompanied oculomotor palsy in this patient.*
+
+
+**Thrombosis, Cavernous Sinus**
+*Axial T1 C+ FS MR shows sphenoid sinusitis
causing CS thrombosis. The right CS is expanded with low signal intensity nonenhancing thrombus
. Loss of the cavernous ICA flow void
is indicative of spasm and possible occlusion. This patient presented with right CNIII, CNIV, and CNVI palsies.*
+
+
+**Thrombosis, Cavernous Sinus**
+*Coronal T1 C+ FS MR reveals right CS septic thrombosis. Note the narrowed caliber of the right ICA flow void
due to spasm. The CS clot is seen as low signal intensity nonenhancing material around the cavernous carotid artery
. The opacified right sphenoid sinus
was sources of infection in this patient with a life-threatening complication of rhinosinusitis.*
+
+
+**Idiopathic Orbital Inflammation (Pseudotumor)**
+*Coronal T1 C+ MR demonstrates idiopathic orbital inflammatory disease in the orbital apex
. Notice the adjacent enhancement of the meninges just superomedial to the orbital apex disease
. Such proximal intracranial disease is not uncommon.*
+
+
+**Intracranial Idiopathic Inflammatory Pseudotumor**
+*Coronal T1 C+ MR reveals an enhancing left CS mass
encasing and narrowing the intracavernous ICA
. Notice the enhancing intracranial pseudotumor extends inferolaterally to enlarge the foramen ovale
.*
+
+
+**Carotid-Cavernous Fistula, Traumatic**
+*Axial T1WI MR shows large left CS
filled with multiple high-velocity flow voids. This patient had severe head trauma and returned for evaluation of left pulsatile exophthalmos and palsies of CNIII and CNVI.*
+
+
+**Saccular Aneurysm, Cavernous ICA**
+*Axial NECT demonstrates a hyperdense giant thrombosed ICA aneurysm extending from the CS into the right middle cranial fossa
. The aneurysm erodes the tip of the anterior clinoid
.*
+
+
+**Pituitary Macroadenoma**
+*Coronal T1 C+ MR shows a pituitary macroadenoma
that invades the left CS
. Notice the cavernous ICA on the left is completely surrounded by adenoma
.*
+
+
+**Cavernous Malformation**
+*Axial T2WI MR shows a hemorrhagic midbrain cavernous malformation
with low signal associated with blood products. Note the associated edema tracking within the midbrain white matter tracts
.*
+
+
+**Schwannoma, Cisternal (CNIII, IV, VI)**
+*Axial T1 C+ FS MR reveals an abducens nerve schwannoma as an enhancing mass "pointing" inferomedially toward the pontomedullary junction
. The lesion remodels the clivus-petrous apex area
. Intramural cysts are present.*
+
+
+**Schwannoma, Cisternal (CNIII, IV, VI)**
+*Axial NECT demonstrates an ovoid left prepontine cistern abducens schwannoma that remodels the petrous apex
and shows posterior margin rim calcification
.*
+
+
+**Direct Cranial Nerve Involvement by Glioma**
+*Axial SPGR C+ shows a poorly enhancing pontine glioma
with direct cranial nerve involvement. Note asymmetric enhancement of the trigeminal nerves
with mass-like enlargement of the left trigeminal nerve.*
+
+
+**Direct Cranial Nerve Involvement by Glioma**
+*Axial SPGR C+ in a patient with pontine glioma (excluded on this image) demonstrates direct cranial nerve involvement. Note abnormal thickening and enhancement of the bilateral oculomotor nerves
. Direct involvement of the cranial nerves by a brainstem glioma is an extraordinarily rare, but increasingly recognized phenomenon.*
+
diff --git a/docs_md/articles/peripheral-facial-nerve-paralysis_cff540e8-4e2c-4cc7-aa46-8e6ca277bb85.md b/docs_md/articles/peripheral-facial-nerve-paralysis_cff540e8-4e2c-4cc7-aa46-8e6ca277bb85.md
new file mode 100644
index 0000000..451bf07
--- /dev/null
+++ b/docs_md/articles/peripheral-facial-nerve-paralysis_cff540e8-4e2c-4cc7-aa46-8e6ca277bb85.md
@@ -0,0 +1,418 @@
+---
+title: "Peripheral Facial Nerve Paralysis"
+docid: "cff540e8-4e2c-4cc7-aa46-8e6ca277bb85"
+authors:
+ - key: "eef2f839-5706-47b9-89c3-60d8315b2b3a"
+ value: "Nicholas A. Koontz, MD"
+breadcrumbs:
+ -
+ name: "Head and Neck"
+ slug: "head-and-neck"
+ treeNodeId: "5c1f8e17-7acd-48d8-9d55-f9f8c2cad850"
+ -
+ name: "Differential Diagnosis"
+ slug: "differential-diagnosis"
+ treeNodeId: "deb55065-e1d6-4b6f-b3e3-181fafb4e218"
+ -
+ name: "Temporal Bone"
+ slug: "temporal-bone"
+ treeNodeId: "190f7e44-415d-4f68-9b5c-21e874422431"
+ -
+ name: "Clinically Based Differentials"
+ slug: "clinically-based-differentials"
+ treeNodeId: "4ddba7d0-151c-4087-bc01-43393e025ce7"
+ -
+ name: "Peripheral Facial Nerve Paralysis"
+ slug: "peripheral-facial-nerve-paralysis"
+ treeNodeId: null
+category: "Head and Neck"
+documentVersionId: "73046d95-3db4-4747-b432-b889a1d41987"
+imageCount: 49
+lastUpdated: "04/13/26"
+pageDescription: "Peripheral Facial Nerve Paralysis"
+pageKeywords: "Head and Neck, Differential Diagnosis, Temporal Bone, Clinically Based Differentials, Peripheral Facial Nerve Paralysis"
+pageTitle: "Peripheral Facial Nerve Paralysis | STATdx"
+enhancedTitle: "Peripheral Facial Nerve Paralysis"
+type: "DDX"
+references: true
+breadcrumbs:
+ - "Head and Neck"
+ - "Differential Diagnosis"
+ - "Temporal Bone"
+ - "Clinically Based Differentials"
+ - "Peripheral Facial Nerve Paralysis"
+---
+## ESSENTIAL INFORMATION
+
+- ### Key Differential Diagnosis Issues
+
+
+ - Peripheral facial nerve paralysis
+ - Definition: Unilateral facial nerve injury between pontine motor nucleus & proximal extracranial facial nerve trunk after it emerges from stylomastoid foramen
+ - Clinical manifestation of peripheral facial nerve injury: All muscles of facial expression, including forehead muscles, are paralyzed
+ - Motor: Facial expression muscles; stapedius muscles
+ - Parasympathetic: Lacrimal, submandibular, & sublingual glands
+ - Special sensory: Anterior 2/3 tongue taste
+ - Imaging hints in searching for causes
+ - **Typical** Bell palsy: Does not need imaged routinely
+ - **Atypical** or unresolving Bell palsy**always**needs imaged to exclude ominous etiology (e.g., cancer)
+ - Best examined by thin-section enhanced (C+) MR with fat saturation; increasing role of 3D FLAIR C+
+ - Subtle MR abnormalities: Imaged with temporal bone CT to exclude facial nerve venous malformation
+- ### Helpful Clues for Common Diagnoses
+
+
+ - **Idiopathic Facial Nerve Paralysis (Bell Palsy)**
+ - Entire intratemporal facial nerve enhances on MR without mass effect
+ - Enhancing fundal IAC "tuft" along CNVII commonly seen on T1 C+ MR
+ - **Temporal Bone****Fractures**
+ - Tympanic segment of facial nerve most vulnerable
+ - **Metastases****in****CPA-IAC**
+ - Poorly marginated enhancing mass in CPA-IAC ± dural enhancement
+ - History of primary cancer usually known
+ - Caveat: CPA-IAC mass with associated facial nerve paralysis is **not** vestibular schwannoma
+ - **Pars Flaccida Acquired Cholesteatoma**
+ - Temporal bone CT: Focal middle ear opacification filling Prussak space with ossicle + bone erosion ± involving CNVII canal
+ - MR: Nonenhancing mass with reduced diffusivity
+ - Reduced diffusivity better seen on nonecho-planar (e.g., HASTE) DWI than echo-planar DWI
+ - **Acute Cerebral Ischemia-Infarction in Pons**
+ - MR: Reduced diffusivity + increased T2/FLAIR signal
+- ### Helpful Clues for Less Common Diagnoses
+
+
+ - **Facial Nerve Perineural Tumor**
+ - Parotid space malignancy ascends through stylomastoid foramen to mastoid facial nerve
+ - **Facial Nerve Schwannoma in****Temporal Bone**
+ - Fusiform enhancing mass along CNVII canal with expansile bone margins
+ - Geniculate fossa most common location
+ - **Jugular****Paraganglioma**
+ - Bone CT: Permeative destructive bone changes along jugular foramen (JF) margins
+ - T1 C+ MR: Enhancing JF mass with high-velocity flow voids projects superolateral through middle ear floor
+ - DOTATATE avid
+ - Clinical clues: Vascular retrotympanic mass, pulsatile tinnitus
+ - **Meningioma in CPA-IAC**
+ - Bone CT: Underlying bone ± hyperostosis or permeative sclerosis
+ - T1 C+ MR: Lobulated, enhancing CPA mass with dural base ± dural tails
+ - **Congenital Cholesteatoma in Middle Ear**
+ - Otoscopy: White mass behind **intact** tympanic membrane in child
+ - Bone CT: Mass-like middle ear opacification medial to ossicles ± erosions (particularly when large)
+ - MR: Nonenhancing mass with reduced diffusivity
+ - Reduced diffusivity better seen on nonecho-planar (e.g., HASTE) DWI than echo-planar DWI
+ - **Meningioma****in Temporal Bone**
+ - Bone CT: Hyperostosis or permeative sclerosis of tegmen, JF margins
+ - T1 C+ MR: Middle ear enhancing tumor comes from tegmen tympani, JF, or inner ear
+ - **Metastasis in Temporal Bone**
+ - Destructive temporal bone mass in patient with known cancer
+ - **Multiple Sclerosi****s****in Brainstem**
+ - Young adult with predominantly supratentorial white matter disease ± pontine plaques
+ - Pontine plaques may or may not be visible in setting of facial nerve paralysis
+- ### Helpful Clues for Rare Diagnoses
+
+
+ - **Adenoid Cystic Carcinoma****in****Parotid**
+ - Flame-shaped enhancing parotid space invasive mass
+ - Tumor replaces normal fat in stylomastoid foramen
+ - **Mucoepidermoid Carcinoma in****Parotid**
+ - Ovoid invasive, heterogeneous signal parotid space mass
+ - Decreased T2 and ADC signal indicate higher grade malignancy
+ - **Facial Nerve Venous Malformation ("Hemangioma")**
+ - Bone CT: Poorly marginated mass in geniculate fossa with honeycomb matrix (50%)
+ - T1 C+ MR: Lesion enhances avidly
+ - Geniculate fossa most common location
+ - **Cavernous Malformation in Pons**
+ - CT: Hyperdense pontine lesion ± popcorn calcifications
+ - MR: Pontine lesion with complex signal from blood products ± calcifications, nearby DVA
+ - **Ramsay Hunt Syndrome**
+ - Herpes zoster oticus affects facial nerve ± vestibulocochlear nerve ± inner ear
+ - External auditory canal vesicles usually precede facial nerve symptoms
+ - T1 C+ MR: Enhancing facial nerve in IAC & temporal bone
+ - Other MR findings: Enhancement of inner ear structures & vestibulocochlear nerve variable
+ - 3D-FLAIR C+ enhancement of CNVIII, IAC wall, & inner ear more typical of Ramsay Hunt than Bell palsy
+ - **Sarcoidosis****in****CPA-IAC**
+ - MR: Dural-based enhancing "meningioma mimic" that may track along cistern-IAC CNVII
+ - **Langerhans Histiocytosis****in****Temporal Bone**
+ - Expansile punched-out T-bone lytic lesion in child
+ - **Rhabdomyosarcoma in Temporal bone**
+ - Destructive lesion of temporal bone in child
+ - Often centered in middle ear
+ - **F****acial Nerve****Schwannoma in CPA-IAC**
+ - CPA-IAC enhancing "vestibular schwannoma mimic"
+ - Diagnosis confirmed if schwannoma projects into labyrinthine segment CNVII canal, which is often widened
+ - Looks like "labyrinthine tail" projecting off IAC fundus
+- ### Alternative Differential Approaches
+
+
+ - Organize by **anatomic segment of CNVII**
+ - Divide facial nerve into its discrete segments
+ - **Intramedullary**: 3 nuclei (1 motor & 2 sensory) tracts; motor fibers circle CNVI nucleus to create facial colliculus in floor of 4th ventricle
+ - **CPA-IAC cistern**: Root exit zone of lateral brainstem to fundus of IAC
+ - **Intratemporal**: Labyrinthine, tympanic, & mastoid intratemporal CNVII
+ - **Extracranial**: Stylomastoid foramen to functional terminal segment of CNVII
+ - By looking at CNVII injury from this vantage point, a method is created for designing your imaging protocol
+ - Scanning protocol must include pons, CPA-IAC cistern, temporal bone, & parotid space
+ - Segmental anatomic approach to CNVII paralysis DDx
+ - **Intramedullary lesions**
+ - [Cavernous malformation in pons](/document/cavernous-malformation/cc2415f4-75a6-458d-800e-be31b3cf50c3)
+ - [Multiple sclerosis in brainstem](/document/multiple-sclerosis/abe95a5e-394f-411b-aca6-72ab160a1d0d)
+ - [Acute cerebral ischemia-infarction in pons](/document/acute-cerebral-ischemiainfarction/7a3ed4a9-ae05-4d64-ae8e-6a30105501e1)
+ - **CPA-IAC lesions**
+ - [Metastases in CPA-IAC](/document/cpa-iac-metastases/f8ed3e6c-c693-4537-80a6-53890702554b)
+ - [Meningioma in CPA-IAC](/document/cpa-iac-meningioma/6b77ba6f-ceb0-43f7-bc36-ef3cbeddc7ae)
+ - [Sarcoidosis in CPA-IAC](/document/cpa-iac-neurosarcoid/753df677-73a1-4b90-abad-93f78d880686)
+ - [Ramsay Hunt Syndrome (Herpes Zoster Oticus)](/document/ramsay-hunt-syndrome/c0dbd018-4d75-4b06-9b5b-85dba42afe97)
+ - [Facial nerve schwannoma in CPA-IAC](/document/cpa-iac-facial-nerve-schwannoma/9e47ebb0-b89d-48d6-9ac9-54f946ac66a0)
+ - **T-bone lesions**
+ - [Idiopathic Facial Nerve Paralysis (Bell Palsy)](/document/bell-palsy/90f9c6f0-6748-4700-9eed-c1e67a7d1fe6)
+ - [Temporal bone fractures](/document/temporal-bone-fractures/e0013293-52d5-4ad1-a71b-7f4c92a389b5)
+ - [Pars flaccida acquired cholesteatoma](/document/pars-flaccida-cholesteatoma/01af3b33-08eb-465e-8020-1584bba38547)
+ - [Facial nerve schwannoma in temporal bone](/document/temporal-bone-facial-nerve-schwann-/13c97224-d062-45b2-8ef9-df32b97a6f32)
+ - [Facial nerve venous malformation in temporal bone](/document/temporal-bone-facial-nerve-venous--/86356905-9bc4-41af-9965-e0d48658c1f2)
+ - Jugular paraganglioma
+ - [Congenital cholesteatoma in middle ear](/document/congenital-middle-ear-cholesteatoma/64bb557f-6fa3-4650-936a-5efcabbf16d0)
+ - [Meningioma in temporal bone](/document/temporal-bone-meningioma/e50133ce-c32f-4464-b1ef-9e9b6a702278)
+ - Metastasis in temporal bone
+ - [Langerhans histiocytosis in temporal bone](/document/temporal-bone-langerhans-cell-hist-/2fe7ea1c-9935-4954-a6fb-33ebf1668f16)
+ - [Rhabdomyosarcoma in temporal bone](/document/temporal-bone-rhabdomyosarcoma/d6eda8bd-9b4d-4d9b-90dc-ae184ba70efd)
+ - **Parotid lesions**
+ - Facial nerve perineural tumor
+ - [Adenoid cystic carcinoma](/document/parotid-adenoid-cystic-carcinoma/5a484251-6571-4d3a-a3dd-d0581e314bb0)
+ - [Mucoepidermoid carcinoma](/document/parotid-mucoepidermoid-carcinoma/dc717719-95a8-415f-90c8-cff1227f2491)
+
+## References
+
+## Selected References
+
+1. [Chahine R et al: Imaging insights into perineural tumor spread. Radiol Clin North Am. 64(1):107-22, 2026](http://www.ncbi.nlm.nih.gov/pubmed/?term=41233048%5Bpmid%5D)
+1. [Fancello V et al: Acute onset of peripheral facial nerve palsy in children: an overview. Pediatr Rep. 16(4):844-53, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=39449399%5Bpmid%5D)
+1. [Ottaiano AC et al: The facial nerve: anatomy and pathology. Semin Ultrasound CT MR. 44(2):71-80, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37055142%5Bpmid%5D)
+1. [George E et al: Facial nerve palsy: clinical practice and cognitive errors. Am J Med. 133(9):1039-44, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32445717%5Bpmid%5D)
+1. [Kim SJ et al: Acute peripheral facial palsy: recent guidelines and a systematic review of the literature. J Korean Med Sci. 35(30):e245, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32743989%5Bpmid%5D)
+1. [Jindal G et al: Imaging evaluation and treatment of vascular lesions at the skull base. Radiol Clin North Am. 55(1):151-66, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27890183%5Bpmid%5D)
+1. [Kuya J et al: Usefulness of high-resolution 3D multi-sequences for peripheral facial palsy: differentiation between Bell's palsy and Ramsay Hunt syndrome. Otol Neurotol. 38(10):1523-7, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=29135869%5Bpmid%5D)
+1. [Lingam RK et al: A meta-analysis on the diagnostic performance of non-echoplanar diffusion-weighted imaging in detecting middle ear cholesteatoma: 10 years on. Otol Neurotol. 38(4):521-8, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28195998%5Bpmid%5D)
+1. [Chevallier KM et al: Differentiating pediatric rhabdomyosarcoma and Langerhans cell histiocytosis of the temporal bone by imaging appearance. AJNR Am J Neuroradiol. 37(6):1185-9, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26869468%5Bpmid%5D)
+1. [Chung MS et al: The clinical significance of findings obtained on 3D-FLAIR MR imaging in patients with Ramsay-Hunt syndrome. Laryngoscope. 125(4):950-5, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25346250%5Bpmid%5D)
+1. [Gamss C et al: Imaging evaluation of the suprahyoid neck. Radiol Clin North Am. 53(1):133-44, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25476177%5Bpmid%5D)
+1. [Ho ML et al: Anatomy and pathology of the facial nerve. AJR Am J Roentgenol. 204(6):W612-9, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26001250%5Bpmid%5D)
+1. [Singh AK et al: Imaging spectrum of facial nerve lesions. Curr Probl Diagn Radiol. 44(1):60-75, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=24975082%5Bpmid%5D)
+1. [Baugh RF et al: Clinical practice guideline: Bell's Palsy executive summary. Otolaryngol Head Neck Surg. 149(5):656-63, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24190889%5Bpmid%5D)
+1. [McRackan TR et al: Facial nerve outcomes in facial nerve schwannomas. Otol Neurotol. 2012 Jan;33(1):78-82. Erratum in: Otol Neurotol. 33(3):472, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22143290%5Bpmid%5D)
+1. [Magliulo G et al: Facial nerve dehiscence and cholesteatoma. Ann Otol Rhinol Laryngol. 120(4):261-7, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21585157%5Bpmid%5D)
+1. [Benoit MM et al: Facial nerve hemangiomas: vascular tumors or malformations? Otolaryngol Head Neck Surg. 142(1):108-14, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20096233%5Bpmid%5D)
+1. [Nakata S et al: 3D-FLAIR MRI in facial nerve paralysis with and without audio-vestibular disorder. Acta Otolaryngol. 130(5):632-6, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=19916898%5Bpmid%5D)
+1. [Finsterer J: Management of peripheral facial nerve palsy. Eur Arch Otorhinolaryngol. 265(7):743-52, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18368417%5Bpmid%5D)
+1. [Moody MW et al: Incidence of dehiscence of the facial nerve in 416 cases of cholesteatoma. Otol Neurotol. 28(3):400-4, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17159491%5Bpmid%5D)
+1. [Quaranta N et al: Facial paralysis associated with cholesteatoma: a review of 13 cases. Otol Neurotol. 28(3):405-7, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17414046%5Bpmid%5D)
+1. [Critchley EP: Multiple sclerosis initially presenting as facial palsy. Aviat Space Environ Med. 75(11):1001-4, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15559004%5Bpmid%5D)
+1. [Park SU et al: The usefulness of MR imaging of the temporal bone in the evaluation of patients with facial and audiovestibular dysfunction. Korean J Radiol. 3(1):16-23, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=11919474%5Bpmid%5D)
+1. [Martin N et al: Haemangioma of the petrous bone: MRI. Neuroradiology. 34(5):420-2, 1992](http://www.ncbi.nlm.nih.gov/pubmed/?term=1407526%5Bpmid%5D)
+1. [Curtin HD et al: "Ossifying" hemangiomas of the temporal bone: evaluation with CT. Radiology. 164(3):831-5, 1987](http://www.ncbi.nlm.nih.gov/pubmed/?term=3112865%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+**Idiopathic Facial Nerve Paralysis (Bell Palsy)**
+*Axial T1 C+ FS MR in a patient with Bell palsy shows the classic faint, tuft-like enhancement at the fundus of the internal auditory canal (IAC)
, which extends as a linear enhancement along the labyrinthine segment
, anterior genu
, and anterior tympanic
segments of the facial nerve.*
+
+
+**Idiopathic Facial Nerve Paralysis (Bell Palsy)**
+*Axial T1 C+ FS MR in a patient with Bell palsy shows the classic faint, tuft-like enhancement at the fundus of the internal auditory canal (IAC)
, which extends as a linear enhancement along the labyrinthine segment
, anterior genu
, and anterior tympanic
segments of the facial nerve.*
+
+
+**Idiopathic Facial Nerve Paralysis (Bell Palsy)**
+*Coronal T1 C+ FS MR shows a classic "tuft" of enhancement at the superior fundus of the IAC
as well as along the tympanic segment
of the facial nerve in a patient with Bell palsy.*
+
+
+**Temporal Bone Fractures**
+*Axial NECT shows a complex temporal bone fracture with a longitudinal component
traversing the anterior genu
of the facial nerve canal, resulting in facial nerve injury. Note a large, distracted fragment of the anterior petrous ridge
and temporal squamosal fracture component
.*
+
+
+**Temporal Bone Fractures**
+*Axial NECT shows a complex temporal bone fracture with a transverse component
violating the anterior genu
and anterior tympanic
segments of CNVII canal.*
+
+
+**Metastases in CPA-IAC**
+*Axial T1 C+ MR in a patient with breast cancer and progressive facial weakness shows enhancement along CNVII and CNVIII within the right IAC
due to leptomeningeal metastatic disease. Additional enhancing focus is present at the left IAC fundus
.*
+
+
+**Metastases in CPA-IAC**
+*Axial FLAIR C+ MR in a melanoma patient with progressive facial weakness, vestibular symptoms, and hearing loss shows avid enhancement in IAC
and cochlea
bilaterally as well as left trigeminal cave
due to florid leptomeningeal metastatic disease.*
+
+
+**Pars Flaccida Acquired Cholesteatoma**
+*Axial NECT in a patient with cholesteatoma post mastoidectomy and new facial weakness shows a large soft tissue mass
within the mastoidectomy bowl corresponding to recurrent cholesteatoma, which has eroded into the descending mastoid segment
of CNVII canal.*
+
+
+**Pars Flaccida Acquired Cholesteatoma**
+*Coronal nonecho-planar (HASTE) DWI in a patient with pars flaccida cholesteatoma shows a markedly hyperintense mass
in the right middle ear. Cholesteatomas characteristically show reduced diffusivity on DWI.*
+
+
+**Acute Cerebral Ischemia-Infarction in Pons**
+*Axial T2 MR demonstrates a subacute lateral pontine infarction
on the right. This patient presented with acute onset of right facial nerve paralysis and facial numbness.*
+
+
+**Facial Nerve Perineural Tumor**
+*Axial T1 C+ FS MR demonstrates adenoid cystic carcinoma spreading along a branch of the extracranial facial nerve
. In cases where antegrade perineural tumor spreads from the parotid distally along CNVII, peripheral facial nerve paralysis may be only partial.*
+
+
+**Facial Nerve Schwannoma in Temporal Bone**
+*Axial NECT in a patient with CN7VII schwannoma shows a soft tissue middle ear mass
extending eccentrically from tympanic segment
of CNVII. Widening of the CNVII canal at the posterior genu
was a helpful feature in recognizing the facial nerve origin of this lesion.*
+
+
+**Jugular Paraganglioma**
+*Axial NECT shows a permeative-destructive lesion of the left jugular foramen
, typical of a paraganglioma. Facial weakness was due to invasion of the descending mastoid segment of CNVII
. Note normal appearance of right jugular foramen
.*
+
+
+**Meningioma in CPA-IAC**
+*Axial T1 C+ FS MR shows an avidly enhancing extraaxial mass
centered in the CPA but extending into the IAC
. Note the prominent dural tails
of enhancement, characteristic of meningioma.*
+
+
+**Congenital Cholesteatoma in Middle Ear**
+*Coronal bone CT shows a large congenital cholesteatoma of the middle ear that has eroded the lateral bony wall of the anterior tympanic segment of the facial nerve canal
as well as the ossicles. This child presented with a middle ear mass behind the intact tympanic membrane and CNVII paresis.*
+
+
+**Meningioma in Temporal Bone**
+*Axial T1 C+ FS MR shows an enhancing extraaxial mass
with the dural tail sign
, intraosseous extension, and hyperostosis
, consistent with aggressive meningioma that obliterates the CPA. Note schwannomas of left CNV
and right IAC
in this neurofibromatosis type 2 patient.*
+
+
+**Metastasis in Temporal Bone**
+*Axial T1 C+ MR shows a large left temporal bone mass
obliterating the middle ear, including the expected course of CNVII
. Note a similar-appearing but smaller mass in the right occipital bone
. Multiplicity is a helpful clue to metastatic disease, in this case, from neuroblastoma.*
+
+
+**Multiple Sclerosis in Brainstem**
+*Axial FLAIR MR demonstrates a large pontine multiple sclerosis plaque
in this patient with florid supratentorial white matter disease (not shown).*
+
+
+**Adenoid Cystic Carcinoma in Parotid**
+*Axial T1 MR in a patient with adenoid cystic carcinoma of the parotid shows an irregular-shaped, infiltrative mass replacing the deep lobe of the parotid
and extending through the stylomandibular tunnel into the superficial parotid
, obliterating the plane of the facial nerve.*
+
+
+**Mucoepidermoid Carcinoma in Parotid**
+*Axial T2 FS MR shows an infiltrating, ill-defined mass spanning deep & superficial lobes of parotid gland, obliterating plane of CN7. Note T2-bright mucous cystic regions
as well as areas of dark T2 signal intensity
from high cellularity portions of the tumor.*
+
+
+**Facial Nerve Venous Malformation ("Hemangioma")**
+*Axial NECT in a patient with CNVII venous malformation shows a permeative expansile lesion centered at geniculate fossa
that widens labyrinthine segment
of CNVII canal. Note the characteristic honeycomb matrix within the lesion.*
+
+
+**Cavernous Malformation in Pons**
+*Coronal T2* GRE MR reveals a blooming cavernous malformation in the left pons
. T1 images (not shown) showed hyperintense foci of methemoglobin, consistent with prior hemorrhage.*
+
+
+**Ramsay Hunt Syndrome (Herpes Zoster Oticus)**
+*Coronal T1 C+ FS MR in Ramsay Hunt syndrome shows enhancement of CNVII and CNVIII in the IAC
. Note enhancement of the external auditory canal
and external ear
, which corresponds with areas involved by vesicular rash on clinical exam.*
+
+
+**Sarcoidosis in CPA-IAC**
+*Axial T1 C+ FS MR reveals sarcoid affecting the CNVII and CNVIII in the IAC
. Notice that the tympanic segment of the facial nerve is also avidly enhancing
.*
+
+
+**Langerhans Histiocytosis in Temporal Bone**
+*Axial T1 C+ FS MR in a child with Langerhans histiocytosis shows a destructive middle ear/mastoid mass
with ossicular encasement
and encroachment of the CNVII tympanic segment
. Note aggressive periosteal reaction
not commonly seen with Langerhans calvarial lesions.*
+
+
+**Rhabdomyosarcoma in Temporal Bone**
+*Coronal T1 C+ FS MR shows transspatial mass of middle ear
, external auditory canal
, and infratemporal fossa
. Note obliteration of the tympanic segment of CNVII
. Histology revealed rhabdomyosarcoma.*
+
+
+**Facial Nerve Schwannoma in CPA-IAC**
+*Axial T2 MR shows a CPA mass
extending along posterior IAC
into CNVII labyrinthine segment
. While CNVII lives anteriorly in IAC, close attention shows a near-CSF signal-associated arachnoid cyst
displacing CNVII schwannoma posteriorly. Note CSF
is slightly brighter.*
+
+
+### Additional Images
+
+
+**Idiopathic Facial Nerve Paralysis (Bell Palsy)**
+*Axial T1 C+ FS MR shows a tuft sign
in this patient with acute-onset left peripheral CNVII paralysis. The enhancing fundal portion of the IAC CNVII often has this diffuse, less linear appearance.*
+
+
+**Idiopathic Facial Nerve Paralysis (Bell Palsy)**
+*Axial T1 C+ FS MR demonstrates enhancement of the labyrinthine
and tympanic
segments of the intratemporal facial nerve. Mastoid segment enhancement was also present (not shown).*
+
+
+**Idiopathic Facial Nerve Paralysis (Bell Palsy)**
+*Coronal T1 C+ FS MR demonstrates avid enhancement of the mastoid segment of the facial nerve
and the proximal extracranial segment in the stylomastoid foramen
in this patient with typical acute-onset Bell palsy.*
+
+
+**Temporal Bone Fractures**
+*Axial bone CT shows healing bone fragments in the lateral geniculate fossa
. This patient suffered a bone fracture with persistent facial nerve paralysis 6 weeks before this CT.*
+
+
+**Temporal Bone Fractures**
+*Sagittal bone CT demonstrates disrupted ossicles in the attic
and healing bone in the lateral roof of the geniculate fossa
. Six weeks after temporal bone fracture, persistent conductive hearing loss and facial nerve paralysis were still present.*
+
+
+**Metastases in CPA-IAC**
+*Axial T1 C+ MR shows breast carcinoma metastases in both IACs
. This type of linear enhancement is seen when the metastasis is in the pia-arachnoid of CNVII and CNVIII.*
+
+
+**Metastases in CPA-IAC**
+*Axial T2WI MR reveals bilateral IAC breast carcinoma metastases
thickening the facial and vestibulocochlear nerve bundles. Pia-arachnoid metastasis has a floating in CSF appearance.*
+
+
+**Pars Flaccida Acquired Cholesteatoma**
+*Axial bone CT demonstrates an epitympanic cholesteatoma that has eroded the anterior superior wall of the attic, shaved off the anterior head of the malleus
, and dehisced the lateral bony wall of the anterior tympanic segment of the facial nerve canal
.*
+
+
+**Pars Flaccida Acquired Cholesteatoma**
+*Sagittal bone CT shows an anterior middle ear cholesteatoma that has both eroded the tegmen tympani
and dehisced the lateral bony wall of the geniculate fossa
.*
+
+
+**Facial Nerve Schwannoma in Temporal Bone**
+*Axial T1 C+ FS MR in a patient with facial nerve schwannoma shows an avidly enhancing middle ear mass
growing along the expected course of the tympanic segment
of the facial nerve.*
+
+
+**Facial Nerve Schwannoma in Temporal Bone**
+*Coronal T1 C+ FS MR in a patient with facial nerve schwannoma shows an avidly enhancing middle ear mass
growing below the level of the lateral semicircular canal, which is the typical course of the tympanic segment of the facial nerve
. Note the eccentric growth pattern relative to the nerve of origin, typical of schwannomas.*
+
+
+**Facial Nerve Schwannoma in Temporal Bone**
+*Axial bone CT shows smooth enlargement of the geniculate fossa
by a facial nerve schwannoma. T1-enhanced MR (not shown) revealed enhancing tissue within the enlarged geniculate fossa.*
+
+
+**Jugular Paraganglioma**
+*Coronal T1 C+ FS MR shows a jugular paraganglioma filling the jugular foramen
and spreading superolaterally through the middle ear floor to involve the tympanic segment of CNVII
.*
+
+
+**Meningioma in CPA-IAC**
+*Axial T2 FS MR reveals a lobulated dural-based meningioma with a CSF vascular cleft
and asymmetric relationship to the porus acusticus
.*
+
+
+**Meningioma in Temporal Bone**
+*Axial bone CT reveals the permeative-sclerotic bone changes of an anterior tegmen tympani meningioma
. Notice that the lateral wall of the anterior tympanic segment of the facial nerve canal is affected by the meningioma
.*
+
+
+**Metastasis in Temporal Bone**
+*Axial bone CT shows a large floor of middle cranial fossa destructive metastasis eroding the anterior wall of the middle ear cavity
and invading the geniculate fossa
. This patient with known colon cancer presented with acute onset of facial nerve paralysis.*
+
+
+**Adenoid Cystic Carcinoma in Parotid**
+*Axial CECT shows a poorly marginated adenoid cystic carcinoma
of the parotid gland with deep invasion toward the stylomastoid foramen and proximal extracranial facial nerve
.*
+
+
+**Mucoepidermoid Carcinoma in Parotid**
+*Axial T1 C+ FS MR shows an aggressive-appearing enhancing left parotid mucoepidermoid carcinoma that involves both the superficial
and the deep
lobes. Note the spread into the lower portion of the stylomastoid foramen
.*
+
+
+**Facial Nerve Venous Malformation ("Hemangioma")**
+*Axial bone CT reveals a venous malformation enlarging the geniculate fossa
. Note the central tumor matrix calcifications.*
+
+
+**Ramsay Hunt Syndrome (Herpes Zoster Oticus)**
+*Axial T1 C+ MR in Ramsay Hunt syndrome shows enhancement along the IAC
and intratemporal facial nerve
. A compelling clinical history is helpful, as distinguishing from Bell palsy on imaging alone is challenging. 3D FLAIR C+ has shown promise in this regard.*
+
+
+**Langerhans Histiocytosis in Temporal Bone**
+*Axial bone CT demonstrates a lesion of the right temporal bone eroding the bones of the petrous apex
and middle ear
. The lateral wall of the middle ear/mastoid is absent
with periauricular soft tissue mass visible.*
+
+
+**Rhabdomyosarcoma in Temporal Bone**
+*Axial T1 C+ FS MR shows a very large skull base
and temporal bone
rhabdomyosarcoma. The inner ear bony otic capsule appears to be "floating" in the tumor
.*
+
+
+**Facial Nerve Schwannoma in CPA-IAC**
+*Axial T1 C+ MR shows an enhancing tumor of the CPA
and IAC
. This tumor can be correctly identified as a facial nerve schwannoma because of the "labyrinthine tail" of enhancement of the facial nerve
.*
+
diff --git a/docs_md/articles/posttraumatic-brain-swelling_21a74d44-6e0c-40c6-b865-a4267453d629.md b/docs_md/articles/posttraumatic-brain-swelling_21a74d44-6e0c-40c6-b865-a4267453d629.md
new file mode 100644
index 0000000..95e0654
--- /dev/null
+++ b/docs_md/articles/posttraumatic-brain-swelling_21a74d44-6e0c-40c6-b865-a4267453d629.md
@@ -0,0 +1,479 @@
+---
+title: "Posttraumatic Brain Swelling"
+docid: "21a74d44-6e0c-40c6-b865-a4267453d629"
+authors:
+ - key: "b2e6dabb-ee1c-42a4-a332-9f0814c1c607"
+ value: "Surjith Vattoth, MD"
+ - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
+ value: "Anne G. Osborn, MD, FACR"
+breadcrumbs:
+ -
+ name: "Brain"
+ slug: "brain"
+ treeNodeId: "0361042c-9c90-48e4-864e-a2e6428ee9c9"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "948c6f1e-8e18-45f3-bd9d-7a04ab0b8708"
+ -
+ name: "Pathology-Based Diagnoses"
+ slug: "pathology-based-diagnoses"
+ treeNodeId: "d60fe914-7897-46a0-9cd2-f88ffb322f16"
+ -
+ name: "Trauma"
+ slug: "trauma"
+ treeNodeId: "5ba86a0e-d2d2-4c84-b223-f6f31a3b90e9"
+ -
+ name: "Secondary Effects of CNS Trauma"
+ slug: "secondary-effects-of-cns-trauma"
+ treeNodeId: "a8eb9c3c-8751-4d76-bb9a-702ca66f895d"
+ -
+ name: "Posttraumatic Brain Swelling"
+ slug: "posttraumatic-brain-swelling"
+ treeNodeId: null
+category: "Brain"
+documentVersionId: "f91cb770-50a0-48d1-8b72-ce114ae1c912"
+imageCount: 21
+lastUpdated: "08/14/25"
+pageDescription: "Posttraumatic Brain Swelling"
+pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Trauma, Secondary Effects of CNS Trauma, Posttraumatic Brain Swelling"
+pageTitle: "Posttraumatic Brain Swelling | STATdx"
+enhancedTitle: "Posttraumatic Brain Swelling"
+type: "DX"
+references: true
+breadcrumbs:
+ - "Brain"
+ - "Diagnosis"
+ - "Pathology-Based Diagnoses"
+ - "Trauma"
+ - "Secondary Effects of CNS Trauma"
+ - "Posttraumatic Brain Swelling"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Vasogenic edema (VE), cytotoxic edema (CTE), cerebral edema (CE), diffuse brain swelling (DBS)
+ - 2 forms of brain edema: VE and CTE (often coexist)
+ - VE: Extracellular edema, follows blood-brain barrier (BBB) breakdown
+ - CTE: Intracellular (closed barrier) edema
+ - **Juvenile head trauma syndrome**: Brain swelling after single trivial head injury
+ - Functional channelopathy/ion channel subunits disturbances
+- ### Imaging
+
+
+ - Compressed ventricles, effaced sulci
+ - Vasogenic more prominent in white matter (WM), cytotoxic more prominent in gray matter (GM)
+ - Secondary effects of CE
+ - Brain herniation(s)
+ - Vascular compression → infarction
+ - DWI together with ADC differentiates VE from CTE
+ - CTE: Cellular swelling (↑ signal on DWI, ↓ ADC)
+ - VE: ↑ extracellular brain water (↑ ADC)
+ - Brain edema accompanied by ↑ ICP, ↑ pulsatility index, ↓ blood flow velocity within 24 hours → poor prognosis
+ - Disturbed cerebral autoregulation during first 48 hours correlates with poor outcome
+- ### Top Differential Diagnoses
+
+
+ - Anoxic encephalopathy
+ - Metabolic encephalopathy
+ - Pressure-related edema
+- ### Pathology
+
+
+ - Dynamic, multifactorial, including genetic, excitotoxicity, contact-kinin system, and plasminogen activator inhibitor-2
+- ### Clinical Issues
+
+
+ - Goal = maintain cerebral perfusion pressure (CPP) without inducing hydrostatic VE
+ - DBS more common in children than adults
+- ### Diagnostic Checklist
+
+
+ - Suspect severe brain swelling if mass effect ≥ 3 mm more than maximum width of extraaxial hemorrhage
+
+## TERMINOLOGY
+
+- ### Synonyms
+
+
+ - Vasogenic edema (VE), cytotoxic edema (CTE), cerebral edema (CE), diffuse brain swelling (DBS)
+- ### Definitions
+
+
+ - Brain, CSF, and blood coexist in closed intracranial compartment
+ - To maintain normal intracranial pressure (ICP), ↑ pressure in one compartment must be balanced by ↓ in others (Monro-Kellie doctrine)
+ - Dynamic, multifactorial, including genetic, excitotoxicity, contact-kinin system, and PAI-2
+ - 2 basic forms of brain edema in trauma: VE and CTE
+ - VE: Extracellular edema, follows blood-brain barrier (BBB) breakdown; initially
+ - CTE: Intracellular (closed barrier) edema; later
+ - **Juvenile head trauma syndrome**: Diffuse brain or hemispheric swelling after single trivial injury
+ - Due to rapidly progressive reactive hyperemia
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Compressed ventricles, effaced sulci; usually minimal subfalcine herniation only
+ - Suspect and treat early; potentially catastrophic brain swelling if disproportionate mass effect
+ - ≥ 3 mm more than maximum width of extraaxial hemorrhage or collection
+ - #### Location
+
+
+ - VE earlier, more prominent in white matter (WM); CTE later, more prominent in gray matter (GM); often coexist
+ - #### Morphology
+
+
+ - Compressed ventricles, effaced sulci
+ - Secondary effects of CE
+ - Brain herniation(s); vascular compression → infarction
+- ### Radiographic Findings
+
+
+ - #### Radiography
+
+
+ - ± fractures, split sutures
+- ### CT Findings
+
+
+ - #### NECT
+
+
+ - Compressed ventricles, effaced sulci
+ - Low-attenuation brain parenchyma: WM > GM
+ - Subcortical WM less resistant to fluid accumulation than GM; initially GM-WM differentiation maintained
+ - Later loss of GM-WM interface
+ - VE more prominent in WM
+ - CTE more prominent in GM
+ - ↓ supratentorial density with preservation of infratentorial density → **white cerebellum** sign or **cerebellar reversal** sign
+ - Multifocal hemorrhages often present
+ - #### CECT
+
+
+ - Usually no enhancement unless BBB disrupted
+ - Xenon CT
+ - Edema major contributor to brain swelling
+ - Cerebral blood volume actually ↓ in proportion to cerebral blood flow
+- ### MR Findings
+
+
+ - #### T1WI
+
+
+ - Hypointense edema
+ - #### T2WI
+
+
+ - Hyperintense edema
+ - #### FLAIR
+
+
+ - Hyperintense edema
+ - Less useful in newborn due to normally ↑ water content of neonatal brain
+ - #### T2* GRE
+
+
+ - ± blood products
+ - #### DWI
+
+
+ - DWI together with ADC differentiates VE from CTE
+ - CTE: Cellular swelling (↓ ADC)
+ - VE: ↑ extracellular brain water (↑ ADC)
+ - Diffusion tensor imaging (DTI): ↓ diffusion anisotropy early, when MR/DWI still normal
+ - DTI identifies traumatic penumbra, potentially salvageable brain
+ - #### T1WI C+
+
+
+ - Patchy enhancement if BBB breakdown
+ - #### MRA
+
+
+ - ± ↓ flow (thinned arteries)
+ - Vascular obstruction (compression or dissection during herniation) → posttraumatic infarction
+ - #### MRV
+
+
+ - Sinus compression with severe edema
+ - #### MRS
+
+
+ - ↓ NAA, elevated Cho (membrane breakdown), presence of lactate predicts poor prognosis
+ - Perfusion: ↓ brain perfusion with progressive ↑ ICP
+- ### Ultrasonographic Findings
+
+
+ - #### Pulsed Doppler
+
+
+ - Brain edema accompanied by ↑ ICP, ↑ pulsatility index, ↓ blood flow velocity within 24 hours → poor prognosis
+ - Moving correlation index between mean arterial BP and ICP = PRx (measures cerebral vasomotor reactivity)
+ - PRx < 0.3 = intact reactivity; PRx > 0.3 = impaired reactivity
+ - Disturbed cerebral autoregulation during first 48 hours correlates with poor outcome
+ - #### Color Doppler
+
+
+ - Hyperemia precedes brain edema in some patients
+- ### Angiographic Findings
+
+
+ - Conventional
+ - Slow arteriovenous transit if ↑ ICP
+- ### Nuclear Medicine Findings
+
+
+ - #### PET
+
+
+ - PET/SPECT: ↓ rCBV, hypometabolism (dependent upon timing)
+- ### Imaging Recommendations
+
+
+ - NECT performed due to accessibility in critically ill trauma patients
+ - DWI with ADC maps (or DTI) important to differentiate VE, CTE
+ - Multiplanar MR allows characterization of acquired cerebral herniations
+ - Subfalcine (cingulate), tonsillar, uncal, transtentorial, transalar, transdural/transcranial
+
+## DIFFERENTIAL DIAGNOSIS
+
+- [Anoxic Encephalopathy](/document/carbon-monoxide-poisoning/827ae14b-3d0f-4c3a-937a-e450a7eec716)
+ - Hypoxic-ischemic encephalopathy, drowning, cardiopulmonary arrest
+- ### Pressure-Related Edema
+
+
+ - [Posterior reversible encephalopathy syndrome](/document/posterior-reversible-encephalopath-/84176f2c-fc9d-4497-8af9-1430b9f0187c)
+ - Hypertensive encephalopathy, cyclosporine/FK506 encephalopathy, L-asparaginase, eclampsia
+ - Predominantly VE in parietooccipital subcortical WM
+ - Venous obstruction with ↑ venous pressure
+- ### Metabolic Encephalopathy
+
+
+ - [Uremia, mitochondrial disorders](/document/urea-cycle-disorders/dcbc692c-e682-4992-94b6-524c6fe5a1b5)
+- [Meningitis/Encephalitis](/document/meningitis/a08411a0-492d-4fa1-a504-dd97cce22a0e)
+ - Diffuse sulcal effacement, leptomeningeal ± parenchymal enhancement
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Etiology
+
+
+ - Posttraumatic inflammation and brain edema mediated by **contact-kinin system**
+ - Pathway activation triggered by **activated factor XII**
+ - Genetic deficiency or pharmacologic blocking of activated factor XII in mice diminishes brain injury-induced **bradykinin** release by contact-kinin system
+ - Reduce brain lesion size, BBB breakdown, brain edema and inflammation
+ - **Kallikrein-kinin system**inflammatory pathway activate and ↑ brain edema formation in acute phase via activation of **bradykinin**2 receptors (B2R)
+ - Inhibition of B2R can reduce long-term neurocognitive deficits after traumatic brain injury
+ - **PAI-2** contributes to posttraumatic brain edema
+ - Genetic deficiency of PAI-2 in mice diminishes posttraumatic brain inflammation and edema
+ - Trigeminovascular system may mediate brain swelling associated with SDH
+ - VE
+ - ↑ BBB permeability
+ - Endothelial tight junctions disrupted → leakage of proteins/Na⁺⁺/water → fluid shift into extracellular spaces
+ - Primarily WM, myelin (major association bundles, relative sparing of commissural/projection fibers)
+ - CTE
+ - Intracellular (closed barrier) edema
+ - Energy failure → loss of Na⁺⁺/K homeostasis
+ - Intracellular water uptake causes cell swelling, compression of extracellular space
+ - Other brain water disturbances
+ - Hydrocephalic (interstitial)
+ - ↑ intraventricular volume/pressure drives CSF through ependymal lining
+ - Hydrostatic (congestive)
+ - ↑ intravascular pressure → cerebrovascular resistance ↑ → flooding capillary beds
+ - Hypoosmotic
+ - Flooding with IV fluids; inappropriate secretion of antidiuretic hormone; multiple, bilateral lesions in 90%
+ - **Juvenile head trauma syndrome**: Functional **channelopathy/ion channel**subunits disturbances
+ - Mutation in calcium channel subunit gene (*CACNA1A*) associated with familial hemiplegic migraine in many
+ - General pathology comments
+ - ↑ brain water, astroglial swelling (not reactive astrogliosis)
+ - CTE: GM predominance, intracellular, ↓ ADC
+ - VE: WM, extracellular, ↑ ADC
+ - DBS more common in children
+ - ↑ oxidative stress endangers BBB
+ - Sustained posttraumatic cerebral hypoperfusion
+ - Childhood transient ↑ NMDA receptor expression may allow ↑ intracellular Na⁺⁺ accumulation in brain cells
+ - #### Genetics
+
+
+ - Differential expression of genes controlling destructive and neuroprotective cascades determine cellular response to injury
+ - Genes govern inflammation, transcription regulation, inflammation, cell adhesion, extracellular matrix
+- ### Gross Pathologic & Surgical Features
+
+
+ - ↑ brain water, obliteration of cisterns/ventricles/sulci
+- ### Microscopic Features
+
+
+ - Extracellular fluid of cortex neuropil → pre-/postsynaptic swelling and shrinkage, synaptic disassembly
+ - Effects of hypoxia and cell death
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - Altered consciousness, coma
+ - Severe brain swelling usually takes 24-48 hours to develop; occasionally rapidly after trauma
+ - #### Clinical profile
+
+
+ - < 2 years old: Inflicted injury in 80% of cases
+ - Teens and adults: Motor vehicle crash, assaults
+ - Car crashes, especially automobiles without seat belts, motorcyclists/bicyclists without helmets
+ - > 65 years old: Accidental falls
+- ### Demographics
+
+
+ - #### Age
+
+
+ - Children, young adults, and patients with repetitive concussions or subconcussive injuries most prone
+ - 2x more likely than older adults
+ - #### Sex
+
+
+ - M:F = 1.6:2.1
+ - #### Ethnicity
+
+
+ - Black, Native American patients overrepresented
+ - #### Epidemiology
+
+
+ - 1.5 million traumatic brain injuries per year (USA)
+ - Highest incidence in children < 5 years old
+ - Focal, regional, or DBS in 10-20% of traumatic brain injuries
+ - Acute subdural hemorrhage patients with young age, low platelet:WBC ratio, and traumatic SAH: ↑ risk of developing posttraumatic acute brain swelling
+- ### Natural History & Prognosis
+
+
+ - Slowly expanding lesions can be accommodated without elevated ICP
+ - Rapid expansion (trauma, rapid tumor growth, abscess) → rapid rise of ICP
+ - Followed by cascade of sequelae (e.g., excitotoxin release) → cell death
+ - Mortality approaches 50%
+ - Posttraumatic edema generally resolves within 2 weeks, atrophy (due to cellular death) ensues
+- ### Treatment
+
+
+ - Goal = maintain cerebral perfusion pressure (CPP) without inducing hydrostatic VE
+ - ↑ CPP in selected patients with intact cerebral vasomotor reactivity
+ - Decompressive surgery
+ - Osmotherapy, neuroprotective agents, steroids, all controversial
+ - Pharmacologic blocking of activated factor XII, and PAI-2 may be used to diminish posttraumatic brain swelling based on mouse model studies
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - Suspect severe brain swelling if mass effect ≥ 3 mm more than maximum width of extraaxial hemorrhage
+- ### Image Interpretation Pearls
+
+
+ - Image timing crucial: VE (1st hours) replaced by CTE
+
+ 6c9bb638-1030-423d-ac14-98e98e2bbbc5
+
+## References
+
+## Selected References
+
+1. [Fu S et al: Incidence, risk factors, and clinical outcomes of acute brain swelling associated with traumatic acute subdural hematoma: a retrospective study utilizing novel diagnostic criteria. Ther Adv Neurol Disord. 17:17562864241242944, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38638672%5Bpmid%5D)
+1. [Wehn AC et al: Bradykinin 2 receptors mediate long-term neurocognitive deficits after experimental traumatic brain injury. J Neurotrauma. 41(21-22):2442-54, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38818807%5Bpmid%5D)
+1. [Griemert EV et al: Deficiency of plasminogen activator inhibitor type 2 limits brain edema formation after traumatic brain injury. J Neurotrauma.36(14):2272-8, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30755137%5Bpmid%5D)
+1. [Hopp S et al: Alleviation of secondary brain injury, posttraumatic inflammation, and brain edema formation by inhibition of factor XIIa. J Neuroinflammation. 14(1):39, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28219400%5Bpmid%5D)
+1. [Mckee AC et al: The neuropathology of traumatic brain injury. Handb Clin Neurol. 127:45-66, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25702209%5Bpmid%5D)
+1. [Alves JL: Blood-brain barrier and traumatic brain injury. J Neurosci Res. 92(2):141-7, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24327344%5Bpmid%5D)
+1. [Lu H et al: The apparent diffusion coefficient does not reflect cytotoxic edema on the uninjured side after traumatic brain injury. Neural Regen Res. 9(9):973-7, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25206920%5Bpmid%5D)
+1. [Paiva WS et al: Delayed unilateral traumatic brain swelling in a child. J Pediatr Neurosci. 9(2):169-71, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25250079%5Bpmid%5D)
+1. [Wu H et al: The diagnosis and surgical treatment of central brain herniations caused by traumatic bifrontal contusions. J Craniofac Surg. 25(6):2105-8, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25304144%5Bpmid%5D)
+1. [Bor-Seng-Shu E et al: Posttraumatic refractory intracranial hypertension and brain herniation syndrome: cerebral hemodynamic assessment before decompressive craniectomy. Biomed Res Int. 2013:750809, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24377095%5Bpmid%5D)
+1. [Ren W et al: Occludin and connexin 43 expression contribute to the pathogenesis of traumatic brain edema. Neural Regen Res. 8(29):2703-12, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=25206581%5Bpmid%5D)
+1. [Squier W et al: The pathophysiology of brain swelling associated with subdural hemorrhage: the role of the trigeminovascular system. Childs Nerv Syst. 28(12):2005-15, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22885686%5Bpmid%5D)
+1. [Greve MW et al: Pathophysiology of traumatic brain injury. Mt Sinai J Med. 76(2):97-104, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19306379%5Bpmid%5D)
+1. [Tollard E et al: Experience of diffusion tensor imaging and 1H spectroscopy for outcome prediction in severe traumatic brain injury: Preliminary results. Crit Care Med. 37(4):1448-55, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19242330%5Bpmid%5D)
+1. [Galloway NR et al: Diffusion-weighted imaging improves outcome prediction in pediatric traumatic brain injury. J Neurotrauma. 25(10):1153-62, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18842104%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+*Axial NECT in an adult with small hemorrhagic contusions in the left temporal lobe (not shown) shows posttraumatic brain swelling in the left cerebral hemisphere
. Note obliteration of left sulci compared with normal right sulci
.*
+
+
+*Axial T2 MR in a patient with posttraumatic left peritrigonal hemorrhage
, surrounding edema
, and left decompressive craniectomy shows transcranial brain herniation through the craniectomy defect
. Edema in the splenium of the corpus callosum
is also noted.*
+
+
+*Axial DWI MR demonstrates increased signal in the corpus callosum and cerebral white matter (WM)
, which was consistent with cytotoxic edema on ADC showing low signal (not shown). Vasogenic edema
(being bright on ADC) is also noted surrounding the left peritrigonal hematoma
.*
+
+
+*Axial NECT in a patient with posttraumatic diffuse brain swelling shows hypodense cerebrum
with relatively hyperdense unaffected cerebellum
(white cerebellum sign or cerebellar reversal sign).*
+
+
+*Axial NECT in an adult with trauma demonstrates subdural hemorrhage (SDH)
along the right convexity and falx with brain edema. There is mass effect on the right lateral ventricle
and leftward midline shift with subfalcine herniation
. Suspect posttraumatic brain swelling if the mass effect is disproportionately greater than (≥ 3 mm) the maximum width of the extraaxial hemorrhage/collection.*
+
+
+*Axial DWI MR in a toddler post trauma shows left hemispheric edema
in a nonvascular distribution.*
+
+
+*Axial ADC map in the same patient shows extensive left hemispheric decreased ADC
, confirming cytotoxic edema.*
+
+
+*Axial NECT at follow-up shows left hemiatrophy with compensatory enlargement of the ventricles
secondary to atrophy of the cortex
and underlying WM, particularly posteriorly. If the neurologic deterioration is associated with a single injury in a young child, it is referred to as juvenile head trauma syndrome and 2nd-impact syndrome if after a subsequent minor trauma.*
+
+
+*Axial T1 MR in a 2-year-old boy with juvenile head trauma syndrome after a fall from a height shows tiny SDHs along the bilateral posterior fossa convexities
.*
+
+
+*Axial T2 MR in the same patient shows hyperintense disproportionate bilateral cerebellar edema
underlying the thin SDHs, consistent with posttraumatic brain swelling. DWI showed facilitated diffusion suggestive of vasogenic edema; there was patchy contrast enhancement suggestive of blood-brain barrier breakdown (not shown).*
+
+
+### Additional Images
+
+
+*Sagittal T1 MR in a child with brain swelling shows herniation of the cerebellar tonsils
. Note the pyramidal shape of the tonsils, indicative of compression.*
+
+
+*Sagittal T2 MR shows a ventricular catheter
, abnormal signal in impacted cervicomedullary cord
, and edema of cerebellar tonsils
following tonsillar herniation.*
+
+
+*Axial NECT shows severe midline herniation, obstruction of contralateral and effacement of ipsilateral ventricles, subdural hematoma with active bleeding, and diffuse loss of gray matter (GM)-WM junction.*
+
+
+*Sagittal brain scan in the same patient shows absence of cerebral perfusion at follow-up. The study is diagnostic of brain death.*
+
+
+*Axial NECT demonstrates diffusely decreased GM-WM differentiation and hypodensity of the cerebral hemispheres. Effacement of the sulci and basal cisterns, as well as the small ventricles, indicate elevated intracranial pressure.*
+
+
+*Axial NECT demonstrates diffuse hypodensity, markedly decreased GM-WM differentiation, and diffuse sulcal effacement throughout the cerebral hemispheres, findings indicative of cerebral edema and increased intracranial pressure There is also traumatic subarachnoid hemorrhage
.*
+
+
+*Axial CECT demonstrates severe cerebral edema with complete effacement of internal landmarks.*
+
+
+*Axial NECT shows a mixed density right subdural hematoma
with right cerebral edema. There is effacement of the right lateral ventricle and sulci, entrapment of the left lateral ventricle
, and midline shift to the left
with developing edema in the left anterior cerebral artery (ACA) distribution
.*
+
+
+*Axial NECT demonstrates acute left periatrial hemorrhage
with minimal surrounding vasogenic edema. Note diffuse sulcal effacement, midline shift to the right, and early trapping of the right lateral ventricle
.*
+
+
+*Axial NECT in a 3-year-old boy who was not belted into a child car seat and was ejected during a high-impact motor vehicle accident shows that the cerebral hemispheres are diffusely hypodense without GM-WM differentiation. Some subarachnoid and peritentorial subdural blood is present.*
+
+
+*More cephalad scan in the same patient shows complete lack of GM-WM differentiation. The lateral ventricles are compressed and almost inapparent. There is severe posttraumatic brain swelling.*
+
diff --git a/docs_md/articles/primary-arteritis-of-cns_490b3aed-37e2-4ec6-95dd-76efc734490f.md b/docs_md/articles/primary-arteritis-of-cns_490b3aed-37e2-4ec6-95dd-76efc734490f.md
new file mode 100644
index 0000000..bc06638
--- /dev/null
+++ b/docs_md/articles/primary-arteritis-of-cns_490b3aed-37e2-4ec6-95dd-76efc734490f.md
@@ -0,0 +1,572 @@
+---
+title: "Primary Arteritis of CNS"
+docid: "490b3aed-37e2-4ec6-95dd-76efc734490f"
+authors:
+ - key: "8d5254e9-8dda-478b-8f08-bdee97a32c79"
+ value: "Karen L. Salzman, MD, FACR"
+ - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
+ value: "Anne G. Osborn, MD, FACR"
+breadcrumbs:
+ -
+ name: "Brain"
+ slug: "brain"
+ treeNodeId: "0361042c-9c90-48e4-864e-a2e6428ee9c9"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "948c6f1e-8e18-45f3-bd9d-7a04ab0b8708"
+ -
+ name: "Pathology-Based Diagnoses"
+ slug: "pathology-based-diagnoses"
+ treeNodeId: "d60fe914-7897-46a0-9cd2-f88ffb322f16"
+ -
+ name: "Stroke"
+ slug: "stroke"
+ treeNodeId: "7a135176-0a69-4fc9-b200-59569fbf5166"
+ -
+ name: "Nonatheromatous Vasculopathy"
+ slug: "nonatheromatous-vasculopathy"
+ treeNodeId: "2ccf261b-3d7a-42a7-8041-8ed08dd81bff"
+ -
+ name: "Primary Arteritis of CNS"
+ slug: "primary-arteritis-of-cns"
+ treeNodeId: null
+category: "Brain"
+documentVersionId: "72c99f81-89ac-42f6-83a0-79a0088de463"
+imageCount: 39
+lastUpdated: "08/19/25"
+pageDescription: "Primary Arteritis of CNS"
+pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Stroke, Nonatheromatous Vasculopathy, Primary Arteritis of CNS"
+pageTitle: "Primary Arteritis of CNS | STATdx"
+enhancedTitle: "Primary Arteritis of CNS"
+type: "DX"
+references: true
+anatomy:
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+ - "{'authors': 'Jeffrey S. Anderson, MD, PhD', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/e1a20b61-b2c1-44c5-ba04-59843855bfef', 'category': 'Brain', 'compareUrl': '/compare/document/e1a20b61-b2c1-44c5-ba04-59843855bfef/related-anatomy/treeNode?subContext=Limbic Network', 'documentId': 'e1a20b61-b2c1-44c5-ba04-59843855bfef', 'documentType': 'ANATOMY', 'documentUrl': '/document/limbic-network/e1a20b61-b2c1-44c5-ba04-59843855bfef', 'enhancedTitle': 'Limbic Network', 'entryDate': '10/20/20', 'imageCount': 3, 'imageUrl': '/image/thumbnail/17c31997-0c69-473f-ac55-0b912c1cef1a?size=174&quality=85', 'inCompareCart': False, 'rank': 2, 'referenceCount': 11, 'showCompareButton': False, 'title': 'Limbic Network'}"
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+ - "{'authors': 'Anne G. Osborn, MD, FACR; Edward P. Quigley, III, MD, PhD; Adriene C. Eastaway, MD, MS', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/5658d6a5-aa61-4c8a-a6c7-a8d5aa5c41c4', 'category': 'Brain', 'compareUrl': '/compare/document/5658d6a5-aa61-4c8a-a6c7-a8d5aa5c41c4/related-anatomy/treeNode?subContext=Middle Cerebral Artery', 'documentId': '5658d6a5-aa61-4c8a-a6c7-a8d5aa5c41c4', 'documentType': 'ANATOMY', 'documentUrl': '/document/middle-cerebral-artery/5658d6a5-aa61-4c8a-a6c7-a8d5aa5c41c4', 'enhancedTitle': 'Middle Cerebral Artery', 'entryDate': '10/20/20', 'imageCount': 18, 'imageUrl': '/image/thumbnail/7c2f30b1-e387-44db-8578-e9fe14c174f6?size=174&quality=85', 'inCompareCart': False, 'rank': 6, 'referenceCount': 0, 'showCompareButton': False, 'title': 'Middle Cerebral Artery'}"
+cases: 2
+breadcrumbs:
+ - "Brain"
+ - "Diagnosis"
+ - "Pathology-Based Diagnoses"
+ - "Stroke"
+ - "Nonatheromatous Vasculopathy"
+ - "Primary Arteritis of CNS"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Primary arteritis of CNS (PACNS)
+ - No evidence of secondary (systemic) vasculitis
+ - Pathology: Inflammation, necrosis of blood vessel wall
+- ### Imaging
+
+
+ - Imaging can be normal; requires clinical/laboratory correlation
+ - MR
+ - Punctate, linear T2/FLAIR hyperintensities
+ - ± linear or patchy parenchymal enhancement
+ - DWI variable (may restrict)
+ - 3T vessel wall imaging (VWI)
+ - Circumferential, smooth, long-segment enhancement
+ - DSA
+ - Once considered imaging gold standard
+ - "Beaded" arteries (irregular stenoses, dilatations) on DSA
+ - Peripheral branches > circle of Willis
+- ### Top Differential Diagnoses
+
+
+ - Systemic CNS vasculitis (imaging indistinguishable)
+ - Intracranial atherosclerotic disease (atherosclerotic vascular disease)
+ - Reversible cerebral vasoconstriction syndrome (RCVS)
+ - Intravascular large B-cell lymphoma
+ - Cerebral amyloid-β-related angiitis (ABRA)
+- ### Pathology
+
+
+ - Brain biopsy may be required to confirm diagnosis
+ - 75-80% sensitive
+ - Negative biopsy does not exclude PACNS
+- ### Clinical Issues
+
+
+ - Peak: 40-60 years; range = childhood to older adults
+- ### Diagnostic Checklist
+
+
+ - Atherosclerosis is by far most common cause of vasculitis-like DSA pattern in older adults, not PACNS
+
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - Primary arteritis of CNS (PACNS)
+- ### Synonyms
+
+
+ - Vasculitis, vasculopathy
+- ### Definitions
+
+
+ - Descriptive term rather than specific disease
+ - 2 cardinal features required for histopathologic diagnosis of vasculitis
+ - Inflammation and necrosis/destruction of blood vessel wall
+ - Often associated with occlusion, infarction
+ - PACNS: Arteritis confined to intracranial CNS without evidence for systemic vasculitis
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Circumferential, smooth, long-segment wall enhancement
+ - "Beaded" arteries (irregular stenoses, dilatations) on DSA
+ - Nonspecific [intracranial atherosclerotic vascular disease (ASVD) > vasculitis]
+ - Note: Imaging work-up can be normal; requires clinical/laboratory correlation
+ - #### Location
+
+
+ - Intracranial vessels of any size
+ - Brain is primary site, but spinal cord can also be involved
+ - #### Size
+
+
+ - Degree of vessel narrowing may range from normal or minimally stenotic to completely occluded
+ - #### Morphology
+
+
+ - Vasculitic pattern (common)
+ - Areas of smooth or slightly irregularly shaped stenoses alternating with dilated segments
+ - Nonspecific (appearance similar to other vasculitides)
+ - Tumefactive pattern (rare)
+ - Solitary tumor-like enhancing mass with extensive vasogenic edema
+- ### CT Findings
+
+
+ - #### NECT
+
+
+ - Relatively insensitive; often normal
+ - May see subarachnoid hemorrhage (SAH), particularly convexal
+ - May see secondary signs, such as ischemia or infarction
+ - Multifocal low-density areas, especially in basal ganglia, subcortical white matter
+ - #### CECT
+
+
+ - May see patchy areas of enhancement
+- ### MR Findings
+
+
+ - #### T1WI
+
+
+ - Multifocal deep gray and subcortical hypointensities
+ - #### FLAIR
+
+
+ - Multifocal deep gray and subcortical hyperintensities
+ - #### T2* GRE
+
+
+ - ± petechial hemorrhages (SWI more sensitive)
+ - SAH may be present
+ - #### DWI
+
+
+ - Restricted diffusion in acute stages
+ - #### T1WI C+
+
+
+ - May see patchy areas of parenchymal enhancement
+ - May be linear, punctate, or nodular
+ - #### MRA
+
+
+ - Relatively insensitive, most often normal
+ - May see some classic angiographic signs if larger vessels involved
+ - Vessel wall imaging (VWI)
+ - Smooth, concentric, long-segment enhancement
+ - SWI
+ - ± petechial hemorrhages, SAH
+- ### Ultrasonographic Findings
+
+
+ - #### Color Doppler
+
+
+ - Transcranial Doppler may be used to monitor cerebral blood flow velocities and evaluate therapy if large arteries are involved
+- ### Angiographic Findings
+
+
+ - DSA (so-called "vasculitic" changes below are neither specific nor diagnostic)
+ - Typical: Alternating stenosis with dilatation primarily involving 2nd-, 3rd-order branches
+ - Less common: Long-segment stenoses, pseudoaneurysms, occlusions
+ - Consider: Imaging renal arteries to evaluate for systemic vasculitis
+ - N.B.: Normal DSA does not exclude CNS vasculitis
+- ### Nuclear Medicine Findings
+
+
+ - 11C-(R)-PK11195 PET shows ↑ binding
+ - Specific ligand for peripheral benzodiazepine binding site
+ - Particularly abundant on cells of mononuclear phagocyte lineage
+ - May be helpful in patients of suspected vasculitis with normal or ambiguous MR findings
+- ### Imaging Recommendations
+
+
+ - #### Best imaging tool
+
+
+ - VWI with thin-section, high-resolution (3T) T1 C+
+ - DSA
+ - Nonspecific (ASVD much more common than vasculitis)
+ - May differentiate reversible cerebral vasoconstriction syndrome (RCVS) from vasculitis
+ - #### Protocol advice
+
+
+ - CTA/MRA is useful for screening; spatial resolution insufficient for subtle disease
+ - DSA if lab studies positive, MR/MRA negative, and high clinical suspicion
+
+## DIFFERENTIAL DIAGNOSIS
+
+- [Intracranial Atherosclerotic Disease (Atherosclerotic Vascular Disease)](/document/intracranial-atherosclerosis/c5ef0315-edbd-4ace-8930-484a116610b9)
+ - Advanced patient age
+ - Typical distribution (carotid siphon, proximal intracranial vessels)
+ - VWI
+ - Multiple, eccentric, focal with wall remodeling
+ - Plaque T2 hyperintense
+ - Heterogeneously enhancing
+ - Short > segment
+- [Systemic CNS Vasculitis](/document/miscellaneous-vasculitis/221f737a-6bfa-4331-b296-402e40973f7e)
+ - DSA, VWI appearance indistinguishable from PACNS
+ - Secondary CNS involvement of systemic vasculitis, polyarteritis, systemic lupus erythematosus (SLE) are most common
+ - VWI enhancement
+ - DSA: Multifocal irregularities, stenoses, and vascular occlusions
+- [Reversible Cerebral Vasoconstriction Syndrome](/document/reversible-cerebral-vasoconstricti-/c3a19be2-1f91-4dcb-b63a-cbfdaeaca5c1)
+ - Reversible; posterior circulation, watershed
+ - Can appear identical on DSA
+ - Verapamil infusion results in ↓ vascular irregularity, helps make diagnosis of RCVS
+ - VWI
+ - 50% no enhancement
+ - 50% mild, reversible
+- [Vasospasm](/document/vasospasm/397752a7-6998-4375-8090-e097d775d180)
+ - Temporal relationship to SAH
+ - Proximal trunks (circle of Willis) > peripheral branches
+- [Intravascular Large B-Cell Lymphoma](/document/intravascular-large-b-cell-lymphoma/9afdbab5-a2c4-41a6-a463-41e907f753b1)
+ - Lacks vessel wall enhancement
+ - Hemorrhage, infarcts more common
+ - MR: Linear parenchymal enhancement
+- ### Cerebral Amyloid-β-Related Angiitis
+
+
+ - Microbleeds + superficial siderosis more common
+ - Generally older patient with dementia
+- ### Neurosarcoidosis
+
+
+ - Granulomatous vasculitis/angiitis
+ - Small arterial perforators most frequently affected
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - Brain biopsy may be required to confirm diagnosis
+ - Definite diagnosis made from mononuclear inflammation of vessel wall
+ - 75-80% sensitive
+ - Negative biopsy does **not** necessarily exclude PACNS
+ - Must be distinguished from other causes of CNS inflammation and noninflammatory vascular disease
+- ### Staging, Grading, & Classification
+
+
+ - PACNS: Highly heterogeneous group of vasculitides limited to CNS
+ - Spectrum from granulomatous angiitis of CNS (GACNS) to benign angiopathy of CNS (BACNS) to reversible vasoconstrictive syndrome (RCVS)
+ - BACNS, RCVS have more favorable outcome
+ - All have indistinguishable angiographic appearance
+ - Clinical manifestations may be identical
+- ### Gross Pathologic & Surgical Features
+
+
+ - Ischemic lesions and small petechial hemorrhages
+ - Vessels of any size can be involved
+ - May see venulitis with parenchymal hemorrhages
+- ### Microscopic Features
+
+
+ - Mononuclear inflammation with necrosis of blood vessel walls is PACNS hallmark
+ - Variable degree of granulomatous and nongranulomatous angiitis of small vessels
+ - Typically involves media and adventitia of small leptomeningeal arteries and veins
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - Stroke from vascular involvement (stenoses, occlusion, aneurysm)
+ - Headache is also common
+ - #### Clinical profile
+
+
+ - Clinical presentation is highly variable: Focal to diffuse manifestations and acute to chronic evolution
+ - Subacute presentation over weeks or months is typical (mean = 5 months to diagnosis)
+ - Headache and mental status change with focal deficits
+ - No evidence of secondary vasculitis or other diseases mentioned in differential diagnosis should arouse suspicion
+- ### Demographics
+
+
+ - #### Age
+
+
+ - 50% between 40-60 years; range from age 3 to older adults
+ - BACNS, RCVS patients tend to be young women
+ - #### Sex
+
+
+ - Distribution of PACNS is nearly equal between sexes with perhaps slight male predominance
+ - #### Epidemiology
+
+
+ - Rare; 2.4 cases per 1 million person-years
+- ### Natural History & Prognosis
+
+
+ - Prognosis greatly improved with early recognition and therapy
+ - Delay in diagnosis may lead to additional morbidity
+ - PACNS: More likely to develop symptoms subacutely and remain undiagnosed for months
+ - BACNS: More likely to have relatively acute presentations and be diagnosed within weeks of onset
+ - Untreated PACNS: Risk of permanent cognitive dysfunction
+ - Often diagnosed posthumously; high index of suspicion is necessary to make correct diagnosis on timely basis
+- ### Treatment
+
+
+ - Few controlled studies on treatment of vasculitis, with considerable variation among centers on current therapeutic regimens
+ - Therapy typically comprises aggressive immunosuppressive approach
+ - High-dose steroid therapy with prolonged course and gradual taper controls disease in most cases
+ - Close monitoring of patients mandatory
+ - Without treatment, patients with PACNS tend to have progressively downhill courses often leading to death
+ - BACNS, RCVS patients may respond to less aggressive corticosteroid and Ca⁺⁺ channel blocker therapy
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - DSA when clinical suspicion of PACNS is strong, regardless of findings on MR
+ - VWI
+ - Some investigators suggest PACNS should be diagnosed only if patient presents with headache and combination of
+ - Focal neurologic deficits of at least 6 months duration (except in cases of "devastating onset")
+ - Several areas of segmental arterial narrowing demonstrated on cerebral angiography
+ - Systemic inflammation or infection has been excluded
+ - Leptomeningeal or parenchymal biopsy demonstrates vascular inflammation, but no signs of infection, atherosclerosis, or neoplastic disease
+- ### Image Interpretation Pearls
+
+
+ - Atherosclerosis is by far most common cause of vasculitis-like DSA pattern in older adults, not PACNS
+
+ 1d4f7eb2-2fc1-4f6a-b720-9235d9860603
+
+## References
+
+## Selected References
+
+1. [Hoffmann A et al: 7T MRI as a powerful tool to detect small- and medium-size vessel CNS vasculitis. AJNR Am J Neuroradiol. 46(6):1283-6, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=39694614%5Bpmid%5D)
+1. [Bangad A et al: Imaging of amyloid-beta-related arteritis. Neuroimaging Clin N Am. 34(1):167-73, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=37951701%5Bpmid%5D)
+1. [Hoshina Y et al: Vasculitis in the central nervous system: etiology, characteristics, and outcomes in a large single-center cohort. Neurohospitalist. 14(2):129-39, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38666288%5Bpmid%5D)
+1. [Hung SC et al: Imaging of childhood cerebral vasculitis. Neuroimaging Clin N Am. 34(1):149-66, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=37951700%5Bpmid%5D)
+1. [Salvarani C et al: Primary central nervous system vasculitis. N Engl J Med. 391(11):1028-37, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=39292929%5Bpmid%5D)
+1. [Sherri A et al: Primary angiitis of the CNS and ANCA-associated vasculitis: from pathology to treatment. Rheumatol Int. 44(2):211-22, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=37777632%5Bpmid%5D)
+1. [Corrêa DG et al: Intracranial vessel wall magnetic resonance imaging features of infectious vasculitis. Clin Imaging. 98:26-35, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36996597%5Bpmid%5D)
+1. [Pascarella R et al: European Stroke Organisation (ESO) guidelines on primary angiitis of the central nervous system (PACNS). Eur Stroke J. 8(4):842-79, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37903069%5Bpmid%5D)
+1. [Arnett N et al: Vessel wall MR imaging of central nervous system vasculitis: a systematic review. Neuroradiology. 64(1):43-58, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=33938989%5Bpmid%5D)
+1. [Hanafi R et al: COVID-19 neurologic complication with CNS vasculitis-like pattern. AJNR Am J Neuroradiol. 41(8):1384-7, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32554425%5Bpmid%5D)
+1. [Leao DJ et al: Intracranial vessel wall imaging: applications, interpretation, and pitfalls. Clin Radiol. 75(10):730-9, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32197916%5Bpmid%5D)
+1. [Rice CM et al: The diagnosis of primary central nervous system vasculitis. Pract Neurol. 20(2):109-14, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31649101%5Bpmid%5D)
+1. [Saygin D et al: Differentiation between neurosarcoidosis and primary central nervous system vasculitis based on demographic, cerebrospinal and imaging features. Clin Exp Rheumatol. 38 Suppl 124(2):135-8, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31928590%5Bpmid%5D)
+1. [Guggenberger K et al: High-resolution compressed-sensing T1 black-blood MRI: a new multipurpose sequence in vascular neuroimaging? Clin Neuroradiol. 31(1):207-16, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31853612%5Bpmid%5D)
+1. [Huang NF et al: Cerebellar PACNS in an elderly patient present as a tumor-like mass lesion: a case report. Acta Neurol Taiwan. 28(2):38-43, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31867705%5Bpmid%5D)
+1. [Huang X et al: Susceptibility-weighted imaging in the differential diagnosis of autoimmune central nervous system vasculitis and multiple sclerosis. Mult Scler Relat Disord. 33:70-4, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31158805%5Bpmid%5D)
+1. [Jin H et al: Primary angiitis of the central nervous system mimicking glioblastoma: a case report and literature review. Front Neurol. 10:1208, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31798527%5Bpmid%5D)
+1. [Kern KC et al: Vessel wall imaging of cerebrovascular disorders. Curr Treat Options Cardiovasc Med. 21(11):65, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31728661%5Bpmid%5D)
+1. [Moseley BD et al: Primary angiitis of the central nervous system presenting with microhemorrhages on gradient echo imaging. Neurol India. 67(5):1374-5, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31744984%5Bpmid%5D)
+1. [Rayfield C et al: Vasculitis on brain angiography is not always vasculitis: intravascular large B-cell lymphoma mimicking central nervous system vasculitis. BMJ Case Rep. 12(8):e230753, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31451471%5Bpmid%5D)
+1. [Soun JE et al: Central nervous system vasculopathies. Radiol Clin North Am. 57(6):1117-31, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31582039%5Bpmid%5D)
+1. [Strunk D et al: Biomarkers in vasculitides of the nervous system. Front Neurol. 10:591, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31244756%5Bpmid%5D)
+1. [Thaler C et al: Neuroradiologic characteristics of primary angiitis of the central nervous system according to the affected vessel size. Clin Neuroradiol. 29(1):37-44, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=28875326%5Bpmid%5D)
+1. [Bathla G et al: Cerebrovascular manifestations of neurosarcoidosis: an underrecognized aspect of the imaging spectrum. AJNR Am J Neuroradiol. 39(7):1194-200, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29284603%5Bpmid%5D)
+1. [Chen CY et al: Vascular wall imaging in reversible cerebral vasoconstriction syndrome - a 3-T contrast-enhanced MRI study. J Headache Pain. 19(1):74, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30167985%5Bpmid%5D)
+1. [Ide S et al: Intracranial vessel wall lesions in patients with systematic lupus erythematosus. J Magn Reson Imaging. 48(5):1237-46, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29473985%5Bpmid%5D)
+1. [Mehdipoor G et al: Imaging manifestations of Behcet's disease: key considerations and major features. Eur J Radiol. 98:214-25, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29196115%5Bpmid%5D)
+1. [Dutra LA et al: Central nervous system vasculitis in adults: an update. Autoimmun Rev. 16(2):123-31, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28087351%5Bpmid%5D)
+1. [Mandal J et al: Primary angiitis of the central nervous system. Rheum Dis Clin North Am. 43(4):503-18, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=29061238%5Bpmid%5D)
+1. [Mossa-Basha M et al: Added value of vessel wall magnetic resonance imaging for differentiation of nonocclusive intracranial vasculopathies. Stroke. 48(11):3026-33, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=29030476%5Bpmid%5D)
+1. [Schuster S et al: Subtypes of primary angiitis of the CNS identified by MRI patterns reflect the size of affected vessels. J Neurol Neurosurg Psychiatry. 88(9):749-55, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28705900%5Bpmid%5D)
+1. [Miller TR et al: Reversible cerebral vasoconstriction syndrome, part 2: diagnostic work-up, imaging evaluation, and differential diagnosis. AJNR Am J Neuroradiol. 36(9):1580-8, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25614476%5Bpmid%5D)
+1. [Abdel Razek AA et al: Imaging spectrum of CNS vasculitis. Radiographics. 34(4):873-94, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25019429%5Bpmid%5D)
+
+## Anatomy
+
+### Basal Ganglia
+Brain/ANATOMY:a9de3815-ec59-4c78-adf0-94974065a7e3
+
+### Limbic Network
+Brain/ANATOMY:e1a20b61-b2c1-44c5-ba04-59843855bfef
+
+### Aortic Arch and Great Vessels
+Brain/ANATOMY:a7a252f0-2ac6-402a-8c87-cfce8adc799b
+
+### Anterior Cerebral Artery
+Brain/ANATOMY:1da0f3ae-7858-4ad7-bc22-ea2a245a4214
+
+### Posterior Cerebral Artery
+Brain/ANATOMY:7bad3118-2ae8-4727-8f8d-fd8175c4e8c2
+
+### Middle Cerebral Artery
+Brain/ANATOMY:5658d6a5-aa61-4c8a-a6c7-a8d5aa5c41c4
+
+## Cases
+
+- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '006be825-ccf3-4ca5-8057-286b5737eea2', 'description': 'Axial T2WIs (#1-3) and FLAIR (#4,5) scans show multifocal hyperintensities (arrows) in the pons, white matter and the right "hand knob" . T2*GRE (not shown) disclosed no evidence for microhemorrhages. No lesions enhanced. DWI (#6) and ADC (#7) did show several foci of restricted diffusion in the cortex of the right hemisphere (arrows). \n\nCerebral angiogram was performed. Minor nonhemodynamically significant atherosclerotic plaques were seen at both carotid bifurcations (not shown). Multiple areas of alternating stenoses and dilatations were identified on the intracranial arteries distal to the circle of Willis (arrows, #8-14) without evidence for significant atherosclerosis on either the carotid or vertebrobasilar arteries. While this pattern is consistent with vasculitis, in an elderly patient the most common etiology for this angiographic appearance is still atherosclerosis. Imaging findings prompted further evaluation. Laboratory workup was positive for antinuclear antibodies.\n\nThe patient died of unrelated causes two months later and a brain-only limited autopsy was performed. Microscopic examination of the intracranial vessels (#15-22) showed intimal and adventitial thickening in leptomeningeal arteries and numerous medium-sized branches of the circle of Willis. Inflammatory changes and necrosis in blood vessel walls consistent with vasculitis were noted and are best seen on image #16. Giant cells were seen in some sections (arrows, #17,18) so the final diagnosis was giant cell arteritis of the CNS. Note cortical infarct on image #19 (arrow), taken from the site of the acute infarct seen on image #6. Image #20 shows a thrombosed arteriole (arrow) with surrounding spongiosis in the parenchyma. Images #21 and 22 show a lacunar infarct in the region of the red nucleus with foamy degenerative changes.\n\nGiant cell arteritis is a systemic granulomatous inflammatory vasculitis that involves medium to large-sized arteries. With autopsy limited to the brain, it is not known whether this patient had any other organ involvement.', 'history': 'History of T12 spinal infarct, paraplegia following surgery for spinal stenosis. Presented with 10 day history of decreased fluid intake, one day of nausea and vomiting followed by symptoms of TIA. ', 'imagePoolId': '9616c0cd-d3d6-4722-96bf-a3908095435d', 'name': 'Path-proven giant cell arteritis', 'teachingPoint': None, 'demographics': '71 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '857b45a5-bbf3-4aa5-abf5-680d8a43f64f', 'description': 'Axial T2WI (#1) shows high signal intensity in the right cerebral peduncle, substantia nigra, and pars compacta of the midbrain (arrows). Note adjacent parahippocampal gyrus involvement (curved arrow). An enlarged, abnormal midbrain perivascular space is seen (open arrow). Post-contrast T1WI (#2) shows enhancement in the affected area.\n\nComment: The midbrain is a common site for vasculitic involvement. Both PACNS and other vasculitides such as Behcet disease have a predilection for this location.', 'history': 'Known PACNS vasculitis.', 'imagePoolId': '74c99d6f-1367-4731-bf83-eb3068f08583', 'name': 'Perivascular spaces of midbrain', 'teachingPoint': None}, {'authors': [{'key': 'ba63ec29-d7f8-4a21-a70a-6776e9506c9b', 'value': 'Gary M. Nesbit, MD, FSNIS'}], 'caseVersionId': '6091ab71-fbf5-4e67-b1e1-f062fa79d728', 'description': 'DWI (#1) shows multifocal regions of diffusion restriction (arrows). T2 (#2) and FLAIR (#3) hyperintensity in the deep white matter and corpus callosum (arrows) is an unusual distribution for embolic or hypoperfusion infarcts. Several views of the digital subtraction angiogram in this patient (#4-8) show multiple areas of stenosis and slight fusiform enlargement in the middle cerebral artery, anterior cerebral artery, and PCA distributions with tapered occlusion of the left anterior cerebral artery. The right vertebral artery (arrow, #4) is especially severely involved.', 'history': 'Patient admitted with acute on subacute course of encephalopathy for 6 months; problems with job duties previously performed without difficulty; fatigue and headache; then acutely decompensated. Brain biopsy negative; tissue sample was very small and did not include the meninges; treated with IV methylprednisolone and began to improve clinically on day 2 of this therapy.\n\n', 'imagePoolId': '88b180b6-8a67-4ca4-86f9-ed1b4f7c9bcb', 'name': 'Corpus callosum, multifocal primary arteritis of CNS', 'teachingPoint': None, 'demographics': '60 Years old female'}, {'authors': [{'key': '8d5254e9-8dda-478b-8f08-bdee97a32c79', 'value': 'Karen L. Salzman, MD, FACR'}, {'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '1496ee92-d43e-4b0c-a1a1-e4ba2ae3584f', 'description': 'Typical MR case of primary arteritis of the CNS affecting the cerebral hemispheres with a posterior circulation predominance with DWI restriction and contrast-enhancement.\n\nAxial FLAIR MR images (#1-3) show abnormal hyperintensity in the cortex and subcortical white matter of the occipital lobes, parietal lobes and posterior temporal lobes bilaterally (arrows) related to ischemia. Contrast-enhanced T1 MR images (#4-7) show extensive gyriform and patchy enhancement. DWI MR images (#8-10) show bright diffusion restriction indicating acute ischemia. The corresponding ADC maps confirmed the diffusion restriction.\n\nComment: Primary arteritis of the CNS typically involves all vascular distributions and does not have a posterior circulation predominance as in this case. Although the MR findings are highly suspicious in this case, conventional angiography remains the "gold standard" for diagnosis.', 'history': None, 'imagePoolId': '4818cdd3-2e67-49c7-acca-06ec6bbb8d5e', 'name': 'Enhancement, posterior predominance', 'teachingPoint': None, 'demographics': '50 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'a4d956e3-0b79-4aa1-8e72-191066786184', 'description': 'A series of axial (#1, 2) and coronal (#3, 4) T1 C+ scans show patchy and linear enhancement in the midbrain and both temporal lobes (arrows). Note abnormal enhancing areas in the basal ganglia as well as the insular and subinsular cortex (arrows, #2, 3). \n\nComment: Patchy and linear areas of contrast-enhancement, especially in the basal ganglia, midbrain, and cortical/subcortical regions, should raise suspicion of vasculitis. Other causes of this imaging appearance include intravascular lymphoma.', 'history': 'Proven PACNS.', 'imagePoolId': 'd8b9954e-ca2d-4860-866e-f67ff876de74', 'name': 'Midbrain, temporal lobes', 'teachingPoint': None, 'demographics': '42 Years old female'}, {'authors': [{'key': '8d5254e9-8dda-478b-8f08-bdee97a32c79', 'value': 'Karen L. Salzman, MD, FACR'}], 'caseVersionId': '657bd0f0-1cee-4c84-a3c1-1380930ec086', 'description': 'Typical MR and DSA case of primary arteritis of the CNS.\n\nAxial T2 MR images (#1-5) show multifocal areas of abnormal hyperintensity related to ischemic lesions. Note involvement of the pons, brachium pontis, midbrain, and basal ganglia (open arrows). Post-contrast T1 MR images (#6-7) show abnormal enhancement of the pons and midbrain lesions (arrows), commonly seen with primary arteritis of the CNS.\n\nLateral DSA images (#8-9) from an internal carotid injection shows subtle arterial stenosis and dilatation (curved arrows) typical for primary arteritis of the CNS. Conventional angiography remains the "gold standard" for diagnosis.', 'history': 'Patient with headaches and mental status changes.', 'imagePoolId': '7081b72e-c23a-4e0e-b900-24cdd577e31b', 'name': 'Classic', 'teachingPoint': None, 'demographics': '40 Years old female'}], 'caseType': 'typical', 'name': 'TYPICAL'}
+- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '666b4c11-930c-45cf-9dae-2f483d9a7557', 'description': 'A series of axial NECT scans (#1-4) show patchy increased density and more widespread decreased density in the left temporoparietal area. The sulci and cisterns are obliterated and the ventricles appear smaller than normal, indicating diffuse brain swelling. Hypercoagulability with thrombosed transverse sinus and vein of Labbe was the initial impression given the CT findings. \n\nAn MR scan was requested for further evaluation. Axial T1WI (#5) and FLAIR (#6) scans show acute parenchymal hemorrhage with edema. Note high signal in the perimesencephalic cistern and sulci (arrows, #6) indicating the presence of subarachnoid hemorrhage. Postcontrast axial (#7) and coronal (#8) show no enhancement. The coronal T1 C+ scan (#8) shows an empty delta sign (open arrow) and there is some adjacent dural thickening (arrow). Venous sinus occlusion was confirmed on MRV (not shown).', 'history': 'Post partum with increasing headaches, drowsiness, papilledema. Normotensive.', 'imagePoolId': '79530d74-084a-4d1b-81ad-5be1ad7aa486', 'name': 'Post partum with venous thrombosis', 'teachingPoint': 'Further evaluation disclosed clinical and laboratory evidence of vasculitis. Postpartum vasculitis can affect either the arterial or venous circulation and dural sinus or cortical vein thrombosis is a known complication.', 'demographics': '22 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '94ae3153-0a97-454a-a3dd-7004d5bba9db', 'description': 'A series of axial FLAIR scans (#1-4) show striking hyperintensity in the occipital gyri and subcortical white matter along with diffuse high signal intensity in the subarachnoid spaces (arrows). The sagittal pre-contrast T1WI shows subacute gyriform hemorrhage (arrows, #5). The post-contrast axial T1WIs (#6-8) show enhancement in the sulci, posterior gyri and suboccipital white matter (arrow). DWI scans show multifocal patchy areas of restriction (arrows, #9, 10), especially in the occipital lobes.\n\nFrank cortical/subcortical and subarachnoid hemorrhage in vasculitis is atypical but not rare. In this case, the occipital predominance might make posterior reversible encephalopathy syndrome (PRES) a diagnostic consideration. However, the patient was normotensive and DWI scans in PRES are usually negative.\n\nGiven the history, a diagnosis of drug-related (ephedrine) angiopathy should be considered. Angiography was not performed in this patient but high dose steroid therapy resulted in significant improvement.', 'history': 'Headaches with mental status changes, meningismus, visual symptoms progressing to decreased consciousness and coma in a normotensive patient. History of cold treated with over-the-counter pseudoephedrine tablets for a week. ', 'imagePoolId': 'd3d556fb-1834-4d28-877f-b7e2c31b2785', 'name': 'DWI positive', 'teachingPoint': None, 'demographics': '81 Years old female'}], 'caseType': 'variant', 'name': 'VARIANT'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*Coronal oblique graphic illustrates alternating segmental areas of narrowing and dilatation of the opercular and superficial middle cerebral artery (MCA) branches as well as patchy multifocal ischemia
within the underlying brain from primary arteritis of the CNS (PACNS).*
+
+
+*Coronal oblique graphic illustrates alternating segmental areas of narrowing and dilatation of the opercular and superficial middle cerebral artery (MCA) branches as well as patchy multifocal ischemia
within the underlying brain from primary arteritis of the CNS (PACNS).*
+
+
+*H&E photomicrograph shows a thick arteriolar wall with inflammation and necrosis, the 2 cardinal histologic features of vasculitis. (Courtesy R. Hewlett, MD.)*
+
+
+*Precontrast high-resolution vessel wall T1 MR in a 14-year-old girl with a "wake-up" stroke 3 days prior to the study shows no abnormality.*
+
+
+*Axial T1 C+ FS black-blood vessel wall MR (VW-MR) study shows circumferential and linear enhancement in the right M1 and M2 vessel walls
. Note gyriform enhancement in the subacute infarct
. This is proven antineutrophil cytoplasmic autoantibody (ANCA) vasculitis. Imaging is identical to PACNS. VW-MR is often key to the diagnosis.*
+
+
+*Coronal T1 C+ MR shows bilateral, prominent linear patterns of enhancement
. This is biopsy-proven PACNS. Vasculitis often presents with convexal subarachnoid hemorrhage (SAH) and small infarcts.*
+
+
+*Axial FLAIR MR in a 60-year-old with new strokes shows nonspecific periventricular white matter (WM) disease
suggestive of chronic microvascular ischemia and a chronic lacunar infarct
. Subtle SAH
is also present. In a patient with new strokes and SAH, vasculitis should be considered.*
+
+
+*Axial C+ VW-MR in the same patient shows arterial wall enhancement
related to vasculitis in the anterior and posterior vascular distributions. Note the enhancement in the temporal lobe and pons
related to recent strokes.*
+
+
+*Axial C+ VW-MR in the same patient shows bilateral MCA arterial wall enhancement
. PACNS can be diagnosed with imaging and laboratory analysis, though DSA and biopsy may be required. Prognosis of PACNS is greatly improved with early recognition and therapy.*
+
+
+*Axial FLAIR MR in a young adult shows focal SAH
and surrounding hyperintense infarct in the right insula. DTI showed additional foci of small infarcts in multiple vascular distributions (not shown).*
+
+
+*Axial C+ VW-MR of the same patient shows focal enhancement in the sulcus and surrounding an MCA branch
related to PACNS. Patients typically respond well to medical management with steroids and aggressive immunosuppressive therapy. Prevention of future strokes is a key treatment goal.*
+
+
+*Axial T1 C+ FS MR in a patient with progressive ataxia and slowly worsening diplopia for 6 years shows multifocal punctate and linear enhancing foci
in the medulla and cerebellar hemispheres.*
+
+
+*More cephalad T1 C+ FS MR in the same patient shows innumerable linear and dot-like areas of enhancement
in the subcortical and deep WM of both hemispheres. Enhancement pattern suggests an infectious or inflammatory process.*
+
+
+*Axial DWI MR in the same patient shows multiple small, rounded, and linear foci of restricted diffusion
related to acute infarcts.*
+
+
+*Lateral DSA of the internal carotid angiogram subsequently obtained shows subtle areas of irregular narrowing
in distal cortical arteries, suggestive of vasculitis. DSA imaging differential considerations include vasospasm or the much more common atherosclerotic disease.*
+
+
+*Micrograph with H&E stain of the biopsy obtained in the same patient shows necrosis and inflammatory infiltrate thickening the wall of this penetrating cortical artery.*
+
+
+*Immunohistochemistry in the same patient is positive for CD68 T-cell lymphocytes. The final histopathologic diagnosis was PACNS. With the advent of VW-MR, DSA and biopsy for pathologic diagnosis is not always required. Patients are often treated medically on the basis of VW-MR and lumbar puncture analysis.*
+
+
+*Axial T2 MR in a 42-year-old man with 2 months of blurry vision and 4 days of nausea, vomiting, and increasing headache shows a right parietooccipital mass with WM edema
surrounding a mixed iso- and hypointense component
. There is a right frontal heterogeneously hyperintense lesion
.*
+
+
+*Axial FLAIR MR shows the right parietooccipital mass
does not suppress, while part of the right frontal mass exhibits fluid suppression
.*
+
+
+*Axial T2* GRE shows gradient susceptibility with hemorrhage
in both the parietooccipital and frontal masses.*
+
+
+*Axial T1 C+ MR in the same patient shows intense but heterogeneous enhancement in the right parietooccipital mass
. Note ependymal enhancement
adjacent to the mass. The right frontal mass
does not enhance. The preoperative diagnosis was glioblastoma. The histologic diagnosis was lymphocytic vasculitis. Tumefactive PACNS is an uncommon presentation of vasculitis.*
+
+
+*Axial GRE MR in a 60-year-old woman shows a right frontal lobe mass with hypointensity
related to blood products. T1 hyperintensity was noted in the cortex in a gyriform pattern on T1 MR images (not shown).*
+
+
+*Axial T1 C+ in the same patient shows a complex heterogeneously enhancing mass in the right frontal lobe. Imaging suggested a hemorrhagic neoplasm or subacute infarct. Tumefactive PACNS was diagnosed at biopsy.*
+
+
+### Additional Images
+
+
+*Axial gross pathology of vasculitis shows multiple hemorrhagic infarcts in the basal ganglia
and cortex
.*
+
+
+*Sagittal T1 C+ MR shows nodular and linear enhancement along the pial surface (folia) of the cerebellum and in the deep medullary veins of the frontal lobe
. This has a mixed pattern of arterial and venous involvement in biopsy-proven granulomatous angiitis of the CNS.*
+
+
+*Axial FLAIR in a 63-year-old woman with headaches shows patchy
as well as punctate
and linear
hyperintensities in the subcortical and deep periventricular WM.*
+
+
+*Axial DWI in the same patient shows multiple foci of restricted diffusion
in the subcortical and deep WM.*
+
+
+*Axial T1 C+ FS MR in the same case shows multiple punctate foci of contrast enhancement in the basal ganglia
and WM
.*
+
+
+*More cephalad T1 C+ FS MR in the same patient shows linear enhancing foci
perpendicular to the lateral ventricles.*
+
+
+*Coronal T1 C+ MR shows the multiple enhancing foci concentrated in the basal ganglia
.*
+
+
+*More posterior T1 C+ MR in the same patient shows some of the linear enhancing foci
.*
+
+
+*Coronal T1 C+ MR in the same patient shows enhancing lesions
in the occipital lobe WM and cerebellum. This was biopsy-proven PACNS.*
+
+
+*Axial T2 MR in a 56-year-old man with 1 week of increasing headache and gait imbalance shows a heterogeneously hyperintense mass
in the left cerebellar hemisphere.*
+
+
+*Axial T1 C+ FS MR shows faint enhancement in the cerebellar hemisphere
, but the most striking finding is multiple tortuous, corkscrew vessels
lying along the folia.*
+
+
+*Axial T2* SWI MR shows multiple rounded and punctate foci of susceptibility
and hypointensity along the cerebellar folia
.*
+
+
+*Axial DWI
at the lateral aspect of the cerebellar hemisphere shows abnormal hypointensity of the cerebellar folia
.*
+
+
+*Left vertebral artery DSA, lateral view, arterial phase, shows only mild irregularity of the posterior inferior cerebellar artery
.*
+
+
+*Late venous phase of the DSA in the same patient shows multiple dilated small venous pouches
. The imaging diagnosis was venous vasculitis ("venulitis"), possibly Behçet disease. No biopsy was performed. Patient was placed on steroids and symptoms rapidly improved. Follow-up imaging at 3 months and 10 months showed only mild residual gliosis in the left lateral cerebellum.*
+
+
+*Axial NECT in a 42-year-old man with 2 months of blurry vision and 4 days of nausea, vomiting, and increasing headache shows a hypointense mass
in the right parietal and occipital lobes extending from the subcortical WM into the corpus callosum and deep periventricular WM.*
+
+
+*Axial T1 MR shows the mass
exhibits patchy hypointensity throughout the right parietal and occipital lobes. A 2nd, more hypointense mass is present in the right frontal lobe
. Final diagnosis after resection was PACNS. Imaging mimics neoplasm in this rare presentation of vasculitis.*
+
diff --git a/docs_md/articles/renal-vasculature-anatomy_3ff759c8-2856-4e6e-888c-ade01f1960c7.md b/docs_md/articles/renal-vasculature-anatomy_3ff759c8-2856-4e6e-888c-ade01f1960c7.md
new file mode 100644
index 0000000..17e3e95
--- /dev/null
+++ b/docs_md/articles/renal-vasculature-anatomy_3ff759c8-2856-4e6e-888c-ade01f1960c7.md
@@ -0,0 +1,198 @@
+---
+title: "Renal Vasculature Anatomy"
+docid: "3ff759c8-2856-4e6e-888c-ade01f1960c7"
+authors:
+ - key: "91e93745-f376-45a8-9b33-eae419cd3322"
+ value: "T. Gregory Walker, MD, FSIR"
+breadcrumbs:
+ -
+ name: "Vasculature"
+ slug: "vasculature"
+ treeNodeId: "6de1ee4d-afe9-419c-a868-d4074ec0fb7e"
+ -
+ name: "Anatomy"
+ slug: "anatomy"
+ treeNodeId: "3bbfaa8b-2dbc-41eb-b3da-3bd83a9737c4"
+ -
+ name: "Renal Vasculature Anatomy"
+ slug: "renal-vasculature-anatomy"
+ treeNodeId: null
+category: "Vasculature"
+documentVersionId: "6f31f41e-6db9-4742-89a0-0e3b3ac94f60"
+imageCount: 5
+lastUpdated: "08/06/20"
+pageDescription: "Renal Vasculature Anatomy"
+pageKeywords: "Vasculature, Anatomy, Renal Vasculature Anatomy"
+pageTitle: "Renal Vasculature Anatomy | STATdx"
+enhancedTitle: "Renal Vasculature Anatomy"
+type: "ANATOMY"
+references: true
+breadcrumbs:
+ - "Vasculature"
+ - "Anatomy"
+ - "Renal Vasculature Anatomy"
+---
+## GROSS ANATOMY
+
+- ### Renal Arteries
+
+
+ - Kidney is supplied by 1 renal artery in most individuals
+ - Renal artery usually 5-6 mm in diameter, 4-6 cm long
+ - 30-40% incidence of multiple renal arteries
+ - More common on right side if present
+ - Renal arteries arise from abdominal aorta at ~ L2 level
+ - Right renal artery arises anterolaterally from aorta
+ - Courses behind inferior vena cava (IVC) and renal vein
+ - Left renal artery arises laterally from aorta
+ - Courses posterior to corresponding renal vein
+ - Main renal arteries divide at renal hilum into anterior and posterior segmental arteries
+- ### Intrarenal Arterial Anatomy
+
+
+ - Anterior segmental artery supplies upper, middle, and lower renal segments; also often supplies apical segment
+ - Posterior segmental artery usually supplies only posterior segment of kidney
+ - May supply apical segment in minority of patients
+ - Segmental arteries yield interlobar arteries
+ - Course alongside renal pyramids toward periphery
+ - Yield arcuate arteries at corticomedullary junction
+ - Arcuate arteries travel across top of renal pyramids and give rise to interlobular arteries
+ - Interlobular arteries are tiny parenchymal branches that course toward kidney surface and subdivide into afferent glomerular arterioles
+- ### Branches of Renal Arteries
+
+
+ - Inferior adrenal artery
+ - Arises superiorly from proximal main renal artery
+ - Supplies inferior aspect of adrenal gland
+ - Adrenal gland is also supplied by middle adrenal artery arising from aorta and superior adrenal artery originating from inferior phrenic artery
+ - Superior capsular artery
+ - Arises adjacent to, or with, inferior adrenal artery
+ - Primarily supplies perirenal (capsular) fat
+ - May anastomose with retroperitoneal arterial plexus
+ - Plexus is derived from several tiny aortic branches
+ - Intrarenal arteries communicate via perforating branches
+ - Middle capsular artery
+ - Arises from main renal artery or its branches
+ - Medial course toward renal sinus; gives off branches that supply ventral and dorsal perirenal fat
+ - Perforating middle capsular arteries arise from renal interlobular arteries, penetrate capsule, and anastomose with perirenal branches
+ - May be difficult to distinguish from ureteric artery
+ - Inferior capsular artery
+ - Commonly arises from gonadal artery
+ - Usually not well developed
+ - When present, anastomoses with superior capsular artery; forms arcade along lateral margin of kidney
+ - Arcade communicates with perforating capsular arteries and other retroperitoneal arteries
+ - Capsular arteries may provide collateral flow to kidney
+ - Occurs with severe renal artery stenosis/occlusion located distal to capsular artery origin
+ - Exophytic renal neoplasms may derive portion of arterial supply from these vessels
+ - e.g., angiomyolipoma, renal cell carcinoma
+ - Ureteric arteries
+ - Superior ureter is supplied by branches arising from renal, inferior adrenal, or testicular artery
+ - Distal ureter is supplied by tiny branches from common, internal, and external iliac arteries
+ - May provide collateral flow to kidneys in presence of renal artery stenosis/occlusion
+ - Enlarged collaterals may cause "ureteral notching"
+- ### Variant Renal Arterial Anatomy
+
+
+ - 30-40% incidence of variations in number, location, and branching patterns of renal arteries
+ - Accessory renal arteries usually arise from aorta
+ - May arise from common iliac arteries
+ - Rarely arise above superior mesenteric artery (SMA)
+ - Horseshoe kidneys always have multiple renal arteries
+ - Fused portion of horseshoe kidney (isthmus) may be supplied by distal aorta and iliac arteries
+ - Other congenital variations in renal configuration/position (e.g., pelvic kidney, crossed fused ectopia) have high incidence of variant arterial anatomy
+- ### Renal Veins
+
+
+ - Usually each kidney is drained by single renal vein
+ - Right renal vein drains directly into IVC
+ - Multiple right renal veins occur in 28% of patients
+ - Right gonadal vein drains into right renal vein in < 10% of individuals
+ - Right adrenal vein drains directly into IVC rather than into right renal vein
+ - Left renal vein courses between aorta and SMA
+ - Enters IVC directly opposite right renal vein
+ - In 99% of individuals, left gonadal vein drains into left renal vein before latter crosses aorta
+ - Left adrenal and inferior phrenic veins drain into left renal vein, usually via common trunk
+ - Compression of left renal vein between aorta and SMA can result in nutcracker syndrome
+- ### Variant Renal Venous Anatomy
+
+
+ - Circumaortic left renal vein is most common variant
+ - Vein divides; encircles aorta anteriorly and posteriorly
+ - Each venous moiety enters IVC separately
+ - Occurs in ~ 2-6% of patients
+ - Retroaortic left renal vein is another common variant
+ - Vein courses posterior to aorta to enter IVC
+ - May be compressed between aorta and spine, causing left renal vein outflow obstruction
+ - Implications for optimal IVC filter placement
+
+ 2cefb342-b121-4a67-862e-9386c0b288cc
+
+## References
+
+## Selected References
+
+1. [Kang WY et al: Perihilar branching patterns of renal artery and extrarenal length of arterial branches and tumour-feeding arteries on multidetector CT angiography. Br J Radiol. 86(1023):20120387, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23418206%5Bpmid%5D)
+1. [Khamanarong K et al: Anatomy of renal arterial supply. Clin Anat. 17(4):334-6, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15108340%5Bpmid%5D)
+1. [Hoeltl W et al: Renal vein anatomy and its implications for retroperitoneal surgery. J Urol. 143(6):1108-14, 1990](http://www.ncbi.nlm.nih.gov/pubmed/?term=2342169%5Bpmid%5D)
+1. [Meyers MA et al: The significance of the renal capsular arteries. Br J Radiol. 40(480):949-56, 1967](http://www.ncbi.nlm.nih.gov/pubmed/?term=4952886%5Bpmid%5D)
+
+
+## Images
+
+
+### Normal Renal Arterial and Venous Anatomy
+
+
+*Graphic shows the normal renal arterial and venous anatomy. Each kidney is typically supplied by a single renal artery and is drained by a single renal vein, although variations in both arterial and venous anatomy are fairly common. The right renal artery and left renal vein are each longer than their counterparts as a result of their relationships to the abdominal aorta and inferior vena cava (IVC), respectively. Each renal artery yields several small branches proximally, including the inferior adrenal, capsular, and ureteric arteries. These vessels can provide important collateral perfusion to the kidney in some cases of stenosis or occlusion of the main renal artery. The left renal vein courses between the aorta and superior mesenteric artery (SMA) to enter the IVC directly opposite the right renal vein. The left gonadal vein drains into the left renal vein while its counterpart on the right enters the IVC directly.*
+
+
+*Graphic shows the normal renal arterial and venous anatomy. Each kidney is typically supplied by a single renal artery and is drained by a single renal vein, although variations in both arterial and venous anatomy are fairly common. The right renal artery and left renal vein are each longer than their counterparts as a result of their relationships to the abdominal aorta and inferior vena cava (IVC), respectively. Each renal artery yields several small branches proximally, including the inferior adrenal, capsular, and ureteric arteries. These vessels can provide important collateral perfusion to the kidney in some cases of stenosis or occlusion of the main renal artery. The left renal vein courses between the aorta and superior mesenteric artery (SMA) to enter the IVC directly opposite the right renal vein. The left gonadal vein drains into the left renal vein while its counterpart on the right enters the IVC directly.*
+
+
+*Graphic shows the normal renal arterial and venous anatomy. Each kidney is typically supplied by a single renal artery and is drained by a single renal vein, although variations in both arterial and venous anatomy are fairly common. The right renal artery and left renal vein are each longer than their counterparts as a result of their relationships to the abdominal aorta and inferior vena cava (IVC), respectively. Each renal artery yields several small branches proximally, including the inferior adrenal, capsular, and ureteric arteries. These vessels can provide important collateral perfusion to the kidney in some cases of stenosis or occlusion of the main renal artery. The left renal vein courses between the aorta and superior mesenteric artery (SMA) to enter the IVC directly opposite the right renal vein. The left gonadal vein drains into the left renal vein while its counterpart on the right enters the IVC directly.*
+
+
+*Graphic shows the normal renal arterial and venous anatomy. Each kidney is typically supplied by a single renal artery and is drained by a single renal vein, although variations in both arterial and venous anatomy are fairly common. The right renal artery and left renal vein are each longer than their counterparts as a result of their relationships to the abdominal aorta and inferior vena cava (IVC), respectively. Each renal artery yields several small branches proximally, including the inferior adrenal, capsular, and ureteric arteries. These vessels can provide important collateral perfusion to the kidney in some cases of stenosis or occlusion of the main renal artery. The left renal vein courses between the aorta and superior mesenteric artery (SMA) to enter the IVC directly opposite the right renal vein. The left gonadal vein drains into the left renal vein while its counterpart on the right enters the IVC directly.*
+
+
+### Normal Renal Arterial Anatomy
+
+
+*Graphic shows normal renal artery anatomy. The adrenal artery arises from the proximal renal artery. It often has a common trunk with the superior capsular artery, that supplies the perirenal fat. Normally, the renal artery extends to the hilum before branching into segmental renal arteries. The latter yield interlobar arteries, which course along the renal pyramids toward the periphery where they yield arcuate arteries. The latter course over the renal pyramids and give rise to interlobular arteries (parenchymal branches that course toward the kidney surface and subdivide into the afferent glomerular arterioles).*
+
+
+*Graphic shows normal renal artery anatomy. The adrenal artery arises from the proximal renal artery. It often has a common trunk with the superior capsular artery, that supplies the perirenal fat. Normally, the renal artery extends to the hilum before branching into segmental renal arteries. The latter yield interlobar arteries, which course along the renal pyramids toward the periphery where they yield arcuate arteries. The latter course over the renal pyramids and give rise to interlobular arteries (parenchymal branches that course toward the kidney surface and subdivide into the afferent glomerular arterioles).*
+
+
+*Graphic shows normal renal artery anatomy. The adrenal artery arises from the proximal renal artery. It often has a common trunk with the superior capsular artery, that supplies the perirenal fat. Normally, the renal artery extends to the hilum before branching into segmental renal arteries. The latter yield interlobar arteries, which course along the renal pyramids toward the periphery where they yield arcuate arteries. The latter course over the renal pyramids and give rise to interlobular arteries (parenchymal branches that course toward the kidney surface and subdivide into the afferent glomerular arterioles).*
+
+
+*Left renal arteriogram shows a normal arterial branching pattern. The anterior segmental renal artery supplies the anterior, upper, middle, and lower segments; the posterior segmental artery supplies the posterior segment. The superior capsular artery courses over the upper renal pole and anastomoses with small retroperitoneal branches. Here, the superior capsular artery has a separate origin from the inferior adrenal artery. The middle capsular artery arises from the main renal artery and gives supply to the renal pelvis and proximal ureter.*
+
+
+*Left renal arteriogram shows a normal arterial branching pattern. The anterior segmental renal artery supplies the anterior, upper, middle, and lower segments; the posterior segmental artery supplies the posterior segment. The superior capsular artery courses over the upper renal pole and anastomoses with small retroperitoneal branches. Here, the superior capsular artery has a separate origin from the inferior adrenal artery. The middle capsular artery arises from the main renal artery and gives supply to the renal pelvis and proximal ureter.*
+
+
+*Left renal arteriogram shows a normal arterial branching pattern. The anterior segmental renal artery supplies the anterior, upper, middle, and lower segments; the posterior segmental artery supplies the posterior segment. The superior capsular artery courses over the upper renal pole and anastomoses with small retroperitoneal branches. Here, the superior capsular artery has a separate origin from the inferior adrenal artery. The middle capsular artery arises from the main renal artery and gives supply to the renal pelvis and proximal ureter.*
+
+
+### Normal Renal Venous Anatomy
+
+
+*Graphic shows the venous drainage of the kidneys. Normally, each kidney is drained by a single renal vein, although multiple renal veins may occur (more common on the right). Variant venous anatomy is prevalent on the left; common variants include circumaortic and retroaortic renal veins. Any retroaortic component of the left renal vein may be compressed between the aorta and the underlying vertebra, potentially causing left renal vein outflow obstruction. Also, the left renal vein can be compressed between the aorta and SMA, causing nutcracker syndrome.*
+
+
+*Graphic shows the venous drainage of the kidneys. Normally, each kidney is drained by a single renal vein, although multiple renal veins may occur (more common on the right). Variant venous anatomy is prevalent on the left; common variants include circumaortic and retroaortic renal veins. Any retroaortic component of the left renal vein may be compressed between the aorta and the underlying vertebra, potentially causing left renal vein outflow obstruction. Also, the left renal vein can be compressed between the aorta and SMA, causing nutcracker syndrome.*
+
+
+*Graphic shows the venous drainage of the kidneys. Normally, each kidney is drained by a single renal vein, although multiple renal veins may occur (more common on the right). Variant venous anatomy is prevalent on the left; common variants include circumaortic and retroaortic renal veins. Any retroaortic component of the left renal vein may be compressed between the aorta and the underlying vertebra, potentially causing left renal vein outflow obstruction. Also, the left renal vein can be compressed between the aorta and SMA, causing nutcracker syndrome.*
+
+
+*Left renal venogram shows detailed normal anatomy of the intrarenal and extrarenal veins. The branching pattern of the intrarenal veins corresponds to that of the intrarenal arterial circulation. The left kidney is drained by a single renal vein that courses across the midline to drain into the IVC opposite the right renal vein. The latter drains directly into the IVC. The left adrenal gland is drained by a single adrenal vein that joins with the inferior phrenic vein to drain into the superior aspect of the left renal vein via a common trunk, while the right adrenal vein drains directly into the IVC via a short venous trunk.*
+
+
+*Left renal venogram shows detailed normal anatomy of the intrarenal and extrarenal veins. The branching pattern of the intrarenal veins corresponds to that of the intrarenal arterial circulation. The left kidney is drained by a single renal vein that courses across the midline to drain into the IVC opposite the right renal vein. The latter drains directly into the IVC. The left adrenal gland is drained by a single adrenal vein that joins with the inferior phrenic vein to drain into the superior aspect of the left renal vein via a common trunk, while the right adrenal vein drains directly into the IVC via a short venous trunk.*
+
+
+*Left renal venogram shows detailed normal anatomy of the intrarenal and extrarenal veins. The branching pattern of the intrarenal veins corresponds to that of the intrarenal arterial circulation. The left kidney is drained by a single renal vein that courses across the midline to drain into the IVC opposite the right renal vein. The latter drains directly into the IVC. The left adrenal gland is drained by a single adrenal vein that joins with the inferior phrenic vein to drain into the superior aspect of the left renal vein via a common trunk, while the right adrenal vein drains directly into the IVC via a short venous trunk.*
+
diff --git a/docs_md/articles/reversible-cerebral-vasoconstriction-syndrome_c3a19be2-1f91-4dcb-b63a-cbfdaeaca5c1.md b/docs_md/articles/reversible-cerebral-vasoconstriction-syndrome_c3a19be2-1f91-4dcb-b63a-cbfdaeaca5c1.md
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@@ -0,0 +1,319 @@
+---
+title: "Reversible Cerebral Vasoconstriction Syndrome"
+docid: "c3a19be2-1f91-4dcb-b63a-cbfdaeaca5c1"
+authors:
+ - key: "8d5254e9-8dda-478b-8f08-bdee97a32c79"
+ value: "Karen L. Salzman, MD, FACR"
+breadcrumbs:
+ -
+ name: "Brain"
+ slug: "brain"
+ treeNodeId: "0361042c-9c90-48e4-864e-a2e6428ee9c9"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "948c6f1e-8e18-45f3-bd9d-7a04ab0b8708"
+ -
+ name: "Pathology-Based Diagnoses"
+ slug: "pathology-based-diagnoses"
+ treeNodeId: "d60fe914-7897-46a0-9cd2-f88ffb322f16"
+ -
+ name: "Stroke"
+ slug: "stroke"
+ treeNodeId: "7a135176-0a69-4fc9-b200-59569fbf5166"
+ -
+ name: "Nonatheromatous Vasculopathy"
+ slug: "nonatheromatous-vasculopathy"
+ treeNodeId: "2ccf261b-3d7a-42a7-8041-8ed08dd81bff"
+ -
+ name: "Reversible Cerebral Vasoconstriction Syndrome"
+ slug: "reversible-cerebral-vasoconstricti-"
+ treeNodeId: null
+category: "Brain"
+documentVersionId: "b46340ff-198e-449f-bfc4-fe7974414202"
+imageCount: 17
+lastUpdated: "08/19/25"
+pageDescription: "Reversible Cerebral Vasoconstriction Syndrome"
+pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Stroke, Nonatheromatous Vasculopathy, Reversible Cerebral Vasoconstriction Syndrome"
+pageTitle: "Reversible Cerebral Vasoconstriction Syndrome | STATdx"
+enhancedTitle: "Reversible Cerebral Vasoconstriction Syndrome"
+type: "DX"
+references: true
+breadcrumbs:
+ - "Brain"
+ - "Diagnosis"
+ - "Pathology-Based Diagnoses"
+ - "Stroke"
+ - "Nonatheromatous Vasculopathy"
+ - "Reversible Cerebral Vasoconstriction Syndrome"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Reversible cerebral vasoconstriction syndrome (RCVS) a.k.a. Call-Fleming syndrome
+ - Group of disorders characterized by
+ - Reversible, multifocal cerebral artery vasoconstrictions
+ - Severe headaches ± focal neurologic deficits
+- ### Imaging
+
+
+ - Acute/recurrent headaches with vasculitic pattern (DSA)
+ - DSA = crucial for diagnosis (100% sensitive)
+ - Involves large-/medium-sized arteries
+ - Diffuse, multifocal, segmental narrowing
+ - Sometimes string of beads or sausage strings appearance
+ - NECT often negative
+ - Small cortical subarachnoid hemorrhage (SAHs) (20%) ± parenchymal hemorrhage
+ - CTA/MRA: May be normal if subtle changes (10%)
+ - Diffuse segmental arterial constriction in 90%
+ - VW-MR: May show diffuse, uniform wall thickening
+ - TCD: ↑ arterial velocities in MCA, ICA, ACA
+- ### Pathology
+
+
+ - Thought to represent transient disturbance in control of cerebral vascular tone → vasoconstriction → ischemia, stroke, death
+ - Spontaneous (1/3 of cases) or precipitated by
+ - Postpartum state
+ - Exposure to vasoactive substances
+ - Autoimmune disease; immunosuppressive therapy
+ - Associated with posterior reversible encephalopathy syndrome (PRES)
+- ### Clinical Issues
+
+
+ - Symptoms: Severe, acute thunderclap headache
+ - Often recurrent (95%)
+ - Ischemia/stroke (visual disturbance, aphasia, hemiparesis)
+ - Treatment
+ - Discontinuation of vasoactive medications
+ - Vasodilators (e.g., Ca⁺⁺ antagonists)
+
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - Reversible cerebral vasoconstriction syndrome (RCVS)
+- ### Synonyms
+
+
+ - Call-Fleming syndrome
+- ### Definitions
+
+
+ - Group of disorders characterized by
+ - Reversible, multifocal cerebral artery vasoconstriction
+ - Severe headaches (HAs) ± focal neurologic deficits
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Acute-onset, usually recurrent HA with vasculitic pattern on DSA
+- ### CT Findings
+
+
+ - #### NECT
+
+
+ - May see small cortical subarachnoid hemorrhage (SAH) in 20% of parenchymal hemorrhages
+ - Cortical ± subcortical hypodensity secondary to ischemia/stroke
+ - #### CECT
+
+
+ - Usually normal unless ischemic stroke ensues → gyriform enhancement
+ - CTA: May be normal or can see undulations of large-/medium-sized arteries
+- ### MR Findings
+
+
+ - #### FLAIR
+
+
+ - May see uni- or bilateral cortical SAH
+ - Focal regions of ↑ signal secondary to stroke
+ - #### DWI
+
+
+ - Most sensitive for regions of ischemia/stroke
+ - #### MRA
+
+
+ - May be normal if subtle changes (10%); require DSA for diagnosis
+ - Diffuse segmental arterial constriction in 90%
+ - MR-vessel wall (VW-MR)
+ - May show diffuse, uniform wall thickening
+ - No vessel wall enhancement is typical, though mild enhancement may be seen
+- ### Ultrasonographic Findings
+
+
+ - Transcranial Doppler (TCD): ↑ arterial velocities (vasospasm) + ↓ luminal diameter of middle cerebral artery (MCA), internal carotid artery (ICA), anterior cerebral artery (ACA) in 70%
+- ### Angiographic Findings
+
+
+ - Crucial for diagnosis (100% sensitive)
+ - Diffuse, multifocal, segmental narrowing of large-/medium-sized arteries
+ - Occasional dilated segments may appear like string of beads or sausage strings
+
+## DIFFERENTIAL DIAGNOSIS
+
+- [Cerebral Vasculitis](/document/primary-arteritis-of-cns/490b3aed-37e2-4ec6-95dd-76efc734490f)
+ - Similar appearance on DSA, CTA, MRA
+ - VW-MR typically shows more robust vessel wall enhancement with vessel wall abnormalities persisting longer than RCVS
+ - Clinical onset more insidious, CSF abnormalities
+ - e.g., primary angiitis of CNS (PACNS), systemic lupus, infection, sarcoidosis
+- [Subarachnoid Hemorrhage](/document/aneurysmal-subarachnoid-hemorrhage/9109b698-5ee5-49c4-ba0c-1a86f1fbede4)
+ - Aneurysm, dural fistula, trauma, arteriovenous malformation
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Etiology
+
+
+ - Thought to represent transient disturbance in control of cerebral vascular tone → vasoconstriction → ischemia, stroke
+ - #### Associated abnormalities
+
+
+ - Occurs spontaneously (1/3 of cases) or may be precipitated by
+ - Postpartum state
+ - Exposure to vasoactive substances
+ - Cannabis, cocaine, ecstasy, amphetamine derivatives, LSD
+ - Selective serotonin reuptake inhibitors (SSRIs)
+ - Nasal decongestants, pseudoephedrine
+ - Ergotamine tartrate, bromocriptine, sumatriptan
+ - Pheochromocytoma, bronchial carcinoid tumor
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - Severe, acute-onset (thunderclap) HA
+ - Often recurrent (95%)
+ - Mimics SAH secondary to ruptured aneurysm
+ - Less common: Ischemia/stroke may result in visual disturbance, aphasia, hemiparesis; seizures
+- ### Natural History & Prognosis
+
+
+ - Stroke occurs in 7-54%
+ - M:F = 1:2
+ - Up to 10% permanent disability
+ - Associated with posterior reversible encephalopathy syndrome (PRES)
+ - Associated with autoimmune diseases, immunosuppressive therapy
+ - Vascular abnormalities resolve within several months
+- ### Treatment
+
+
+ - Discontinuation of vasoactive medications
+ - Vasodilators (e.g., Ca⁺⁺ antagonists) PO/IV/IA infusion
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - RCVS in patient with history of thunderclap HA but no SAH or limited cortical SAH
+- ### Image Interpretation Pearls
+
+
+ - Interval DSA may show **improvement**with **vasodilator**Rx (i.e., IA verapamil)
+
+ 1125516a-407a-4e56-a618-1ff5a898773d
+
+## References
+
+## Selected References
+
+1. [Girfanova M et al: Retrospective analysis of 80 patients diagnosed with reversible cerebral vasoconstriction syndrome in the Helsinki Metropolitan Area. Eur J Neurol. 32(2):e16564, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=39878370%5Bpmid%5D)
+1. [Kim SA et al: Beyond the "string of beads": case-based exploration of diagnostic pitfalls and solutions in reversible cerebral vasoconstriction syndrome. J Headache Pain. 26(1):89, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=40289076%5Bpmid%5D)
+1. [Battal B et al: Imaging of reversible cerebral vasoconstriction syndrome and posterior reversible encephalopathy syndrome. Neuroimaging Clin N Am. 34(1):129-47, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=37951698%5Bpmid%5D)
+1. [Madapoosi A et al: Hemorrhagic versus non-hemorrhagic presentation of presumed reversible cerebral vasoconstriction syndrome (RCVS): presentations and outcomes. Interv Neuroradiol. 15910199241285501, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=39311023%5Bpmid%5D)
+1. [Jeanneret V et al: PRES and RCVS: two distinct entities or a spectrum of the same disease? J Stroke Cerebrovasc Dis. 31(6):106472, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35390732%5Bpmid%5D)
+1. [Ospel JM et al: Intra-arterial verapamil treatment in oral therapy-refractory reversible cerebral vasoconstriction syndrome. AJNR Am J Neuroradiol. 41(2):293-9, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31879333%5Bpmid%5D)
+1. [Sequeiros JM et al: Quantifying intra-arterial verapamil response as a diagnostic tool for reversible cerebral vasoconstriction syndrome. AJNR Am J Neuroradiol. 41(10):1869-75, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32943423%5Bpmid%5D)
+1. [Rocha EA et al: RCVS2 score and diagnostic approach for reversible cerebral vasoconstriction syndrome. Neurology. 92(7):e639-47, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30635475%5Bpmid%5D)
+1. [Chen CY et al: Vascular wall imaging in reversible cerebral vasoconstriction syndrome - a 3-T contrast-enhanced MRI study. J Headache Pain. 19(1):74, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30167985%5Bpmid%5D)
+1. [Shimoda M et al: Centripetal propagation of vasoconstriction at the time of headache resolution in patients with reversible cerebral vasoconstriction syndrome. AJNR Am J Neuroradiol. 37(9):1594-8, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27079368%5Bpmid%5D)
+1. [Calic Z et al: The reversible cerebral vasoconstriction syndrome. Intern Med J. 45(6):599-608, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25511128%5Bpmid%5D)
+1. [Kass-Hout T et al: A novel approach to diagnose reversible cerebral vasoconstriction syndrome: a case series. J Stroke Cerebrovasc Dis. 24(1):e31-7, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25440342%5Bpmid%5D)
+1. [Miller TR et al: Reversible cerebral vasoconstriction syndrome, part 1: epidemiology, pathogenesis, and clinical course. AJNR Am J Neuroradiol. 36(8):1392-9, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25593203%5Bpmid%5D)
+1. [Miller TR et al: Reversible cerebral vasoconstriction syndrome, part 2: diagnostic work-up, imaging evaluation, and differential diagnosis. AJNR Am J Neuroradiol. 36(9):1580-8, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25614476%5Bpmid%5D)
+1. [Agarwal R et al: Posterior reversible encephalopathy and cerebral vasoconstriction in a patient with hemolytic uremic syndrome. Pediatr Neurol. 50(5):518-21, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24731845%5Bpmid%5D)
+1. [Grooters GS et al: How often is thunderclap headache caused by the reversible cerebral vasoconstriction syndrome? Headache. 54(4):732-5, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24822246%5Bpmid%5D)
+1. [Katz BS et al: Clinical worsening in reversible cerebral vasoconstriction syndrome. JAMA Neurol. 71(1):68-73, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24190097%5Bpmid%5D)
+1. [Obusez EC et al: High-resolution MRI vessel wall imaging: spatial and temporal patterns of reversible cerebral vasoconstriction syndrome and central nervous system vasculitis. AJNR Am J Neuroradiol. 35(8):1527-32, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24722305%5Bpmid%5D)
+1. [Wolff V et al: Ischaemic strokes with reversible vasoconstriction and without thunderclap headache: a variant of the reversible cerebral vasoconstriction syndrome? Cerebrovasc Dis. 39(1):31-8, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25547150%5Bpmid%5D)
+1. [Muehlschlegel S et al: Differentiating reversible cerebral vasoconstriction syndrome with subarachnoid hemorrhage from other causes of subarachnoid hemorrhage. JAMA Neurol. 70(10):1254-60, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23939614%5Bpmid%5D)
+1. [Marder CP et al: Multimodal imaging of reversible cerebral vasoconstriction syndrome: a series of 6 cases. AJNR Am J Neuroradiol. 33(7):1403-11, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22422190%5Bpmid%5D)
+1. [Ducros A et al: The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain. 130(Pt 12):3091-101, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=18025032%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+*Axial NECT in a 62-year-old man with acute-onset thunderclap headache shows subarachnoid hemorrhage (SAH)
along the right parietal lobe as hyperdensity in the sulci. MR was performed and showed the SAH but no ischemia (not shown). DSA confirmed RCVS.*
+
+
+*Axial DWI trace MR in a 61-year-old with RCVS shows multiple foci of acute ischemia
, a common complication of RCVS. MR imaging mimics vasculitis. Vessel wall MR (VW-MR) &/or DSA helps to distinguish vasculitis vs. RCVS.*
+
+
+*Right ICA DSA shows multifocal arterial narrowing
. Imaging suggests vasculitis or RCVS. The narrowing improved after Verapamil infusion, typical for RCVS. A persistent trigeminal artery
is present.*
+
+
+*Axial DTI in a patient on immunosuppressants shows multifocal diffusion restriction
from recent infarcts. VW-MR showed vascular irregularity without enhancement, suggestive of RCVS (not shown). DSA was performed to evaluate for vasculitis vs. RCVS and confirmed RCVS.*
+
+
+### Additional Images
+
+
+*Axial NECT in a patient with acute-onset severe headache shows limited cortical SAH in the sulci of the right frontal lobe
. DSA was performed to exclude a vascular lesion and showed multifocal arterial narrowings (not shown).*
+
+
+*MR studies obtained 4 days after admission in the same patient when she noticed visual disturbances are shown. FLAIR MR (top) shows multifocal hyperintensities in both occipital lobes
. DWI MR (bottom) shows restricted diffusion
consistent with recent infarcts.*
+
+
+*Axial NECT in a female patient with acute-onset of severe headache shows limited cortical SAH in the sulci of the frontal lobes
. DSA was performed to exclude a vascular lesion and showed multifocal arterial narrowings (not shown) related to RCVS.*
+
+
+*AP right vertebral artery DSA shows diffuse luminal irregularity and focal stenoses involving the basilar artery and posterior cerebral and superior cerebellar arteries
. Similar changes were seen in the anterior circulation (not shown). The patient was treated with IA verapamil over the next 10 days.*
+
+
+*DSA repeated 2 weeks later shows significant interval resolution of the posterior circulation vasospasm. There are a few foci
of mild residual stenoses still evident.*
+
+
+*Axial DWI trace MR shows multiple foci of hyperintensity
related to acute ischemia in this patient with RCVS who presented with an acute onset of a "thunderclap" headache.*
+
+
+*AP left ICA injection DSA in a 41-year-old man with left frontal SAH and RCVS shows multifocal vascular stenoses, most pronounced at the left anterior cerebral artery (ACA)
distribution.*
+
+
+*Axial NECT in a 40-year-old man with acute-onset headache after cocaine and methamphetamine use shows SAH along the left frontal lobe
as hyperdensity in the sulci. Subsequently, DSA was performed and showed multifocal arterial narrowing (not shown).*
+
+
+*AP left vertebral artery DSA in a patient with focal SAH shows multifocal luminal irregularity and focal stenoses involving the posterior circulation: Basilar artery
, posterior cerebral
, and superior cerebellar arteries
. Similar changes were seen in the anterior circulation (not shown). The patient was treated with 8-mg IA verapamil.*
+
+
+*DSA repeated after IA verapamil injection shows some improvement of the posterior circulation vasospasm. Mild residual stenoses are still evident
.*
+
+
+*Right ICA DSA shows multifocal arterial narrowing
in the anterior circulation in a patient with headache and focal SAH. Imaging suggests vasculitis or RCVS. The narrowing improved after verapamil infusion, typical for RCVS. Note the persistent trigeminal artery
.*
+
+
+*Axial FLAIR MR in a thunderclap headache patient shows SAH
in the right parietal sulci. Subsequently, DSA was performed and showed multifocal arterial narrowing (not shown). RCVS is often complicated by SAH, ischemia, and, less commonly, parenchymal hemorrhage or posterior reversible encephalopathy syndrome (PRES).*
+
+
+*Right ICA oblique DSA shows multifocal, segmental narrowing
of large and medium-sized arteries. There are dilated segments with a sausage appearance
, typical for RCVS. Response to IA verapamil confirms the diagnosis.*
+
diff --git a/docs_md/articles/spontaneous-nontraumatic-intracranial-hemorrhage_15aa4f01-2d4b-45ee-a1d4-8a079150569d.md b/docs_md/articles/spontaneous-nontraumatic-intracranial-hemorrhage_15aa4f01-2d4b-45ee-a1d4-8a079150569d.md
new file mode 100644
index 0000000..2bb56fa
--- /dev/null
+++ b/docs_md/articles/spontaneous-nontraumatic-intracranial-hemorrhage_15aa4f01-2d4b-45ee-a1d4-8a079150569d.md
@@ -0,0 +1,592 @@
+---
+title: "Spontaneous Nontraumatic Intracranial Hemorrhage"
+docid: "15aa4f01-2d4b-45ee-a1d4-8a079150569d"
+authors:
+ - key: "8d5254e9-8dda-478b-8f08-bdee97a32c79"
+ value: "Karen L. Salzman, MD, FACR"
+ - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
+ value: "Anne G. Osborn, MD, FACR"
+breadcrumbs:
+ -
+ name: "Brain"
+ slug: "brain"
+ treeNodeId: "0361042c-9c90-48e4-864e-a2e6428ee9c9"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "948c6f1e-8e18-45f3-bd9d-7a04ab0b8708"
+ -
+ name: "Pathology-Based Diagnoses"
+ slug: "pathology-based-diagnoses"
+ treeNodeId: "d60fe914-7897-46a0-9cd2-f88ffb322f16"
+ -
+ name: "Stroke"
+ slug: "stroke"
+ treeNodeId: "7a135176-0a69-4fc9-b200-59569fbf5166"
+ -
+ name: "Nontraumatic Intracranial Hemorrhage"
+ slug: "nontraumatic-intracranial-hemorrha-"
+ treeNodeId: "fec768e1-2e80-4b63-a846-579ea0a9ef90"
+ -
+ name: "Spontaneous Nontraumatic Intracranial Hemorrhage"
+ slug: "spontaneous-nontraumatic-intracran-"
+ treeNodeId: null
+category: "Brain"
+documentVersionId: "8f759c71-0761-48ae-aa77-1d810236f401"
+imageCount: 39
+lastUpdated: "08/12/25"
+pageDescription: "Spontaneous Nontraumatic Intracranial Hemorrhage"
+pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Stroke, Nontraumatic Intracranial Hemorrhage, Spontaneous Nontraumatic Intracranial Hemorrhage"
+pageTitle: "Spontaneous Nontraumatic Intracranial Hemorrhage | STATdx"
+enhancedTitle: "Spontaneous Nontraumatic Intracranial Hemorrhage"
+type: "DX"
+references: true
+anatomy:
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+cases: 3
+breadcrumbs:
+ - "Brain"
+ - "Diagnosis"
+ - "Pathology-Based Diagnoses"
+ - "Stroke"
+ - "Nontraumatic Intracranial Hemorrhage"
+ - "Spontaneous Nontraumatic Intracranial Hemorrhage"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Primary intraparenchymal hemorrhage (pICH)
+ - Acute nontraumatic intracranial hemorrhage (ICH)
+- ### Imaging
+
+
+ - Acute round or oval intracerebral hematoma
+ - Subcentimeter microbleeds to massive ICH
+ - Hematoma location for common causes of pICH
+ - Hypertension (HTN): Basal ganglia > thalamus > pons > cerebellum
+ - Amyloid angiopathy: Lobar
+ - Arteriovenous malformation (AVM): Any location
+ - Cavernous malformation: Any location
+ - Venous sinus thrombosis: Subcortical white matter
+ - Neoplasm: Any location
+ - Recommended imaging protocol
+ - If HTN with striatocapsular hematoma → stop
+ - If atypical hematoma → CTA or MR/MRA
+ - Atypical hematoma or unclear history: MR (T2*, DWI, C+)
+ - If standard study suggests vascular etiology → CTA/MRA
+ - If concern for venous infarct → CTV/MRV
+- ### Pathology
+
+
+ - **Pediatric patients**, < 18 years old: Vascular malformation (~ 50%) > hematologic disorders, vasculopathy, venous infarct, neoplasm
+ - **Young adults**, < 45 years old: Vascular malformation, drug abuse, venous thrombosis, posterior reversible encephalopathy syndrome (PRES), vasculitis, reversible cerebral vasoconstriction syndrome (RCVS), neoplasm
+ - **Adults**, > 45 years old: HTN, amyloid > neoplasm (primary or metastatic), venous infarct, coagulopathy
+- ### Clinical Issues
+
+
+ - ICH causes ~ 15% of acute strokes
+ - Treatment: Control of intracranial pressure, hydrocephalus
+ - Surgical evacuation when clinically indicated
+ - If positive spot sign indicates active bleeding, predicts hematoma expansion and poor outcome
+ - 1-year mortality approaches 60%
+
+## TERMINOLOGY
+
+- ### Synonyms
+
+
+ - Primary intraparenchymal hemorrhage (pICH), hemorrhagic stroke
+- ### Definitions
+
+
+ - Acute nontraumatic intracranial hemorrhage (ICH)
+ - Etiology often initially unknown
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Acute nontraumatic intracerebral hematoma
+ - #### Location
+
+
+ - Varies with etiology
+ - Hypertension (HTN): Deep gray matter [basal ganglia (BG), thalamus], pons, cerebellar hemisphere
+ - Amyloid angiopathy: Lobar
+ - Arteriovenous malformation (AVM): Any location
+ - Cavernous malformation (CM): Any location, common in brainstem
+ - Venous sinus thrombosis: Subcortical white matter (WM) adjacent to occluded sinus
+ - Neoplasm: Any location, posterior fossa common
+ - #### Size
+
+
+ - Subcentimeter microbleeds to massive hemorrhage
+ - #### Morphology
+
+
+ - Typically round or oval; often irregular when large
+ - Patterns with HTN and amyloid angiopathy
+ - Acute parenchymal hematoma
+ - Multiple subacute/chronic microbleeds in deep gray matter (HTN > amyloid) &/or subcortical WM (amyloid > HTN)
+ - Microbleeds often seen only on GRE or SWI MR
+- ### CT Findings
+
+
+ - #### NECT
+
+
+ - Acute hyperdense round/elliptical mass with surrounding low-density edema
+ - Hematoma size = ABC/2 (width x length x height/2)
+ - Hematoma may be mixed iso-/hyperdense
+ - May have fluid-fluid level
+ - Coagulopathy
+ - Brisk bleeding
+ - Bleed into cystic mass
+ - Deep (BG, thalamus) ICH may rupture into ventricles
+ - #### CTA
+
+
+ - Often normal or shows displaced vessels from mass effect
+ - Spot sign shows acute contrast extravasation
+ - ± underlying vascular malformation (AVM, aneurysm)
+ - Look for dural sinus venous thrombosis
+- ### MR Findings
+
+
+ - #### T1WI
+
+
+ - Hyperacute (< 24 hours)
+ - Isointense center (oxygenated Hgb)
+ - Isointense periphery (deoxygenated Hgb, clot-tissue interface)
+ - Hypointense rim (vasogenic edema)
+ - #### T2WI
+
+
+ - Hyperacute (< 24 hours)
+ - Hyperintense, heterogeneous center
+ - May have subtle hypointense periphery
+ - Hyperintense rim of edema
+ - #### T2* GRE
+
+
+ - Hypointense
+ - Multifocal hypointense lesions ("black dots") T2*/SWI
+ - BG and thalami suggest HTN
+ - Subcortical WM suggests amyloid angiopathy
+ - #### DWI
+
+
+ - Hyperacute: Bright; acute/early subacute: Dark; late subacute: Bright; chronic: Dark
+ - T2 shine-through common; may see "DWI restriction" in core
+ - #### T1WI C+
+
+
+ - Often none in acute hematoma
+ - May enhance if underlying neoplasm, vascular malformation
+ - May see enhancement in subacute hematoma
+ - MRA: Often normal; look for vascular lesion
+ - MRV: Look for dural venous sinus thrombosis
+- ### Angiographic Findings
+
+
+ - DSA, often negative
+ - Look for dural sinus occlusion, "stagnating vessels" (thrombosed AVM)
+- ### Imaging Recommendations
+
+
+ - #### Best imaging tool
+
+
+ - Screening: NECT
+ - If patient with HTN and BG hematoma → stop
+ - Consider CTA to exclude underlying vascular lesion
+ - Standard MR (include T2*, SWI, DWI)
+ - If no clear cause of hemorrhage or atypical appearance on CT
+ - If T2*/SWI shows multifocal "black dots" → stop
+ - T1WI C+ to assess for underlying tumor
+ - If standard study suggests vascular etiology → MRA/CTA
+ - Follow-up: Repeat MR if etiology unclear ± DSA if initial MRA/CTA negative
+ - #### Protocol advice
+
+
+ - Atypical hematoma or unclear history: MR (with T2*, SWI, DWI, T1WI C+)
+ - Add MRV if concern for venous infarct
+
+## DIFFERENTIAL DIAGNOSIS
+
+- [Hypertensive Intracranial Hemorrhage](/document/hypertensive-intracranial-hemorrha-/6f6d2768-06ba-4eae-93cf-3b639800522c)
+ - Patients usually older
+ - BG hematoma most common location
+- [Vascular Malformation](/document/arteriovenous-malformation/84792183-949d-4b25-a586-572dbe2e11a2)
+ - AVM, CM most common
+ - ICH rate in AVMs of BG or thalamus (9.8% per year) much higher than AVMs in other locations (3%)
+ - High risk of incurring neurological deficit with each hemorrhagic event
+- [Cerebral Amyloid Angiopathy](/document/cerebral-amyloid-disease/18edc9f3-9218-410c-8280-29c3e6df4c91)
+ - Older patients (70 years old, normotensive)
+ - Usually lobar hemorrhage
+ - Microbleeds ("black dots") on T2*/SWI peripheral location
+- [Underlying Neoplasm](/document/parenchymal-metastases/2cf0bd40-4597-4c0b-83e4-74340304b98f)
+ - Causes 2-15% of nontraumatic ICHs
+ - Primary (glioblastoma) or metastasis (i.e., renal cell, melanoma)
+ - Typically shows enhancement
+- [Venous Thrombosis](/document/cortical-venous-thrombosis/8efaec3c-b3ac-46e2-9da8-6c68880f8236)
+ - May result in hemorrhagic venous infarct
+ - Risk factors: Dehydration, pregnancy, oral contraceptives
+- ### Anticoagulation
+
+
+ - "Growing" hematoma, fluid-fluid levels common
+ - Check history
+- [Drug Abuse](/document/drug-abuse/48859403-0b26-44d8-ba74-e0919e4c3147)
+ - May have hypertensive striatocapsular hemorrhage
+ - Uncommon = pseudoaneurysm rupture into cerebrum
+- [Vasculitis](/document/miscellaneous-vasculitis/221f737a-6bfa-4331-b296-402e40973f7e)
+ - Often results in subarachnoid hemorrhage (SAH) rather than ICH, ± small infarcts
+ - Patients usually younger
+- ### Reversible Cerebral Vasoconstriction Syndrome
+
+
+ - Reversible, multifocal cerebral artery vasoconstriction
+ - Acute thunderclap headache with SAH ± small strokes
+- [Dural Arteriovenous Fistula](/document/dural-av-fistula/dd83ba51-7fd9-406f-9950-9a944c02c291)
+ - Dilated venous flow voids
+ - Often posterior fossa and skull base
+ - Hemorrhage in those with cortical venous drainage
+- [Ruptured Pseudoaneurysm](/document/pseudoaneurysm/1df27abc-b596-45bc-aba5-441253f39327)
+ - Mycotic (endocarditis)
+ - Traumatic
+ - Vasculopathy
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Etiology
+
+
+ - **Pediatric** patients, < 18 years old: Vascular malformation (~ 50%) > hematologic disorders, vasculopathy, venous infarct, neoplasm
+ - **Young adults**, < 45 years old: Vascular malformation, drug abuse, venous thrombosis, posterior reversible encephalopathy syndrome (PRES), vasculitis, reversible cerebral vasoconstriction syndrome (RCVS), neoplasm
+ - **Adults**, > 45 years old: HTN, amyloid > neoplasm (primary or metastatic), venous infarct, coagulopathy > vascular malformation [usually dural arteriovenous fistula (dAVF)]
+ - #### Genetics
+
+
+ - *MMP-9*, cytokine gene expression ↑ after acute spontaneous ICH
+ - Apolipoprotein E (*APOE*) gene and its ε2 and ε4 alleles are strongly associated with ICH
+ - **Multiple spontaneous ICH**
+ - **Children/young adults**: CMs, hematologic disorder/malignancy
+ - **Middle-aged/older adults**: HTN, amyloid angiopathy > hemorrhagic metastatic disease, coagulopathy, anticoagulation
+ - **All ages**: Venous infarcts > PRES, vasculitis, septic emboli > thrombotic microangiopathy, acute hemorrhagic leukoencephalopathy
+- ### Staging, Grading, & Classification
+
+
+ - Clinical "ICH score" correlates with 30-day mortality
+ - Admission Glasgow Coma Scale
+ - > 80 years old, ICH volume
+ - Infratentorial
+ - Presence of intraventricular hemorrhage
+- ### Gross Pathologic & Surgical Features
+
+
+ - Findings range from petechial microbleeds to gross parenchymal hematoma
+- ### Microscopic Features
+
+
+ - Coexisting microangiopathy common in amyloid, HTN
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - 90% of patients with recurrent pICH are hypertensive
+ - Large ICHs present with sensorimotor deficits, impaired consciousness
+- ### Demographics
+
+
+ - Age: Any age
+ - Epidemiology: Parenchymal hemorrhage causes ~ 10-15% of acute strokes
+- ### Natural History & Prognosis
+
+
+ - Prognosis related to location, size of ICH
+ - Hematoma enlargement common in first 24-48 hours
+ - Risk factors: EtOH, coagulopathy, HTN
+ - ~ 25-40% of patients
+ - 20-30% of patients die within 48 hours despite intervention
+ - Edema associated with poor outcome
+ - Mortality: 30-55% in 1st month
+ - 1-year mortality approaches 60%
+ - 30% rebleed within 1 year
+ - Most survivors have significant deficits
+ - Incidence ~ 25 per 100,000 per year worldwide
+ - Spot sign indicates active bleeding, predicts hematoma expansion and poor outcome
+ - Death or dependent state is outcome in > 70% of patients
+- ### Treatment
+
+
+ - Control of intracranial pressure (ICP), hydrocephalus
+ - Surgical evacuation when clinically indicated
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - Consider underlying etiology for hemorrhage (AVM, amyloid, neoplasm, drug use, etc.)
+- ### Image Interpretation Pearls
+
+
+ - Unexplained ICH → search for microbleeds on T2*/SWI MR
+ - Fluid-fluid level, iso-/mildly hyperdense clot may indicate coagulopathy
+
+ 1a7a1222-d6a1-436c-b2a7-5ace924ce719
+
+## References
+
+## Selected References
+
+1. [Horn M et al: Timing of spot sign appearance, spot sign volume, and leakage rate among phases of multiphase CTA predict intracerebral hemorrhage growth. AJNR Am J Neuroradiol. 45(6):693-700, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38782592%5Bpmid%5D)
+1. [Tartarin H et al: Uncommon causes of nontraumatic intracerebral hemorrhage. Stroke. 55(5):1416-27, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38572651%5Bpmid%5D)
+1. [Camargo A et al: Value of CTA/MRA in the setting of intraparenchymal hemorrhage in the emergency department. Neuroradiology. 65(1):97-103, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36385589%5Bpmid%5D)
+1. [Ducroux C et al: NCCT markers of intracerebral hemorrhage expansion using revised criteria: an external validation of their predictive accuracy. AJNR Am J Neuroradiol. 44(6):658-64, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37169542%5Bpmid%5D)
+1. [Szidonya L et al: Cerebral amyloid angiopathy. Radiol Clin North Am. 61(3):551-62, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36931769%5Bpmid%5D)
+1. [Anderson E et al: Prognosis and futility in neurosurgical emergencies: a review. Clin Neurol Neurosurg. 195:105851, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32422469%5Bpmid%5D)
+1. [Beer-Furlan A et al: Endovascular management of symptomatic intracranial pseudoaneurysm and intimal flow-limiting dissection with a single device. World Neurosurg. ePub, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32492541%5Bpmid%5D)
+1. [Hoffman H et al: Prediction of mortality after evacuation of supratentorial intracerebral hemorrhage using NSQIP data. J Clin Neurosci. 77:148-56, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32376154%5Bpmid%5D)
+1. [Ironside N et al: Fully automated segmentation algorithm for perihematomal edema volumetry after spontaneous intracerebral hemorrhage. Stroke. 51(3):815-23, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32078476%5Bpmid%5D)
+1. [Nawabi J et al: Inter- and intrarater agreement of spot sign and noncontrast CT markers for early intracerebral hemorrhage expansion. J Clin Med. 9(4), 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32260409%5Bpmid%5D)
+1. [Patel VD et al: Natural history of infratentorial intracerebral hemorrhages: two subgroups with distinct presentations and outcomes. J Stroke Cerebrovasc Dis. 104920, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32423853%5Bpmid%5D)
+1. [Sembolini A et al: Acute hematoma expansion after spontaneous intracerebral hemorrhage: risk factors and impact on long-term prognosis. Neurol Sci. 41(9):2503-9, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32215850%5Bpmid%5D)
+1. [Wu J et al: Emergency surgery is an effective way to improve the outcome of severe spontaneous intracerebral hemorrhage patients on long-term oral antiplatelet therapy. Neurosurg Rev. 44(2):1205-16, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32468316%5Bpmid%5D)
+1. [Ng D et al: The CT swirl sign is associated with hematoma expansion in intracerebral hemorrhage. AJNR Am J Neuroradiol. 39(2):232-7, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29217744%5Bpmid%5D)
+1. [Rammos SK et al: Aneurysms associated with brain arteriovenous malformations. AJNR Am J Neuroradiol. 37(11):1966-71, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27339951%5Bpmid%5D)
+1. [Alexander MD et al: Association between venous angioarchitectural features of sporadic brain arteriovenous malformations and intracranial hemorrhage. AJNR Am J Neuroradiol. 36(5):949-52, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25634722%5Bpmid%5D)
+1. [Kranz PG et al: Spontaneous brain parenchymal hemorrhage: an approach to imaging for the emergency room radiologist. Emerg Radiol. 22(1):53-63, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=24894555%5Bpmid%5D)
+1. [Miller TR et al: Reversible cerebral vasoconstriction syndrome, part 1: epidemiology, pathogenesis, and clinical course. AJNR Am J Neuroradiol. 36(8):1392-9, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25593203%5Bpmid%5D)
+1. [Sampath Kumar NS et al: Multiple spontaneous hypertensive intracerebral hemorrhages. J Stroke Cerebrovasc Dis. 24(1):e25-7, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25541521%5Bpmid%5D)
+1. [Ciura VA et al: Nontraumatic acute intraparenchymal hemorrhage: algorithm for workup and differential diagnosis. Semin Roentgenol. 49(1):112-26, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24342680%5Bpmid%5D)
+1. [Koivunen RJ et al: Predictors of early mortality in young adults after intracerebral hemorrhage. Stroke. 45(8):2454-6, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24947290%5Bpmid%5D)
+1. [Caceres JA et al: Intracranial hemorrhage. Emerg Med Clin North Am. 30(3):771-94, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22974648%5Bpmid%5D)
+1. [Gazzeri R et al: Minimal craniotomy and matrix hemostatic sealant for the treatment of spontaneous supratentorial intracerebral hemorrhage. J Neurosurg. 110(5):939-42, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19061356%5Bpmid%5D)
+1. [Hanley DF: Intraventricular hemorrhage: severity factor and treatment target in spontaneous intracerebral hemorrhage. Stroke. 40(4):1533-8, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19246695%5Bpmid%5D)
+1. [Jeffree RL et al: Warfarin related intracranial haemorrhage: a case-controlled study of anticoagulation monitoring prior to spontaneous subdural or intracerebral haemorrhage. J Clin Neurosci. 16(7):882-5, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19342242%5Bpmid%5D)
+1. [Kumar R et al: Spontaneous intracranial hemorrhage in children. Pediatr Neurosurg. 45(1):37-45, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19221461%5Bpmid%5D)
+1. [Lou M et al: The relationship between hematoma iron content and perihematoma edema: an MRI study. Cerebrovasc Dis. 27(3):266-71, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19202331%5Bpmid%5D)
+1. [Tejero MA et al: [Multiple spontaneous cerebral haemorrhages. Description of a series and review of the literature.] Rev Neurol. 48(7):346-8, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19319814%5Bpmid%5D)
+1. [van Beijnum J et al: Outcome after spontaneous and arteriovenous malformation-related intracerebral haemorrhage: population-based studies. Brain. 132(Pt 2):537-43, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19042932%5Bpmid%5D)
+1. [Walsh M et al: Developmental venous anomaly with symptomatic thrombosis of the draining vein. J Neurosurg. 109(6):1119-22, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=19035729%5Bpmid%5D)
+1. [Harden SP et al: Cranial CT of the unconscious adult patient. Clin Radiol. 62(5):404-15, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17398264%5Bpmid%5D)
+1. [Chao CP et al: Cerebral amyloid angiopathy: CT and MR imaging findings. Radiographics. 26(5):1517-31, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16973779%5Bpmid%5D)
+1. [Finelli PF: A diagnostic approach to multiple simultaneous intracerebral hemorrhages. Neurocrit Care. 4(3):267-71, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16757837%5Bpmid%5D)
+1. [Leach JL et al: Imaging of cerebral venous thrombosis: current techniques, spectrum of findings, and diagnostic pitfalls. Radiographics. 26 Suppl 1:S19-41; discussion S42-3, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=17050515%5Bpmid%5D)
+1. [Thanvi B et al: Sporadic cerebral amyloid angiopathy--an important cause of cerebral haemorrhage in older people. Age Ageing. 35(6):565-71, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16982664%5Bpmid%5D)
+1. [Abilleira S et al: Matrix metalloproteinase-9 concentration after spontaneous intracerebral hemorrhage. J Neurosurg. 99(1):65-70, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12854746%5Bpmid%5D)
+1. [Fewel ME et al: Spontaneous intracerebral hemorrhage: a review. Neurosurg Focus. 15(4):E1, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=15344894%5Bpmid%5D)
+1. [Kalaria RN et al: Introduction: Non-atherosclerotic cerebrovascular disorders. Brain Pathol. 12(3):337-42, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12146802%5Bpmid%5D)
+1. [Skidmore CT et al: Spontaneous intracerebral hemorrhage: epidemiology, pathophysiology, and medical management. Neurosurg Clin N Am. 13(3):281-8, v, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12486918%5Bpmid%5D)
+1. [Qureshi AI et al: Spontaneous intracerebral hemorrhage. N Engl J Med. 344(19):1450-60, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11346811%5Bpmid%5D)
+1. [Roob G et al: Magnetic resonance imaging of cerebral microbleeds. Curr Opin Neurol. 13(1):69-73, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10719653%5Bpmid%5D)
+
+## Anatomy
+
+### Sensorimotor Network
+Brain/ANATOMY:e1563250-4c6c-4730-bbad-4430ae16a5c6
+
+### White Matter Tracts
+Brain/ANATOMY:846101a2-e892-4c70-9a32-c9fa887d073a
+
+### Basal Ganglia
+Brain/ANATOMY:a9de3815-ec59-4c78-adf0-94974065a7e3
+
+### Limbic System
+Brain/ANATOMY:f2a117ed-9429-441d-baa0-5e99e05722ac
+
+### Language Overview
+Brain/ANATOMY:40f2ed79-0d31-4943-aaa2-7c3244a7e87b
+
+### Limbic Network
+Brain/ANATOMY:e1a20b61-b2c1-44c5-ba04-59843855bfef
+
+### Memory Overview
+Brain/ANATOMY:40e2e25f-421b-4653-94e3-641b09b47e4d
+
+### Brainstem Overview
+Brain/ANATOMY:bf889ffb-646b-429d-8699-d9bd34e8e193
+
+### Cerebellar Overview
+Brain/ANATOMY:b624e76f-15b9-4c1f-910e-8f5581ac2cfe
+
+### Pons
+Brain/ANATOMY:fc185efd-1f7a-4804-95f6-a49d0fa8aefb
+
+### Gyral/Sulcal Anatomy
+Brain/ANATOMY:849da2a0-4a32-4a07-8f00-c69291e59434
+
+### Gyral/Sulcal Anatomy
+Brain/ANATOMY:299a5990-1805-4018-85b5-191d8416385b
+
+### Brain
+Ultrasound/ANATOMY:080771c2-02f3-408d-ad70-04a80d849500
+
+### Thalamus
+Brain/ANATOMY:b7f0cd6b-4ba2-4ed4-8dd0-5ca56d9ae92b
+
+## Cases
+
+- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '8eac95b8-8e45-48d0-a98d-432263728d5d', 'description': 'In this case, an axial NECT scan (#1) shows what appears to be a classic basal ganglionic hemorrhage that has dissected into the lateral ventricles. Because the patient was only mildly hypertensive, digital subtraction angiography (#2) was performed to look for an underlying vascular lesion. Selective injection of the right internal carotid artery shows a large avascular mass in the deep basal ganglia with a "round" shift of the anterior cerebral artery across the midline. Closer inspection shows a tangle of abnormal arteries (arrow) fed by a very large, medially displaced anterior choroidal artery. A mostly thrombosed arteriovenous malformation was confirmed at surgery.\n\nComment: When a middle-aged or younger patient has a spontaneous basal ganglia hemorrhage that looks like classic hypertensive striatocapsular hemorrhage, predisposing or underlying etiologies must be considered. Hypertensive hemorrhage from drug abuse is increasingly common.', 'history': 'Acute onset left hemiparesis, followed by coma.', 'imagePoolId': '0f3cbf2e-9b5c-48d5-8e6d-9bba1fcc870f', 'name': 'Mimic', 'teachingPoint': None, 'demographics': '46 Years old female'}], 'caseType': 'Other', 'name': 'OTHER'}
+- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '1b73f09a-1d13-4e32-a80f-d05e70aafdf9', 'description': 'NECT scan shows hypodense mass centered in the putamen/external capsule (arrows, #1). A punctate area of hyperdensity is seen within the mass (open arrow, #1). Sagittal (#3) and coronal (#4,5) images from the CTA obtained after the NECT scan show faint rim enhancement around the lesion (arrows) and a hyperdense linear mass within the lesion (open arrows) that appears to extend from a lenticulostriate artery (curved arrow).\n\nMR was obtained. Axial T1WI (#6) shows the mass (arrow) is a subacute hemorrhage. Note hypointensity within the mass (open arrow) that corresponds to the hyperdense lesion seen on CTA. The mass (arrows) remains mostly hyperintense on T2WI (#7) and FLAIR (#8) and is surrounded by a hypointense rim. The "dot" within the mass--a subacute hematoma (open arrow, #7,8) --remains hypointense. It "blooms" on T2*GRE (open arrow, #9), as does the hemosiderin rim surrounding the clot (arrow). DWI (#10) and ADC (#11) show typical changes of evolving hemorrhage. The enhanced scans (#12,13) show some rim enhancement around the subacute hematoma (arrows).', 'history': 'Hypertensive patient with several days of decreased right-sided motor function.', 'imagePoolId': '2fd06d58-0180-40df-852d-5584932259a0', 'name': 'Charcot-Bouchard aneurysms', 'teachingPoint': 'Findings on both CT and MR are those of a classic late subacute/early chronic hypertensive hemorrhage. What is most interesting is the identification of the small tubular structure within it, suggesting this is a thrombosed Charcot-Bouchard aneurysm, sometimes called a "bleeding globe." These are occasionally identified at autopsy but rarely seen on imaging studies.', 'demographics': '57 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'f6502fd1-f002-4c48-8bd5-bc53e0329515', 'description': 'Axial NECT (image 1) shows an unusual hyperdense mass in the left temporal lobe that is causing edema and uncal herniation. Axial precontrast T1 (image 2) and T2-weighted (image 3) MR scans show the mass has heterogeneous signal intensity. Postcontrast axial scans (images 4, 5) show the mass has some rim-enhancement around the hematoma (arrows) as well as a solid enhancing nodule (image 4, open arrow). A second, unsuspected enhancing mass is present in the right temporal lobe (image 4, curved arrow). Biopsy disclosed a metastatic spindle cell carcinoma, primary unknown.', 'history': 'Sudden onset of right-sided weakness, left pupil-involving third nerve palsy.', 'imagePoolId': '2b3f0919-d5cb-4bc9-8a72-334ec0c565fd', 'name': 'Metastases', 'teachingPoint': "Both primary and metastatic brain tumors can cause spontaneous intracranial hemorrhage (ICH) and should be considered in older, normotensive, nondemented patients who present with unexplained cerebral hemorrhage. This case is unusual because of the patient's relatively young age.", 'demographics': '34 Years old female'}, {'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}], 'caseVersionId': '0be5dde9-dd3d-402c-a759-f07258779dec', 'description': 'CT images (#1,2) show hyperdense hematoma is present at site of GBM (arrows). Surrounding hypodensity is from vasogenic edema (open arrows). Initial MR shows clot is mostly iso-/hypointense on T1 (arrow, #3) and heterogeneously hyperintense on T2WI (arrow, #4). Edema is hypointense on T1WI and hyperintense on T2WIs (curved arrows).\n\nFollow-up imaging several days later demonstrates the typical aging appearance of intracellular methemoglobin as T1 hyperintense (arrow, #5) and T2 hypointense (arrow, #6) with peripheral edema (curved arrows). Rim enhancement is seen on T1C+ scans (open arrows, #7-9) along with persisting edema (curved arrows). Some of the enhancement can be attributed to the underlying GBM. Diffusion at this stage (#10) simply represents the same hypointense findings as that seen on T2 (#6).\n\nAs expected, surrounding vasogenic edema is hypointense on T1 and hyperintense on T2 (curved arrows) and does not restrict with diffusion.', 'history': 'Patient with known glioblastoma multiforme (GBM).', 'imagePoolId': 'e89f2d31-5b26-45c8-a9be-5a53629d947b', 'name': 'Tumor hematoma', 'teachingPoint': 'This is the typical appearance of an aging cerebral hematoma secondary to GBM hemorrhage.'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '942eae44-9af9-44df-bc97-c4016915748d', 'description': 'Axial T1WI (#1) shows an odd lesion in the left centrum semiovale (arrows). It is mildly hyperintense but inhomogeneous. On T2WI (#2) it is mixed hypo- and hyperintense. Again, the inhomogeneity is rather striking. Axial T1 C+ scan (#3) shows some very mild enhancement around the margins of the lesion (arrows). The coronal scan (#4), obtained slightly later, is very revealing. It shows an unsuspected enhancing lesion in the right hemisphere (arrow).', 'history': 'Poorly controlled hypertension, right-sided weakness increasing over 2 days.', 'imagePoolId': 'ece0a824-513a-47b3-be78-b06872d5edfb', 'name': 'Inhomogeneous clot', 'teachingPoint': 'The clot was evacuated and the right-sided lesion was biopsied. Metastatic melanoma was found. The patient had had a small melanoma resected many years before but had not reported it to the ER team. The lesson here is that although the history was suggestive of hypertensive hemorrhage, the MR was atypical by being very inhomogeneous and suspicious-appearing. In such cases the use of contrast may be definitive.', 'demographics': '62 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'ad4df766-126a-4712-b0d8-9c3027c1973a', 'description': 'Axial NECT (#1) shows large left posterior temporal lobe hematoma (arrow) with edema, mass effect. Brain appears diffusely swollen, with obliterated sulci. Subtle subarachnoid hemorrhage present in left sylvian fissure (open arrow). Emergency MR was obtained. Axial T1WI (#2) shows a mixed signal intensity mass (arrows). Adjacent brain is swollen and there is evidence for early uncal and hippocampal herniation (open arrows). FLAIR scan (#3) shows moderate edema surrounding the clot (arrow) and confirms presence of subarachnoid hemorrhage (open arrows). Axial T1 C+ scan (#4) shows no abnormal enhancement. Coronal scan (#5) shows empty delta sign of nonenhancing thrombus surrounded by enhancing dura of left transverse sinus (arrow).', 'history': 'Postpartum headache, followed by seizure.', 'imagePoolId': '1c6e8746-a1f6-44d5-8651-02ba41ab1b8b', 'name': 'Large temporal lobe hematoma', 'teachingPoint': 'Dural sinus and cortical vein thrombosis are known risks in the postpartum hypercoagulable period. In this case, the thrombosed left transverse sinus caused a large spontaneous intracranial hemorrhage in the adjacent temporal lobe. These are often caused by concomitant occlusion of a dominant anastomotic vein of Labbe.', 'demographics': '24 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'd0869488-2889-4c5c-a4da-27f785830e74', 'description': 'Axial NECT scan (image 1) shows hemorrhage in the right parietal lobe. The venous phase from a selective right internal carotid angiogram is shown in image 2. The right transverse and sigmoid sinus do not opacify and there is collateral drainage into the cavernous sinus and clival venous plexus (arrows). Dural sinus thrombosis may cause spontaneous parenchymal hemorrhage.', 'history': 'This young woman with severe headache presented in the emergency room. There is no history of trauma or drug abuse.', 'imagePoolId': 'f8afb207-8be3-44a6-8bd1-b2b16b3ac4c4', 'name': 'Vein Occlusion', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'd95d6987-e865-42b4-a3b6-c3a498bea81b', 'description': 'Axial NECT scan (#1) shows a high density "cast" of the lateral ventricles, caused by intraventricular hemorrhage. No parenchymal abnormalities were identified. AP (#2) and lateral (#3) views of the selective left internal carotid angiogram performed in this patient show a tightly-packed tangle of vessels in the cingulate gyrus (arrows). Note an "early draining" vein (open arrow) with a contrast meniscus around a filling defect (curved arrow), probably in the vein of Galen.', 'history': 'Sudden onset of severe headache, followed by loss of consciousness. No history of hypertension, drug abuse, or other predisposing factor identified.', 'imagePoolId': 'af4c7949-3c20-469c-bfd9-4d3255b7b0de', 'name': 'AVM with IVH', 'teachingPoint': 'Spontaneous, nontraumatic intraventricular hemorrhage (IVH) in a normotensive young adult is uncommon. In this case, a previously undiagnosed AVM was present. When the draining vein spontaneously thrombosed, the intranidal AVM pressure rose and caused the hemorrhage.', 'demographics': '35 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'da464bb3-4293-4962-8c4f-ce7e6b3f508a', 'description': 'Axial NECT scans (#1, 2) show a focal hematoma in the right frontal lobe. There is significant surrounding edema and mass effect on the right lateral ventricle. Sagittal T1WI (#3) shows a very mixed signal mass with some hyperintense foci mixed into a predominately isointense mass (arrows). A series of axial T2WIs (#4-6) show the mass is largely hypointense, but with some hyperintense areas and a definite fluid-fluid level (#4, 6, arrows). DWI (#7) shows no restriction (a small amount of T2 "shine through" from the edema is seen peripheral to the mass). Postcontrast T1WIs (#8-10) show a "rind" of enhancement surrounding the mass. Glioblastoma multiforme (GBM) was found at surgery.', 'history': 'Older adult normotensive patient functioning normally until sudden onset of severe headache, collapse, with left hemiparesis.', 'imagePoolId': '0ab9e185-8db7-4049-967e-367d707e6884', 'name': 'Underlying GBM', 'teachingPoint': 'Spontaneous ICH in a normotensive nondemented elderly patient, particularly when the clot shows very mixed signal intensities, should raise the suspicion of underlying neoplasm. The enhancing rim is typical of most primary GBMs. GBM is the most common intracranial neoplasm to hemorrhage. Other considerations would include solitary metastasis.'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '067d9a70-e50a-4308-bdb0-672a817f968c', 'description': 'A series of NECT scans (#1-8) show a hyperdense clot centered in the left external capsule and putamen (open arrows, #1-5, 8). Some intraventricular hemorrhage is present (arrows, #1-5). Moderate mass effect with left to right subfalcine herniation of the 3rd and lateral ventricles is seen. The mixed density of the clot, appreciated especially well on images #4-6, indicates some active bleeding in the clot. A fluid-fluid level is seen (curved arrow, #6). The site where the clot probably ruptured deep into the left lateral ventricle is seen on (arrow, #7).', 'history': 'Patient with untreated hypertension presented in the ER with "stroke," manifested as sudden onset of right-sided weakness.', 'imagePoolId': 'e872f238-ce30-4f42-b89b-a9ff159a05f6', 'name': 'With intraventricular blood', 'teachingPoint': None, 'demographics': '72 Years old female'}], 'caseType': 'typical', 'name': 'TYPICAL'}
+- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '5b646ce4-5ce2-4f4e-97a6-a368e9fd656c', 'description': 'Initial NECT scan (#1,2) was normal. Repeat scan 24 hours later (#3-6) showed interval development of cortical hemorrhages, mostly in the high right posterior frontal lobe (arrows). Because of continued clinical deterioration, a third scan was obtained another 24 hours later. Scan (#7-11) shows slightly more hemorrhage, especially on the most superior image (arrows, #11). \n\nBecause the clinical deterioration was disproportionate to the interval change on CT, an MR was obtained. Axial T2WIs (#12-14) and FLAIR scans (#15-17) are notable for not just the hemorrhage (open arrows) but multifocal hyperintensities within the cortex of both cerebral hemispheres (arrows). Some subarachnoid hemorrhage is visible (#17, curved arrows). T2*GRE images (#18,19) show the confluent large cortical hemorrhages (open arrows) but with a few exceptions (arrows) most of the small peripheral lesions are nonhemorrhagic.\n\nDWI scans (#20-25) really "tell the story." Note multiple foci of diffusion restriction through the cortex of both hemispheres (arrows), most striking on images #21-25 where they are too numerous to count. These represent multiple areas of cortical ischemia caused by occlusion of small arteries and capillaries.', 'history': 'Very obese patient with several days of flu-like symptoms prompted visit to an Insta-Care outpatient facility where patient was found to have pneumonia, anemia (leukopenic or pancytopenic). He refused treatment but after continued fevers and chills his family found him with altered mental status and brought him to the ER. White count was 9.3 with 23% bands, hematocrit 31%, platelets 22,000. He was treated for beta streptococcal pneumonia and sepsis. Mental status continued to deteriorate and he became unresponsive.\n\n', 'imagePoolId': '407bb55e-acf1-4d4d-abf6-68ba718e5923', 'name': 'Disseminated intravascular coagulopathy', 'teachingPoint': 'Disseminated intravascular coagulopathy was diagnosed. Thrombotic microangiopathy with thrombocytopenia and intravascular hemolysis are characteristic of three disorders: Malignant hypertension (MH), disseminated intravascular coagulation (DIC) and thrombocytopenic thrombotic purpura (TTP).', 'demographics': '64 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '9801dbf5-4fda-4550-8903-9caa04ec02da', 'description': 'Axial T1WI shows a large mixed signal intensity left temporal/parietal mass (arrows, #1). T1 shortening (open arrow) indicates hemorrhage. A cystic pineal gland is incidentally noted (curved arrow). FLAIR (#2) shows diffuse hyperintensity with focal areas of even more striking hyperintensity (open arrow). Axial (#3) and coronal (#4) T1C+ scans show irregular rim enhancing mass (arrows) with adjacent hypointense edema.\n\nThe DWI (#5) and ADC (#6) images show restriction characteristic of acute infarction (arrows). Pathology specimen showed extensive areas of vascular thrombosis both within the tumor and adjacent cortex.', 'history': 'Five month history of cognitive problems, difficulty with concentration and reading comprehension had sudden onset of stroke-like symptoms.', 'imagePoolId': '23bf4090-7509-41df-9d28-781b819b658d', 'name': 'Intratumoral hemorrhage, cortical infarct', 'teachingPoint': 'Approximately 2% of primary brain tumors present with stroke-like symptoms, either from intratumoral bleeding (most common) or frank cerebral ischemia-infarction.', 'demographics': '61 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '950335f4-8122-4711-99fe-dc16a2e2138d', 'description': 'Axial NECT scans (#1-5) show intraventricular as well as right frontal intraparenchymal hemorrhage. Note absence of subarachnoid hemorrhage. Because the location was atypical for hypertensive bleed, CTA was performed. MIP images (#6, 7) showed an anterior communicating artery aneurysm (arrows), especially well demonstrated on the shaded surface display (#8). Conventional DSA (#9-12) demonstrated the aneurysm (arrows) which was subsequently clipped. \n\nSpontaneous intraparenchymal and intraventricular hemorrhage without subarachnoid hemorrhage is an uncommon presentation of a saccular aneurysm. When the hemorrhage is atypical for hypertension, further investigation is warranted as happened in this case.', 'history': 'Elderly hypertensive patient presented in the ER with severe headache and loss of consciousness.', 'imagePoolId': '2898c70a-30b8-45d0-a245-6658dd147e33', 'name': 'Intraventricular hemorrhage', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '8177d0f1-a552-42a4-8f3d-ef76acd2078e', 'description': 'Axial NECT scans (#1,2) show a high density mass in the vermis. Cerebral angiogram was then performed. Lateral (#3,4) and AP (#5,6) views of the vertebrobasilar angiogram demonstrate an avascular mass effect with an enlarged posterior meningeal artery (arrow, #4) that communicates directly with the adjacent transverse sinus (open arrows). Note filling defect (curved arrow, #4, 6) in the transverse sinus. At surgery, a small dural arteriovenous fistula was identified with thrombus filling the transverse sinus.', 'history': 'Sudden onset of headache, followed by loss of consciousness. Patient was normotensive on admission to the emergency room.', 'imagePoolId': '1794b6cc-63f4-4ca9-be7d-1f709ec6d8e6', 'name': 'Thrombosed dAVF', 'teachingPoint': 'Hemorrhage into an underlying vascular malformation is a relatively uncommon cause of spontaneous intracranial hemorrhage (ICH) in an older adult patient. In this case, the outlet vein (the transverse sinus) partially thrombosed and the elevated venous pressure caused the ICH.', 'demographics': '75 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '884ce0f4-f7a2-42f7-98f4-be1480464365', 'description': 'Primary as well as metastatic brain tumors can hemorrhage spontaneously, as happened in this case.\n\nAxial NECT scan (image 1) shows lobulated hyper density in the region of the upper 4th ventricle and aqueduct (arrow). Axial precontrast T1- (image 2) and T2- weighted MR scans (images 3, 4) show a mass that is isointense with brain on T1WI and mixed signal intensity on T2WIs. Note profoundly hypointense rim, seen best on image 4 (arrow). Postontrast T1-weighted scan (image 5) shows the mass (arrow) enhances strongly but inhomogeneously.', 'history': 'Acute onset of severe headache followed by drowsiness, decreased mental status. Papilledema on neurologic examination.', 'imagePoolId': 'bb2dba7f-a997-4e4c-96f0-a377ae12767a', 'name': 'Subependymoma', 'teachingPoint': 'A lateral view of the vertebrobasilar angiogram performed in this case shows only avascular mass effect. Posterior fossa craniotomy disclosed a hemorrhagic subependymoma of the 4th ventricle. Spontaneous (nontraumatic) intraventricular hemorrhage is uncommon and should raise suspicion of an underlying lesion such as neoplasm or vascular malformation.', 'demographics': '53 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '20616280-ef69-44dc-b57b-25630a95fbec', 'description': 'A single NECT scan is selected in this case and shows a large hyperdense mass in the right temporal lobe with subfalcine herniation of the right lateral ventricle. Spontaneous ICH in an elderly patient is often caused by hypertension or amyloid angiopathy. In this case, the patient was normotensive and had a history of normal cognitive function. Cerebral angiogram (not shown) demonstrated only an avascular mass effect. The hematoma was evacuated at surgery. The surgeon noted some "unusual-appearing" tissue mixed with the hematoma. Pathologic examination disclosed adenocarcinoma, primary source unknown.\n\nSudden hemorrhage into a neoplasm in an elderly patient is most commonly caused by high grade astrocytoma but metastasis, often from breast or lung, may present as spontaneous ICH. Other causes besides hypertension and amyloid angiopathy that should not be overlooked include venous occlusion with hemorrhagic venous infarct. Further evaluation in this case identified an adenocarcinoma of the colon.', 'history': 'Elderly normotensive patient presented with sudden onset of left hemiparesis, decreasing mental status. Pupil-involving right third cranial nerve palsy was identified on initial examination.', 'imagePoolId': 'a92fe82d-17b2-4c81-a8de-52793625e70c', 'name': 'Sudden', 'teachingPoint': None}], 'caseType': 'variant', 'name': 'VARIANT'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*Axial CT shows a hyperdense hematoma in the right basal ganglia
with local mass effect and extension to the thalamus. Intraventricular extension of hemorrhage
is common in hypertensive hemorrhage. The basal ganglia is the most common location.*
+
+
+*Axial CT shows a hyperdense hematoma in the right basal ganglia
with local mass effect and extension to the thalamus. Intraventricular extension of hemorrhage
is common in hypertensive hemorrhage. The basal ganglia is the most common location.*
+
+
+*Axial CTA in a hypertensive patient shows a hematoma
in the basal ganglia with a spot sign
indicating active extravasation. The spot sign indicates active bleeding, predicts hematoma expansion, and is associated with a poor outcome.*
+
+
+*Axial GRE MR in a 71-year-old man shows an acute hypertensive hemorrhage in the left cerebellar hemisphere
with extension to the 4th ventricle
. The posterior fossa accounts for ~ 10% of all hypertensive hemorrhages.*
+
+
+*Axial NECT in an 26-year-old shows a hypertensive basal ganglia hemorrhage
with intraventricular hemorrhage
. However, in this young patient, underlying etiologies must be considered, including drug abuse and vascular lesions, such as an arteriovenous malformation (AVM).*
+
+
+*Axial gross pathology, through the middle of the cerebellum, shows an acute spontaneous parenchymal hemorrhage
. Note the atherosclerosis in the basilar artery
. No underlying lesion was identified in this patient with chronic hypertension. (Courtesy R. Hewlett, MD.)*
+
+
+*Axial SWI MR in an older adult with acute hemorrhage shows multiple foci of susceptibility artifact, related to hemosiderin deposition in this patient with cerebral amyloid disease (CAA). CAA may result in lobar hemorrhage.*
+
+
+*Axial CT in a 27-year-old pregnant woman with preeclampsia and hypertension shows a parenchymal hematoma
with intraventricular hemorrhage
.*
+
+
+*Axial CTA in the same patient showed no underlying vascular lesion and no spot sign to suggest rapid hematoma expansion. Given the young age, additional considerations include vasculitis and drug abuse. Additional imaging ultimately revealed reversible cerebral vasoconstriction syndrome (RCVS).*
+
+
+*Axial NECT in a 26-year-old woman with severe headache shows left frontal hemorrhage
and edema
related to a venous infarct. Note hyperdense thrombus in the superior sagittal sinus
and cortical veins
.*
+
+
+*Axial NECT shows a thalamic hemorrhage
with focal Ca++
. In this young patient, underlying vascular lesions should be considered. Cavernous malformation was the final diagnosis. In children, vascular lesions represent ~ 50% of all intracranial hemorrhages.*
+
+
+### Additional Images
+
+
+*Axial NECT shows lobar hematoma in a 68-year-old normotensive, demented patient. Differential diagnosis includes cerebral amyloid angiopathy (CAA), underlying neoplasm, cortical vein occlusion, and coagulopathy.*
+
+
+*Axial T1 MR shows a mostly isointense left anterior parietal mass
. No other abnormalities were seen on this sequence.*
+
+
+*Axial T2* GRE MR in the same patient with probable CAA shows a hematoma blooming strongly but inhomogeneously. Note the multifocal cortical/subcortical hypointensities ("black dots")
.*
+
+
+*Axial NECT in an elderly patient shows a hyperdense mass in the right centrum semiovale
. Significant cerebral edema
is present surrounding the lesion.*
+
+
+*Axial T1 C+ MR in the same patient shows a thick, irregular rind of enhancement around the hematoma. Glioblastoma (GBM) was found at surgery.*
+
+
+*Coronal gross pathology in an elderly patient who died from spontaneous Primary intraparenchymal hemorrhage
shows large lobar hemorrhage from cerebral amyloid angiopathy. (Courtesy J. Townsend, MD.)*
+
+
+*Axial NECT shows large acute right frontal intraparenchymal hemorrhage with considerable mass effect and subfalcine herniation. Subarachnoid hemorrhage is also present. This hemorrhage was secondary to an M3 branch aneurysm bleed.*
+
+
+*Axial NECT shows acute hematoma in the right frontal lobe
, secondary to GBM, which has hemorrhaged. Note the small halo of edema along the posteromedial cortex
.*
+
+
+*Axial T1 C+ MR shows peripheral ring enhancement
around GBM in the same patient. This image is slightly more superior above the level of the acute hemorrhage.*
+
+
+*Axial NECT shows bilateral hematomas in a patient with underlying coagulopathy. Layering fluid/fluid levels are present. There is also a small left frontal hematoma
.*
+
+
+*Axial NECT shows acute posterior frontal hemorrhage within underlying tumor. Note the large "halo" of surrounding edema
, more than would be expected from a simple or "bland" hemorrhage.*
+
+
+*Axial T1 MR shows hemorrhagic transformation of small, watershed-distribution right parietal infarction
with hyperintense intracellular methemoglobin.*
+
+
+*Axial T2 MR in the same patient shows surrounding edema
better appreciated on fluid-weighted sequences.*
+
+
+*Axial CT shows a hyperdense hematoma centered in the left external capsule and putamen
with associated edema and mass effect. This is the most common location for a hypertensive hemorrhage.*
+
+
+*Axial CT in a 58-year-old hypertensive woman shows a hyperdense hemorrhage
in the right thalamus with surrounding edema. No additional imaging is required in this patient.*
+
+
+*Axial NECT in a 46-year-old mildly hypertensive man shows basal ganglionic hemorrhage with intraventricular blood. No history of drug abuse could be elicited.*
+
+
+*AP DSA from a right internal carotid artery injection shows an avascular mass in the basal ganglia (hematoma) with midline shift of the anterior cerebral artery
. There is a cluster of abnormal arteries (AVM)
fed by a large anterior choroidal artery.*
+
+
+*Axial T2* GRE MR shows a large occipital lobe hemorrhage
and multiple other foci of susceptibility artifact
or blooming, related to hemosiderin deposition in this elderly patient with CAA. The MR was done to exclude an underlying mass.*
+
+
+*Axial T1 C+ MR shows lobular cerebral hematoma in the left temporal lobe. Note fluid-fluid levels
in the hematoma from brisk bleeding. The more avidly enhancing region in sulcus represented pseudoaneurysm
.*
+
+
+*AP DSA of selective left internal carotid injection shows a fusiform mycotic aneurysm
arising from a distal left middle cerebral artery branch. Note the mass effect on middle cerebral artery branches from the associated cerebral hematoma
.*
+
+
+*Axial CT in this 5 year old shows a large frontal lobe hemorrhage
with surrounding edema and mass effect. Additional imaging revealed an underlying cavernous malformation. In children, vascular lesions represent ~ 50% of all intracranial hemorrhages.*
+
+
+*Axial CT in a 73-year-old man shows an acute occipital lobe hemorrhage
. Subtle subarachnoid hemorrhage
was also present. Biopsy disclosed renal cell carcinoma metastatic disease.*
+
+
+*Axial CT in a young postpartum woman with severe headaches shows a hemorrhagic venous infarct
with surrounding edema. CTV showed a left transverse sinus thrombosis. Risk factors for venous thrombosis include dehydration, pregnancy, and oral contraceptives.*
+
+
+*Axial CT shows a hyperdense hematoma in the right putamen
with local mass effect and medial extension to the thalamus. Intraventricular extension of hemorrhage
is common in hypertensive hemorrhage. The basal ganglia is the most common location for a hypertensive hemorrhage.*
+
+
+*Axial NECT in an 18-year-old shows what appears to be a classic hypertensive basal ganglia hemorrhage
. However, in this young patient, underlying etiologies must be considered, including drug abuse and vascular lesions, such as an AVM.*
+
+
+*Coronal CTA in the same patient shows the hematoma
with no underlying vascular mass or evidence of a spot sign to suggest hematoma expansion. The blood toxicology screen was positive for amphetamines.*
+
+
+*Axial T2 MR in this young patient shows a classic cavernous malformation
. In children, vascular lesions represent ~ 50% of all intracranial hemorrhages.*
+
+
+*AP DSA from a left vertebral artery injection in a patient with an occipital hematoma shows a cluster of abnormal vessels, or "bag of worms," related to an AVM
fed by a large posterior cerebral artery
. AVM may present at any age but is typically seen in children or young adults with a peak age of 20-40 years. 25% of AVMs are symptomatic by 15 years.*
+
+
+*Axial SWI MIP MR in an older adult with acute hemorrhage shows multiple foci of susceptibility artifact related to hemosiderin deposition
in this patient with CAA. CAA may result in lobar hemorrhage and superficial siderosis
, as noted in this patient.*
+
diff --git a/docs_md/articles/subclavian-steal-syndrome_02e57d99-5b25-4652-a7af-b8941c86a16d.md b/docs_md/articles/subclavian-steal-syndrome_02e57d99-5b25-4652-a7af-b8941c86a16d.md
new file mode 100644
index 0000000..a2dbf5e
--- /dev/null
+++ b/docs_md/articles/subclavian-steal-syndrome_02e57d99-5b25-4652-a7af-b8941c86a16d.md
@@ -0,0 +1,405 @@
+---
+title: "Subclavian Steal Syndrome"
+docid: "02e57d99-5b25-4652-a7af-b8941c86a16d"
+authors:
+ - key: "91e93745-f376-45a8-9b33-eae419cd3322"
+ value: "T. Gregory Walker, MD, FSIR"
+breadcrumbs:
+ -
+ name: "Vasculature"
+ slug: "vasculature"
+ treeNodeId: "6de1ee4d-afe9-419c-a868-d4074ec0fb7e"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "53de565c-37fe-4b4b-802e-7ea3610cc666"
+ -
+ name: "Extracranial Cerebral Arteries"
+ slug: "extracranial-cerebral-arteries"
+ treeNodeId: "f83006c9-b9f0-4c4b-a4ad-92ec5a46547f"
+ -
+ name: "Subclavian Steal Syndrome"
+ slug: "subclavian-steal-syndrome"
+ treeNodeId: null
+category: "Vasculature"
+documentVersionId: "ff91c6b5-997d-482b-a1f6-d36ea1eba782"
+imageCount: 17
+lastUpdated: "04/20/16"
+pageDescription: "Subclavian Steal Syndrome"
+pageKeywords: "Vasculature, Diagnosis, Extracranial Cerebral Arteries, Subclavian Steal Syndrome"
+pageTitle: "Subclavian Steal Syndrome | STATdx"
+enhancedTitle: "Subclavian Steal Syndrome"
+type: "DX"
+references: true
+anatomy:
+ - "{'authors': 'Anne G. Osborn, MD, FACR', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/a7a252f0-2ac6-402a-8c87-cfce8adc799b', 'category': 'Brain', 'compareUrl': '/compare/document/a7a252f0-2ac6-402a-8c87-cfce8adc799b/related-anatomy/treeNode?subContext=Aortic Arch and Great Vessels', 'documentId': 'a7a252f0-2ac6-402a-8c87-cfce8adc799b', 'documentType': 'ANATOMY', 'documentUrl': '/document/aortic-arch-and-great-vessels/a7a252f0-2ac6-402a-8c87-cfce8adc799b', 'enhancedTitle': 'Aortic Arch and Great Vessels', 'entryDate': '10/20/20', 'imageCount': 8, 'imageUrl': '/image/thumbnail/5a451d53-7fe4-426e-b816-7f0e0a1df745?size=174&quality=85', 'inCompareCart': False, 'rank': 1, 'referenceCount': 0, 'showCompareButton': False, 'title': 'Aortic Arch and Great Vessels'}"
+cases: 1
+breadcrumbs:
+ - "Vasculature"
+ - "Diagnosis"
+ - "Extracranial Cerebral Arteries"
+ - "Subclavian Steal Syndrome"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Vertebral artery (VA) provides collateral blood flow to upper extremity because of vascular blockage
+ - Caused by stenosis/occlusion of proximal subclavian artery (SCA) or, occasionally, brachiocephalic artery
+ - Leads to reversed flow in VA to perfuse SCA distal to stenosis/occlusion
+ - Exacerbated by demand for increased arterial blood flow in arm supplied by affected SCA
+- ### Imaging
+
+
+ - US is very reliable in diagnosing subclavian steal
+ - CTA, MRA, or DSA to confirm or delineate anatomy
+ - With MRA, use phase contrast technique to show flow direction/reversal in affected VA
+ - If abnormal VA flow is not detected at rest, then induce flow reversal with ipsilateral arm exercise or artificial arm hyperemia (after cuff compression)
+ - DSA usually combined with endovascular treatment
+- ### Pathology
+
+
+ - Atherosclerosis is most common cause of SCA obstruction leading to subclavian steal
+ - Rarely, steal-inducing SCA obstruction may result from vasculitis, dissection, trauma, or vessel compression from adjacent neoplastic mass
+- ### Clinical Issues
+
+
+ - **Clinical profile:** Diminished arm pulses and blood pressure ipsilateral to SCA obstruction
+ - Linear correlation between increasing arm blood pressure difference and occurrence of symptoms
+ - Usually involves left side (85%); can occur on right
+ - Conservative management is appropriate in asymptomatic or minimally symptomatic patients
+ - Address atherosclerosis risk factors
+ - SCA angioplasty/stenting is preferred over surgical bypass in symptomatic individuals
+
+## TERMINOLOGY
+
+- ### Definitions
+
+
+ - Vertebral artery (VA) provides collateral blood flow to upper extremity because of vascular blockage
+ - Caused by stenosis/occlusion of proximal subclavian artery (SCA) or, occasionally, brachiocephalic artery
+ - Leads to reversed flow in VA to perfuse SCA distal to stenosis/occlusion
+ - Exacerbated by demand for increased arterial blood flow in arm supplied by affected SCA
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Reversed or biphasic VA flow ipsilateral to stenosed/obstructed SCA; antegrade flow in contralateral VA
+ - Increased volume/velocity of flow in contralateral VA
+ - #### Location
+
+
+ - More common on left side but can occur on right
+- ### Ultrasonographic Findings
+
+
+ - **Duplex Doppler ultrasound****(US)**
+ - **Mild subclavian steal****(grade 1)**
+ - Systolic deceleration of VA flow
+ - **Moderate subclavian steal (grade 2)**
+ - Biphasic (to-and-fro) flow in affected VA at rest
+ - Provocative dynamic tests with ipsilateral arm exercise or tourniquet-induced arm hyperemia may better demonstrate flow reversal
+ - **Severe subclavian steal (grade 3)**
+ - Reversed flow in affected VA
+ - **Additional findings**
+ - Increased blood flow in contralateral VA
+ - Not precisely defined, but peak systolic velocity > 60 cm/s suggests abnormality
+ - Damped Doppler waveforms in affected SCA
+ - Possible Doppler evidence of SCA stenosis
+ - Focal high velocity, turbulence
+- ### CT Findings
+
+
+ - #### NECT
+
+
+ - May show atherosclerotic calcifications in vessel wall of stenotic or occluded proximal SCA
+ - Large plaques may show low-density foci (lipid)
+ - #### CECT
+
+
+ - High-grade SCA stenosis or occlusion
+ - Lesion must involve SCA proximal to VA origin
+ - #### CTA
+
+
+ - Permits 3D reconstruction of vasculature
+ - Allows better estimation of degree of stenosis
+ - May differentiate severe stenosis from occlusion
+- ### MR Findings
+
+
+ - #### T1WI
+
+
+ - May identify high-signal lipid/hemorrhage in atherosclerotic plaque of stenosed SCA
+ - #### T2WI
+
+
+ - Wall thickening and luminal narrowing in SCA
+ - Absence of flow void may occur if vessel is severely stenotic or occluded
+ - #### MRA
+
+
+ - Determine degree of SCA stenosis
+ - With severe stenosis, intravascular signal may not be evident on noncontrast 2D time-of-flight MRA
+ - Can mimic SCA occlusion
+ - 3D C+ MRA is much better in delineating true presence and severity of SCA stenosis
+ - Determine flow direction in VA
+ - Reversed flow in VA will be inapparent on noncontrast 2D time-of-flight MRA
+ - Can mimic VA occlusion
+ - Due to superior saturation pulse used to remove inferiorly flowing venous signal
+ - 3D C+ MRA shows vessel patency, not flow direction
+ - Time-resolved 3D C+ MRA is better for showing retrograde VA flow
+ - Phase contrast MRA can confirm
+ - Vessel patency
+ - Reversal of VA flow
+ - Location of stenosis
+- ### Angiographic Findings
+
+
+ - **DSA**
+ - Severe SCA stenosis or occlusion proximal to VA
+ - Reversed or to-and-fro flow in affected VA
+ - Antegrade flow in contralateral VA
+ - Best demonstrated on thoracic arch aortogram
+ - Usually combined with endovascular treatment
+- ### Imaging Recommendations
+
+
+ - #### Best imaging tool
+
+
+ - US is very reliable in diagnosing subclavian steal
+ - CTA, MRA, or DSA to confirm or delineate anatomy
+ - #### Protocol advice
+
+
+ - If abnormal VA flow is not detected at rest, then induce flow reversal with ipsilateral arm exercise or artificial arm hyperemia (following cuff compression)
+ - With MRA, use phase contrast technique to show flow direction/reversal in affected VA
+
+## DIFFERENTIAL DIAGNOSIS
+
+- ### Right Common Carotid Artery (CCA) Steal
+
+
+ - Associated with high-grade brachiocephalic artery stenosis/occlusion (right side only)
+ - Analogous to subclavian steal, but blood is "stolen" from right CCA as well as from right VA
+ - To-and-fro or reversed right CCA and right VA flow
+- ### Vertebral Arteriovenous Fistula (AVF)
+
+
+ - Abnormal direct communication between vertebral artery and vein
+ - High-velocity turbulent flow with low resistance in affected vessels
+ - Surrounding venous engorgement
+ - May see reversal of VA flow distal to site of AVF
+- ### Vertebral Artery Occlusion/Severe Stenosis
+
+
+ - Flow is not identified in VA; only venous flow is noted
+ - May be confused with slow reversal of flow in VA but will not demonstrate arterial waveform
+ - Adjacent vessels (usually from external carotid artery or proximal SCA) may mimic VA patency
+ - May even mimic reversal of VA flow
+ - Cervical collateral vessels can also reconstitute VA distal to occlusion/stenosis resulting in dampened distal VA and alternating or high-resistance flow pattern
+- ### Vertebral Artery Hypoplasia
+
+
+ - May not detect normal VA flow in severe hypoplasia
+ - Usually on right side but may occur on left side
+ - Hypoplastic VA often supplies only ipsilateral posterior inferior cerebellar artery
+ - Hypoplastic left VA may arise directly from aortic arch
+ - Should demonstrate normal antegrade flow direction
+ - May have high-resistance flow pattern
+ - Due to increased flow friction in small vessel
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Etiology
+
+
+ - Atherosclerotic occlusive disease by far most common cause of SCA obstruction leading to subclavian steal
+ - Rarely, steal-inducing SCA obstruction may result from vasculitis, dissection, trauma, or vessel compression from adjacent neoplastic mass
+ - **Anatomy**
+ - Unique vertebrobasilar arterial system permits subclavian steal: 2 VAs join to form basilar artery
+ - With proximal SCA occlusion/severe stenosis, blood is "stolen" from ipsilateral VA to perfuse arm
+ - Reversal of flow in ipsilateral VA and hyperdynamic flow in contralateral VA
+ - Usually requires ≥ 70% SCA diameter stenosis
+- ### Staging, Grading, & Classification
+
+
+ - Grading of subclavian steal phenomenon
+ - **Grade 0:** No subclavian steal
+ - Normal antegrade VA flow
+ - **Grade 1:**Mild subclavian steal
+ - Systolic deceleration of VA flow
+ - **Grade 2:**Moderate subclavian steal
+ - Alternating (biphasic) flow in VA at rest
+ - **Grade 3:** Severe subclavian steal
+ - Reversed flow in affected VA
+ - Clinical classification
+ - Complete (persistent): Subclavian steal phenomenon is always present
+ - Implies SCA occlusion/severe stenosis
+ - Intermittent: Subclavian steal phenomenon occurs only when arm exercise increases blood flow demand
+ - Implies moderate SCA stenosis
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - Generally harmless, asymptomatic phenomenon
+ - Can be symptomatic, especially during arm exercise
+ - Arm claudication (pain with arm exercise)
+ - If vertebrobasilar insufficiency is present, may cause dizziness (most common), vertigo, ataxia, diplopia, dysarthria, homonymous hemianopsia
+ - #### Other signs/symptoms
+
+
+ - Very rarely causes vertebrobasilar infarct of brainstem, cerebellum, or posterior cerebral hemispheres
+ - Concomitant carotid/vertebral/cerebral vascular disease is invariably present
+ - Angina may occur in patients who have undergone left internal mammary artery bypass surgery for coronary artery disease and have subclavian steal
+ - Known as coronary subclavian steal syndrome
+ - Myocardial ischemia due to reversed flow in internal mammary artery bypass graft
+ - #### Clinical profile
+
+
+ - Diminished arm pulses and blood pressure ipsilateral to SCA obstruction (differential of 20-30 mm Hg when comparing blood pressure in arms)
+ - Linear correlation between increasing arm blood pressure difference and occurrence of symptoms
+ - Usually involves left side (85%); can occur on right
+- ### Demographics
+
+
+ - #### Age
+
+
+ - Older individuals (average age: 60 years)
+ - Atherosclerotic occlusive disease
+ - May occur in younger individuals with Takayasu arteritis causing SCA obstruction
+ - #### Gender
+
+
+ - M > F (slight male predominance)
+- ### Natural History & Prognosis
+
+
+ - Usually remains asymptomatic
+ - May become symptomatic if atherosclerosis progresses in affected SCA/cerebral vasculature
+- ### Treatment
+
+
+ - Conservative management is appropriate in asymptomatic or minimally symptomatic patients
+ - Address atherosclerosis risk factors
+ - SCA angioplasty/stenting is preferred over surgical bypass in symptomatic individuals
+ - Surgical bypass options include innominate-to-SCA, CCA-to-SCA, or axillary-to-axillary artery bypass
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - Subclavian steal is likely if cerebral symptoms are exacerbated with arm exercise
+ - SCA obstruction is most likely due to atherosclerosis
+ - Must exclude vasculitis, dissection, adjacent neoplasm
+- ### Image Interpretation Pearls
+
+
+ - Sonography is easiest and best test to confirm diagnosis
+ - If abnormal VA flow is not detected at rest, then induce flow reversal with ipsilateral arm exercise or hyperemia
+ - VA flow reversal will be inapparent on noncontrast 2D time-of-flight MRA; use C+ or phase contrast MRA
+
+ 244fbcf9-4521-4048-a17c-4f86f2ffaddd
+
+## References
+
+## Selected References
+
+1. [Osiro S et al: A review of subclavian steal syndrome with clinical correlation. Med Sci Monit. 18(5):RA57-63, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22534720%5Bpmid%5D)
+1. [Song L et al: Endovascular stenting vs. extrathoracic surgical bypass for symptomatic subclavian steal syndrome. J Endovasc Ther. 19(1):44-51, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22313201%5Bpmid%5D)
+1. [Betensky BP et al: Unequal blood pressures: a manifestation of subclavian steal. Am J Med. 124(8):e1-2, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21787894%5Bpmid%5D)
+1. [Tan TY et al: Hemodynamic effects of subclavian steal phenomenon on contralateral vertebral artery. J Clin Ultrasound. 34(2):77-81, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16547976%5Bpmid%5D)
+1. [Sheehy N et al: Contrast-enhanced MR angiography of subclavian steal syndrome: value of the 2D time-of-flight "localizer" sign. AJR Am J Roentgenol. 185(4):1069-73, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16177436%5Bpmid%5D)
+1. [Bitar R et al: MR angiography of subclavian steal syndrome: pitfalls and solutions. AJR Am J Roentgenol. 183(6):1840-1, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15547242%5Bpmid%5D)
+
+## Anatomy
+
+### Aortic Arch and Great Vessels
+Brain/ANATOMY:a7a252f0-2ac6-402a-8c87-cfce8adc799b
+
+## Cases
+
+- {'cases': [{'authors': [{'key': 'd69a0534-8989-4b83-b19d-c3b640f22e53', 'value': 'Megan R. Saettele, MD'}, {'key': '2e78a4ff-418e-46f9-93e4-c6d95d960e35', 'value': 'Brandt C. Wible, MD'}], 'caseVersionId': '152051e0-0d53-471a-9f3d-141991f08731', 'description': 'Initial DSA ascending aortogram image (#1) shows a 4 cm segment of the left subclavian artery that remains unopacified (open arrows). The distal subclavian fills from retrograde vertebral artery flow. Spot fluoroscopic image (#2) shows an attempt to re-open stenosed left subclavian artery with balloon angioplasty (arrows). Note the "neck" in the balloon, which represents the area of stenosis. Subsequent DSA image (#3) shows this to be only partially successful with multiple areas of narrowing present throughout the left subclavian artery. Spot fluoroscopic image (#4) shows deployment of a balloon expandable stent (arrows). Subsequent DSA image (#5) shows expandable stent in place within the left subclavian artery (arrows). Further DSA images (#6-8) confirm opacification with contrast upon subsequent contrast injection through the expandable stent (arrows, #6), indicating left subclavian recannulization (open arrows).', 'history': 'Patient with 3 month history of increasing intermittent upper extremity pain, as well as dizziness when using left hand.', 'imagePoolId': 'e53fcf69-8f0c-4d4c-9b85-c34784f7bdba', 'name': 'Subclavian steal syndrome', 'teachingPoint': None, 'demographics': '38 Years old female'}], 'caseType': 'typical', 'name': 'TYPICAL'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*Color Doppler US shows (A) normal antegrade arterial waveforms
in the right vertebral artery, indicating appropriate blood flow directionality. (B) There is reversed directionality of the left vertebral artery waveform
, indicating retrograde flow.*
+
+
+*Color Doppler US shows (A) normal antegrade arterial waveforms
in the right vertebral artery, indicating appropriate blood flow directionality. (B) There is reversed directionality of the left vertebral artery waveform
, indicating retrograde flow.*
+
+
+*Left anterior oblique DSA of the thoracic aortic arch shows occlusion
of the left subclavian artery, proximal to the origin of the left vertebral artery. This is the typical anatomy that results in the subclavian steal phenomenon and is usually caused by atherosclerosis.*
+
+
+*Delayed image from the aortic arch DSA demonstrates contrast opacification of the left vertebral artery
and filling of the left subclavian artery
beyond the occlusion. This is the classic DSA appearance of subclavian steal.*
+
+
+*(C) DSA after selective catheterization of the right vertebral artery
shows contrast flowing retrograde into the left vertebral artery
and reconstituting the left subclavian artery
. (D) Yellow arrows on the graphic show the directionality of blood flow in subclavian steal phenomenon.*
+
+
+*Axial CECT in a patient with dizziness and left arm claudication shows (A) a nonopacified proximal left subclavian artery
, consistent with occlusion. (B) More cephalad, the distal left subclavian artery
is opacified, indicating reconstitution via collateral blood flow.*
+
+
+*Coronal CT reconstruction shows atherosclerotic calcification
and segmental occlusion
of the proximal left subclavian artery. The distal left subclavian artery
is reconstituted via a large left vertebral artery
.*
+
+
+*Preferred treatment of a symptomatic subclavian steal due to a severely stenotic or occluded subclavian artery is via endovascular revascularization. DSA shows that the subclavian occlusion has been recanalized and an intravascular stent
has been placed, with care taken to preserve patency of the vertebral artery
.*
+
+
+*DSA of the thoracic aortic arch following endovascular revascularization shows a patent stent
and normal antegrade filling of the left vertebral
and subclavian
arteries.*
+
+
+*Although subclavian steal usually occurs on the left, right-sided cases also occur. Color Doppler US shows (A) reversed flow
in the right VA and (B) biphasic internal carotid artery flow
, worrisome for brachiocephalic artery obstruction in a patient with dizziness and right arm claudication.*
+
+
+*Thoracic aorta DSA shows a severely stenotic brachiocephalic artery
& a faintly opacified right subclavian artery
. Retrograde flow via the right VA and CCA collateralizes the subclavian artery.*
+
+
+### Additional Images
+
+
+*Coronal oblique 3D TOF contrast-enhanced MRA MIP image shows occlusion of the proximal left subclavian artery (SCA)
with reconstitution of SCA via reversal of flow in the left vertebral artery
.*
+
+
+*Axial CECT shows stenosis of the proximal left subclavian artery with calcification (high-density foci) and lipid (low-density area) in the atheromatous plaque
.*
+
+
+*Axial 2D PC MRA image shows subclavian steal with upward flow (black) in carotid and right vertebral arteries
and downward flow (white) in jugular veins and left vertebral artery
.*
+
+
+*Color Doppler ultrasound shows antegrade left vertebral artery flow with the left arm cuff inflated and reversal of flow with the cuff deflated, a maneuver utilized to evoke subclavian steal.*
+
+
+*Color Doppler ultrasound of the left vertebral artery shows biphasic flow with late systolic reversal of flow
indicative of moderate left subclavian steal.*
+
+
+*Coronal PC MRA MIP image shows patency of the entire vertebrobasilar arterial system. 2D TOF MRA (not shown) demonstrated lack of flow in the left vertebral artery. This was a case of left subclavian steal.*
+
+
+*Coronal oblique 2D TOF MRA MIP image shows lack of signal in the left vertebral artery, mimicking occlusion. This is actually due to superior saturation pulse nulling the signal of downward flow.*
+
diff --git a/docs_md/articles/systemic-lupus-erythematosus_f15d3007-90bd-4ab8-8d53-db18509f1a7a.md b/docs_md/articles/systemic-lupus-erythematosus_f15d3007-90bd-4ab8-8d53-db18509f1a7a.md
new file mode 100644
index 0000000..3618b4b
--- /dev/null
+++ b/docs_md/articles/systemic-lupus-erythematosus_f15d3007-90bd-4ab8-8d53-db18509f1a7a.md
@@ -0,0 +1,583 @@
+---
+title: "Systemic Lupus Erythematosus"
+docid: "f15d3007-90bd-4ab8-8d53-db18509f1a7a"
+authors:
+ - key: "b2e6dabb-ee1c-42a4-a332-9f0814c1c607"
+ value: "Surjith Vattoth, MD"
+breadcrumbs:
+ -
+ name: "Brain"
+ slug: "brain"
+ treeNodeId: "0361042c-9c90-48e4-864e-a2e6428ee9c9"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "948c6f1e-8e18-45f3-bd9d-7a04ab0b8708"
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+ name: "Pathology-Based Diagnoses"
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+ name: "Systemic Lupus Erythematosus"
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+lastUpdated: "09/04/25"
+pageDescription: "Systemic Lupus Erythematosus"
+pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Stroke, Nonatheromatous Vasculopathy, Systemic Lupus Erythematosus"
+pageTitle: "Systemic Lupus Erythematosus | STATdx"
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+cases: 2
+breadcrumbs:
+ - "Brain"
+ - "Diagnosis"
+ - "Pathology-Based Diagnoses"
+ - "Stroke"
+ - "Nonatheromatous Vasculopathy"
+ - "Systemic Lupus Erythematosus"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Systemic lupus erythematosus (SLE); neuropsychiatric SLE (NPSLE): Primary or secondary
+ - Multisystem autoimmune disorder that affects respiratory, cardiovascular, GI, GU, MSK systems and CNS
+- ### Imaging
+
+
+ - Brain MR abnormalities more common in patients with SLE with antiphospholipid antibody (APLA) syndrome (APS)
+ - Multifocal T2/FLAIR white matter hyperintensities (WMH)
+ - Inflammatory lesion ± gray matter involvement, small vessel disease (SVD), large vessel disease (LVD)
+ - Intracerebral hemorrhage: Cerebral microbleeds (CMBs) more common in SLE with WMH, mainly inflammatory
+ - Other findings: Migratory edema, postictal edema, posterior reversible encephalopathy syndrome (PRES), demyelinating syndrome, optic neuritis, autoimmune antibody associated encephalitis, longitudinally extensive transverse myelitis
+ - Mild SLE: PET/SPECT more sensitive than MR
+ - Restricted diffusion (cytotoxic edema) in ischemia/infarct
+ - ↑ diffusion (vasogenic edema) in vasculopathy
+ - Acute/active CNS lesions may enhance
+- ### Top Differential Diagnoses
+
+
+ - Arteriolosclerosis (microvascular disease)
+ - Other vasculitides (e.g., PACNS)
+ - Multiple sclerosis (MS), Susac syndrome, Lyme disease
+- ### Pathology
+
+
+ - Neuronal dysfunction mediated by antibodies → diffuse neuropsychiatric symptoms
+ - Circulating immune complexes → vascular injury
+ - Endothelial cell activation by cytokines and complement activation → occlusive vasculopathy
+ - Antiphospholipid antibodies (APL-Ab) → macro- and microvascular thrombosis
+ - Antibody-mediated [aquaporin-4, myelin oligodendrocyte glycoprotein (MOG)] → myelopathy and cranial neuropathy
+- ### Clinical Issues
+
+
+ - Cerebral involvement may precede full-blown SLE picture or may develop during course of disease
+ - SLE associated with APS, PRES, Libman-Sacks endocarditis, emboli
+- ### Diagnostic Checklist
+
+
+ - Role of imaging in SLE: Assess acute neurologic deficits
+ - Negative brain MR does not exclude cerebral lupus
+
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - Systemic lupus erythematosus (SLE)
+ - Neuropsychiatric SLE (NPSLE)
+- ### Definitions
+
+
+ - **Primary NPSLE**: If resulting directly from immune-mediated injury
+ - **Secondary NPSLE**: If related to treatment, infections, metabolic disturbances, or other systemic manifestations not related to SLE; ~ 60%
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Most common finding: Multifocal T2/FLAIR**white matter hyperintensities (WMH)**
+ - WMH could be due to inflammatory lesions or small vessel disease (SVD)
+ - **Inflammatory lesion WMH**: Defined as mass-like, medium- to large-sized WMH ± involvement of **gray matter (GM)**
+ - Not dependent on vascular territories
+ - Acute: Contrast enhancement and diffusion restriction
+ - Responsive to steroids
+ - **SVD WMH**: Defined as recent small subcortical infarcts, lacunes, microbleeds, and brain atrophy
+ - According to standards for reporting vascular changes on neuroimaging (STRIVE)
+ - Includes basal ganglia and infratentorial involvement
+ - **Large vessel disease (LVD)**: Focal infarcts of various sizes may be seen in
+ - SVD or LVD infarcts restricts diffusion in acute stage
+ - **Intracerebral hemorrhage**: In 0.4-7%, due to hypertension, anticoagulation, antiphospholipid antibody (APLA) syndrome (APS), low factor XIII activity, and comorbidities
+ - APS characterized by APLA and thromboembolic phenomena, such as arterial and deep venous thromboses, spontaneous abortions
+ - Brain MR abnormalities more common in patients with **SLE with APS** than in those without APS
+ - Large territory infarcts, lacunar infarcts in deep WM, localized cortical infarcts in MCA territory, bilateral borderzone infarcts, anterior basal ganglia lesions, microbleeds, arterial stenoses
+ - **Cerebral microbleeds (CMBs)** in SWI more common in SLE with WMH (than in those without WMH)
+ - In WMH group, inflammatory lesion subgroup shows more frequent CMBs than in SVD subgroup
+ - WMH group shows ↑ SLE disease activity index, longer duration of disease, and ↑ incidence of APS (than in those without WMH)
+ - May have symptomatic **"migratory" edematous** areas
+ - **Postictal edema** after seizure
+ - May show transient diffusion restriction
+ - **Posterior reversible encephalopathy syndrome (PRES)**: Typical and central variant (brainstem and thalamus/basal ganglia)
+ - **Optic neuritis**: Segmental or diffuse bilateral optic nerve &/or optic chiasm involvement with optic nerve head sparing
+ - Mimics neuromyelitis optics (NMO) usually
+ - **Demyelinating syndrome**: Rare, in 3%, mimics NMO
+ - Lesions in areas of aquaporin (AQP4) expression like periependymal regions of medial thalamus, hypothalamus, periaqueductal GM, dorsal pons and medulla including area postrema, periventricular WM, and corpus callosum
+ - Demyelination and optic neuritis may rarely mimic multiple sclerosis (MS)
+ - **Autoimmune antibody-mediated encephalitis**: Rare, anti-NMDAR, antiphospholipid or antiribosomal P-protein antibodies
+ - T2/FLAIR hyperintense amygdala, hippocampus, and basal ganglia
+ - Occasional basal ganglia punctate enhancement and diffusion restriction
+ - T1 hyperintensity suggesting coagulative necrosis may be seen
+ - Extremely rarely **hypertrophic pachymeningitis** and cranial nerve palsies
+ - **Transverse myelitis**: Usually longitudinally extensive transverse myelitis (LETM) spanning > 3 vertebral bodies
+ - Involve both halves of spinal cord, variable swelling
+ - Enhancement absent/minimal, patchy in active cases
+ - **Salivary gland abnormalities** like duct strictures, sialectasis, enlargement, or atrophy may be seen in autoimmune disorders like SLE and Sjögren syndrome
+ - Evaluate parotid glands on brain MR and CT
+ - **Cervical lymphadenopathy** in SLE, may see perinodal inflammatory fat stranding
+ - Rarely associated with Kikuchi-Fujimoto disease (subacute necrotizing histiocytosis), especially if posterior triangle lymphadenopathy
+ - #### Location
+
+
+ - WM, GM
+ - Frontal, parietal subcortical WM most common
+ - #### Morphology
+
+
+ - Rounded or patchy lesions
+- ### CT Findings
+
+
+ - #### NECT
+
+
+ - Scattered patchy cortical/subcortical hypodensities
+ - May be normal, diffuse atrophy common
+ - May see focal infarcts, cerebral calcification, or potentially life-threatening cerebral edema
+ - #### CECT
+
+
+ - ↑ sensitivity for acute/subacute lesions
+ - #### CTA
+
+
+ - Often completely normal in NPSLE
+- ### MR Findings
+
+
+ - #### T2WI
+
+
+ - WMH in T2 and FLAIR
+ - Acute lesions on T2 suggesting active NPSLE
+ - New infarct, discrete GM lesions, diffuse GM hyperintensities, cerebral edema
+ - #### FLAIR
+
+
+ - Multifocal WMH
+ - #### T2* GRE
+
+
+ - GRE and SWI for hemorrhage, including CMB
+ - #### DWI
+
+
+ - Restricted diffusion (cytotoxic edema) in acute ischemia/infarct
+ - ↑ diffusion (vasogenic edema) in vasculopathy
+ - #### T1WI C+
+
+
+ - Acute/active CNS lesions may enhance
+ - #### MRA
+
+
+ - Look for extra-/intracranial thrombosis
+ - #### MRV
+
+
+ - May show dural venous sinus thrombosis
+ - Especially in antiphospholipid syndrome
+ - #### MRS
+
+
+ - ¹H-MRS in NPSLE patients
+ - ↓ N-acetyl aspartate in lesions, as well as normal-appearing WM/GM
+ - ↑ choline related to disease activity, stroke, inflammation, chronic WM disease
+ - No ↑ in lactate → anaerobic metabolism not fundamental characteristic of NPSLE
+ - ↑ in lactate in acute infarcts
+ - ↓ glutamine and glutamate (neuronal and axonal loss with ↓ synapses; ↑ myoinositol (astrogliosis and neuronal/axonal damage, marker of poor prognosis)
+ - MRS findings directly correlate with severity of neuropsychiatric symptoms
+- ### Angiographic Findings
+
+
+ - CTA/MRA/DSA rarely detects cerebral lupus vasculitis
+- ### Nuclear Medicine Findings
+
+
+ - #### PET
+
+
+ - Parietooccipital hypometabolism = most conspicuous finding in MR-negative NPSLE
+ - Tc-99m ethyl cysteinate dimer brain SPECT
+ - Sensitive tool for early detection of brain abnormalities in SLE (more sensitive than MR)
+ - Relatively nonspecific regional cerebral cortical hypoperfusion
+ - Most hypoperfused areas: Parietal, frontal, and temporal lobes (MCA territory)
+ - Least hypoperfused area: Cerebellum
+ - Positive findings also seen in patients without neuropsychiatric signs/symptoms
+ - Secondary to subclinical brain involvement or cerebral atrophy (due to steroid therapy)
+ - Occasionally may show transient hyperperfusion
+- ### Imaging Recommendations
+
+
+ - #### Best imaging tool
+
+
+ - MR more sensitive than CT
+ - #### Protocol advice
+
+
+ - MR with T2, FLAIR, DWI; consider PET if MR normal
+
+## DIFFERENTIAL DIAGNOSIS
+
+- [Arteriolosclerosis](/document/arteriolosclerosis/07e561a5-0554-4867-b811-448c36890ee3)
+ - Caused by diabetes, HTN, hypercholesterolemia
+ - T2-hyperintense lesions within deep GM (basal ganglia, thalamus), centrum semiovale
+ - Diffuse, confluent regions of periventricular hyperintense WM involvement (leukoaraiosis)
+- [Multiple Sclerosis](/document/multiple-sclerosis/abe95a5e-394f-411b-aca6-72ab160a1d0d)
+ - T2-hyperintense WM lesions
+ - Lesions radially oriented along WM tracts
+ - Periventricular WM (callososeptal interface), juxtacortical/cortical, infratentorial, and cord
+ - SLE lesions not confined to periventricular WM, favor gray-white junction or involve cortex/deep nuclei
+- ### Antiphospholipid Antibody Syndrome
+
+
+ - May or may not be associated with SLE
+ - Spontaneous fetal loss, thrombocytopenia
+ - Early stroke, recurrent arterial + venous thromboses
+ - Infarcts of various sizes and T2-hyperintense WM foci
+ - Characteristic atrophy pattern in **parietal lobes** with relative sparing of frontotemporal lobes
+ - Cardiac valve abnormalities in 1/3, most commonly mitral valve thickening
+ - Dementia in 10%, seizures in 13%
+- [Lyme Encephalopathy (Neuroborreliosis)](/document/lyme-disease/082aad00-d3cf-4b75-a9aa-5ed2c724959c)
+ - Can mimic MS
+ - Hyperintense periventricular and other WM lesions on T2 MR, may show enhancement
+- [Susac Syndrome](/document/susac-syndrome/5408755d-e5f0-488c-9f84-a4e4d91664eb)
+ - Microangiopathy of unknown etiology
+ - Triad of encephalopathy, branch retinal artery stenoses, hearing loss
+ - Deep WM, corpus callosum multifocal hyperintense lesions on T2, FLAIR MR
+ - Central CC > callososeptal interface
+ - May enhance (acute)
+ - Central callosal "holes" in subacute/chronic
+ - Usually self-limited, fluctuating, monophasic illness
+ - Duration 2-4 years (from 6 months up to 5 years)
+- [Other Vasculitides](/document/miscellaneous-vasculitis/221f737a-6bfa-4331-b296-402e40973f7e)
+ - [Primary angiitis of CNS, polyarteritis nodosa (PAN), Granulomatosis with polyangiitis (GPA), Behçet disease, syphilis, Sjögren syndrome](/document/primary-arteritis-of-cns/490b3aed-37e2-4ec6-95dd-76efc734490f)
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Etiology
+
+
+ - Pathogenesis of NPSLE likely multifactorial
+ - No pathognomonic brain lesion
+ - Diverse nonspecific lesions of varying etiology
+ - Diffuse neuropsychiatric symptoms
+ - Neuronal dysfunction mediated by antibodies: Antineuronal, antiribosomal P-protein, and anticytokines
+ - Focal neurologic symptoms
+ - Circulating immune complexes → vascular injury
+ - Endothelial cell activation by cytokines and complement activation → occlusive vasculopathy
+ - APLA → macro- and microvascular thrombosis
+ - Antibody-mediated [aquaporin-4, myelin oligodendrocyte glycoprotein (MOG)] → myelopathy and cranial neuropathy
+ - Late stage SLE: Accelerated atherosclerosis
+ - ↑ intravascular complement turnover and APL-Ab
+ - #### Genetics
+
+
+ - Genetic predisposition to SLE
+ - HLA-DR2, HLA-DR3, null complement alleles
+ - Congenital deficiencies of complement (C4, C2)
+ - #### Associated abnormalities
+
+
+ - Libman-Sacks endocarditis, emboli, APS, PRES
+- ### Staging, Grading, & Classification
+
+
+ - Central or peripheral NPSLE
+ - Active &/or previously active NPSLE
+ - Acute or chronic NPSLE
+- ### Gross Pathologic & Surgical Features
+
+
+ - Cortical atrophy, infarcts, hemorrhage
+ - Vasculitis → CNS ischemia or hemorrhage (intraparenchymal/subarachnoid)
+ - Edema → reversible leukoencephalopathy
+ - WM degeneration, myelin vacuolation of spinal cord
+- ### Microscopic Features
+
+
+ - Vasculopathy
+ - Most common, but nonspecific finding
+ - Hyalinization, endothelial proliferation, and perivascular gliosis, mainly of small blood vessels
+ - True vasculitis of CNS is rare in SLE
+ - Hemorrhage, ischemic demyelination, MS-like demyelination, gliosis
+ - Subtle cerebral edema in diffuse NPSLE
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - NPSLE prevalence variable (40-90%), ~ 56% in high-quality studies
+ - Migraine, seizures, stroke, chorea
+ - Transverse myelopathy, cranial neuropathies, aseptic meningitis
+ - Psychosis, mood disorders, acute confusional state, cognitive dysfunction
+ - Subclinical CNS disease in SLE: Transient event
+ - #### Clinical profile
+
+
+ - Multisystem autoimmune disorder that affects respiratory, cardiovascular, GI, GU, MSK systems and CNS
+ - Cerebral involvement may precede full-blown SLE picture or may develop during course of disease
+ - Most frequently within first 3 years
+ - Diffuse psychiatric or focal neurologic symptoms
+ - Movement disorders (chorea, parkinsonism)
+ - 25-40% of SLE patients have APS
+- ### Demographics
+
+
+ - #### Age
+
+
+ - All groups affected; peak incidence: 20-45 years
+ - #### Sex
+
+
+ - Strong female predominance (as high as 5:1 during childbearing years)
+ - #### Ethnicity
+
+
+ - High prevalence in Black women
+ - #### Epidemiology
+
+
+ - Incidence of SLE (USA): 14.6-50.8 in 100,000 people
+- ### Natural History & Prognosis
+
+
+ - Neurologic complications worsen prognosis of SLE
+ - Transient neurologic deficits, chronic brain injury
+ - SLE patients with APL-Ab have additional risk for neuropsychiatric events
+ - Mortality rate in NPSLE: 7-40%
+- ### Treatment
+
+
+ - High-dose glucocorticoids and IV cyclophosphamide for severe symptoms
+ - Rituximab, IV immunoglobulins, plasmapheresis if no response
+ - Azathioprine and mycophenolate for mild to moderate symptoms or for maintenance therapy
+ - Lifelong anticoagulation for APL-Ab-mediated thromboembolic events
+ - Primary prevention of accelerated ASVD and narrowing of blood vessels: Prophylactic aspirin, lipid-lowering drugs
+
+## DIAGNOSTIC CHECKLIST
+
+- ### Consider
+
+
+ - Difficult to differentiate active from old NPSLE lesions
+ - Obtain MR with DWI within 24 hours of neurologic event
+- ### Image Interpretation Pearls
+
+
+ - Most important role of imaging in NPSLE: Assessment of acute focal (stroke-like) neurologic deficits
+ - Lupus-related CNS vasculitis, inflammatory lesions
+ - Thromboembolic events due to vasculopathy or endocarditis (Libman-Sacks)
+ - APL-Ab-mediated thrombosis
+ - Microangiopathy (including thrombotic thrombocytopenic purpura)
+ - Accelerated ASVD
+ - Negative brain MR does not exclude cerebral lupus
+
+ 43e060a8-0974-40b6-9530-b6b95d044eae
+
+## References
+
+## Selected References
+
+1. [Bai H et al: Global research landscape on antiphospholipid syndrome and systemic lupus erythematosus: trends, collaborations, and future directions. Autoimmun Rev. 24(1):103696, 2025](http://www.ncbi.nlm.nih.gov/pubmed/?term=39561865%5Bpmid%5D)
+1. [Ota Y et al: Central nervous system systemic lupus erythematosus: pathophysiologic, clinical, and imaging features. Radiographics. 42(1):212-32, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=34990324%5Bpmid%5D)
+1. [John TJ et al: SLE pachymeningitis and multiple cranial nerve palsies: a case report and review of the literature. Lupus. 28(9):1154-7, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31333063%5Bpmid%5D)
+1. [Mvambo N et al: Clinical, neuroimaging and immunological phenotype of South African neuropsychiatric systemic lupus erythematosus patients. Lupus. 28(5):685-94, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31018814%5Bpmid%5D)
+1. [Yeoh H et al: Relationship between cerebral microbleeds and white matter MR hyperintensities in systemic lupus erythematosus: a retrospective observational study. Neuroradiology. 61(3):265-74, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30415319%5Bpmid%5D)
+1. [McGlasson S et al: Neurological disease in lupus: toward a personalized medicine approach. Front Immunol. 9:1146, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29928273%5Bpmid%5D)
+1. [Vivaldo JF et al: Definition of NPSLE: does the ACR nomenclature still hold? Front Med (Lausanne). 5:138, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29904630%5Bpmid%5D)
+1. [Magro-Checa C et al: Management of neuropsychiatric systemic lupus erythematosus: current approaches and future perspectives. Drugs. 76(4):459-83, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26809245%5Bpmid%5D)
+1. [Nardone R et al: Longitudinally extensive transverse myelitis in systemic lupus erythematosus: case report and review of the literature. Clin Neurol Neurosurg. 129C:57-61, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25544684%5Bpmid%5D)
+1. [Piga M et al: Twenty-year brain magnetic resonance imaging follow-up study in systemic lupus erythematosus: factors associated with accrual of damage and central nervous system involvement. Autoimmun Rev. ePub, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25617815%5Bpmid%5D)
+1. [Saison J et al: Systemic lupus erythematosus-associated acute transverse myelitis: manifestations, treatments, outcomes, and prognostic factors in 20 patients. Lupus. 24(1):74-81, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25117654%5Bpmid%5D)
+1. [Sarbu N et al: Advanced and Conventional Magnetic Resonance Imaging in Neuropsychiatric Lupus. F1000Res. 4:162, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26236469%5Bpmid%5D)
+1. [Jeong HW et al: Brain MRI in neuropsychiatric lupus: associations with the 1999 ACR case definitions. Rheumatol Int. 14(6):510-6, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25315702%5Bpmid%5D)
+1. [Kaichi Y et al: Brain MR findings in patients with systemic lupus erythematosus with and without antiphospholipid antibody syndrome. AJNR Am J Neuroradiol. 35(1):100-5, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=23886740%5Bpmid%5D)
+1. [Steup-Beekman GM et al: Neuropsychiatric manifestations in patients with systemic lupus erythematosus: epidemiology and radiology pointing to an immune-mediated cause. Ann Rheum Dis. 72 Suppl 2:ii76-9, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23253914%5Bpmid%5D)
+1. [Borowoy AM et al: Neuropsychiatric lupus: the prevalence and autoantibody associations depend on the definition: results from the 1000 faces of lupus cohort. Semin Arthritis Rheum. 42(2):179-85, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22595642%5Bpmid%5D)
+1. [Curiel R et al: PET/CT imaging in systemic lupus erythematosus. Ann N Y Acad Sci. 1228:71-80, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21718325%5Bpmid%5D)
+1. [Lim KS et al: Periodic lateralized epileptiform discharges in neuropsychiatric lupus: association with cerebritis in magnetic resonance imaging and resolution after intravenous immunoglobulin. Lupus. 19(6):748-52, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20133346%5Bpmid%5D)
+1. [Baizabal-Carvallo JF et al: Posterior reversible encephalopathy syndrome as a complication of acute lupus activity. Clin Neurol Neurosurg. 111(4):359-63, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19128872%5Bpmid%5D)
+1. [Appenzeller S et al: Quantitative magnetic resonance imaging analyses and clinical significance of hyperintense white matter lesions in systemic lupus erythematosus patients. Ann Neurol. 64(6):635-43, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=19107986%5Bpmid%5D)
+1. [Petri M et al: Brain magnetic resonance imaging in newly diagnosed systemic lupus erythematosus. J Rheumatol. 35(12):2348-54, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18793003%5Bpmid%5D)
+1. [Janardhan V et al: Anticardiolipin antibodies and risk of ischemic stroke and transient ischemic attack: the Framingham cohort and offspring study. Stroke. 35(3):736-41, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14764933%5Bpmid%5D)
+1. [Jennings JE et al: Value of MRI of the brain in patients with systemic lupus erythematosus and neurologic disturbance. Neuroradiology. 46(1):15-21, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14648006%5Bpmid%5D)
+1. [Susac JO et al: MRI findings in Susac's syndrome. Neurology. 61(12):1783-7, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14694047%5Bpmid%5D)
+
+## Anatomy
+
+### White Matter Tracts
+Brain/ANATOMY:846101a2-e892-4c70-9a32-c9fa887d073a
+
+### Language Overview
+Brain/ANATOMY:40f2ed79-0d31-4943-aaa2-7c3244a7e87b
+
+### Aortic Arch and Great Vessels
+Brain/ANATOMY:a7a252f0-2ac6-402a-8c87-cfce8adc799b
+
+### Posterior Cerebral Artery
+Brain/ANATOMY:7bad3118-2ae8-4727-8f8d-fd8175c4e8c2
+
+### Intracranial Venous System Overview
+Brain/ANATOMY:fa5537f5-50aa-4a7d-abd1-a469b8a8b55f
+
+### Dural Sinuses
+Brain/ANATOMY:deb22ea4-1ac5-4542-a85a-8945376ad725
+
+### Posterior Fossa Veins
+Brain/ANATOMY:7b5caf92-e4eb-4a23-ba29-45d1dbb1af65
+
+### Brain
+Ultrasound/ANATOMY:080771c2-02f3-408d-ad70-04a80d849500
+
+### Transcranial Doppler
+Ultrasound/ANATOMY:914fd68b-ddab-4640-bcc4-883c425d13f8
+
+### Extracranial Veins
+Brain/ANATOMY:83693722-9b65-4d3c-8f5b-88c9824b4252
+
+## Cases
+
+- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '8d7e0238-550a-493f-9442-cb39f8b8c964', 'description': 'Neuropsychiatric lupus is not uncommon as CNS involvement may occur in up to 75% of patients with SLE. Frank vasculitis is rare.\n\nLateral views from a digital subtraction angiogram of the left internal carotid artery (image 1) and vertebrobasilar circulation (image 2) in this case show multifocal areas of alternating stenosis (arrows) and dilatation. These findings are suggestive of lupus vasculitis in this patient.', 'history': 'Migraine headaches, psychosis and mood disorders in a patient with a positive family history of SLE. ', 'imagePoolId': 'e8a19d87-4696-4b26-9fd2-00bc39e8790d', 'name': 'Vasculitis', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '453e121c-9222-4005-8744-d141ed679550', 'description': 'CNS lupus has many manifestations, both direct (such as lupus vasculitis and emboli from endocarditis) and indirect. A serious complication is hypertensive encephalopathy secondary to severe renal involvement, which is what happened in this case.\n\nAxial NECT scans show diffuse confluent low density areas in the deep white matter of both hemispheres, extending into the subcortical white matter. Diffuse cerebral edema caused by CNS lupus was diagnosed. If the involvement is primarily in the posterior circulation, it may be a manifestation of posterior reversible encephalopathy syndrome (PRES).', 'history': 'Known SLE with coma, severe systemic hypertension.', 'imagePoolId': '63b04e2e-ab45-4b67-b519-9b50d33c54dc', 'name': 'Complications', 'teachingPoint': None}], 'caseType': 'typical', 'name': 'TYPICAL'}
+- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '5295d165-0635-45ce-8065-529d46189998', 'description': 'A follow-up MR scan was obtained ten days after blood pressure had normalized. The axial T1WI (#1) shows multifocal areas of gyriform hyperintensity in the occipital cortex as well as the frontal gray matter near the anterior watershed zone. T2WI (#2) shows changes of late subacute hemorrhage in the frontal lobes (arrows) with some persisting edema in the subcortical white matter of the occipital lobes (curved arrows). \n\nComment: Young patients with SLE may have predominately renal involvement, as happened in this case. Renal failure leading to acute hypertension may cause PRES. PRES is an uncommon--but not unheard of--manifestation of SLE.', 'history': 'Child with known SLE, renal failure, acute onset of hypertension and seizures. Initial CT scan (not shown) demonstrated classic findings of posterior reversible encephalopathy syndrome (PRES).', 'imagePoolId': 'f16887a7-977e-44d8-a4af-bb49b8363eb8', 'name': 'Chronic renal failure', 'teachingPoint': None, 'demographics': '11 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '4a806fea-e092-4c08-857f-864229536dc2', 'description': 'Close-up view of the axial NECT scan (#1) obtained in the emergency room shows the basal ganglia appear hypodense and the deep gray matter/white matter interfaces are obliterated. \n\nInitial MR scan is shown in images #2-6. Axial T2WI (#2) shows extensive hyperintensity in the basal ganglia, which appear somewhat swollen and compress the lateral ventricles. FLAIR scans (#3-5) show lesions in the periaqueductal gray matter, both hippocampi, and the basal ganglia. Contrast-enhanced axial T1WIs (#6, 7) show some punctate and linear foci of perivascular enhancement in the basal ganglia and thalami (arrows). \n\nImages #8, 9 are from the MR scan approximately one week later. The axial T1 C+ MR (#8) now shows cavitation in the basal ganglia lesions. DWI (#9) shows bithalamic areas of restricted diffusion indicating frank infarction. \n\nImages #10-12 are from the cervical and thoracic spine MR obtained when the patient subsequently developed symptoms of transverse myelopathy. They show multisegmental areas of increased signal intensity in the central gray matter of the spinal cord (arrows), consistent with lupus myelopathy possibly caused by vasculitis in the penetrating spinal arteries.', 'history': 'Known SLE, presented with chorea and seizures. As these resolved, the patient developed symptoms of transverse myelopathy.', 'imagePoolId': '7a5512ef-fcef-43f5-8094-6d34e8624e1a', 'name': 'Brain and spinal cord lesions', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '200f8dfa-8249-4244-a545-d624097da97e', 'description': 'Cortical atrophy with multifocal infarcts may occur in patients with SLE. These patients often have elevated anticardiolipin and lupus anticoagulant antibodies. Two axial FLAIR scans in this case show both cortical (arrows) and confluent deep white matter infarcts in the setting of generalized atrophy. The multiple clustered hyperintense foci seen on image 2 (curved arrow) probably represent microinfarcts.', 'history': 'Longstanding anticardiolipin antibody syndrome with multiple ischemic episodes. ', 'imagePoolId': 'd771270d-b333-415e-901f-aed9d30adab1', 'name': 'Multiple infarcts', 'teachingPoint': None, 'demographics': '51 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '6b18ef81-dc09-4e07-b01c-e0cf979ac635', 'description': 'True CNS lupus vasculitis is actually relatively rare. When it occurs, as in this case, it may be manifested by multifocal linear and punctate enhancing foci in the basal ganglia, brainstem, and subcortical white matter.', 'history': 'Patient with known SLE.', 'imagePoolId': 'bf6a613d-7525-4f86-8bf1-17034d77ae2f', 'name': 'Florid', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '323db410-3965-46ff-bf08-12cc84f93e03', 'description': 'Sagittal pre-contrast T1WI shows a somewhat swollen brainstem with mixed iso- and hypointense signal (#1). Axial (#2) and coronal (#3) T2WIs show hyperintensity in the pons, major cerebellar peduncles, and basal ganglia (especially the thalami). Axial (#4) and coronal post-contrast T1WIs (#5, 6) show some ring-enhancement in the areas of ischemic necrosis (curved arrows, #4, 6) as well as the white matter of the external capsule (arrows, #6). \n\nComment: True lupus vasculitis, which this patient has, is uncommon. Necrotic infarcts are even less common.', 'history': 'Known SLE with multiple cranial neuropathies.', 'imagePoolId': '5310b568-c886-4bc8-b556-e07a5f560704', 'name': 'Vasculitis with necrotic ring-enhancing infarcts', 'teachingPoint': None}], 'caseType': 'variant', 'name': 'VARIANT'}
+
+
+## Images
+
+
+### Selected Images
+
+
+*Axial FLAIR MR in neuropsychiatric SLE (NPSLE) shows a large right frontal white matter hyperintensity (WMH)
extending into gray matter
, appearing as an inflammatory lesion WMH.*
+
+
+*Axial FLAIR MR in neuropsychiatric SLE (NPSLE) shows a large right frontal white matter hyperintensity (WMH)
extending into gray matter
, appearing as an inflammatory lesion WMH.*
+
+![Coronal T1 C+ MR in the same patient shows linear enhancement in WMH
. Brain MR findings in NPSLE include WMH [inflammatory lesions or small vessel disease (SVD)], vasculitis, large vessel disease (LVD), cerebral microbleeds (CMBs), migratory edema, postictal edema, and PRES. Biopsy showed CNS lupus vasculitis.](images/app.statdx.com_image_thumbnail_c9c4ede1-faa4-476c-a99b-57b44b441cfd_annotated_true_size_900_quality_90_bf5e5efff042a34df874ee1a9020eaec8609528a.jpg)
+*Coronal T1 C+ MR in the same patient shows linear enhancement in WMH
. Brain MR findings in NPSLE include WMH [inflammatory lesions or small vessel disease (SVD)], vasculitis, large vessel disease (LVD), cerebral microbleeds (CMBs), migratory edema, postictal edema, and PRES. Biopsy showed CNS lupus vasculitis.*
+
+
+*Axial FLAIR MR in a patient with NPSLE and seizures shows bilateral frontal and parietooccipital gyral hyperintensities
with intervening perirolandic cortex sparing
. These resolved on follow-up MR after a few days (not shown), suggesting postictal edema/PRES. Note multifocal tiny chronic SVD WMH
and an acute left frontal scalp hematoma
due to head hitting floor during seizure.*
+
+
+*Axial T2 MR in a patient with SLE shows a large infarct in the right middle cerebral artery territory (LVD)
.*
+
+
+*Axial T2 MR in a patient with NPSLE shows extensive hyperintensity and edema in basal ganglia
and thalami
. Note hyperintensity in the external and extreme capsules
. The findings could be due to a neurotoxic response related to antineuronal antibodies or central variant of PRES.*
+
+
+*Axial FLAIR MR in a 32-year-old woman with NPSLE shows marked enlargement and hyperintensity of medulla
. Following treatment, her symptoms and imaging findings completely resolved, suggesting PRES.*
+
+
+*Sagittal T2 MR (left) in lupus myelitis shows T2-hyperintense, longitudinally extensive transverse myelitis (LETM) in the mid to lower thoracic cord
. Sagittal T1 C+ MR (right) shows patchy enhancement
.*
+
+
+*Axial T2 MR (left) shows that both halves of the spinal cord are involved, mainly central gray matter, with extension into left more than right peripheral WM
. Axial T1 C+ MR (right) shows the patchy enhancement
. SLE myelopathy is antibody mediated (aquaporin-4, myelin oligodendrocyte glycoprotein).*
+
+
+*Axial NECT in SLE patient shows bilateral heterogeneous parotid glands with small hypodense areas of fatty atrophy
. SLE and Sjögren syndrome can have this and should be looked for, especially in the young with brain SVD.*
+
+
+*Axial FLAIR MR in a 35-year-old woman with SLE with APS shows mild diffuse brain atrophy and WMH
and cortex
SVD. Note the parietal lobe atrophy
, characteristic of APS. Brain MR abnormalities, including infarcts and CMB, are more common in SLE with APS than in SLE without APS.*
+
+
+### Additional Images
+
+
+*Axial T2 MR shows typical WM lesions in NPSLE. There are numerous foci of abnormal signal in the WM of the frontal and parietal lobes.*
+
+
+*Axial FLAIR MR shows periventricular WM hyperintensities, large bilateral infarcts with cortical atrophy in a patient with SLE.*
+
+
+*Lateral view of carotid angiogram in a different patient with SLE shows multiple areas of arterial narrowing, consistent with vasculitis. This is a rare finding in neuropsychiatric lupus.*
+
+
+*Axial NECT shows diffuse WM hypodensity, consistent with cerebral edema in a patient with SLE.*
+
+
+*Sagittal T1 MR in another patient with SLE myelopathy shows expansion of the cervical spinal cord with hypointense signal abnormality.*
+
+
+*Axial FLAIR MR shows infarction in the right anterior cerebral artery and middle cerebral artery territory in a patient with SLE.*
+
+
+*Axial T1 C+ MR in a different patient with SLE shows multiple enhancing infarcts in the thalami and basal ganglia.*
+
+
+*Axial FLAIR MR in an SLE patient with new neurologic symptoms shows multiple foci of FLAIR hyperintensity in the WM
and corpus callosum
. This patient also had antiphospholipid syndrome, which is common in SLE.*
+
+
+*Lateral vertebrobasilar DSA shows multifocal stenoses
typical of a nonspecific vasculitis. This is uncommonly seen with lupus, which is more of a small-vessel vasculitis than other inflammatory vasculitides. DSA is often normal in SLE patients.*
+
+
+*Axial T1 MR shows multifocal areas of gyriform hyperintensity in the occipital cortex, as well as the frontal gray matter near the anterior watershed zone due to PRES, a secondary complication of systemic renal involvement from lupus.*
+
+
+*Sagittal T2 MR shows hyperintensity in the cervical spinal cord related to systemic lupus erythematosus myelitis, a type of transverse myelitis.*
+
+
+*Axial T2 MR shows hyperintensity in the enlarged pons and middle cerebellar peduncles
in this unusual variant of lupus vasculitis. Although small-vessel vasculitis commonly involves the posterior fossa, it is usually in a multifocal peripheral pattern.*
+
+
+*Axial T1 C+ MR in the same patient shows 2 regions of ring enhancement
in the central pons due to areas of ischemic necrosis. Lupus vasculitis, which this patient has, is uncommon. Necrotic infarcts in lupus are even less common.*
+
+
+*Coronal T2 MR in a 25-year-old woman with NPSLE and seizures shows bilateral multicystic parotid glands
. Note a tiny right frontal SVD WMH
. Multiple other SVD WMH were seen in other images.*
+
diff --git a/docs_md/articles/thoracic-aorta-and-great-vessel-anatomy_a87ab2f8-5c92-4e50-9fe6-d9e63d2b8cab.md b/docs_md/articles/thoracic-aorta-and-great-vessel-anatomy_a87ab2f8-5c92-4e50-9fe6-d9e63d2b8cab.md
new file mode 100644
index 0000000..81f0e4a
--- /dev/null
+++ b/docs_md/articles/thoracic-aorta-and-great-vessel-anatomy_a87ab2f8-5c92-4e50-9fe6-d9e63d2b8cab.md
@@ -0,0 +1,372 @@
+---
+title: "Thoracic Aorta and Great Vessel Anatomy"
+docid: "a87ab2f8-5c92-4e50-9fe6-d9e63d2b8cab"
+authors:
+ - key: "3d1e4c57-c1cf-4c89-b0f0-5d82b29a31e1"
+ value: "Suhny Abbara, MD, FACR, MSCCT, FNASCI"
+ - key: "2f6bbf02-1f58-4c88-94b9-48328b94d527"
+ value: "Michael T. Lu, MD"
+breadcrumbs:
+ -
+ name: "Vasculature"
+ slug: "vasculature"
+ treeNodeId: "6de1ee4d-afe9-419c-a868-d4074ec0fb7e"
+ -
+ name: "Anatomy"
+ slug: "anatomy"
+ treeNodeId: "3bbfaa8b-2dbc-41eb-b3da-3bd83a9737c4"
+ -
+ name: "Thoracic Aorta and Great Vessel Anatomy"
+ slug: "thoracic-aorta-and-great-vessel-an-"
+ treeNodeId: null
+category: "Vasculature"
+documentVersionId: "25945f40-ffee-482e-ad2c-dfc52e71938a"
+imageCount: 21
+lastUpdated: "08/06/20"
+pageDescription: "Thoracic Aorta and Great Vessel Anatomy"
+pageKeywords: "Vasculature, Anatomy, Thoracic Aorta and Great Vessel Anatomy"
+pageTitle: "Thoracic Aorta and Great Vessel Anatomy | STATdx"
+enhancedTitle: "Thoracic Aorta and Great Vessel Anatomy"
+type: "ANATOMY"
+references: true
+breadcrumbs:
+ - "Vasculature"
+ - "Anatomy"
+ - "Thoracic Aorta and Great Vessel Anatomy"
+---
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - Common carotid artery (CCA)
+ - Internal carotid artery (ICA)
+ - External carotid artery (ECA)
+ - Subclavian artery (SCA)
+ - Vertebral artery (VA)
+ - Sinus of Valsalva (SoV)
+ - Sinotubular junction (STJ)
+ - Ascending aorta (AsAo)
+ - Descending thoracic aorta (DsAo)
+- ### Definitions
+
+
+ - Aortic root
+ - Anulus to STJ
+ - AsAo
+ - Extends up to origin of brachiocephalic trunk
+ - Aortic arch or transverse aorta
+ - From brachiocephalic trunk to ligamentum arteriosum
+ - Ligamentum arteriosum is remnant of ductus arteriosus and typically lies immediately distal to origin of left SCA
+ - Aortic isthmus
+ - Segment of distal aortic arch between left subclavian origin and ligamentum arteriosum
+ - DsAo
+ - Ligamentum arteriosum to diaphragmatic hiatus
+
+## IMAGING ANATOMY
+
+- ### Overview
+
+
+ - Thoracic aorta is divided into 4 segments from proximal to distal
+ - Aortic root
+ - AsAo
+ - Aortic arch
+ - DsAo
+ - Aortic root extends from aortic anulus to STJ
+ - Aortic anulus
+ - Virtual ring at base of aortic root defined by lowest attachment point of aortic cusps; cusp attachment site has complex crown shape
+ - Typically elliptical shape
+ - Important for sizing of aortic valve replacement
+ - SoV
+ - 3 sinuses defined by coronary origins
+ - Left coronary artery arises from left coronary sinus
+ - Right coronary artery arises from right coronary sinus
+ - Interatrial septum points toward noncoronary sinus, which is typically located posteriorly and to right on axial images
+ - SoV is typically greatest caliber segment of thoracic aorta
+ - STJ
+ - Anatomical landmark dividing aortic root from tubular AsAo
+ - Narrower than SoV
+ - AsAo extends from STJ to origin of brachiocephalic trunk
+ - Typically greatest in diameter and nearly orthogonal to axial plane at right pulmonary artery level, convenient and standard level of measurement
+ - Aortic arch extends from brachiocephalic trunk to ligamentum arteriosum
+ - Distal arch or aortic isthmus short (~ 2 cm) segment between left subclavian origin and remnant of ductus arteriosus
+ - Aortic isthmus is typically narrower than adjoining aortic segments
+ - If ligamentum arteriosum cannot be identified, aortic arch can also be defined as extending past left subclavian origin
+ - Ductus diverticulum (or "bump") is focal smooth bulge at site of obliterated ductus arteriosus along undersurface of isthmus
+ - Normal variant that can be mistaken for traumatic aortic injury, which also occurs at this location
+ - May become aneurysmal (> 3 cm)
+ - Aortic arch branch vessels to head, neck, upper extremities, and chest wall are termed great vessels
+ - Brachiocephalic trunk (innominate artery) is 1st and largest of great vessels of aortic arch; divides into right CCA and SCA
+ - Right SCA branches include right internal mammary, VA, thyrocervical, costocervical, and long thoracic arteries, and continues as axillary artery after margin of 1st rib
+ - Right CCA divides into ICA and ECA in neck
+ - Left CCA is 2nd great vessel from arch
+ - Divides into ICA and ECA
+ - Left SCA is 3rd and final great vessel from arch
+ - Gives off internal mammary, VA, thyrocervical, costocervical, and long thoracic arteries and continues as axillary artery
+ - Rare (3%) thyroid ima or thyroidea ima with inferior thyroid artery arises directly from aortic arch or innominate artery as opposed to normal origin from thyrocervical trunk
+ - DsAo extends from distal arch to diaphragmatic hiatus, where it continues as abdominal aorta
+ - Descending aorta is typically smaller in caliber than AsAo
+ - Aortic spindle is bulge in proximal descending aorta just distal to isthmus
+ - Commonly seen in children but can persist into adulthood
+ - Descending aorta gives off important small arteries
+ - Bronchial arteries
+ - Intercostal arteries
+ - Supreme intercostals supply T1-T3; arise from costocervical trunk of SCAs
+ - Paired intercostals arise directly from descending aorta from T4-T12
+ - Thoracic spinal cord supply comes from DsAo
+ - Anterior spinal artery is supplied from intercostal and bronchial arteries at T4-T5
+ - Artery of Adamkiewicz arises from intercostal arteries at T6-T12 (75%)
+ - Esophageal, pericardial, superior phrenic, and other miscellaneous mediastinal branches
+ - Central venous anatomy
+ - Jugular veins
+ - Internal jugular veins drain head and neck; joined by external jugular veins draining face and scalp
+ - Subclavian veins
+ - Originate at axillary vein transition at 1st rib margin
+ - Typically valveless; joined by cephalic vein
+ - Brachiocephalic veins
+ - Formed by junction of subclavian and internal jugular veins
+ - Right is short and vertical; left is longer and crosses mediastinum anterior to great vessels
+ - Tributaries: Internal mammary, vertebral, pericardiophrenic, 1st intercostal, inferior thyroidal
+ - Superior vena cava (SVC)
+ - Formed by right and left brachiocephalic veins
+ - 6-8 cm long, up to 2 cm in diameter
+ - Azygos vein joins above pericardium; SVC enters right atrium
+- ### Anatomy Relationships
+
+
+ - Aortic arch variants
+ - Right aortic arch (< 0.1%); 2 types
+ - Mirror-image branching (65%); associated with cyanotic congenital heart disease in 90% of cases
+ - Aberrant left SCA or other great vessel origin (35%); not associated with cyanotic congenital heart disease
+ - Dilated origin of aberrant left SCA in 60% of cases; Kommerell diverticulum; if also ligamentum arteriosum → vascular ring and tracheal compression
+ - Double (duplicated) aortic arch (< 0.1%)
+ - Arises from 3rd rather than 4th branchial arch
+ - High location in chest, near lung apex
+ - May have anomalous great vessel origins
+ - Coarctation (< 0.1%)
+ - Congenital narrowing of aortic arch, usually distal to left subclavian origin
+ - May be preductal (infantile), juxtaductal, or postductal (adult)
+ - Common with other congenital aortic pathology, such as bicuspid aortic valve and Turner syndrome
+ - Great vessel origin variants
+ - Bovine arch (20%): Left CCA may have common origin with or arise from innominate artery
+ - 4-vessel arch (5%): Left VA may arise directly from aortic arch between left CCA and left SCA rather than from left SCA
+ - Aberrant right SCA: Right SCA may arise separately from aortic arch, distal to left SCA
+ - Diverticulum of Kommerell: Dilatation at origin of aberrant right SCA; can be associated with dysphagia (dysphagia lusoria) when large
+
+## ANATOMY IMAGING ISSUES
+
+- ### Imaging Recommendations
+
+
+ - Thoracic aorta is imaged with catheter angiography, transthoracic or transesophageal echocardiography, CT angiography, and MR angiography
+ - CT angiography: Protocol may include noncontrast, arterial, and delayed-phase imaging
+ - Noncontrast images are helpful in cases of extensive calcium, prior surgery, or suspicion for intramural hematoma
+ - Delayed images better delineate mediastinal anatomy and are also helpful in postsurgical patients when there is concern for endoleak
+ - Noncontrast and delayed images are often not necessary for routine follow-up of known aortic aneurysm
+ - Thin-section (≤1.25 mm) reconstruction is preferred
+ - ECG-gated or high-pitch dual-source CT is preferred for accurate evaluation of aortic root due to cardiac motion artifact if root pathology is suspected or followed
+ - MR angiography: Contrast angiography is preferred
+ - Noncontrast sequences often give diagnostic study and are test of choice when there is contraindication to iodinated and gadolinium contrast agent
+ - In general, for follow-up exams it is best to employ consistent imaging modality and measurement technique
+ - "Candy cane" oblique view places thoracic aorta in profile and is commonly employed for catheter, CT, and MR angiography
+ - Aortic measurement should be performed in plane orthogonal to longitudinal axis of aorta
+ - Measurements made in axial plane may be oblique to aorta and less accurate and reproducible
+- ### Transcatheter Aortic Valve Implantation/Replacement Assessment
+
+
+ - For severe aortic stenosis in nonsurgical patients
+ - Transfemoral or transapical approach may be chosen
+ - CT angiography plays increasing role in sizing of aortic anulus and determining suitability of iliofemoral approach
+ - Indications and criteria are evolving
+ - PARTNER trial exclusion criteria
+ - Native aortic anulus size < 18 mm or > 25 mm
+ - Iliofemoral vessels too calcified or small to accommodate 22F or 24F introducer sheath (minimum luminal diameter of 7-8 mm, respectively)
+ - Severe aortic or iliofemoral disease that would preclude safe placement, such as aneurysm, tortuosity, extensive atheroma, or dissection
+ - Bulky calcified aortic valve leaflets in close proximity to coronary ostia
+
+ 51823a80-9158-49b5-940f-aff30a224967
+
+## References
+
+## Selected References
+
+1. [Volonghi P et al: Automatic extraction of three-dimensional thoracic aorta geometric model from phase contrast MRI for morphometric and hemodynamic characterization. Magn Reson Med. 75(2):873-82, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=25754538%5Bpmid%5D)
+1. [Ippolito D et al: Low kV settings CT angiography (CTA) with low dose contrast medium volume protocol in the assessment of thoracic and abdominal aorta disease: a feasibility study. Br J Radiol. 88(1049):20140140, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25784185%5Bpmid%5D)
+1. [Smith CR et al: Transcatheter versus surgical aortic-valve replacement in high-risk patients. N Engl J Med. 364(23):2187-98, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21639811%5Bpmid%5D)
+1. [Hiratzka LF et al: 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease. Circulation. 121(13):e266-369, 2010. Erratum in: Circulation. 122(4):e410, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20233780%5Bpmid%5D)
+1. [Agarwal PP et al: Multidetector CT of thoracic aortic aneurysms. Radiographics. 29(2):537-52, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19325064%5Bpmid%5D)
+1. [Leipsic J et al: The evolving role of MDCT in transcatheter aortic valve replacement: a radiologists' perspective. AJR Am J Roentgenol. 193(3):W214-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19696262%5Bpmid%5D)
+1. [Davies M et al: Developmental abnormalities of the great vessels of the thorax and their embryological basis. Br J Radiol. 76(907):491-502, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12857711%5Bpmid%5D)
+
+
+## Images
+
+
+### Thoracic Aorta and Great Vessels
+
+
+*Graphic demonstrates the thoracic aorta and great vessel origins.*
+
+
+*Graphic demonstrates the thoracic aorta and great vessel origins.*
+
+
+*Graphic demonstrates the thoracic aorta and great vessel origins.*
+
+
+*Graphic depicts the branches of the descending thoracic aorta, including the intercostal, esophageal, and bronchial arteries. Typically, there are both superior and inferior left bronchial arteries and a single right bronchial artery (not pictured).*
+
+
+*Graphic depicts the branches of the descending thoracic aorta, including the intercostal, esophageal, and bronchial arteries. Typically, there are both superior and inferior left bronchial arteries and a single right bronchial artery (not pictured).*
+
+
+### Normal Anatomy of Thoracic Aorta and Great Vessels
+
+
+*Frontal chest radiograph shows a normal thoracic aorta. The aortic knob shadow is created by a superimposition of the aortic arch and proximal descending aorta. The lateral margin of the descending thoracic aorta should always be visible, but the medial margin is usually not perceptible. The lateral margin of the ascending aorta is visible as part of the right mediastinal border.*
+
+
+*Frontal chest radiograph shows a normal thoracic aorta. The aortic knob shadow is created by a superimposition of the aortic arch and proximal descending aorta. The lateral margin of the descending thoracic aorta should always be visible, but the medial margin is usually not perceptible. The lateral margin of the ascending aorta is visible as part of the right mediastinal border.*
+
+
+*Corresponding frontal projection of a catheter angiogram (left) and digital subtraction image (right) of the thoracic aorta illustrate normal anatomy of the aorta and great vessel origins.*
+
+
+*Corresponding frontal projection of a catheter angiogram (left) and digital subtraction image (right) of the thoracic aorta illustrate normal anatomy of the aorta and great vessel origins.*
+
+
+*Corresponding frontal projection of a catheter angiogram (left) and digital subtraction image (right) of the thoracic aorta illustrate normal anatomy of the aorta and great vessel origins.*
+
+
+### Aortic Root CT Anatomy
+
+
+*"Candy cane" oblique MPR of the thoracic aorta depicts the segments of the thoracic aorta. The aortic root (red) extends from the aortic anulus to the sinotubular junction. The ascending aorta (blue) extends to the origin of the brachiocephalic trunk. The aortic arch (yellow) extends to the ligamentum arteriosum. The descending thoracic aorta (green) extends to the diaphragmatic hiatus, where it continues as the abdominal aorta. Note the smooth outpouching along the inferior surface of the aortic arch at the remnant of the ductus arteriosum. This is a normal ductus diverticulum and should not be mistaken for a traumatic aortic injury.*
+
+
+*"Candy cane" oblique MPR of the thoracic aorta depicts the segments of the thoracic aorta. The aortic root (red) extends from the aortic anulus to the sinotubular junction. The ascending aorta (blue) extends to the origin of the brachiocephalic trunk. The aortic arch (yellow) extends to the ligamentum arteriosum. The descending thoracic aorta (green) extends to the diaphragmatic hiatus, where it continues as the abdominal aorta. Note the smooth outpouching along the inferior surface of the aortic arch at the remnant of the ductus arteriosum. This is a normal ductus diverticulum and should not be mistaken for a traumatic aortic injury.*
+
+
+*"Candy cane" oblique MPR of the thoracic aorta depicts the segments of the thoracic aorta. The aortic root (red) extends from the aortic anulus to the sinotubular junction. The ascending aorta (blue) extends to the origin of the brachiocephalic trunk. The aortic arch (yellow) extends to the ligamentum arteriosum. The descending thoracic aorta (green) extends to the diaphragmatic hiatus, where it continues as the abdominal aorta. Note the smooth outpouching along the inferior surface of the aortic arch at the remnant of the ductus arteriosum. This is a normal ductus diverticulum and should not be mistaken for a traumatic aortic injury.*
+
+
+*"Candy cane" view MRA shows a thoracic aorta.*
+
+
+*"Candy cane" view MRA shows a thoracic aorta.*
+
+
+*"Candy cane" view MRA shows a thoracic aorta.*
+
+
+*Three-chamber view from a CT angiogram depicts the anatomy of the left ventricular outflow tract and aortic root.*
+
+
+*Three-chamber view from a CT angiogram depicts the anatomy of the left ventricular outflow tract and aortic root.*
+
+
+*Three-chamber view from a CT angiogram depicts the anatomy of the left ventricular outflow tract and aortic root.*
+
+
+*Graphic depicts the most common configuration of the aortic arch, the 3-vessel arch.*
+
+
+*Graphic depicts the most common configuration of the aortic arch, the 3-vessel arch.*
+
+
+*Graphic depicts the most common configuration of the aortic arch, the 3-vessel arch.*
+
+
+*Graphic depicts common aortic arch variants. In the most common variant (upper left), the brachiocephalic trunk and left common carotid share a common origin. In the 2nd most common variant (upper right), the left common carotid arises from the brachiocephalic trunk. The left vertebral artery may arise directly from the aortic arch (lower left), between the left common carotid and subclavian arteries. The aberrant right subclavian arises from the distal aortic arch after the takeoff of the left subclavian (lower right), courses behind the trachea and esophagus to the right, and therefore may cause dysphagia (termed dysphagia lusoria).*
+
+
+*Graphic depicts common aortic arch variants. In the most common variant (upper left), the brachiocephalic trunk and left common carotid share a common origin. In the 2nd most common variant (upper right), the left common carotid arises from the brachiocephalic trunk. The left vertebral artery may arise directly from the aortic arch (lower left), between the left common carotid and subclavian arteries. The aberrant right subclavian arises from the distal aortic arch after the takeoff of the left subclavian (lower right), courses behind the trachea and esophagus to the right, and therefore may cause dysphagia (termed dysphagia lusoria).*
+
+
+*Graphic depicts common aortic arch variants. In the most common variant (upper left), the brachiocephalic trunk and left common carotid share a common origin. In the 2nd most common variant (upper right), the left common carotid arises from the brachiocephalic trunk. The left vertebral artery may arise directly from the aortic arch (lower left), between the left common carotid and subclavian arteries. The aberrant right subclavian arises from the distal aortic arch after the takeoff of the left subclavian (lower right), courses behind the trachea and esophagus to the right, and therefore may cause dysphagia (termed dysphagia lusoria).*
+
+
+*Digital subtraction catheter angiogram in the "candy cane" oblique view demonstrates 2 common aortic arch variants: Common origin of the brachiocephalic trunk and left common carotid artery (bovine arch) and a left vertebral artery arising directly from the aortic arch.*
+
+
+*Digital subtraction catheter angiogram in the "candy cane" oblique view demonstrates 2 common aortic arch variants: Common origin of the brachiocephalic trunk and left common carotid artery (bovine arch) and a left vertebral artery arising directly from the aortic arch.*
+
+
+### Standard Measurements
+
+
+*Volume-rendered 3D CT angiogram in "candy cane" view from a patient with bicuspid aortic valve shows characteristic aneurysmal bowing of the ascending aorta. White lines denote standard aortic measurement planes, orthogonal to the long axis of the respective aortic segment. From proximal to distal, they include aortic anulus, sinus of Valsalva, sinotubular junction, ascending aorta at the level of the right pulmonary artery, aortic arch between the origins of the left subclavian and common carotid arteries, and descending aorta.*
+
+
+*Volume-rendered 3D CT angiogram in "candy cane" view from a patient with bicuspid aortic valve shows characteristic aneurysmal bowing of the ascending aorta. White lines denote standard aortic measurement planes, orthogonal to the long axis of the respective aortic segment. From proximal to distal, they include aortic anulus, sinus of Valsalva, sinotubular junction, ascending aorta at the level of the right pulmonary artery, aortic arch between the origins of the left subclavian and common carotid arteries, and descending aorta.*
+
+
+*Multiplanar reformation of the aortic anulus shows the typical ovoid shape of the anulus. Accurate measurement of the anulus is important for sizing aortic valve replacements. Long- and short-axis diameters are reported. Anular circumference and area may also be helpful.*
+
+
+*Multiplanar reformation of the aortic anulus shows the typical ovoid shape of the anulus. Accurate measurement of the anulus is important for sizing aortic valve replacements. Long- and short-axis diameters are reported. Anular circumference and area may also be helpful.*
+
+
+*Image immediately above the aortic anulus depicts portions of the aortic valve cusps. The anulus is measured as a virtual ring defined by the attachment of the lowest points of each aortic cusp.*
+
+
+*Image immediately above the aortic anulus depicts portions of the aortic valve cusps. The anulus is measured as a virtual ring defined by the attachment of the lowest points of each aortic cusp.*
+
+
+### Aortic Root Short-Axis Planes
+
+
+*Multiplanar reformat through the sinus of Valsalva is shown. The sinus of Valsalva diameters are measured from commissure to cusp. Note that the interatrial septum points towards the noncoronary cusp in all projections. Inset shows the 3 diameter measurements (commissure to contralateral sinus) obtained in this plane.*
+
+
+*Multiplanar reformat through the sinus of Valsalva is shown. The sinus of Valsalva diameters are measured from commissure to cusp. Note that the interatrial septum points towards the noncoronary cusp in all projections. Inset shows the 3 diameter measurements (commissure to contralateral sinus) obtained in this plane.*
+
+
+*Oblique maximum-intensity projection image through the sinus of Valsalva depicts the coronary origins.*
+
+
+*Oblique maximum-intensity projection image through the sinus of Valsalva depicts the coronary origins.*
+
+
+*Oblique MPR orthogonal to the aorta at the level of the sinotubular junction shows that the sinotubular junction is of lower caliber than the sinus of Valsalva. Aortic diameters are most accurately and reproducibly measured in the plane orthogonal to the centerline of the aorta.*
+
+
+*Oblique MPR orthogonal to the aorta at the level of the sinotubular junction shows that the sinotubular junction is of lower caliber than the sinus of Valsalva. Aortic diameters are most accurately and reproducibly measured in the plane orthogonal to the centerline of the aorta.*
+
+
+### Standard Planes of Aorta
+
+
+*Axial CT at the level of the right pulmonary artery shows the ascending and descending aorta. The ascending aorta is often greatest in diameter and nearly orthogonal to the axial plane at this level.*
+
+
+*Axial CT at the level of the right pulmonary artery shows the ascending and descending aorta. The ascending aorta is often greatest in diameter and nearly orthogonal to the axial plane at this level.*
+
+
+*MPR orthogonal to the aortic long axis at the level of the aortic arch, between the origins of the left common carotid and left subclavian arteries, is the standard plane for aortic arch diameter measurement.*
+
+
+*MPR orthogonal to the aortic long axis at the level of the aortic arch, between the origins of the left common carotid and left subclavian arteries, is the standard plane for aortic arch diameter measurement.*
+
+
+*Oblique MIP in C view depicts the course of the right coronary artery and the origins of the left and right coronary arteries from the sinus of Valsalva.*
+
+
+*Oblique MIP in C view depicts the course of the right coronary artery and the origins of the left and right coronary arteries from the sinus of Valsalva.*
+
+
+### TAVI/R Planning
+
+
+*Curved multiplanar reformation high-pitch gated CT angiogram shows the entire aorta and left iliofemoral system (right). Upper left inset is a coned-down lateral radiograph of a transcatheter aortic valve replacement (TAVR). CT angiogram is increasingly used for TAVR planning. The dimensions of the aortic anulus (mid left inset) are critical for valve sizing. The minimum luminal diameter of the iliofemoral arteries (lower left inset) and the degree of tortuosity and calcification of the aorta and iliofemoral system (right) determine whether a transfemoral approach is possible.*
+
+
+*Curved multiplanar reformation high-pitch gated CT angiogram shows the entire aorta and left iliofemoral system (right). Upper left inset is a coned-down lateral radiograph of a transcatheter aortic valve replacement (TAVR). CT angiogram is increasingly used for TAVR planning. The dimensions of the aortic anulus (mid left inset) are critical for valve sizing. The minimum luminal diameter of the iliofemoral arteries (lower left inset) and the degree of tortuosity and calcification of the aorta and iliofemoral system (right) determine whether a transfemoral approach is possible.*
+
+
+*Oblique MIP depicts the coronary artery origins. Obstruction of the coronary ostia by displaced aortic valve leaflets is an infrequent but reported complication of TAVR, so the distance from the aortic anulus plane (yellow line) to the closest coronary ostium is provided. In this case, the distance to the right coronary ostium corresponds to the double-headed black arrow.*
+
+
+*Oblique MIP depicts the coronary artery origins. Obstruction of the coronary ostia by displaced aortic valve leaflets is an infrequent but reported complication of TAVR, so the distance from the aortic anulus plane (yellow line) to the closest coronary ostium is provided. In this case, the distance to the right coronary ostium corresponds to the double-headed black arrow.*
+
diff --git a/docs_md/articles/transcranial-doppler_914fd68b-ddab-4640-bcc4-883c425d13f8.md b/docs_md/articles/transcranial-doppler_914fd68b-ddab-4640-bcc4-883c425d13f8.md
new file mode 100644
index 0000000..9713b1e
--- /dev/null
+++ b/docs_md/articles/transcranial-doppler_914fd68b-ddab-4640-bcc4-883c425d13f8.md
@@ -0,0 +1,507 @@
+---
+title: "Transcranial Doppler"
+docid: "914fd68b-ddab-4640-bcc4-883c425d13f8"
+authors:
+ - key: "2a5e84a9-1a3f-42a4-b5ae-d081e54c8a55"
+ value: "Stella Sin Yee Ho, RDMS, RVT, PhD"
+ - key: "944c1fa1-404a-4d64-a8fd-ea6184a7c876"
+ value: "Deyond Y. W. Siu, MBChB, FRCR"
+ - key: "961f3a7f-ad62-43bc-98f4-5116b17ab812"
+ value: "Paula J. Woodward, MD, FSRU"
+breadcrumbs:
+ -
+ name: "Ultrasound"
+ slug: "ultrasound"
+ treeNodeId: "517432cb-177e-4b78-bd9f-4c2e751d132e"
+ -
+ name: "Anatomy"
+ slug: "anatomy"
+ treeNodeId: "43bd9326-ab1c-493e-8d96-8ad0aa58c66a"
+ -
+ name: "Brain and Spine"
+ slug: "brain-and-spine"
+ treeNodeId: "615c6d75-0c7c-43e3-87a7-df4e7ac2304b"
+ -
+ name: "Transcranial Doppler"
+ slug: "transcranial-doppler"
+ treeNodeId: null
+category: "Ultrasound"
+documentVersionId: "9b4aa905-077a-45cb-8a60-ef66bad53fde"
+imageCount: 61
+lastUpdated: "10/20/20"
+pageDescription: "Transcranial Doppler"
+pageKeywords: "Ultrasound, Anatomy, Brain and Spine, Transcranial Doppler"
+pageTitle: "Transcranial Doppler | STATdx"
+enhancedTitle: "Transcranial Doppler"
+type: "ANATOMY"
+breadcrumbs:
+ - "Ultrasound"
+ - "Anatomy"
+ - "Brain and Spine"
+ - "Transcranial Doppler"
+---
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - **Carotid arteries**
+ - Common carotid (CCA); internal carotid (ICA)
+ - **Cerebral arteries**
+ - Anterior (ACA); middle (MCA); posterior (PCA)
+ - **Communicating arteries**
+ - Anterior (ACoA); posterior (PCoA)
+ - **Posterior fossa vertebrobasilar arteries**
+ - Vertebral (VA); basilar (BA); posterior inferior cerebellar artery (PICA); anterior inferior cerebellar artery (AICA); superior cerebellar artery (SCA)
+ - **Sinuses**
+ - Superior sagittal (SSS); sphenoparietal (SPS); straight (SS); transverse (TS); cavernous (CS)
+ - **Cerebral veins**
+ - Basal vein of Rosenthal (BV); internal cerebral (ICV); great vein of Galen (GV); deep middle cerebral (dMCV); anterior cerebral (ACV)
+
+## GROSS ANATOMY
+
+- ### Intracranial Internal Carotid Artery
+
+
+ - Complex course with several vertical/horizontal segments, 3 genus (1 petrous, 2 cavernous)
+ - 6 intracranial segments (cervical ICA = C1)
+ - Petrous (C2), lacerum (C3), cavernous (C4), clinoid (C5), ophthalmic (C6), communicating (C7)
+- ### Anterior Cerebral Artery
+
+
+ - Smaller, more medial terminal branch of ICA with 3 segments
+ - **A1**: Horizontal or precommunicating segment
+ - Extends anteromedially above optic nerve & chiasm, below medial olfactory stria
+ - **A2**: Vertical or postcommunicating segment
+ - From ACoA junction, ascends in interhemispheric fissure, anterior to corpus callosum rostrum
+ - **A3**: Distal segment
+ - Pericallosal artery; begins distal to origin of callosomarginal artery
+ - **ACoA**: Communicates between A1 segments; completes anterior portion of circle of Willis
+ - Usually single, may be duplicated/triplicated/hypoplastic
+ - **Cortical branches**supply anterior 2/3 of medial hemispheres
+ - Medial orbitofrontal artery (1st cortical branch)
+ - Arises from proximal A2/from pericallosal artery
+ - Ramifies over inferior surface of frontal lobe
+ - Frontopolar artery arises from mid A2
+ - Extends anteriorly along medial surface of hemisphere to frontal pole
+ - Pericallosal artery begins distal to origin of callosomarginal artery
+ - Larger of 2 major distal ACA branches
+ - Courses posterosuperiorly above corpus callosum, below cingulate gyrus
+ - Callosomarginal artery is smaller of 2 distal ACA branches
+ - Courses posterosuperiorly in cingulate sulcus, above cingulate gyrus
+ - **Perforating branches**supply medial basal ganglia, corpus callosum genu, internal capsule anterior limb
+ - Medial lenticulostriate arteries arise from A1, ACoA
+ - Course superiorly through anterior perforated substance
+ - Recurrent artery of Heubner arises from distal A1/proximal A2
+ - Curves back parallel to A1 in anterior perforated substance
+- ### Middle Cerebral Artery
+
+
+ - Larger, lateral terminal branch of supraclinoid ICA with 4 main segments
+ - **M1**: Horizontal segment
+ - From terminal ICA bifurcation to MCA bifurcation
+ - Usually bi- or trifurcates just before sylvian fissure
+ - Lies lateral to optic chiasm, behind olfactory trigone
+ - Courses laterally under anterior perforated substance
+ - Young adult: Runs laterally/rostrally; bows dorsally
+ - Elderly: Usually straight or tortuous; may bow ventrally coursing closer to sphenoid wing
+ - **M2**: Extends from MCA bifurcation to periinsular sulci
+ - Enter sylvian fissure; course superiorly within it
+ - 6 to 8 stem arteries arise from M2 segments
+ - M2 segments end at top of sylvian fissure
+ - Bifurcate into superior & inferior trunks
+ - Superior trunk: Prefrontal, precentral, central sulcus (rolandic), anterior parietal, orbitofrontal arteries
+ - Inferior trunk: Posterior parietal, middle temporal, posterior temporal, occipital temporal. angular (terminal) arteries
+ - **M3**: Courses inferolaterally through sylvian fissure
+ - **M4**: Exits sylvian fissure & ramifies over cerebral convexity
+ - **Cortical branches** supply most of lateral surface of cerebral hemispheres; except convexity & inferior temporal gyrus
+ - **Penetrating lenticulostriate branches** arise from M1 & supply basal ganglia, internal & external capsules
+- ### Posterior Cerebral Artery
+
+
+ - BA terminates into 2 PCAs, with 4 segments
+ - **P1**: Precommunicating segment; extends laterally from BA to junction with PCoA
+ - Closely related to anteromedial mesencephalon
+ - Courses above cisternal segment of CNIII
+ - Usually with horizontal course but exact course depends on length of BA
+ - Asymmetry (> 50%) & frequently hypoplastic
+ - **P2**: Ambient segment; swings posterolaterally around midbrain
+ - Encircles mesencephalon within ambient cistern
+ - Lies above tentorium, cisternal segment of CNIV
+ - Parallels optic tract, basal vein of Rosenthal
+ - **P3**: Quadrigeminal segment; short segment within lateral aspect of quadrigeminal cistern behind midbrain
+ - Extends behind mesencephalon, beneath splenium of corpus callosum
+ - **P4**: Calcarine segment; PCA terminates above tentorium in calcarine fissure
+ - **Cortical branches**supply posterior 1/3 of medial hemisphere surface, inferior temporal lobe, & occipital lobe (including visual cortex)
+ - Anterior temporal artery arises from P2; courses anterolaterally & anastomoses with MCA branches
+ - Posterior temporal artery arises from P2; courses posterolaterally along hippocampal gyrus
+ - Medial & lateral terminal trunks; major branches include parietooccipital & calcarine arteries
+ - **Penetrating branches** supply mesencephalon, thalami, posterior limb of internal capsule, optic tract
+ - Thalamoperforating arteries arise from P1
+ - Pass posterosuperiorly in interpeduncular fossa
+ - Thalamogeniculate arteries arise from P2
+ - Pass posteromedially into mesencephalon
+ - Peduncular perforating arteries arise from P2, pass directly into cerebral peduncles
+ - Ventricular/choroidal branches arise from P2 & supply choroid plexus of 3rd/lateral ventricles, thalami, posterior commissure, & cerebral peduncles
+ - Splenial branches supply posterior body & splenium of corpus callosum
+- ### Vertebrobasilar System
+
+
+ - **V3** segment (atlas loop) is extracranial/extraspinal; exits at transverse foramen (atlas) to enter into foramen magnum
+ - Rests on groove of posterior arch (atlas)
+ - **V4**segment is intradural/intracranial
+ - Perforates dura, enters skull through foramen magnum, courses superomedially behind clivus
+ - Anterior to medulla oblongata
+ - Unites with contralateral VA at/near pontomedullary junction to form BA
+ - Branches include anterior & posterior spinal arteries, meningeal branches, perforating branches to medulla & PICA
+ - **PICA**: Arises from distal VA, curves around/over cerebellar tonsil, gives off perforating medullary, choroid, tonsillar, cerebellar branches
+ - **BA**large median artery formed by union of VAs
+ - Bifurcates into its terminal branches, PCAs, in interpeduncular or suprasellar cistern behind dorsum sellae
+ - It courses superiorly in prepontine cistern & has multiple branches
+ - Pontine, midbrain perforating branches
+ - **AICA**: Courses inferolaterally, lies ventromedial to CNVII & VIII; often loops into IAC; its size is inversely proportional to size of PICA
+ - **SCA**: Arise from distal BA, course posterolaterally around mesencephalon below CNIII, tentorium; lie above CNV, often in contact with it
+ - **PCA**(terminal BA branches)
+- ### Intracranial Veins
+
+
+ - **Dural venous sinuses**lie between 2 layers of dura mater & drain intracranial veins
+ - **Posterosuperior sinuses**: SSS, inferior sagittal, 2 TS, SS & occipital
+ - **Anteroinferior sinuses**: 2 CS, 2 superior petrosal, 2 inter-CS, 2 inferior petrosal & basilar plexus
+ - Intracranial veins: **Cerebral & cerebellar veins**
+ - Valveless veins which pierce arachnoid membrane & meningeal layer of dura mater
+ - Open into cranial venous sinuses
+ - **Superficial ("external") cerebral veins**: Divided into superior, middle, & inferior divisions
+ - Superior: 8-12 smaller cortical veins over hemispheres, vein of Trolard
+ - Middle: superficial MCV, vein of Labbé
+ - Inferior: dMCV, BVR
+ - **Deep ("internal") cerebral veins**
+ - Drain deep parts of hemisphere & are in pairs
+ - Each formed near interventricular foramen by union of terminal & choroid veins
+ - Run parallel with each other posteriorly beneath splenium of corpus callosum
+ - **BV**: Receives ACV, dMCV, inferior striate veins
+ - Also receives tributaries from interpeduncular fossa, inferior horn of lateral ventricle, hippocampal gyrus & mesencephalon
+ - Each ends in its ICV, which drains into GV
+ - Many variations: May drain in SPS, lateral mesencephalic vein, contralateral BV
+ - **GV**: Receives ICVs, BV, pericallosal veins, & veins draining superior aspect of posterior fossa
+ - Curves backward & upward around splenium of corpus callosum & into SS
+ - **Cerebellar veins**: Superior & inferior
+ - Superior cerebellar veins run anteromedially across superior vermis to end in SS & ICVs
+ - Inferior cerebellar veins (larger) end in TS, superior petrosal, & occipital sinuses
+
+## ANATOMY IMAGING ISSUES
+
+- ### Imaging Recommendations
+
+
+ - Can assess intracranial stenosis & occlusive disease, monitor recanalization after thrombolysis & collateral formation
+ - Preoperative compression test to evaluate collateralizing capacity of circle of Willis
+ - Performed using **low-frequency transducer (1.8-3.6 MHz)**
+ - Grayscale ultrasound: Identify main anatomical structures in correct plane, followed by color Doppler ultrasound
+ - Unless specified, conventional color coding used
+ - Red: Flow toward transducer
+ - Blue: Flow away from transducer
+ - Vessel identification depends on window used, beam angulation, insonation depth, & flow direction
+ - Basal skull arteries are extremely variable in size, development, & course; supplementary CTA/MRA is useful to enhance its diagnostic accuracy
+ - For difficult cases, compression test is helpful for arterial identification & assessment of collaterals
+ - Must be performed by experienced investigator
+ - Exclude risk of embolism in extracranial arteries prior to compression
+ - For anterior circulation, compress CCA in lower neck by 2 fingers
+ - For vertebrobasilar system, compress VA at mastoid slope
+ - Normal adults: Highest velocities in MCA or ACA
+ - Flow velocities in basal cerebral arteries show consistent decrease with increasing age
+- ### Transtemporal Approach (Most Common Approach)
+
+
+ - Transducer on temporal bone superior to zygomatic arch
+ - **Axial mesencephalic plane**
+ - Identify hypoechoic, butterfly-shaped mesencephalon as landmark
+ - Assess C5-C7 segments, A1 segment, M1 & M2 segments, P1 & P2 segments, PCoA
+ - Also assess dMCV, BV, GV, SS, & contralateral TS
+ - **Axial ventricular plane**
+ - Tilting of transducer 10° upward from mesencephalic plane
+ - Identify hypoechoic 3rd ventricle, echogenic pineal gland, & choroid plexus of trigone
+ - Assess A2 segment, M2 & M3 segments, & P3
+ - Assess midline shift due to MCA infarction
+ - **Anterior/posterior coronal planes**
+ - Assess C4-C7 segments, A1 segment, M1-M3 segments, PCA, & distal BA
+ - **Normal mean velocities & depth of insonation**
+ - Terminal ICA: 39 ± 9 cm/sec (60-67 mm)
+ - MCA: 62 ± 12 cm/sec (30-67 mm)
+ - ACA: 50 ± 11 cm/sec (60-80 mm)
+ - PCA: 39 ± 10 cm/sec (55-80 mm)
+- ### Transfrontal Approach
+
+
+ - Insonation depth of 10-16 cm
+ - **Paramedian frontal bone window**
+ - Slightly lateral t midline of forehead
+ - Identify echogenic orbital roof, hypoechoic 3rd ventricle, & corpus callosum
+ - **Lateral frontal bone window**
+ - Just above lateral aspect of eyebrow
+ - Identify echogenic falx cerebri, sylvian fissure, & hypoechoic mesencephalon
+ - Assess A1 & A2 segments, M1 segment, PCA, PCoA, pericallosal artery
+ - Also assess ICV, GV, SS
+- ### Transforaminal/Suboccipital Approach
+
+
+ - Transducer placed between squama occipitalis & spinous process of 1st cervical vertebra
+ - Ultrasound beam aimed at nasal bridge
+ - Identify echogenic processus transversus & clivus
+ - Assess VAs & BA, which are visualized as Y
+ - Normal mean velocities & depth of insonation
+ - VA: 38 ± 10 cm/sec (40-85 mm)
+ - BA: 41 ± 10 cm/sec (> 80 mm)
+- ### Transorbital Approach
+
+
+ - Problem of insonation of orbital lens
+ - Low mechanical index < 0.23 highly recommended
+ - Assess intraocular vessels, C4-C6 segments
+ - Normal mean velocities & depth of insonation
+ - Ophthalmic artery: 21 ± 5 cm/sec (40-60 mm)
+ - Carotid siphon: 47 ± 10 cm/sec (60-80 mm)
+- ### Submandibular Approach
+
+
+ - Assess distal C1 & C2 segments
+ - Normal mean velocities & depth of insonation
+ - Cervical ICA: 37 ± 9 cm/sec (35-70 mm)
+- ### Imaging Pitfalls
+
+
+ - Extensive variations; incomplete circle of Willis
+ - Variable probe-to-vessel angle
+ - Misdiagnosis: Hyperdynamic collaterals for stenosis; vasospasm for stenosis; displacement of basal vessels by mass lesion for occlusion
+
+## CLINICAL IMPLICATIONS
+
+- ### Clinical Importance
+
+
+ - Diagnosis & assessment of intracranial stenosis
+ - Mild stenosis: Mild ↑ peak velocity; waveform unchanged
+ - Moderate/severe stenosis: Greater ↑ in peak velocity, ↑ diastolic velocity ± turbulent flow
+ - Near occlusion: Damped waveform, preocclusive thump, poststenotic drop in peak velocity
+
+ 9260658b-ccb7-4bc8-bc51-1c834cd56971
+
+
+## Images
+
+
+### Distal Internal Carotid artery
+
+
+*Graphic shows intracranial internal carotid artery (ICA) segments. The C2 segment runs in the carotid canal and continues as the C3 segment after leaving the canal. The C4 segment, an extension of C3, has branches anastomosing extensively with external carotid artery (ECA) branches. The C5 segment ends near the anterior clinoid process and the C6 segment extends to just below the posterior communicating artery (PCoA). The C7 segment, after giving rise to the PCoA, branches into anterior cerebral artery (ACA) and middle cerebral artery (MCA).*
+
+
+*Graphic shows intracranial internal carotid artery (ICA) segments. The C2 segment runs in the carotid canal and continues as the C3 segment after leaving the canal. The C4 segment, an extension of C3, has branches anastomosing extensively with external carotid artery (ECA) branches. The C5 segment ends near the anterior clinoid process and the C6 segment extends to just below the posterior communicating artery (PCoA). The C7 segment, after giving rise to the PCoA, branches into anterior cerebral artery (ACA) and middle cerebral artery (MCA).*
+
+
+*Graphic shows numerous ICA to ECA anastomoses through cavernous and deep facial branches of the 2 arteries, respectively. These include numerous anastomoses in and around the orbit; the small vidian artery anastomosing between the internal maxillary artery and petrous C2 segment and the accessory meningeal artery, an important branch that may supply part of the trigeminal ganglion, anastomosing with the inferolateral trunk of the cavernous ICA.*
+
+
+### Distal Internal Carotid Artery
+
+
+*Axial color Doppler ultrasound (transorbital approach) shows the C5 segment of the ICA with flow toward the transducer. C4-C6 segments can be assessed by this approach.*
+
+
+*Axial color Doppler ultrasound (transtemporal approach) at a plane slightly caudad to the standard mesencephalic plane shows the C7 segment of the ICA, ACA, and sphenoparietal sinus.*
+
+
+*Axial oblique color Doppler ultrasound (transtemporal approach) with slight caudad tilting from the standard axial mesencephalic plane shows the anterior choroidal artery and PCoA branching out from the communicating segment of the ICA.*
+
+
+### Anterior Cerebral Artery
+
+
+*Submentovertex graphic shows the relationship of the circle of Willis and its components to the cranial nerves. Note that the normal course of the horizontal (A1) segment is over the optic nerves.*
+
+
+*Sagittal (midline) graphic through the interhemispheric fissure shows the relationship of the ACA and its branches to the underlying brain parenchyma. The A2 segment ascends in front of the 3rd ventricle within the cistern of the lamina terminalis. The A3 segment curves around the corpus callosum genu. The branch point of the distal ACA into the pericallosal and callosomarginal arteries varies. Almost the entire anterior 2/3 of the medial hemisphere surface is supplied by the ACA and its branches. Branches of the posterior and anterior cerebral arteries anastomose around the corpus callosum genu.*
+
+
+*Axial color Doppler ultrasound (transtemporal approach) shows the MCA/ACA junction on the mesencephalic plane, which is the most useful plane for showing the circle of Willis. However, it is limited in depicting more distal segments of the ACA, which can be visualized using a transfrontal approach.*
+
+
+*Axial color Doppler ultrasound (paramedian frontal approach) shows the right A2 segment running along the interhemispheric fissure. Echogenic sphenoid bone and sella turcica provide important landmarks for identification of the A2 segment, which starts close to the sella turcica and tip of the sphenoid bone.*
+
+
+*Sagittal color Doppler ultrasound (paramedian frontal approach) shows the frontopolar artery arising anteriorly from the mid A2 segment and the callosomarginal artery branching out from the distal A2 segment. Note the pericallosal artery (A3 segment) can be seen by this approach.*
+
+
+*Axial color Doppler ultrasound (lateral frontal approach) shows the A2 segment as seen through the frontal bone at the lateral eyebrow. The ipsilateral A2 segment is often seen as a prominent vessel running along the hyperechoic falx cerebri with flow toward the transducer. Sometimes the contralateral distal A1 or proximal A2 segment can be depicted coursing obliquely toward the ipsilateral A2 segment, mimicking the anterior communicating artery (ACoA), which is infrequently discernible in normal subjects.*
+
+
+*Axial spectral Doppler ultrasound (lateral frontal approach) of the A2 segment shows the typical low-resistance waveform with forward flow.*
+
+
+*Corresponding oblique axial reformatted CT arteriogram shows the A1 segments becoming A2 segments after the ACoA junction. They then ascend parallel in the interhemispheric fissure.*
+
+
+*Sagittal color Doppler ultrasound (paramedian frontal approach) illustrates the A2 segment giving off the callosomarginal artery, which is the smaller branch of the distal ACA. It runs posterosuperiorly in cingulate sulcus and on the superior surface of the cingulate gyrus. Its flow is directed toward the transducer.*
+
+
+*Sagittal spectral Doppler ultrasound (paramedian frontal approach) shows the antegrade, low-resistance waveform of callosomarginal artery of the A2 segment.*
+
+
+*Corresponding oblique sagittal reformatted CT arteriogram shows the A2 segment giving off the callosomarginal artery and becoming the A3 segment (pericallosal artery). A similar branching pattern is seen on the opposite side.*
+
+
+### Middle Cerebral Artery
+
+
+*The MCA and its relationship to adjacent structures is depicted on these graphics. The submentovertex graphic shows the left temporal lobe sectioned through the temporal horn of the lateral ventricle. The MCA supplies much of the lateral surface of the brain and is the larger of the 2 terminal branches of the ICA.*
+
+
+*AP graphic shows the MCA and its relationship to the adjacent brain. The MCA courses through the sylvian fissure, and the M1-M4 segments are well delineated. A few medial and numerous lateral lenticulostriate arteries arise from the top of the horizontal (M1) MCA segment, course superiorly through the anterior perforated substance, and supply the lateral basal ganglia and external capsule.*
+
+
+*Axial color Doppler ultrasound (transtemporal approach) of a young adult shows the M1 segment of the MCA on the standard mesencephalic plane where it starts from the ICA termination to MCA bi-/trifurcations. In young adults, the M1 usually bows dorsally.*
+
+
+*Oblique coronal color Doppler ultrasound (transtemporal approach) of the same adult shows the M2 segment in the insula. Note that the flow direction in the M2 segment is directed toward the transducer in the proximal segment and away from the transducer as it ascends within the sylvian fissure.*
+
+
+*Axial color Doppler ultrasound (transtemporal approach) of the MCA in an elderly patient shows a tortuous M1 segment. Its distal portion bows ventrally, bringing the entire segment closer to sphenoid bone. The M1 segment originates near the medial aspect of the lesser wing of the sphenoid and traverses laterally and horizontally to end in the limen insulae.*
+
+
+*Axial spectral Doppler ultrasound (transtemporal approach) of the MCA in a young adult shows that the waveform pattern is of low resistance, and the mean velocity is within normal range of 62 ± 12 cm/sec. The clinical importance of assessing the MCA is its high association with stroke in diseased arteries.*
+
+
+*Oblique, coronal, spectral Doppler ultrasound (transtemporal approach) of a young adult shows a waveform of the M2 segment, which is away from the transducer. The waveform is inseparable from that of a small neighboring cortical branch with flow toward the transducer.*
+
+
+*Axial spectral Doppler ultrasound (transtemporal approach) of an elderly patient shows Doppler waveform of the M1 segment. Note that accurate flow velocity measurement in an elderly patient may be difficult due to uncertainty in angle correction in a tortuous vessel.*
+
+
+### Middle Cerebral Artery
+
+
+*Axial color Doppler ultrasound (transtemporal approach) on the mesencephalic plane illustrates the MCA appearing in the anterior aspect of the circle of Willis. The origin of the M1 segment can be determined accurately with identification of the MCA/ACA junction.*
+
+
+*Axial color Doppler ultrasound (transtemporal approach) on the standard mesencephalic plane depicts the M1 segment arising from the communicating segment of the ICA with flow direction toward the transducer.*
+
+
+*Axial oblique color Doppler ultrasound of the MCA bifurcation (transtemporal approach) shows the superior and inferior trunks of the M2 segments. Note the MCA bifurcates in ~ 75% of normal subjects, and the rest trifurcate with an individual anterior temporal artery.*
+
+
+### Posterior Cerebral Artery
+
+
+*Lateral graphic shows the posterior cerebral artery (PCA) and its branches. The PCA has central (perforating), choroidal, and cortical branches as well as a small branch to the corpus callosum splenium. The tentorium and CNIII lie between the PCA above and the superior cerebellar artery below.*
+
+
+*Submentovertex graphic shows the PCA and the relationship of its segments to the midbrain. The PCA supplies the occipital lobe and almost all of the inferior surface of the temporal lobe (except for its tip). The precommunicating (P1) PCA segment extends from the basilar bifurcation to the PCoA junction. The ambient (P2) segment swings posterolaterally around the midbrain. The quadrigeminal segment (P3) lies behind the midbrain. The PCA terminal segment is the calcarine (P4) segment.*
+
+
+*Axial color Doppler ultrasound (transtemporal approach) on the mesencephalic plane shows the P1 segment extending laterally from the basilar artery to its junction with the PCoA and continuing as the P2 segment around the mesencephalon. The flow direction of P1 and anterior P2 segments is toward the transducer, whereas the posterior P2 segment is away from the transducer.*
+
+
+*Axial color Doppler ultrasound (transtemporal approach) shows the PCoA is identified as a vascular structure connecting the ICA C7 segment to the PCA with flow away from the transducer. The clinical importance of investigation of PCoA is due to the prevalence of aneurysm formation and effectiveness of collateralization.*
+
+
+*Axial color Doppler ultrasound (transtemporal approach) shows the P2 segment curving posteriorly around mesencephalon with change in flow direction around the curvature.*
+
+
+*Axial color Doppler ultrasound (transtemporal approach) on the mesencephalic plane shows the P1 segment emerging in the interpeduncular cistern with the P2 segment encircling the mesencephalon with flow toward transducer in P1 and P2 segments. A short P3 segment is also noted with flow away from the transducer. With adequate acoustic penetration, the contralateral arterial segments can be visualized with flow direction opposite to the ipsilateral arteries.*
+
+
+*Corresponding MR arteriogram in a similar projection plane shows the bilateral PCAs and PCoAs in the circle of Willis.*
+
+
+*Axial spectral Doppler ultrasound (transtemporal approach) of the PCoA on standard the mesencephalic plane shows normal backward flow from the ICA to the PCA in this artery. Note only 75% of the PCoA is discernible transcranially.*
+
+
+*Axial spectral Doppler ultrasound (transtemporal approach) of the P1 segment shows the waveform of P1 flow is of low resistance and on the positive side of the trace. Note that the P1 segment is short and often hypoplastic or absent, making its visualization difficult.*
+
+
+*Axial spectral Doppler ultrasound (transtemporal approach) of the anterior P2 segment on the axial mesencephalic plane shows normal antegrade low-resistance flow with a waveform pattern similar to the P1 segment.*
+
+
+*Axial spectral Doppler ultrasound (transtemporal approach) of the posterior P2 segment on the axial ventricular plane shows the waveform of this segment is of low resistance and on the negative side of the trace, denoting flow away from transducer.*
+
+
+### Vertebrobasilar System
+
+
+*Frontal graphic depicts the vertebrobasilar system. The V3 is the short extraspinal vertebral artery (VA) segment that extends from the top of the C1 to the foramen magnum. The V4 is the intradural (intracranial) segment. A right posterior inferior cerebellar artery (PICA) is shown originating from the VA, while the right anterior inferior cerebellar artery (AICA) is from the basilar artery. A combined AICA-PICA trunk is a common normal variant and is shown on the left side.*
+
+
+*Lateral graphic depicts the vertebrobasilar system. Note the relationship of the PICA loops to the medulla, cerebellar tonsil. Watershed between the SCA, PICA is often near the great horizontal fissure of the cerebellum.*
+
+
+*Axial color Doppler ultrasound (suboccipital approach) through the foramen magnum shows the right V4 segment of the VA and proximal basilar artery with flow directed away from the transducer. Note that reversed basilar artery flow toward the transducer would signify severe intracranial subclavian steal.*
+
+
+*Axial color Doppler ultrasound (suboccipital approach) through the foramen magnum shows the left V4 segment of the VA with flow directed away from the transducer. The convergence of 2 V4 segments forming the basilar artery should give rise to a Y configuration. However, the Y configuration may not be always obtainable because the 3 vessels are not always on the same plane.*
+
+
+*Axial color Doppler ultrasound (transtemporal approach) on the standard mesencephalic plane shows the distal portion of basilar artery, which terminates into the PCAs in the interpeduncular/suprasellar cistern.*
+
+
+*Axial color Doppler ultrasound (suboccipital approach) at a level just above the atlas shows the 2 short extraspinal V3 segments exiting from the transverse foramina of the atlas and running posteromedially at the horizontal groove on the posterior arch (atlas). The flow direction of these vessels is toward the transducer.*
+
+
+*Corresponding spectral Doppler ultrasound of the right V3 segment demonstrates the characteristic antegrade low-resistance flow pattern of this vessel. Note the Doppler signal from the ipsilateral vertebral vein is frequently inseparable from the arterial signal and is detected on the opposite side of the trace.*
+
+
+*Corresponding spectral Doppler ultrasound of the left V3 segment shows similar waveform as the contralateral artery.*
+
+
+### Vertebral Artery V4 Segment
+
+
+*Axial color Doppler ultrasound (suboccipital approach) through the foramen magnum shows the 2 intradural V4 segments of the VA with flow directed away from the transducer. The 2 vertebral veins formed in the suboccipital triangle from numerous small tributaries are also visualized with flow toward the transducer.*
+
+
+*Corresponding spectral Doppler waveform of the right V4 segment shows the normal antegrade low-resistance flow pattern similar to the V3 segment, but with opposite flow direction, as evident by the negative tracing.*
+
+
+*A spectral Doppler waveform of the left V4 segment shows a similar tracing.*
+
+
+### Intracranial veins and sinuses
+
+
+*This 3D rendering of the falx cerebri with major dural sinuses and deep veins shows the interconnections between these 2 venous systems.*
+
+
+*Graphic shows an inferior intracranial view of the deep venous structures. Intracranial veins are valveless veins, which pierce the arachnoid membrane and meningeal layer of dura mater and drain into the cranial venous sinuses.*
+
+
+*Graphic shows the superior intracranial view of the dural venous sinuses. The cerebral hemispheres, midbrain, and pons as well as the left 1/2 of the tentorium cerebelli have been removed. Note the numerous interconnections between both halves of the cavernous sinus, the basilar venous plexus, and the petrosal sinuses.*
+
+
+### Intracranial veins
+
+
+*Axial color Doppler ultrasound (transtemporal approach) illustrates the basal vein of Rosenthal (BV) running posteriorly along the mesencephalon with flow signal away from the transducer. The ipsilateral PCA is often seen closely related to it with opposite flow direction.*
+
+
+*Axial color Doppler ultrasound (transtemporal approach) demonstrates the great vein of Galen (GV). The vein is identified in the midline posterior to mesencephalon in the hyperechoic triangular region of the pineal body and quadrigeminal cistern.*
+
+
+*Axial color Doppler ultrasound (transtemporal approach) shows the 2 tributaries of the BV: The anterior cerebral vein (ACV) and deep middle cerebral vein (dMCV). The ACV courses posteriorly along the base of the brain and joins the dMCV, which runs in the sylvian fissure to form the BV just anterior to the mesencephalon. Note that both the ACV and MCV are accompanied by their respective arteries.*
+
+
+*Axial color Doppler ultrasound (transtemporal approach) shows the posterior segment of the BV. The vein is seen surrounding the mesencephalon and runs almost parallel to the PCA. The blue color coding of the vessel indicates flow directed away from the transducer.*
+
+
+*Axial color Doppler ultrasound (transtemporal approach) with a more anterior tilting approach is shown. The ACV is seen running posteriorly to join the dMCV before draining into the anterior segment of BV. The flow in the ACV and anterior segment of BV is directed toward the transducer, as denoted by the red color coding.*
+
+
+*Axial color Doppler ultrasound (paramedian frontal approach) shows the ACV, which courses posteriorly within the interhemispheric fissure; this small vein, after joining the dMCV, forms the BV. Identification of this vessel is difficult due to its slow flow.*
+
+
+*Axial spectral Doppler ultrasound (transtemporal approach) shows the BV waveform (posterior segment) with typical respiratory phasicity.*
+
+
+*Axial spectral Doppler ultrasound (transtemporal approach) shows the Doppler waveform of the ACV. Note the waveform is on the positive scale, representing flow toward the transducer.*
+
+
+*Axial spectral Doppler ultrasound shows the ACV waveform obtained from the paramedian frontal approach with the flow away from the transducer. Note the flow direction of a vessel depends on the scanning approach used, and the ACV flow is toward the transducer using transtemporal approach but away from the transducer using the transfrontal approach.*
+
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+## UPPER EXTREMITY VENOUS ANATOMY
+
+- ### Upper Extremity Superficial Veins
+
+
+ - Cephalic vein: Courses along radial aspect of arm
+ - Ascends in front of elbow between brachioradialis and biceps brachii muscles
+ - Communicates with basilic vein via median cubital (median basilic) vein at level of elbow
+ - Located in superficial fascia along anterolateral surface of biceps brachii muscle
+ - Passes superiorly between deltoid and pectoralis major muscles in deltopectoral groove
+ - Drains into axillary vein in arch-like configuration
+ - Basilic vein: Courses along ulnar aspect of arm
+ - Ascends medially along biceps in upper arm
+ - Frequently joins brachial vein in upper arm
+- ### Upper Extremity Deep Veins
+
+
+ - Brachial vein: Usually paired
+ - Terminate(s) in axillary vein
+ - Axillary vein: Returns blood from lateral aspect of thorax, axilla, and upper extremity
+ - Starts at border of teres major muscle as continuation of brachial vein; ends at outer edge of 1st rib
+ - Tributaries include basilic and cephalic veins
+ - Subclavian vein: Continuation of axillary vein
+ - Courses from outer border of 1st rib to medial border of anterior scalene muscle
+ - Subclavian vein lies anterior to anterior scalene muscle whereas artery lies posterior
+ - Thoracic duct drains into left subclavian vein
+ - Duct enters near subclavian vein junction with left internal jugular vein
+
+## CERVICOTHORACIC VENOUS ANATOMY
+
+- ### Cervical Veins
+
+
+ - Internal jugular vein: Formed by union of sigmoid and inferior petrosal sinuses with common facial vein
+ - Courses with common carotid artery and vagus nerve
+ - Provides venous drainage for brain, face, and neck
+ - Joins subclavian vein to form brachiocephalic vein
+ - External jugular vein: Formed by union of posterior retromandibular vein and posterior auricular vein
+ - Courses superficial to sternocleidomastoid muscle
+ - Drains into subclavian vein more laterally than does internal jugular vein
+ - Provides venous drainage for exterior of cranium and deep parts of face
+ - Thyroidal veins: Arise from venous plexus surrounding thyroid gland; often multiple
+ - Superior and middle thyroidal drain into internal jugular vein; inferior thyroidal into brachiocephalic veins
+ - Vertebral vein: Derived from small venous tributaries that form plexus around vertebral artery
+ - Plexus ends in single trunk that exits from 6th cervical vertebral transverse foramen
+ - Enters brachiocephalic vein posteriorly near origin
+- ### Thoracic Veins
+
+
+ - Brachiocephalic (innominate) vein: Formed by union of subclavian and internal jugular veins
+ - Veins join at level of sternoclavicular joint
+ - Superior vena cava (SVC): Formed by union of right and left brachiocephalic veins
+ - Courses posterior to sternum on right
+ - Azygos vein: Formed by union of ascending lumbar and right subcostal veins at 12th thoracic vertebral level
+ - Ascends in posterior mediastinum; arches over right mainstem bronchus to join SVC
+ - Drains posterior thorax and abdomen into SVC
+ - Hemiazygos vein: Begins in left ascending lumbar or left renal vein
+ - Passes upward through left crus of diaphragm to enter thorax on left; mirrors lower azygos vein
+ - At ~ 9th thoracic vertebra, courses rightward behind aorta and esophagus to enter azygos vein
+ - Accessory hemiazygos vein: Courses inferiorly along left side of spine, draining upper posterior thorax
+ - Drains 4th-7th posterior intercostal veins
+ - Either courses rightward at ~ 8th thoracic vertebra to join azygos vein or ends in hemiazygos vein
+ - Superior intercostal veins: Right- and left-sided veins that drain 2nd-4th intercostal spaces posteriorly
+ - Right superior intercostal vein drains into azygos vein
+ - Left drains into left brachiocephalic vein
+ - Internal thoracic (mammary) vein: Arises from superior epigastric vein; terminates in brachiocephalic vein
+ - Paired vein that drains anterior chest and breasts
+ - Receives drainage from anterior intercostal veins
+
+## LOWER EXTREMITY VENOUS ANATOMY
+
+- ### Lower Extremity Superficial Veins
+
+
+ - Great saphenous vein (GSV): Originates from dorsal venous pedal arch; courses anterior to medial malleolus
+ - Ascends medially in lower leg, courses over medial epicondyle of femur at knee level, then runs anteromedially along thigh
+ - Joins common femoral vein at saphenofemoral junction (SFJ) in femoral triangle region
+ - Anastomoses freely with small saphenous vein (SSV)
+ - Has tributaries from medial, lateral, and posterior thigh
+ - May form accessory saphenous vein branches that enter GSV at or near SFJ
+ - Superficial epigastric, superficial iliac circumflex, and superficial external pudendal veins join GSV at SFJ
+ - SSV: Originates laterally from dorsal venous pedal arch; courses behind lateral malleolus
+ - Ascends along posterior calf alongside sural nerve; passes between gastrocnemius muscle heads
+ - Often variable anatomy of SSV drainage
+ - Usually enters popliteal vein at saphenopopliteal junction around level of knee joint
+ - May not drain into popliteal vein but instead may enter GSV at variable level
+ - Main SSV may continue as Giacomini vein
+ - Giacomini vein: Communicating vein between GSV and SSV; is usually thigh extension of SSV branch
+ - Ascends along posterior thigh
+ - Typically joins GSV in upper 1/3 of thigh
+ - Found in ~ 60-70% of individuals
+- ### Lower Extremity Deep Veins
+
+
+ - Calf veins: 3 sets of paired veins draining lower leg
+ - Anterior tibial veins: Arise from dorsal pedal veins; run in interosseous membrane between tibia and fibula
+ - Join posterior tibial veins to form popliteal vein
+ - Drain ankle, knee, and tibiofibular joints along with anterior portion of lower leg
+ - Posterior tibial veins: Receive blood from medial and lateral plantar veins
+ - Drain posterior calf and plantar surface of foot
+ - Receive most important calf perforator veins: Cockett perforators (superior, medial, and inferior)
+ - Peroneal (fibular) veins
+ - Return blood from lateral compartment of calf
+ - Drain into posterior tibial veins
+ - Popliteal vein: Formed by posterior and anterior tibial veins
+ - Courses adjacent to popliteal artery behind knee
+ - Returns blood from paired calf veins
+ - Femoral vein: Continuation of popliteal vein
+ - Begins at adductor canal; ends at inguinal ligament
+ - Receives drainage of lower extremity via popliteal veins, profunda femoral veins, and GSVs
+ - Some use term "superficial femoral vein" for lower segment of femoral vein coursing in adductor canal
+ - Differentiates femoral vein segments before and after profunda femoral vein inflow
+ - Usage of term is discouraged; causes confusion as this vein is deep rather than superficial
+- ### Lower Extremity Perforator Veins
+
+
+ - Connect superficial and deep veins
+ - Valves direct blood from superficial to deep system
+ - ~ 150 perforator veins in each leg
+ - Major lower extremity perforators
+ - Foot and ankle perforators: Connect to pedal arches
+ - Leg (calf) perforators: Connect saphenous branches with paired deep veins of calf
+ - Include posterior tibial perforator veins (formerly termed Cockett perforators)
+ - Knee perforators: Connect GSV with popliteal and other deep veins at knee level
+ - Include medial knee perforators (formerly termed Boyd perforators); common site for varicose veins
+ - Thigh perforators: Connect GSV to femoral vein
+ - Include distal thigh perforator (Dodd perforator) and medial thigh perforator (Hunter perforator)
+
+## ABDOMINAL & PELVIC VENOUS ANATOMY
+
+- ### Systemic Abdominal and Pelvic Veins
+
+
+ - Inferior vena cava (IVC) and tributaries: Drain both lower extremities and abdominal and pelvic viscera that are not alimentary tract components
+ - External iliac veins: Arise at inguinal ligament; terminate when joined by internal iliac veins
+ - Connect femoral veins to common iliac veins
+ - Inferior epigastric and deep circumflex iliac veins drain into external iliac veins
+ - Internal iliac (hypogastric) veins: Begin near greater sciatic foramen; join with external iliac vein to form common iliac vein
+ - Tributaries drain external genitalia, uterus, vagina, prostate, bladder, lower rectum, gluteal muscles
+ - Common iliac veins: Formed by union of external and internal iliac veins
+ - Outflow drainage for lower extremities and pelvis
+- ### Portal Venous System
+
+
+ - Portal vein and tributaries: Responsible for directing blood from components of gastrointestinal tract to liver
+
+## VARIANT VENOUS ANATOMY
+
+- ### Superior Vena Cava and Tributaries
+
+
+ - Left SVC: Most common venous anomaly of thorax
+ - Only seen in isolation in 10% of cases; majority accompanied by normal but smaller right SVC
+ - Termed SVC duplication if both present
+ - Can result in right-to-left shunt in minority of cases
+ - Left azygos arch: May occur in association with left SVC
+ - Left superior intercostal vein forms communication between left SVC and accessory hemiazygos vein
+- ### Inferior Vena Cava and Tributaries
+
+
+ - Duplicated IVC: Results from persistence of both supracardinal veins
+ - Left IVC typically ends at left renal vein, which crosses anterior to aorta to join right IVC; prevalence of 0.2-3%
+ - Left IVC: Results from regression of right supracardinal and persistence of left supracardinal vein
+ - Left IVC joins left renal vein, which crosses to unite with right renal vein and form normal suprarenal IVC
+ - Prevalence of 0.2-0.5%
+ - Azygos continuation of IVC: Absence of hepatic IVC
+ - IVC receives blood from kidneys and passes posteriorly to enter thorax as azygos vein
+ - Azygos vein joins SVC at normal location in chest
+ - Circumaortic left renal vein: 2 left renal veins
+ - Superior renal vein receives left adrenal vein and crosses aorta anteriorly
+ - Inferior renal vein receives left gonadal vein and crosses aorta posteriorly
+ - Prevalence may be as high as 8.7%
+ - Retroaortic left renal vein: Single-variant renal vein
+ - Passes posterior to aorta
+ - May result in posterior nutcracker syndrome
+ - Prevalence of 2.1%
+
+ dc96f595-86e1-4ee0-ba97-61a00b4efe48
+
+
+## Images
+
+
+### Normal Abdominal Systemic and Portal Venous Anatomy
+
+
+*Graphic shows abdominal systemic and portal venous anatomy. The inferior vena cava (IVC) is formed by the right and left common iliac venous confluence and provides venous return for the lower extremities and the pelvis and abdomen. As the IVC is located to the right of the midline, there are some normally occurring asymmetries in venous drainage patterns. The gonadal and adrenal veins drain directly into the IVC on the right but into the renal vein on the left. The left and right renal veins drain directly into the IVC. All of the lumbar and hepatic veins usually drain directly into the IVC. The IVC drains into the right atrium and also anastomoses in the abdomen with the azygos venous system. The latter is formed by the ascending lumbar veins along the right side of the spine. Normally, the portal venous system is separate from the IVC and the systemic veins and is responsible for returning blood from various parts of the gastrointestinal tract to the liver. It supplies ~ 70% of the liver perfusion. The portal vein is formed by the union of the superior mesenteric and splenic veins. Other important portal vein tributaries include the inferior mesenteric, gastric, and cystic veins.*
+
+
+*Graphic shows abdominal systemic and portal venous anatomy. The inferior vena cava (IVC) is formed by the right and left common iliac venous confluence and provides venous return for the lower extremities and the pelvis and abdomen. As the IVC is located to the right of the midline, there are some normally occurring asymmetries in venous drainage patterns. The gonadal and adrenal veins drain directly into the IVC on the right but into the renal vein on the left. The left and right renal veins drain directly into the IVC. All of the lumbar and hepatic veins usually drain directly into the IVC. The IVC drains into the right atrium and also anastomoses in the abdomen with the azygos venous system. The latter is formed by the ascending lumbar veins along the right side of the spine. Normally, the portal venous system is separate from the IVC and the systemic veins and is responsible for returning blood from various parts of the gastrointestinal tract to the liver. It supplies ~ 70% of the liver perfusion. The portal vein is formed by the union of the superior mesenteric and splenic veins. Other important portal vein tributaries include the inferior mesenteric, gastric, and cystic veins.*
+
+
+*Graphic shows abdominal systemic and portal venous anatomy. The inferior vena cava (IVC) is formed by the right and left common iliac venous confluence and provides venous return for the lower extremities and the pelvis and abdomen. As the IVC is located to the right of the midline, there are some normally occurring asymmetries in venous drainage patterns. The gonadal and adrenal veins drain directly into the IVC on the right but into the renal vein on the left. The left and right renal veins drain directly into the IVC. All of the lumbar and hepatic veins usually drain directly into the IVC. The IVC drains into the right atrium and also anastomoses in the abdomen with the azygos venous system. The latter is formed by the ascending lumbar veins along the right side of the spine. Normally, the portal venous system is separate from the IVC and the systemic veins and is responsible for returning blood from various parts of the gastrointestinal tract to the liver. It supplies ~ 70% of the liver perfusion. The portal vein is formed by the union of the superior mesenteric and splenic veins. Other important portal vein tributaries include the inferior mesenteric, gastric, and cystic veins.*
+
+
+### Venous Anatomy of Neck, Thoracic Inlet, and Upper Thorax
+
+
+*Graphic shows the venous anatomy of the neck, thoracic inlet, and upper thorax. The sigmoid and inferior petrosal sinuses join to form the internal jugular vein (IJV) and are joined by the common facial vein. The right and left IJVs course with the common carotid artery and vagus nerve inside the carotid sheath and provide venous drainage for the brain, face, and neck. They join the subclavian veins medially to form the right and left brachiocephalic veins, which in turn join to form the superior vena cava (SVC). The external jugular veins (EJVs) are formed by the union of the posterior retromandibular vein and posterior auricular vein. They course superficial to the sternocleidomastoid muscle and drain into the subclavian vein more laterally than do the IJVs. EJVs provide venous drainage for the exterior of the cranium and deep parts of the face.*
+
+
+*Graphic shows the venous anatomy of the neck, thoracic inlet, and upper thorax. The sigmoid and inferior petrosal sinuses join to form the internal jugular vein (IJV) and are joined by the common facial vein. The right and left IJVs course with the common carotid artery and vagus nerve inside the carotid sheath and provide venous drainage for the brain, face, and neck. They join the subclavian veins medially to form the right and left brachiocephalic veins, which in turn join to form the superior vena cava (SVC). The external jugular veins (EJVs) are formed by the union of the posterior retromandibular vein and posterior auricular vein. They course superficial to the sternocleidomastoid muscle and drain into the subclavian vein more laterally than do the IJVs. EJVs provide venous drainage for the exterior of the cranium and deep parts of the face.*
+
+
+*Coronal reformatted CT venogram shows normal cervicothoracic venous anatomy. IJV begins at the jugular foramen at the skull base. Its inferior course is lateral to the carotid arteries. IJVs and EJVs have a relatively superficial course and are thus susceptible to damage but are also easily accessible for venous catheterization.*
+
+
+*Coronal reformatted CT venogram shows normal cervicothoracic venous anatomy. IJV begins at the jugular foramen at the skull base. Its inferior course is lateral to the carotid arteries. IJVs and EJVs have a relatively superficial course and are thus susceptible to damage but are also easily accessible for venous catheterization.*
+
+
+### Anatomy of Thoracic Veins, Superior Vena Cava, and Tributaries
+
+
+*Graphic shows anatomy of thoracic veins. Both IJVs and subclavian veins join to form the right and left brachiocephalic veins. They join to form the SVC, which courses behind the sternum to enter the right atrium. The azygos, hemiazygos, & accessory hemiazygos veins connect the SVC and IVC. They course along both sides of the upper lumbar and thoracic spine and drain the posterior abdomen and thorax. The azygos vein ascends on the right to drain into SVC above the right mainstem bronchus. The hemiazygos vein ascends on the left and crosses to the right, posterior to the aorta, thoracic duct, and esophagus to join the azygos vein. The accessory hemiazygos vein descends on the left and may join the hemiazygos vein or cross to the right to join the azygos vein.*
+
+
+*Graphic shows anatomy of thoracic veins. Both IJVs and subclavian veins join to form the right and left brachiocephalic veins. They join to form the SVC, which courses behind the sternum to enter the right atrium. The azygos, hemiazygos, & accessory hemiazygos veins connect the SVC and IVC. They course along both sides of the upper lumbar and thoracic spine and drain the posterior abdomen and thorax. The azygos vein ascends on the right to drain into SVC above the right mainstem bronchus. The hemiazygos vein ascends on the left and crosses to the right, posterior to the aorta, thoracic duct, and esophagus to join the azygos vein. The accessory hemiazygos vein descends on the left and may join the hemiazygos vein or cross to the right to join the azygos vein.*
+
+
+*DSA venogram in a patient with SVC stenosis shows the anatomy of SVC and some of its tributaries. The right and left brachiocephalic veins join to form the SVC, which drains into the right atrium to return venous blood from the head, neck, and upper extremities. The azygos vein ascends from the abdomen in the posterior mediastinum and arches over the right mainstem bronchus to join the SVC.*
+
+
+*DSA venogram in a patient with SVC stenosis shows the anatomy of SVC and some of its tributaries. The right and left brachiocephalic veins join to form the SVC, which drains into the right atrium to return venous blood from the head, neck, and upper extremities. The azygos vein ascends from the abdomen in the posterior mediastinum and arches over the right mainstem bronchus to join the SVC.*
+
+
+### Anatomic Variants of Inferior Vena Cava
+
+
+*Graphic shows 2 anatomic IVC variants with insets demonstrating cross-sectional anatomy at various levels. In panel 1, the infrarenal IVC is completely left sided (insets C and D) and enters the left renal vein, which then crosses to the right (inset B). The left renal vein may cross anterior to the aorta or may be retroaortic, as in this example. Above the renal veins, the IVC is in a normal right-sided location. Panel 2 shows a duplicated IVC. There is a normal right-sided moiety (insets A-D). The left-sided moiety has the same anatomy as a solitary left-sided IVC as the cava ascends on the left to drain into the left renal vein (inset B). The left renal vein then crosses to the right, and the suprarenal IVC is in a normal right-sided location (inset A).*
+
+
+*Graphic shows 2 anatomic IVC variants with insets demonstrating cross-sectional anatomy at various levels. In panel 1, the infrarenal IVC is completely left sided (insets C and D) and enters the left renal vein, which then crosses to the right (inset B). The left renal vein may cross anterior to the aorta or may be retroaortic, as in this example. Above the renal veins, the IVC is in a normal right-sided location. Panel 2 shows a duplicated IVC. There is a normal right-sided moiety (insets A-D). The left-sided moiety has the same anatomy as a solitary left-sided IVC as the cava ascends on the left to drain into the left renal vein (inset B). The left renal vein then crosses to the right, and the suprarenal IVC is in a normal right-sided location (inset A).*
+
+
+*Duplication of the IVC results from persistence of both supracardinal veins and has a reported prevalence of 0.2-3%. Cross-sectional CECT (panel 3) typically shows large venous structures paralleling the abdominal aorta on either side. Contrast venography (panel 4) shows that the left-sided caval moiety drains into the left renal vein while the right component ascends normally.*
+
+
+*Duplication of the IVC results from persistence of both supracardinal veins and has a reported prevalence of 0.2-3%. Cross-sectional CECT (panel 3) typically shows large venous structures paralleling the abdominal aorta on either side. Contrast venography (panel 4) shows that the left-sided caval moiety drains into the left renal vein while the right component ascends normally.*
+
+
+*Duplication of the IVC results from persistence of both supracardinal veins and has a reported prevalence of 0.2-3%. Cross-sectional CECT (panel 3) typically shows large venous structures paralleling the abdominal aorta on either side. Contrast venography (panel 4) shows that the left-sided caval moiety drains into the left renal vein while the right component ascends normally.*
+
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@@ -0,0 +1,336 @@
+---
+title: "Vocal Cord Paralysis (Right)"
+docid: "b8a07cab-5427-4efe-b55d-2460fec053db"
+authors:
+ - key: "07a2c087-6202-49e7-870b-7aa162d18f06"
+ value: "Bronwyn E. Hamilton, MD"
+breadcrumbs:
+ -
+ name: "Head and Neck"
+ slug: "head-and-neck"
+ treeNodeId: "5c1f8e17-7acd-48d8-9d55-f9f8c2cad850"
+ -
+ name: "Differential Diagnosis"
+ slug: "differential-diagnosis"
+ treeNodeId: "deb55065-e1d6-4b6f-b3e3-181fafb4e218"
+ -
+ name: "Hypopharynx and Larynx"
+ slug: "hypopharynx-and-larynx"
+ treeNodeId: "dfb667ee-219c-4644-9319-3622ba9ab468"
+ -
+ name: "Clinically Based Differentials"
+ slug: "clinically-based-differentials"
+ treeNodeId: "9f892d27-2675-493e-ae6d-3018c0e72a51"
+ -
+ name: "Vocal Cord Paralysis (Right)"
+ slug: "vocal-cord-paralysis-right"
+ treeNodeId: null
+category: "Head and Neck"
+documentVersionId: "369a992f-a6a2-46dd-9c92-785639b7698d"
+imageCount: 26
+lastUpdated: "04/13/26"
+pageDescription: "Vocal Cord Paralysis (Right)"
+pageKeywords: "Head and Neck, Differential Diagnosis, Hypopharynx and Larynx, Clinically Based Differentials, Vocal Cord Paralysis (Right)"
+pageTitle: "Vocal Cord Paralysis (Right) | STATdx"
+enhancedTitle: "Vocal Cord Paralysis (Right)"
+type: "DDX"
+references: true
+breadcrumbs:
+ - "Head and Neck"
+ - "Differential Diagnosis"
+ - "Hypopharynx and Larynx"
+ - "Clinically Based Differentials"
+ - "Vocal Cord Paralysis (Right)"
+---
+## ESSENTIAL INFORMATION
+
+- ### Key Differential Diagnosis Issues
+
+
+ - Right vagus nerve (CNX) extends from brainstem nuclei to level of clavicle
+ - Right recurrent laryngeal nerve (RLN) arises from CNX anterior to right subclavian artery (SCA), then passes beneath SCA over lung apex to ascend in neck within right tracheoesophageal groove
+ - Thoracic diagnoses thus much less common in differential diagnosis
+ - Right vocal cord paralysis (VCP) caused by diseases from medulla to clavicle
+ - If CNX is affected below level of palate, VCP alone is found without other clinical symptoms
+ - If CNX is affected above level of palate, VCP occurs with other symptoms
+ - Other cranial nerve dysfunction: CNIX, CNXI ± CNXII
+ - Ipsilateral soft palate & pharyngeal constrictor malfunction (pharyngeal plexus injury)
+ - May see uvula displaced away from side of lesion &/or ipsilateral pharyngeal constrictor atrophy
+ - Best imaging modality to evaluate CNX
+ - CECT if unsure of location of lesion based on physical examination
+ - Posterior fossa, skull base, suprahyoid neck enhanced MR best if lesion confidently localized above level of palate
+ - Literature not consistent on most common etiology of VCP
+ - Iatrogenic injuries likely #1
+ - Less likely to undergo imaging
+ - Malignancy often cited as #1 nonsurgical cause
+ - Primary cancer &/or metastatic adenopathy
+ - Rare etiology is leptomeningeal carcinomatosis involving cisternal CN X
+ - Idiopathic causes likely include toxic, inflammatory (viral, post viral, granulomatous), &/or ischemic causes
+ - Toxicity: Vincristine, alcohol
+ - Neuropathies: Radiation induced, myasthenia gravis, vitamin B12 deficiency, tuberculosis, neurosarcoid
+- ### Helpful Clues for Common Diagnoses
+
+
+ - **Squamous Cell Carcinoma****, Nodes**
+ - CT/MR: Internal jugular chain extranodal tumor extends into carotid space
+ - **Differentiated Carcinoma, Thyroid**
+ - CT: Enhancing thyroid mass with cystic or nodular enhancing nodes ± calcifications
+ - MR: Enhancing thyroid mass invades tracheoesophageal groove
+ - **Paraganglioma, Glomus Jugulare**
+ - Tumor spreads superolaterally through middle ear floor to enter middle ear cavity
+ - CT: Permeative & destructive bony changes
+ - MR: Enhancing tumor with salt & pepper (T1-bright foci of methemoglobin & flow voids, respectively)
+ - **Nasopharyngeal Carcinoma**
+ - Nasopharyngeal mucosal space tumor invades into posterolateral carotid space
+- ### Helpful Clues for Less Common Diagnoses
+
+
+ - **Cerebral Ischemia-Infarction, Acute**
+ - MR: DWI shows restricted diffusion in lateral medulla & inferior cerebellar hemisphere
+ - **Cavernous Malformation, Hemorrhagic**
+ - CT: High-density focal blood products ± calcification
+ - MR: GRE shows blooming lesion in medulla
+ - Multiple other lesions possible
+ - **Paraganglioma, Carotid Body**
+ - CT: Avidly enhancing tumor in carotid bifurcation
+ - MR: Enhancing tumor with flow voids splays internal carotid artery (ICA) from external carotid artery at bifurcation
+ - **Schwannoma, Jugular Foramen**
+ - CT: Sharply marginated tumor enlarges jugular foramen (JF)
+ - MR: Enhancing fusiform or ovoid lesion spreads superomedially toward medulla
+ - Intramural cysts may be present if large
+ - **Dissection, Carotid Artery, Neck**
+ - MR: T1 with fat saturation shows intramural hematoma (hyperintense crescent adjacent to ICA lumen)
+ - MRA: Luminal narrowing; string sign
+ - CTA: Flame-shaped ICA tapered narrowing or occlusion; may manifest as longitudinal irregularity
+ - Intimal flap with double lumen may be present from true & false lumen (false lumen typically less brightly enhancing)
+ - **Paraganglioma, Glomus Vagale**
+ - MR: Enhancing tumor in carotid space 1-2 cm below skull base with flow voids
+ - **Metastasis, Skull Base**
+ - CT: Destructive bone changes common
+ - MR: Enhancing skull base lesion in vicinity of JF
+ - History of malignancy often known
+ - **Meningioma, Skull Base**
+ - CT: JF lesion causes permeative-sclerotic or hyperostotic bone changes
+ - MR: Enhancing JF mass with dural tails, no flow voids; centrifugal spread pattern
+ - **Lung Cancer, Non-Small Cell**
+ - CT/MR: Paratracheal adenopathy may invade RLN below SCA
+ - **Esophageal Carcinoma, Cervical**
+ - CT: Primary cervical esophageal mass &/or metastatic adenopathy invade tracheoesophageal groove
+ - **Schwannoma, Carotid Space**
+ - MR: Enhancing fusiform mass with intramural cysts possible
+- ### Helpful Clues for Rare Diagnoses
+
+
+ - **Pancoast Tumor**
+ - CT/MR: Invasive apical lung malignancy
+ - **Neurofibroma, Carotid Space**
+ - CT: Low-density, ovoid or fusiform, poorly enhancing mass in carotid space
+ - MR: T2 hyperintense ± central hypointensity
+ - **Anaplastic Carcinoma, Thyroid**
+ - CT/MR: Large, invasive enhancing thyroid mass in older adult patient
+ - May have history of multinodular goiter
+ - **Chondrosarcoma, Skull Base**
+ - CT: Invasive skull base mass centered in petrooccipital fissure
+ - 50% calcified matrix
+ - MR: Low T1, high T2 signal; enhances
+ - **Multiple Sclerosis, Brainstem**
+ - MR: Hyperintense T2/FLAIR periventricular cerebral white matter lesions
+ - Medullary lesions causing VCP not always visible
+ - **Right Subclavian Artery Aneurysm**
+ - Intrathoracic aneurysm usually atherosclerotic
+ - CTA: Focal enhancing outpouching/aneurysm
+ - **Pleural Metastases**
+ - CT/MR: Enhancing apical pleural mass invades SCA
+ - **Parathyroid Carcinoma**
+ - Rare lesion that may cause VCP
+ - Typically more rapid symptom onset, less enhancing, & more invasive appearing on CECT than parathyroid adenoma
+ - **Parathyroid Adenoma**
+ - Rare cause of VCP, potentially reversible
+ - Typically intense enhancement on arterial-phase CECT without venous phase washout
+- ### Alternative Differential Approaches
+
+
+ - Another approach to organizing causes of right VCP is to group lesions by **anatomic segment** of **right CNX**
+ - Intramedullary, skull base, carotid space, tracheoesophageal groove, & SCA/thoracic apex causes
+ - Intramedullary causes
+ - [Cerebral ischemia-infarction, acute](/document/acute-cerebral-ischemiainfarction/7a3ed4a9-ae05-4d64-ae8e-6a30105501e1)
+ - [Cavernous malformation, hemorrhagic](/document/cavernous-malformation/cc2415f4-75a6-458d-800e-be31b3cf50c3)
+ - [Multiple sclerosis, brainstem](/document/multiple-sclerosis/abe95a5e-394f-411b-aca6-72ab160a1d0d)
+ - Skull base/JF causes
+ - Paraganglioma, glomus jugulare
+ - [Schwannoma, JF](/document/jugular-foramen-schwannoma/39fe8b5f-828f-4529-a1a5-2e267c0ab63c)
+ - [Metastasis, skull base](/document/skull-base-metastasis/9f46be56-65de-44f1-bf85-8aa3c6b7d078)
+ - Meningioma, skull base
+ - [Chondrosarcoma, skull base](/document/skull-base-chondrosarcoma/092dd3bf-00db-4a10-b62a-99e4e4c59992)
+ - Carotid space causes
+ - [Squamous cell carcinoma, nodes](/document/nodal-squamous-cell-carcinoma/75d61354-5c63-426a-8a44-7fbf37fec2ee)
+ - [Paraganglioma, glomus vagale](/document/vagal-paraganglioma/55cc2103-a155-4281-ac39-b0b8ab02e98e)
+ - [Nasopharyngeal carcinoma](/document/nasopharyngeal-carcinoma/3b6e5802-e8c2-461a-8efd-1b634b92c8c1)
+ - [Paraganglioma, carotid body](/document/carotid-body-paraganglioma/a5459eb7-9242-4447-9cc2-d7ccc3122ef7)
+ - [Dissection, carotid artery, neck](/document/carotid-artery-dissection-in-neck/95470e51-7671-447c-9c49-30d4e55d04aa)
+ - [Schwannoma, carotid space](/document/carotid-space-schwannoma/77e63e12-2bf6-4295-b73d-501c8ffbbde0)
+ - [Neurofibroma, carotid space](/document/carotid-space-neurofibroma/104a7e79-39b3-4bc8-be70-0c9e142bc1af)
+ - Visceral space/tracheoesophageal groove causes
+ - [Differentiated carcinoma, thyroid](/document/differentiated-thyroid-carcinoma/cb142f02-5c13-4b23-aa67-9b3f444e0632)
+ - [Esophageal carcinoma, cervical](/document/cervical-esophageal-carcinoma/99d6ff5f-3d60-417d-bda8-b176c0f0af7c)
+ - [Anaplastic carcinoma, thyroid](/document/anaplastic-thyroid-carcinoma/29943999-4c05-46ca-80d1-5384b65d870b)
+ - SCA/thoracic apex causes
+ - Pancoast tumor
+ - Right SCA aneurysm
+ - Pleural metastases
+
+## References
+
+## Selected References
+
+1. [Jacks A et al: Computed tomography measurements in assessment of idiopathic vocal fold paralysis. J Voice. 37(2):289.e15-21, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=33358294%5Bpmid%5D)
+1. [Yokoyama K et al: Left parathyroid carcinoma with secondary hyperparathyroidism: a case report. BMC Endocr Disord. 23(1):108, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37193977%5Bpmid%5D)
+1. [Bashir MH et al: Revisiting CT signs of unilateral vocal fold paralysis: a single, blinded study. AJNR Am J Neuroradiol. 43(4):592-6, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35332018%5Bpmid%5D)
+1. [Deng F: Fair performance of CT in diagnosing unilateral vocal fold paralysis. AJNR Am J Neuroradiol. 43(12):E64, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36396337%5Bpmid%5D)
+1. [Shalabi F et al: A case report of unilateral cervical lymphadenopathy and multiple cranial neuropathies following mRNA-COVID-19 vaccination. BMC Neurol. 22(1):369, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36163025%5Bpmid%5D)
+1. [Wang JA et al: Application of 4D-CT scanning in differential diagnosis of arytenoid subluxation and vocal fold paralysis. J Voice. 36(6):859-67, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=33071147%5Bpmid%5D)
+1. [Yamada G et al: Bilateral vocal cord paralysis associated with meningeal carcinomatosis from lung adenocarcinoma. Case Rep Neurol. 14(2):245-50, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35815104%5Bpmid%5D)
+1. [Chew HS et al: Diagnostic yield of computed tomography in the evaluation of unilateral vocal fold palsy. J Laryngol Otol. 135(3):255-8, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33648615%5Bpmid%5D)
+1. [Dean A et al: Stridor due to cranial nerve X palsy progressing to polyneuropathy in a teenager with COVID-19. Pediatrics. 148(6), 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34851417%5Bpmid%5D)
+1. [Jain V: The role of imaging in the evaluation of hoarseness: a review. J Neuroimaging. 31(4):665-85, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34018650%5Bpmid%5D)
+1. [Kheok SW et al: Cardiovascular hoarseness (Ortner's syndrome): a pictorial review. Curr Probl Diagn Radiol. 50(5):749-54, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33036813%5Bpmid%5D)
+1. [Politano S et al: Yield of imaging to evaluate unilateral vocal fold paralysis of unknown etiology. Laryngoscope. 131(8):1840-4, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33009830%5Bpmid%5D)
+1. [Ruda J et al: Multi-institutional evaluation of radiologic findings associated with pediatric congenital idiopathic bilateral vocal fold dysfunction. Otolaryngol Head Neck Surg. 164(6):1314-21, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33019881%5Bpmid%5D)
+1. [Willegers T et al: Bilateral vocal cord paralysis due to an immune-related adverse event of nivolumab: a case report. J Immunother. 43(3):93-4, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32011418%5Bpmid%5D)
+1. [Zhao T et al: Vocal cord paralysis due to ectopic parathyroid adenoma and function recovery: a case report and review of the literature. Endocr J. 67(2):161-5, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31685721%5Bpmid%5D)
+1. [Wu TJ et al: Neurosarcoidosis presenting initially as idiopathic vocal cord paralysis. Ann Otol Rhinol Laryngol. 128(2):157-61, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30360636%5Bpmid%5D)
+1. [Méndez Garrido S et al: Causes and imaging manifestations of paralysis of the recurrent laryngeal nerve. Radiologia. 58(3):225-34, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27066920%5Bpmid%5D)
+1. [Syamal MN et al: Vocal fold paresis: a review of clinical presentation, differential diagnosis, and prognostic indicators. Curr Opin Otolaryngol Head Neck Surg. 24(3):197-202, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27092906%5Bpmid%5D)
+1. [Seyed Toutounchi SJ et al: Vocal cord paralysis and its etiologies: a prospective study. J Cardiovasc Thorac Res. 6(1):47-50, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24753832%5Bpmid%5D)
+1. [Nguyen TT et al: Vocal cord paralysis secondary to spontaneous internal carotid dissection: case report and systematic review of the literature. J Otolaryngol Head Neck Surg. 42:34, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23668480%5Bpmid%5D)
+1. [Kwong Y et al: Radiology of vocal cord palsy. Clin Radiol. 67(11):1108-14, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22608245%5Bpmid%5D)
+1. [Paquette CM et al: Unilateral vocal cord paralysis: a review of CT findings, mediastinal causes, and the course of the recurrent laryngeal nerves. Radiographics. 32(3):721-40, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22582356%5Bpmid%5D)
+1. [Harari A et al: Parathyroid carcinoma: a 43-year outcome and survival analysis. J Clin Endocrinol Metab. 96(12):3679-86, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21937626%5Bpmid%5D)
+1. [Mechchat A et al: Giant aneurysm of the right intra thoracic sub-clavian artery presenting as a dysphonia. Pan Afr Med J. 9:39, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=22355438%5Bpmid%5D)
+1. [Song SW et al: CT evaluation of vocal cord paralysis due to thoracic diseases: a 10-year retrospective study. Yonsei Med J. 52(5):831-7, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21786449%5Bpmid%5D)
+1. [Lee JC et al: Parathyroid adenoma as a cause of vocal fold paralysis. Arch Otolaryngol Head Neck Surg. 135(7):712-3, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19620594%5Bpmid%5D)
+1. [Chin SC et al: Using CT to localize side and level of vocal cord paralysis. AJR Am J Roentgenol. 180(4):1165-70, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12646476%5Bpmid%5D)
+1. [Romo LV et al: Atrophy of the posterior cricoarytenoid muscle as an indicator of recurrent laryngeal nerve palsy. AJNR Am J Neuroradiol. 20(3):467-71, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10219413%5Bpmid%5D)
+1. [Jacobs CJ et al: Vagal neuropathy: evaluation with CT and MR imaging. Radiology. 164(1):97-102, 1987](http://www.ncbi.nlm.nih.gov/pubmed/?term=3588933%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+**Squamous Cell Carcinoma, Nodes**
+*Axial CECT shows a right tonsillar squamous cell carcinoma
with adjacent lateral retropharyngeal nodes
. The loss of soft tissue planes in the oropharyngeal carotid space indicates that an extranodal tumor
is present.*
+
+
+**Squamous Cell Carcinoma, Nodes**
+*Axial CECT shows a right tonsillar squamous cell carcinoma
with adjacent lateral retropharyngeal nodes
. The loss of soft tissue planes in the oropharyngeal carotid space indicates that an extranodal tumor
is present.*
+
+
+**Squamous Cell Carcinoma, Nodes**
+*Axial CECT shows a right tonsillar squamous cell carcinoma
with adjacent lateral retropharyngeal nodes
. The loss of soft tissue planes in the oropharyngeal carotid space indicates that an extranodal tumor
is present.*
+
+
+**Differentiated Carcinoma, Thyroid**
+*Axial CECT demonstrates invasion of the right tracheoesophageal groove
by this differentiated thyroid carcinoma. Also notice the low internal jugular malignant lymph node
just lateral to the internal jugular vein (IJV).*
+
+
+**Nasopharyngeal Carcinoma**
+*Axial T1 C+ FS MR shows a large nasopharyngeal mucosal space mass that invades the right prevertebral muscles
and carotid space
. Cranial nerves IX-XI were all affected at presentation.*
+
+
+**Cerebral Ischemia-Infarction, Acute**
+*Axial FLAIR MR shows a lateral medullary subacute ischemic area secondary to vertebral artery dissection. The high-signal area is confined to the lateral medulla
without inferior cerebellar hemisphere involvement. Focal posterior inferior cerebellar artery embolus was suspected.*
+
+
+**Cavernous Malformation, Hemorrhagic**
+*Sagittal T1 C+ MR shows a large posterior fossa hemorrhagic cavernous malformation with multiple blood-filled "bubbles" within the 4th ventricle and vermis.*
+
+
+**Paraganglioma, Carotid Body**
+*Axial CECT demonstrates clear definition of the avidly enhancing carotid body paraganglioma
sitting in the notch between the external carotid artery
and the internal carotid artery (ICA)
. Vagal neuropathy is less common with this tumor than with glomus vagale paraganglioma.*
+
+
+**Schwannoma, Jugular Foramen**
+*Axial T1 C+ FS MR shows a well-marginated enhancing schwannoma
expanding the right jugular foramen
. Bone CT (not shown) demonstrated smooth remodeling and enlargement without destructive changes.*
+
+
+**Dissection, Carotid Artery, Neck**
+*Axial T1 FS MR shows a tiny flow void
within the right ICA with peripheral crescentic high signal from the intramural hemorrhage
associated with this dissection. The opposite left ICA reveals normal size of flow void
.*
+
+
+**Paraganglioma, Glomus Vagale**
+*Axial T1 C+ FS MR reveals an enhancing high nasopharyngeal carotid space mass
on the right that elevates the ICA
and displaces the styloid process laterally
.*
+
+
+**Metastasis, Skull Base**
+*Axial T1 C+ MR reveals dural metastases in the posterior fossa
with significant invasion of the right jugular foramen
. The bony margins appear irregular. Rapid onset of IX-XI cranial neuropathy was the indication for this examination.*
+
+
+**Meningioma, Skull Base**
+*Axial T1 C+ FS MR shows an enhancing dural-based mass extending through the right jugular foramen
. Bone margins appear preserved without enhancement. An associated dural tail
is present.*
+
+
+**Esophageal Carcinoma, Cervical**
+*Axial CECT in a patient with right vocal cord paralysis shows an infiltrative esophageal carcinoma
extending into the right tracheoesophageal groove
, which may also invade the posterior right thyroid lobe.*
+
+
+**Schwannoma, Carotid Space**
+*Axial CECT shows a hypodense mass in the high right carotid space
posteromedial to the styloid process
, corresponding to histologically confirmed schwannoma. The mass splays the ICA
and IJV
, indicating vagal nerve origin.*
+
+
+**Pancoast Tumor**
+*Axial arterial-phase CECT shows a right apical lung mass
invading beneath and around the right subclavian artery
. Note thoracic spinal and rib osseous metastatic disease with soft tissue extension into epidural space
and mediastinum
. Right pleural effusion
is present.*
+
+
+### Additional Images
+
+
+**Parathyroid Adenoma**
+*Axial arterial-phase CECT shows an intensely enhancing right tracheoesophageal groove parathyroid adenoma
thought to be responsible for this patients' right vocal cord paresis.*
+
+
+**Schwannoma, Jugular Foramen**
+*Axial T1 C+ MR shows a large enhancing mass in the right basal cistern
. This schwannoma can be followed inferolaterally into the jugular foramen
.*
+
+
+**Meningioma, Skull Base**
+*Axial T1 C+ FS MR shows extensive skull base meningioma that involves the right occipital bone
and extends through the jugular foramen into the nasopharyngeal carotid space
.*
+
+
+**Lung Cancer, Non-Small Cell**
+*Axial CECT shows right paratracheal adenopathy
that was the source of a new right vocal cord paralysis in this patient with known left-sided lung cancer
.*
+
+
+**Lung Cancer, Non-Small Cell**
+*Coronal CECT MPR in a patient with right vocal cord paralysis shows an infiltrative mediastinal nodal mass
invading beneath the right subclavian artery at the brachiocephalic junction
where the right recurrent laryngeal nerve arises from the vagus. The small right upper lobe primary tumor was also seen (not shown).*
+
+
+**Esophageal Carcinoma, Cervical**
+*Axial CECT shows a bulky, heterogeneous soft tissue mass that invades the right carotid space
and tracheoesophageal groove
. Note the back of the trachea is destroyed
by this aggressive esophageal malignancy.*
+
+
+**Schwannoma, Carotid Space**
+*Axial CECT demonstrates an ovoid inhomogeneously enhancing right carotid space vagal schwannoma
. Notice the carotid arteries are displaced anteromedially
, although the internal jugular vein is flattened anterolaterally
.*
+
+
+**Neurofibroma, Carotid Space**
+*Axial T2 MR shows a well-defined tumor in the right carotid space
posterior and medial to the common carotid artery
and internal jugular vein
. Schwannoma and neurofibroma of the vagus nerve are both differential diagnosis possibilities.*
+
+
+**Anaplastic Carcinoma, Thyroid**
+*Axial CECT reveals an invasive right thyroid lobe mass that has spread into the carotid space
, perivertebral space
, and endolarynx
.*
+
+
+**Chondrosarcoma, Skull Base**
+*Axial T1 C+ MR demonstrates a mass centered on the low petrooccipital fissure on the right. The lesion extends inferolaterally into the jugular foramen
where it affects cranial nerves IX-XI.*
+
+
+**Multiple Sclerosis, Brainstem**
+*Coronal FLAIR MR reveals a right lateral medullary multiple sclerosis plaque
. An active lesion in this area can affect any of 3 cranial nerves (IX, X, XI).*
+
+
+**Pleural Metastases**
+*Axial CECT shows enhancing pleural metastases from thyroid carcinoma at the right lung apex
beneath the right subclavian artery
. Small right thyroid cancer primary is also visible
.*
+
diff --git a/docs_md/articles/zellweger-syndrome-and-peroxisomal-biogenesis-disorders_5a3e0701-0615-4e17-b102-d67a399a9fc2.md b/docs_md/articles/zellweger-syndrome-and-peroxisomal-biogenesis-disorders_5a3e0701-0615-4e17-b102-d67a399a9fc2.md
new file mode 100644
index 0000000..f74f510
--- /dev/null
+++ b/docs_md/articles/zellweger-syndrome-and-peroxisomal-biogenesis-disorders_5a3e0701-0615-4e17-b102-d67a399a9fc2.md
@@ -0,0 +1,345 @@
+---
+title: "Zellweger Syndrome and Peroxisomal Biogenesis Disorders"
+docid: "5a3e0701-0615-4e17-b102-d67a399a9fc2"
+authors:
+ - key: "838e1722-2479-4fbd-a5fe-d965980a1a2c"
+ value: "Blaise V. Jones, MD"
+breadcrumbs:
+ -
+ name: "Pediatrics"
+ slug: "pediatrics"
+ treeNodeId: "40c0ffd5-41a0-4a6f-99fd-74c72c2e0bd3"
+ -
+ name: "Diagnosis"
+ slug: "diagnosis"
+ treeNodeId: "8c45dd6b-ac86-4be1-b770-4755c799ad00"
+ -
+ name: "Pediatric Neuroradiology"
+ slug: "pediatric-neuroradiology"
+ treeNodeId: "47bf4839-9cae-4078-8a93-b14004d9eef1"
+ -
+ name: "Brain"
+ slug: "brain"
+ treeNodeId: "64b09eb7-b76b-48be-9ef0-064fb3828f85"
+ -
+ name: "Pathology-Based Diagnoses"
+ slug: "pathology-based-diagnoses"
+ treeNodeId: "827f44e9-f4d6-4bf1-814e-08372b32333f"
+ -
+ name: "Inherited Metabolic/Degenerative Disorders"
+ slug: "inherited-metabolicdegenerative-di-"
+ treeNodeId: "da66af2b-69eb-489d-9f88-918e39338204"
+ -
+ name: "Peroxisomal Disorders"
+ slug: "peroxisomal-disorders"
+ treeNodeId: "4bc1d7ee-f43d-4f03-8cc9-a3f195c98827"
+ -
+ name: "Zellweger Syndrome and Peroxisomal Biogenesis Disorders"
+ slug: "zellweger-syndrome-and-peroxisomal-"
+ treeNodeId: null
+category: "Pediatrics"
+documentVersionId: "c0a29a4f-2b01-4be0-87ff-74d735b10b60"
+imageCount: 8
+lastUpdated: "02/13/24"
+pageDescription: "Zellweger Syndrome and Peroxisomal Biogenesis Disorders"
+pageKeywords: "Pediatrics, Diagnosis, Pediatric Neuroradiology, Brain, Pathology-Based Diagnoses, Inherited Metabolic/Degenerative Disorders, Peroxisomal Disorders, Zellweger Syndrome and Peroxisomal Biogenesis Disorders"
+pageTitle: "Zellweger Syndrome and Peroxisomal Biogenesis Disorders | STATdx"
+enhancedTitle: "Zellweger Syndrome and Peroxisomal Biogenesis Disorders"
+type: "DX"
+references: true
+breadcrumbs:
+ - "Pediatrics"
+ - "Diagnosis"
+ - "Pediatric Neuroradiology"
+ - "Brain"
+ - "Pathology-Based Diagnoses"
+ - "Inherited Metabolic/Degenerative Disorders"
+ - "Peroxisomal Disorders"
+ - "Zellweger Syndrome and Peroxisomal Biogenesis Disorders"
+---
+## KEY FACTS
+
+- ### Terminology
+
+
+ - Disorders of peroxisome biosynthesis, assembly, and biochemical functions
+ - Varying phenotypes reflect degree of dysfunction
+ - Severe: Zellweger syndrome (ZS)
+ - Moderate: Neonatal adrenoleukodystrophy (NALD)
+ - Mild: Infantile Refsum disease (IRD), Heimler syndrome (HS)
+- ### Imaging
+
+
+ - Polymicrogyria, pachygyria, hypomyelination, germinolytic cysts, heterotopic gray matter
+ - Leukoencephalopathy with abnormal myelin in corticospinal tracts and dentate nuclei
+ - Volume loss evident after 1 year
+ - Use short TE MRS: ↓ NAA; ↑ Cho
+ - Lipid peaks at 0.9 and 1.33 ppm
+ - Polymicrogyria most severe in perisylvian region, especially posterior insula
+- ### Top Differential Diagnoses
+
+
+ - Congenital CMV
+ - Single peroxisomal enzyme deficiencies
+- ### Pathology
+
+
+ - Autosomal recessive
+ - 70% associated with *PEX1*mutations
+ - 15-20% associated with *PEX6* mutations
+ - Laboratory analysis demonstrates ↓ peroxisomal function
+ - Elevated plasma levels of sphingomyelin, very long chain fatty acids (VLCFA), pristanic acid, phytanic acid, pipecolic acid
+- ### Clinical Issues
+
+
+ - Elevated liver enzymes, hepatomegaly
+ - Vision loss, hearing loss
+ - Severe deficiencies (ZS) manifest at birth
+ - Milder forms (IRD) may present later in childhood
+- ### Diagnostic Checklist
+
+
+ - Polymicrogyria most severe in perisylvian region, especially posterior insula
+
+## TERMINOLOGY
+
+- ### Abbreviations
+
+
+ - Peroxisomal biogenesis disorders (PBD)
+ - Zellweger syndrome (ZS)
+ - Neonatal adrenoleukodystrophy (NALD)
+ - Infantile Refsum disease (IRD)
+ - Rhizomelic chondrodysplasia punctata (RCDP) type 1
+ - Heimler syndrome (HS)
+ - Peroxisomal enzyme deficiencies (PED)
+ - Adrenoleukodystrophy protein deficiency (X-ALD)
+ - Alkyl-DHAP-synthase deficiency (RCDP type 3)
+ - Acyl-CoA oxidase deficiency (ACOX1D)
+ - D-bifunctional protein deficiency (D-BP)
+ - Sterol carrier protein X deficiency (SCPx)
+ - 2-methylacyl CoA racemase deficiency (AMACR)
+ - DHAP-alkyl transferase deficiency (DHAPAT; RCDP type 2)
+ - Adult Refsum disease (ARD)
+ - Primary hyperoxaluria type 1 (PH1)
+ - Acatalasemia (ACATLAS)
+- ### Synonyms
+
+
+ - ZS spectrum (ZSS)
+ - Peroxisome biogenesis disorders (PBD)
+- ### Definitions
+
+
+ - Disorders of peroxisome biosynthesis, assembly, and biochemical functions
+ - Caused by defective peroxisome biogenesis
+ - Varying phenotypes reflect degree of dysfunction
+ - Severe: Zellweger syndrome (ZS)
+ - Moderate: Neonatal adrenoleukodystrophy (NALD)
+ - Mild: Infantile Refsum disease (IRD), Heimler syndrome (HS)
+ - Related disorders
+ - Multiple peroxisomal enzyme deficiencies (PEDs): Rhizomelic chondrodysplasia punctata (RCDP)
+ - Single peroxisomal enzyme deficiency: Acyl-CoA oxidase deficiency, adrenoleukodystrophy protein deficiency (X-ALD), D-bifunctional protein deficiency (D-BP)
+
+## IMAGING
+
+- ### General Features
+
+
+ - #### Best diagnostic clue
+
+
+ - Polymicrogyria, pachygyria, hypomyelination, germinolytic cysts, heterotopic gray matter
+ - Leukoencephalopathy with abnormal myelin in corticospinal tracts and dentate nuclei
+ - Volume loss evident after 1 year
+ - #### Location
+
+
+ - Polymicrogyria most severe in perisylvian region, especially posterior insula
+ - Pachygyria most common frontoparietal
+ - Diffuse hypomyelination; cerebellum and brainstem may be involved, especially if present > 1 year
+ - #### Size
+
+
+ - Central volume loss common
+ - #### Morphology
+
+
+ - ± heterotopia (periventricular or subcortical)
+- ### MR Findings
+
+
+ - #### T1WI
+
+
+ - Polymicrogyria, pachygyria
+ - Germinolytic cysts (near foramina of Monro)
+ - ± ↑ globus pallidus signal from hyperbilirubinemia
+ - #### T2WI
+
+
+ - ↑ white matter (WM) signal in corticospinal tracts and dentate nuclei
+ - #### T1WI C+
+
+
+ - May see enhancement of corticospinal tracts in brainstem
+ - #### MRS
+
+
+ - Use short TE: ↓ NAA; ↑ Cho; lipid peaks at 0.9 and 1.33 ppm
+- ### Imaging Recommendations
+
+
+ - #### Best imaging tool
+
+
+ - MR + MRS
+ - #### Protocol advice
+
+
+ - Volumetric T1, high-resolution T2 for migrational abnormalities
+
+## DIFFERENTIAL DIAGNOSIS
+
+- [Congenital CMV](/document/congenital-cmv/ad51c41b-8389-4adb-b4b0-f1d35a380338)
+ - Ca⁺⁺, periventricular cysts usually not caudothalamic
+- ### Pseudo-TORCH
+
+
+ - Basal ganglia, thalamic, and periventricular Ca⁺⁺
+- ### Single Peroxisomal Enzyme Deficiencies
+
+
+ - Brain MR may be similar; biochemistry different
+
+## PATHOLOGY
+
+- ### General Features
+
+
+ - #### Etiology
+
+
+ - Defect in biogenesis of peroxisomes
+ - Defective transport of proteins into peroxisomal matrix → accumulation of very long chain fatty acids (VLCFA)
+ - Laboratory analysis demonstrating ↓ peroxisomal function
+ - Elevated plasma levels of sphingomyelin, VLCFA, pristanic acid, phytanic acid, pipecolic acid
+ - Deficiency of RBC plasmalogens
+ - #### Genetics
+
+
+ - Autosomal recessive
+ - Caused by mutations in 1 of 14 known PEX genes
+ - 70% associated with *PEX1* mutations
+ - 15-20% associated with *PEX6* mutations
+ - #### Associated abnormalities
+
+
+ - Characteristic facies in ZS: High forehead, broad nasal bridge, hypertelorism
+ - Large fontanelle + sutures
+ - Eye: Brushfield spots, retinal pigment degeneration
+ - Hepatomegaly, renal cortical cysts
+ - Skeletal: Stippled chondral Ca⁺⁺ in RCDP
+- ### Gross Pathologic & Surgical Features
+
+
+ - Leukoencephalopathy, germinolytic cysts, cortical and cerebellar malformations
+ - Subcortical heterotopia, cerebellar hypoplasia
+- ### Microscopic Features
+
+
+ - Pachygyria, polymicrogyria, or microgyria
+ - Sudanophilic leukodystrophy
+
+## CLINICAL ISSUES
+
+- ### Presentation
+
+
+ - #### Most common signs/symptoms
+
+
+ - Severe hypotonia, seizures, poor sucking
+ - Elevated liver enzymes, hepatomegaly
+ - #### Other signs/symptoms
+
+
+ - Vision loss due to retinal dystrophy and abnormal optic nerves
+ - Hearing loss
+ - #### Clinical profile
+
+
+ - Low Apgar scores; very floppy, dysmorphic facies
+- ### Demographics
+
+
+ - #### Age
+
+
+ - Severe deficiencies (ZS) manifest at birth
+ - Milder forms (IRD) may present later in childhood
+ - Rarely diagnosed in adults
+ - #### Epidemiology
+
+
+ - 1:50,000 to 1:100,000
+ - Incidence ↑ with detection of milder forms
+- ### Natural History & Prognosis
+
+
+ - Most severely affected die < 3 months, milder may live > 20 years
+- ### Treatment
+
+
+ - Supportive, no proven therapy
+
+ bd0130f6-98cd-48ae-828f-6de36e1e4cf9
+
+## References
+
+## Selected References
+
+1. [Bose M et al: Characterization of severity in Zellweger spectrum disorder by clinical findings: a scoping review, meta-analysis and medical chart review. Cells. 11(12):1891, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35741019%5Bpmid%5D)
+1. [Tan AP et al: Clinical and neuroimaging spectrum of peroxisomal disorders. Top Magn Reson Imaging. 27(4):241-57, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30086110%5Bpmid%5D)
+1. [Braverman NE et al: Peroxisome biogenesis disorders in the Zellweger spectrum: an overview of current diagnosis, clinical manifestations, and treatment guidelines. Mol Genet Metab. 117(3):313-21, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26750748%5Bpmid%5D)
+1. [Salpietro V et al: Zellweger syndrome and secondary mitochondrial myopathy. Eur J Pediatr. 174(4):557-63, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25287621%5Bpmid%5D)
+1. [Crane DI: Revisiting the neuropathogenesis of Zellweger syndrome. Neurochem Int. 69:1-8, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24607700%5Bpmid%5D)
+1. [van der Knaap MS et al: MRI as diagnostic tool in early-onset peroxisomal disorders. Neurology. 78(17):1304-8, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22459681%5Bpmid%5D)
+1. [Kulkarni KS et al: Contrast enhancement of brainstem tracts in Zellweger spectrum disorder: evidence of inflammatory demyelination? Neuropediatrics. 42(1):32-4, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21557147%5Bpmid%5D)
+1. [Krause C et al: Rational diagnostic strategy for Zellweger syndrome spectrum patients. Eur J Hum Genet. 17(6):741-8, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19142205%5Bpmid%5D)
+1. [Weller S et al: Cerebral MRI as a valuable diagnostic tool in Zellweger spectrum patients. J Inherit Metab Dis. 31(2):270-80, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18415699%5Bpmid%5D)
+1. [Barkovich AJ et al: MR of Zellweger syndrome. AJNR Am J Neuroradiol. 18(6):1163-70, 1997](http://www.ncbi.nlm.nih.gov/pubmed/?term=9194444%5Bpmid%5D)
+
+
+## Images
+
+
+### Selected Images
+
+
+*Axial T1WI MR in an 8-month-old boy with Zellweger syndrome shows polymicrogyria
at the apex of each sylvian fissure.*
+
+
+*Axial T2WI MR in the same patient at 3 years of age shows the thickening of the cortical ribbon on each side with an irregular gray matter-white matter interface. The complexity of white matter rami is diminished in the regions of polymicrogyria
when compared to the normal frontal white matter.*
+
+
+*Axial T2WI MR in a 5-month-old with neonatal adrenoleukodystrophy shows bilateral polymicrogyria
. Subtle areas of cortical malformation are best seen on T2WI in the neonatal period but can be more difficult to discern as myelination progresses.*
+
+
+*Dysmyelination in the periventricular white matter
, corticospinal tracts
, and dentate nuclei of the cerebellum
are evident in this 4-year-old with infantile Refsum disease.*
+
+
+### Additional Images
+
+
+*Axial T2WI MR shows patchy bihemispheric demyelination
, hyperintense subependymal germinolytic cysts
, and abnormal left sylvian fissure operculization
.*
+
+
+*Single voxel MRS using PRESS short TE (35 msec) technique reveals prominent peaks in the lipid and lactate range
in a patient with Zellweger syndrome. (Courtesy C. Glasier, MD.)*
+
+
+*Axial T2WI MR shows caudothalamic groove cysts
and demyelination in a patient with Zellweger syndrome. Note the perisylvian polymicrogyria
.*
+
+
+*Coronal T2WI MR of Zellweger syndrome demonstrates small germinolytic cysts
.*
+
diff --git a/requirements.txt b/requirements.txt
index 1d08318..755207f 100644
--- a/requirements.txt
+++ b/requirements.txt
@@ -4,4 +4,6 @@ playwright>=1.40.0
python-dotenv>=1.0.0
loguru
pyyaml
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+fastapi
+uvicorn
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diff --git a/results.json b/results.json
index ab4b0cd..a6a83e1 100644
--- a/results.json
+++ b/results.json
@@ -199,6 +199,46 @@
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+ {
+ "path": "docs_md/articles/cerebral-amyloid-disease_18edc9f3-9218-410c-8280-29c3e6df4c91.md",
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+ "breadcrumbs": [
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+ "Diagnosis",
+ "Pathology-Based Diagnoses",
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+ "authors": [
+ {
+ "key": "2bca6b86-1eca-4e93-b997-4e18913686a7",
+ "value": "Hediyeh Baradaran, MD, MS"
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+ {
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+ "value": "Karen L. Salzman, MD, FACR"
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@@ -285,7 +325,7 @@
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@@ -301,6 +341,92 @@
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},
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},
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- "path": "missing_45c3147e-3a1b-4fbf-a626-ed6e99a02ac2",
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"reasons": [
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+ "snippet": "\n## KEY FACTS\n\n- ### Terminology\n \n \n - Rapidly enlarging, malignant WHO grade 4 astrocytic tumor characterized by necrosis & neovascularity\n- ### Imaging\n \n \n - Best imaging clue: Thick, irregularly enhancing rind of neoplastic tissue surrounding necrotic core\n - Heterogeneous, hyperin...",
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+ "snippet": "\n## TERMINOLOGY\n\n- ### Abbreviations\n \n \n - Aortic arch (AA); brachiocephalic trunk (BCT)\n - Right common carotid arteries (RCCA)\n - Left common carotid arteries (LCCA)\n - Right subclavian arteries (RSCA)\n - Left subclavian arteries (LSCA)\n - Congenital heart disease (CHD)\n- ### ...",
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+ "snippet": "\n## TERMINOLOGY\n\n- ### Abbreviations\n \n \n - Anterior cerebral artery (ACA)\n\n## GROSS ANATOMY\n\n- ### Overview\n \n \n - Smaller, more medial terminal branch of supraclinoid internal carotid artery (ICA)\n - 3 segments\n - Horizontal or precommunicating (**A1**) segment\n - Ve...",
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+ "snippet": "\n## TERMINOLOGY\n\n- ### Abbreviations\n \n \n - Posterior cerebral artery (PCA)\n - Posterior communicating artery (PCoA)\n - Basilar artery (BA)\n - Internal carotid artery (ICA)\n\n## GROSS ANATOMY\n\n- ### Overview\n \n \n - Main BA terminal branches = 2 PCAs\n - 4 segments\n - Pre...",
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+ {
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+ "authors": [
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+ "snippet": "\n## KEY FACTS\n\n- ### Terminology\n \n \n - Secondary brain tumors (metastases) arise from\n - Tumors outside CNS spreading to CNS\n - Usually via hematogenous dissemination\n - Primary CNS neoplasms spreading from one site to another\n - Usually geog...",
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+ "snippet": "\n## KEY FACTS\n\n- ### Terminology\n \n \n - Many drugs (prescription, illicit, or street) have adverse CNS effects\n - Illicit drug use often causes cerebrovascular disease\n - Amphetamines, cocaine > opioids, cannabis\n - Polydrug abuse (including EtOH) is common\n - Nitrous ox...",
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+ "path": "docs_md/articles/abnormal-shape-configuration-of-corpus-callosum_238ca32d-6bc6-4f5a-81b1-6601dd605856.md",
+ "title": "Abnormal Shape/Configuration of Corpus Callosum",
+ "docid": "238ca32d-6bc6-4f5a-81b1-6601dd605856",
+ "breadcrumbs": [
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+ "pageKeywords": "Pediatrics, Differential Diagnosis, Brain, Abnormal Shape/Configuration of Corpus Callosum",
+ "reasons": [
+ "Linked (Diff Diag of Hypertensive Intracranial Hemorrhage)"
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+ "snippet": "\n# ESSENTIAL INFORMATION\n\n- ## Key Differential Diagnosis Issues\n \n \n - Clinical features to consider\n - Normal corpus callosum (CC) varies in thickness & shape\n - Associated anomalies portend worse prognosis\n - If not congenital anomaly, clinical history is crucial\n ...",
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"is_cached": true
},
{
- "path": "missing_1c0c7b6a-574a-4dcc-bfe9-9b0530b14386",
+ "path": "docs_md/articles/kernicterus_1c0c7b6a-574a-4dcc-bfe9-9b0530b14386.md",
"title": "Kernicterus",
"docid": "1c0c7b6a-574a-4dcc-bfe9-9b0530b14386",
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+ "Kernicterus"
+ ],
+ "authors": [
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+ "value": "Kevin R. Moore, MD"
+ },
+ {
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+ "value": "Miral D. Jhaveri, MD, MBA"
+ },
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+ "value": "Surjith Vattoth, MD"
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"reasons": [
"Linked (Diff Diag of Neonatal Hypoxic-Ischemic Injury)"
],
- "snippet": "",
- "linked_info": null,
- "is_cached": false
+ "snippet": "\n## KEY FACTS\n\n- ### Terminology\n \n \n - Kernicterus or bilirubin encephalopathy: Pathologic bilirubin staining of basal ganglia, brainstem, and hippocampi associated with hyperbilirubinemia\n- ### Imaging\n \n \n - Acute: ↑ T1 signal in globus pallidus (GP), subthalamic nuclei (STN), hippocamp...",
+ "linked_info": {
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+ "links": []
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+ "differential": {
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+ "links": [
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+ "docid": "e3e9538d-fac5-4a1f-8c7a-c989c692ff39"
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"docid": "bd221c1a-5b9c-4213-a0e1-df38acc3bd62"
},
{
- "title": "Gliomatosis Cerebri",
+ "title": "Reversible Cerebral Vasoconstriction Syndrome",
"docid": "c3a19be2-1f91-4dcb-b63a-cbfdaeaca5c1"
},
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- "reasons": [
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- "snippet": "\n## TERMINOLOGY\n\n- ### Abbreviations\n \n \n - Aortic arch (AA); brachiocephalic trunk (BCT)\n - Right common carotid arteries (RCCA)\n - Left common carotid arteries (LCCA)\n - Right subclavian arteries (RSCA)\n - Left subclavian arteries (LSCA)\n - Congenital heart disease (CHD)\n- ### ...",
- "linked_info": {
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@@ -4198,18 +5271,45 @@
"is_cached": true
},
{
- "path": "missing_914fd68b-ddab-4640-bcc4-883c425d13f8",
- "title": "Anatomy Document",
+ "path": "docs_md/articles/transcranial-doppler_914fd68b-ddab-4640-bcc4-883c425d13f8.md",
+ "title": "Transcranial Doppler",
"docid": "914fd68b-ddab-4640-bcc4-883c425d13f8",
- "breadcrumbs": [],
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+ "breadcrumbs": [
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+ "Brain and Spine",
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+ "value": "Stella Sin Yee Ho, RDMS, RVT, PhD"
+ },
+ {
+ "key": "944c1fa1-404a-4d64-a8fd-ea6184a7c876",
+ "value": "Deyond Y. W. Siu, MBChB, FRCR"
+ },
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+ "key": "961f3a7f-ad62-43bc-98f4-5116b17ab812",
+ "value": "Paula J. Woodward, MD, FSRU"
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"reasons": [
"Linked (Anatomy of Moyamoya)"
],
- "snippet": "",
- "linked_info": null,
- "is_cached": false
+ "snippet": "\n## TERMINOLOGY\n\n- ### Abbreviations\n \n \n - **Carotid arteries**\n - Common carotid (CCA); internal carotid (ICA)\n - **Cerebral arteries**\n - Anterior (ACA); middle (MCA); posterior (PCA)\n - **Communicating arteries**\n - Anterior (ACoA); posterior (PCoA)\n - **...",
+ "linked_info": {
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"docid": "2b17f148-bf71-4b13-8373-d3c65459dd67"
},
{
- "title": "Differential Diagnosis",
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},
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"docid": "6d668284-4c8e-4a14-90b6-c7abf86065e6"
},
{
- "title": "Differential Diagnosis",
+ "title": "Vocal Cord Paralysis (Right)",
"docid": "b8a07cab-5427-4efe-b55d-2460fec053db"
}
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"is_cached": true
},
{
- "path": "missing_6471fb1c-d46d-47cd-8322-1671e82335c3",
- "title": "Differential Diagnosis",
+ "path": "docs_md/articles/enhancing-cranial-nerves_6471fb1c-d46d-47cd-8322-1671e82335c3.md",
+ "title": "Enhancing Cranial Nerve(s)",
"docid": "6471fb1c-d46d-47cd-8322-1671e82335c3",
- "breadcrumbs": [],
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+ "Differential Diagnosis",
+ "Cranial Nerves and Brainstem",
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+ "Enhancing Cranial Nerve(s)"
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+ "value": "Daniel E. Meltzer, MD"
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"reasons": [
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],
- "snippet": "",
- "linked_info": null,
- "is_cached": false
+ "snippet": "\n## ESSENTIAL INFORMATION\n\n- ### Key Differential Diagnosis Issues\n \n \n - Enhancement of cisternal, cavernous sinus cranial nerve (CN) segments always abnormal\n - Which CN(s) are affected\n - Optic nerve: Multiple sclerosis (MS), neurofibromatosis type 1 (NF1) (optic glioma), viral/p...",
+ "linked_info": {
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- "title": "Parenchymal Metastases",
- "docid": "2cf0bd40-4597-4c0b-83e4-74340304b98f",
+ "path": "docs_md/articles/herpes-encephalitis-type-1_556f5f76-c20b-44ca-a913-c53b11c93341.md",
+ "title": "Herpes Encephalitis Type 1",
+ "docid": "556f5f76-c20b-44ca-a913-c53b11c93341",
"breadcrumbs": [
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+ "Pediatrics",
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"Pathology-Based Diagnoses",
- "Neoplasms",
- "Metastatic Tumors",
- "Parenchymal Metastases"
+ "Infectious Disease",
+ "Acquired Infections",
+ "Herpes Encephalitis Type 1"
],
"authors": [
{
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- "value": "Anne G. Osborn, MD, FACR"
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],
- "pageKeywords": "Brain, Diagnosis, Pathology-Based Diagnoses, Neoplasms, Metastatic Tumors, Parenchymal Metastases",
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"reasons": [
- "Linked (Diff Diag of Multiple Embolic Cerebral Infarctions)"
+ "Linked (Diff Diag of Childhood Stroke)"
],
- "snippet": "\n## KEY FACTS\n\n- ### Terminology\n \n \n - Secondary brain tumors (metastases) arise from\n - Tumors outside CNS spreading to CNS\n - Usually via hematogenous dissemination\n - Primary CNS neoplasms spreading from one site to another\n - Usually geog...",
+ "snippet": "\n## KEY FACTS\n\n- ### Terminology\n \n \n - **HSV1** brain infection\n - Typically reactivation in immunocompetent patients\n - **Different from neonatal (HSV2)**herpes encephalitis\n- ### Imaging\n \n \n - T2/FLAIR hyperintensity in **mesial temporal, subfrontal, insular, and cingular cortex*...",
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{
- "title": "Glioblastoma",
- "docid": "45c3147e-3a1b-4fbf-a626-ed6e99a02ac2"
+ "title": "Other Encephalitides",
+ "docid": "c2e7e8a3-254a-48b4-8187-bfffaf95407a"
},
{
- "title": "1° CNS Lymphoma",
- "docid": "383f7bff-10a1-40d9-9a8b-bfabd1288520"
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+ "docid": "7a3ed4a9-ae05-4d64-ae8e-6a30105501e1"
},
{
- "title": "Abscess",
- "docid": "8c199d8a-99c4-4087-82e4-a54d5d80ec1e"
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+ "path": "docs_md/articles/group-b-streptococcal-meningitis_bafa10c7-e65b-4432-9959-b8e5e4af708c.md",
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+ ],
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"reasons": [
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- "snippet": "",
- "linked_info": null,
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+ "snippet": "\n## KEY FACTS\n\n- ### Terminology\n \n \n - Group B β-hemolytic streptococcal (GBS) meningitis\n - Leading newborn meningitis cause in developed countries\n- ### Imaging\n \n \n - Acute: Meningitis, cerebritis, vasculitis, ventriculitis, subdural effusion, empyema, arterial and venous infarction...",
+ "linked_info": {
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+ "Cranial Nerves and Brainstem",
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+ "Complex Cranial Nerve 9-12 Neuropathy"
+ ],
+ "authors": [
+ {
+ "key": "eef2f839-5706-47b9-89c3-60d8315b2b3a",
+ "value": "Nicholas A. Koontz, MD"
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+ "pageKeywords": "Head and Neck, Differential Diagnosis, Cranial Nerves and Brainstem, Clinically Based Differentials, Complex Cranial Nerve 9-12 Neuropathy",
"reasons": [
"Linked (Diff Diag of Acute Cerebral Ischemia/Infarction)"
],
- "snippet": "",
- "linked_info": null,
- "is_cached": false
+ "snippet": "\n## ESSENTIAL INFORMATION\n\n- ### Key Differential Diagnosis Issues\n \n \n - Lesions causing injury to CNIX-XII are found anywhere along nerve's course from medulla to end organ\n - CNIX & CNXII remain suprahyoid\n - CNX & CNXI course infrahyoid\n - CNX in carotid s...",
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{
- "path": "missing_5b8be233-f227-4365-9e58-df57a7950b29",
- "title": "Differential Diagnosis",
+ "path": "docs_md/articles/hemifacial-spasm_5b8be233-f227-4365-9e58-df57a7950b29.md",
+ "title": "Hemifacial Spasm",
"docid": "5b8be233-f227-4365-9e58-df57a7950b29",
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"reasons": [
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],
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- "linked_info": null,
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+ "snippet": "\n## ESSENTIAL INFORMATION\n\n- ### Key Differential Diagnosis Issues\n \n \n - Overall statistics\n - In > 95% of cases, **arterial vascular loop** is cause of hemifacial spasm (HFS)\n - All other causes listed account for < 5% of cases\n - HFS\n - Definition: Segmental myo...",
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},
{
- "path": "missing_01092f4c-ec92-43df-a667-b3ee93b8a268",
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+ "path": "docs_md/articles/oculomotor-trochlear-or-abducens-neuropathy_01092f4c-ec92-43df-a667-b3ee93b8a268.md",
+ "title": "Oculomotor, Trochlear, or Abducens Neuropathy",
"docid": "01092f4c-ec92-43df-a667-b3ee93b8a268",
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+ "Oculomotor, Trochlear, or Abducens Neuropathy"
+ ],
+ "authors": [
+ {
+ "key": "eef2f839-5706-47b9-89c3-60d8315b2b3a",
+ "value": "Nicholas A. Koontz, MD"
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"reasons": [
"Linked (Diff Diag of Acute Cerebral Ischemia/Infarction)"
],
- "snippet": "",
- "linked_info": null,
- "is_cached": false
+ "snippet": "\n## ESSENTIAL INFORMATION\n\n- ### Key Differential Diagnosis Issues\n \n \n - Cranial nerves supply 6 extraocular muscles\n - CNIII: All muscles but superior oblique (CNIV) & lateral rectus (CNVI)\n - CNIII, IV, VI all have brainstem, cisternal, cavernous sinus (CS), & orbital components\n...",
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{
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+ "path": "docs_md/articles/peripheral-facial-nerve-paralysis_cff540e8-4e2c-4cc7-aa46-8e6ca277bb85.md",
+ "title": "Peripheral Facial Nerve Paralysis",
"docid": "cff540e8-4e2c-4cc7-aa46-8e6ca277bb85",
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"reasons": [
"Linked (Diff Diag of Acute Cerebral Ischemia/Infarction)"
],
- "snippet": "",
- "linked_info": null,
- "is_cached": false
+ "snippet": "\n## ESSENTIAL INFORMATION\n\n- ### Key Differential Diagnosis Issues\n \n \n - Peripheral facial nerve paralysis\n - Definition: Unilateral facial nerve injury between pontine motor nucleus & proximal extracranial facial nerve trunk after it emerges from stylomastoid foramen\n ...",
+ "linked_info": {
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},
{
- "path": "missing_b8a07cab-5427-4efe-b55d-2460fec053db",
- "title": "Differential Diagnosis",
+ "path": "docs_md/articles/vocal-cord-paralysis-right_b8a07cab-5427-4efe-b55d-2460fec053db.md",
+ "title": "Vocal Cord Paralysis (Right)",
"docid": "b8a07cab-5427-4efe-b55d-2460fec053db",
- "breadcrumbs": [],
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+ "value": "Bronwyn E. Hamilton, MD"
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+ "pageKeywords": "Head and Neck, Differential Diagnosis, Hypopharynx and Larynx, Clinically Based Differentials, Vocal Cord Paralysis (Right)",
"reasons": [
"Linked (Diff Diag of Acute Cerebral Ischemia/Infarction)"
],
- "snippet": "",
- "linked_info": null,
- "is_cached": false
+ "snippet": "\n## ESSENTIAL INFORMATION\n\n- ### Key Differential Diagnosis Issues\n \n \n - Right vagus nerve (CNX) extends from brainstem nuclei to level of clavicle\n - Right recurrent laryngeal nerve (RLN) arises from CNX anterior to right subclavian artery (SCA), then passes beneath SCA over lung ape...",
+ "linked_info": {
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"path": "docs_md/articles/homonymous-hemianopsia_1cdca8f4-95f8-4d19-b28a-19a433d1a624.md",
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},
{
- "path": "missing_5a3e0701-0615-4e17-b102-d67a399a9fc2",
- "title": "Peroxisomal Disorders",
+ "path": "docs_md/articles/zellweger-syndrome-and-peroxisomal-biogenesis-disorders_5a3e0701-0615-4e17-b102-d67a399a9fc2.md",
+ "title": "Zellweger Syndrome and Peroxisomal Biogenesis Disorders",
"docid": "5a3e0701-0615-4e17-b102-d67a399a9fc2",
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+ "breadcrumbs": [
+ "Pediatrics",
+ "Diagnosis",
+ "Pediatric Neuroradiology",
+ "Brain",
+ "Pathology-Based Diagnoses",
+ "Inherited Metabolic/Degenerative Disorders",
+ "Peroxisomal Disorders",
+ "Zellweger Syndrome and Peroxisomal Biogenesis Disorders"
+ ],
+ "authors": [
+ {
+ "key": "838e1722-2479-4fbd-a5fe-d965980a1a2c",
+ "value": "Blaise V. Jones, MD"
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+ "pageKeywords": "Pediatrics, Diagnosis, Pediatric Neuroradiology, Brain, Pathology-Based Diagnoses, Inherited Metabolic/Degenerative Disorders, Peroxisomal Disorders, Zellweger Syndrome and Peroxisomal Biogenesis Disorders",
"reasons": [
"Linked (Diff Diag of White Matter Injury of Prematurity)"
],
- "snippet": "",
- "linked_info": null,
- "is_cached": false
+ "snippet": "\n## KEY FACTS\n\n- ### Terminology\n \n \n - Disorders of peroxisome biosynthesis, assembly, and biochemical functions\n - Varying phenotypes reflect degree of dysfunction\n - Severe: Zellweger syndrome (ZS)\n - Moderate: Neonatal adrenoleukodystrophy (NALD)\n - Mild: Infan...",
+ "linked_info": {
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+ "differential": {
+ "exists": true,
+ "links": [
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+ "title": "Congenital CMV",
+ "docid": "ad51c41b-8389-4adb-b4b0-f1d35a380338"
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+ }
+ },
+ "is_cached": true
+ },
+ {
+ "path": "docs_md/articles/aneurysmal-subarachnoid-hemorrhage_9109b698-5ee5-49c4-ba0c-1a86f1fbede4.md",
+ "title": "Aneurysmal Subarachnoid Hemorrhage",
+ "docid": "9109b698-5ee5-49c4-ba0c-1a86f1fbede4",
+ "breadcrumbs": [
+ "Brain",
+ "Diagnosis",
+ "Pathology-Based Diagnoses",
+ "Subarachnoid Hemorrhage and Aneurysms",
+ "Subarachnoid Hemorrhage",
+ "Aneurysmal Subarachnoid Hemorrhage"
+ ],
+ "authors": [
+ {
+ "key": "8d5254e9-8dda-478b-8f08-bdee97a32c79",
+ "value": "Karen L. Salzman, MD, FACR"
+ },
+ {
+ "key": "5cff4116-3654-4b3a-bb75-5ebe0b8c9850",
+ "value": "Anne G. Osborn, MD, FACR"
+ }
+ ],
+ "pageKeywords": "Brain, Diagnosis, Pathology-Based Diagnoses, Subarachnoid Hemorrhage and Aneurysms, Subarachnoid Hemorrhage, Aneurysmal Subarachnoid Hemorrhage",
+ "reasons": [
+ "Linked (Diff Diag of Reversible Cerebral Vasoconstriction Syndrome)"
+ ],
+ "snippet": "\n## KEY FACTS\n\n- ### Terminology\n \n \n - SAH caused by ruptured aneurysm (aSAH)\n - Saccular (SA) > > dissecting aneurysm (DA)\n- ### Imaging\n \n \n - CT/CTA\n - Hyperdense cisterns/sulci on NECT\n - Within 6 hours after ictus, sensitivity ~ 100%\n - Dis...",
+ "linked_info": {
+ "anatomy": {
+ "exists": false,
+ "links": []
+ },
+ "differential": {
+ "exists": true,
+ "links": [
+ {
+ "title": "Perimesencephalic SAH",
+ "docid": "09db329f-e08a-4075-9a94-4fcc9dfee765"
+ },
+ {
+ "title": "Traumatic SAH",
+ "docid": "3697dced-cf23-47db-bdbd-0b941d63cf42"
+ },
+ {
+ "title": "Reversible Cerebral Vasoconstriction Syndrome",
+ "docid": "c3a19be2-1f91-4dcb-b63a-cbfdaeaca5c1"
+ }
+ ]
+ }
+ },
+ "is_cached": true
+ },
+ {
+ "path": "docs_md/articles/vertebrobasilar-system_0ff112d1-0e58-4694-9b49-0a0327ea1310.md",
+ "title": "Vertebrobasilar System",
+ "docid": "0ff112d1-0e58-4694-9b49-0a0327ea1310",
+ "breadcrumbs": [
+ "Brain",
+ "Anatomy",
+ "Intracranial Arteries",
+ "Vertebrobasilar System"
+ ],
+ "authors": [
+ {
+ "key": "5cff4116-3654-4b3a-bb75-5ebe0b8c9850",
+ "value": "Anne G. Osborn, MD, FACR"
+ },
+ {
+ "key": "095c34cb-da44-4830-98c9-7e1a24bdda5b",
+ "value": "Edward P. Quigley, III, MD, PhD"
+ },
+ {
+ "key": "afaec833-e997-49c4-8eab-e28310c6c2bf",
+ "value": "Adriene C. Eastaway, MD, MS"
+ }
+ ],
+ "pageKeywords": "Brain, Anatomy, Intracranial Arteries, Vertebrobasilar System",
+ "reasons": [
+ "Linked (Anatomy of Intracranial Atherosclerosis)"
+ ],
+ "snippet": "\n## TERMINOLOGY\n\n- ### Abbreviations\n \n \n - Vertebrobasilar (VB), vertebral artery (VA), basilar artery (BA)\n - Superior cerebellar arteries (SCAs), posterior inferior cerebellar artery (PICA), anterior inferior cerebellar artery (AICA)\n - Internal carotid artery (ICA)\n - Anterior spin...",
+ "linked_info": {
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"path": "docs_md/articles/adem_7066708a-9349-472e-b210-849cca6426d7.md",
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"docid": "766451da-d8ce-4ee6-8cb2-cb38609a1dae"
},
{
- "title": "Differential Diagnosis",
+ "title": "Enhancing Cranial Nerve(s)",
"docid": "6471fb1c-d46d-47cd-8322-1671e82335c3"
},
{
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"is_cached": true
},
- {
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{
"path": "docs_md/articles/deep-cerebral-veins_e426b06e-4b91-48ef-956b-78fb179b1de9.md",
"title": "Deep Cerebral Veins",
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"is_cached": true
},
{
- "path": "missing_c5ef0315-edbd-4ace-8930-484a116610b9",
- "title": "Intracranial Atherosclerotic Vascular Disease",
- "docid": "c5ef0315-edbd-4ace-8930-484a116610b9",
+ "path": "docs_md/articles/sensorimotor-network_e1563250-4c6c-4730-bbad-4430ae16a5c6.md",
+ "title": "Sensorimotor Network",
+ "docid": "e1563250-4c6c-4730-bbad-4430ae16a5c6",
+ "breadcrumbs": [
+ "Brain",
+ "Anatomy",
+ "Functional MRI",
+ "Functional Network Anatomy",
+ "Sensorimotor Network"
+ ],
+ "authors": [
+ {
+ "key": "15a1b74a-5576-4e3a-a193-2d84e315fbd0",
+ "value": "Jeffrey S. Anderson, MD, PhD"
+ }
+ ],
+ "pageKeywords": "Brain, Anatomy, Functional MRI, Functional Network Anatomy, Sensorimotor Network",
+ "reasons": [
+ "Linked (Anatomy of Spontaneous Nontraumatic Intracranial Hemorrhage)"
+ ],
+ "snippet": "\n## IMAGING ANATOMY\n\n- ### Overview\n \n \n - Includes primary somatosensory and primary motor cortex, motor and sensory association cortex, subcortical and cerebellar motor regions, and primary auditory cortex\n - Map of sensorimotor homunculus extends along the central sulcus from facial regio...",
+ "linked_info": {
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+ "links": []
+ },
+ "differential": {
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+ "links": []
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+ "is_cached": true
+ },
+ {
+ "path": "docs_md/articles/memory-overview_40e2e25f-421b-4653-94e3-641b09b47e4d.md",
+ "title": "Memory Overview",
+ "docid": "40e2e25f-421b-4653-94e3-641b09b47e4d",
+ "breadcrumbs": [
+ "Brain",
+ "Anatomy",
+ "Brain Network Anatomy",
+ "Memory Overview"
+ ],
+ "authors": [
+ {
+ "key": "15a1b74a-5576-4e3a-a193-2d84e315fbd0",
+ "value": "Jeffrey S. Anderson, MD, PhD"
+ }
+ ],
+ "pageKeywords": "Brain, Anatomy, Brain Network Anatomy, Memory Overview",
+ "reasons": [
+ "Linked (Anatomy of Spontaneous Nontraumatic Intracranial Hemorrhage)"
+ ],
+ "snippet": "\n## TERMINOLOGY\n\n- ### Definitions\n \n \n - Procedural memory: Long-term memory that does not require conscious recollection such as motor skills, also called implicit memory\n - Declarative memory: Long-term memory that requires conscious recollection, can be episodic or semantic, also called ...",
+ "linked_info": {
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+ },
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+ }
+ },
+ "is_cached": true
+ },
+ {
+ "path": "missing_84792183-949d-4b25-a586-572dbe2e11a2",
+ "title": "Vascular Malformation",
+ "docid": "84792183-949d-4b25-a586-572dbe2e11a2",
"breadcrumbs": [],
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"reasons": [
- "Linked (Diff Diag of Miscellaneous Vasculitis)"
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"snippet": "",
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},
{
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- "title": "Arterial Vasospasm",
- "docid": "397752a7-6998-4375-8090-e097d775d180",
+ "path": "missing_dd83ba51-7fd9-406f-9950-9a944c02c291",
+ "title": "Dural Arteriovenous Fistula",
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"authors": [],
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"reasons": [
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{
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- "title": "Temporal relationship to SAH",
- "docid": "9109b698-5ee5-49c4-ba0c-1a86f1fbede4",
+ "path": "missing_1df27abc-b596-45bc-aba5-441253f39327",
+ "title": "Ruptured Pseudoaneurysm",
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"reasons": [
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- "is_cached": false
- },
- {
- "path": "missing_ac1add00-0c2a-4818-8c79-310fa74e2331",
- "title": "Cerebral contusion",
+ "path": "docs_md/articles/cerebral-contusion_ac1add00-0c2a-4818-8c79-310fa74e2331.md",
+ "title": "Cerebral Contusion",
"docid": "ac1add00-0c2a-4818-8c79-310fa74e2331",
- "breadcrumbs": [],
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- "pageKeywords": [],
+ "breadcrumbs": [
+ "Brain",
+ "Diagnosis",
+ "Pathology-Based Diagnoses",
+ "Trauma",
+ "Primary Effects of CNS Trauma",
+ "Cerebral Contusion"
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+ "authors": [
+ {
+ "key": "cdbaaa96-a7b4-4498-a355-a2823a7d9e26",
+ "value": "Kelly A. Dahlstrom, DO"
+ },
+ {
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+ "value": "Anne G. Osborn, MD, FACR"
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+ "pageKeywords": "Brain, Diagnosis, Pathology-Based Diagnoses, Trauma, Primary Effects of CNS Trauma, Cerebral Contusion",
"reasons": [
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+ "snippet": "\n## KEY FACTS\n\n- ### Terminology\n \n \n - Brain surface injuries involving gray matter and contiguous subcortical white matter\n - Coup: Direct injury to brain **beneath** impact site\n - Contrecoup: Injury **opposite** impact site (often more severe than coup)\n- ### Imaging\n \n ...",
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+ "snippet": "\n## TERMINOLOGY\n\n- ### Abbreviations\n \n \n - Anterior, central, posterior skull base (ASB, CSB, PSB)\n - Greater, lesser wings of sphenoid (GWS, LWS)\n- ### Definitions\n \n \n - CSB: Skull base posterior to LWS/planum sphenoidale & anterior to petrous ridge/dorsum sella\n\n## IMAGING ANATOMY\n\n...",
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+ "snippet": "\n## KEY FACTS\n\n- ### Terminology\n \n \n - Brain displaced from one compartment into another\n- ### Imaging\n \n \n - **Subfalcine herniation (SFH)**\n - **Cingulate** **gyrus** displaced under falx\n - Ipsilateral ventricle compressed, contralateral dilated\n - **Unilateral des...",
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{
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+ "snippet": "\n## KEY FACTS\n\n- ### Terminology\n \n \n - Asymmetric, large jugular bulb (JB) flow phenomenon simulates neoplasm or thrombosis on MR sequences\n- ### Imaging\n \n \n - Best diagnostic clue: Complex MR signal in JB with normal jugular foramen (JF) cortex & jugular spine\n - Complex MR sig...",
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"title": "Dural Arteriovenous Fistula",
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+ "value": "Ram Vaidhyanath, DMRD, DNB, FRCR"
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],
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+ "snippet": "\n## KEY FACTS\n\n- ### Terminology\n \n \n - Dural arteriovenous fistula (DAVF)\n - Acquired direct shunt between dural artery and dural venous sinus or cortical vein\n- ### Imaging\n \n \n - Best imaging modality: DSA\n - Most common site: Transverse sinus (TS)\n - CECT findings in DAVF\n ...",
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"is_cached": false
},
{
- "path": "missing_563bc74a-c052-4fdb-a5f2-5a318578bb62",
- "title": "Differential Diagnosis",
+ "path": "docs_md/articles/intrinsic-skull-base-lesion_563bc74a-c052-4fdb-a5f2-5a318578bb62.md",
+ "title": "Intrinsic Skull Base Lesion",
"docid": "563bc74a-c052-4fdb-a5f2-5a318578bb62",
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"breadcrumbs": [
- "Brain",
- "Anatomy",
- "Brain Network Anatomy",
- "Memory Overview"
+ "Head and Neck",
+ "Differential Diagnosis",
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+ "Anatomically Based Differentials",
+ "Intrinsic Skull Base Lesion"
],
"authors": [
{
- "key": "15a1b74a-5576-4e3a-a193-2d84e315fbd0",
- "value": "Jeffrey S. Anderson, MD, PhD"
+ "key": "99f10fe6-7f91-4026-bc60-d769c5d7c7c4",
+ "value": "C. Douglas Phillips, MD, FACR"
}
],
- "pageKeywords": "Brain, Anatomy, Brain Network Anatomy, Memory Overview",
+ "pageKeywords": "Head and Neck, Differential Diagnosis, Skull Base, Anatomically Based Differentials, Intrinsic Skull Base Lesion",
"reasons": [
- "Linked (Anatomy of Adult Hypoxic-Ischemic Injury)"
+ "Linked (Diff Diag of Dural Sinus and Aberrant Arachnoid Granulations)"
],
- "snippet": "\n## TERMINOLOGY\n\n- ### Definitions\n \n \n - Procedural memory: Long-term memory that does not require conscious recollection such as motor skills, also called implicit memory\n - Declarative memory: Long-term memory that requires conscious recollection, can be episodic or semantic, also called ...",
+ "snippet": "\n## ESSENTIAL INFORMATION\n\n- ### Key Differential Diagnosis Issues\n \n \n - Lesion may be focal, diffuse, localized, or part of systemic disease\n - Variable presentation of skull base lesion\n - Headache, cranial neuropathy\n - On occasion, may be incidental imaging finding\n ...",
"linked_info": {
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},
{
- "path": "missing_21a74d44-6e0c-40c6-b865-a4267453d629",
- "title": "Traumatic Cerebral Edema/Ischemia",
+ "path": "docs_md/articles/lyme-disease_082aad00-d3cf-4b75-a9aa-5ed2c724959c.md",
+ "title": "Lyme Disease",
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+ "breadcrumbs": [
+ "Brain",
+ "Diagnosis",
+ "Pathology-Based Diagnoses",
+ "Infectious, Inflammatory, and Demyelinating Diseases",
+ "Acquired Infections",
+ "Lyme Disease"
+ ],
+ "authors": [
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+ "key": "a25c450b-3d34-4f64-bba3-cc0834813df6",
+ "value": "Miral D. Jhaveri, MD, MBA"
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+ "pageKeywords": "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Diseases, Acquired Infections, Lyme Disease",
+ "reasons": [
+ "Linked (Diff Diag of Systemic Lupus Erythematosus)"
+ ],
+ "snippet": "\n## KEY FACTS\n\n- ### Terminology\n \n \n - Lyme disease (LD), Lyme neuroborreliosis (LNB)\n - Multisystem, multistage inflammatory disease\n - Caused by *Borrelia burgdorferi*(USA)\n - Transmitted by *Ixodes* tick bite\n - Reservoirs: White-footed mice/deer\n- ### Imaging\n...",
+ "linked_info": {
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+ "links": []
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+ "differential": {
+ "exists": true,
+ "links": [
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+ "title": "Multiple Sclerosis",
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"docid": "21a74d44-6e0c-40c6-b865-a4267453d629",
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+ "key": "5cff4116-3654-4b3a-bb75-5ebe0b8c9850",
+ "value": "Anne G. Osborn, MD, FACR"
+ }
+ ],
+ "pageKeywords": "Brain, Diagnosis, Pathology-Based Diagnoses, Trauma, Secondary Effects of CNS Trauma, Posttraumatic Brain Swelling",
"reasons": [
"Linked (Diff Diag of Adult Hypoxic-Ischemic Injury)"
],
- "snippet": "",
- "linked_info": null,
- "is_cached": false
+ "snippet": "\n## KEY FACTS\n\n- ### Terminology\n \n \n - Vasogenic edema (VE), cytotoxic edema (CTE), cerebral edema (CE), diffuse brain swelling (DBS)\n - 2 forms of brain edema: VE and CTE (often coexist)\n - VE: Extracellular edema, follows blood-brain barrier (BBB) breakdown\n - CTE: Intra...",
+ "linked_info": {
+ "anatomy": {
+ "exists": false,
+ "links": []
+ },
+ "differential": {
+ "exists": true,
+ "links": [
+ {
+ "title": "Carbon Monoxide Poisoning",
+ "docid": "827ae14b-3d0f-4c3a-937a-e450a7eec716"
+ },
+ {
+ "title": "Posterior Reversible Encephalopathy Syndrome (PRES)",
+ "docid": "84176f2c-fc9d-4497-8af9-1430b9f0187c"
+ },
+ {
+ "title": "Uremia, mitochondrial disorders",
+ "docid": "dcbc692c-e682-4992-94b6-524c6fe5a1b5"
+ },
+ {
+ "title": "Meningitis/Encephalitis",
+ "docid": "a08411a0-492d-4fa1-a504-dd97cce22a0e"
+ }
+ ]
+ }
+ },
+ "is_cached": true
},
{
- "path": "missing_827ae14b-3d0f-4c3a-937a-e450a7eec716",
- "title": "Toxic/Metabolic Disorder",
+ "path": "docs_md/articles/carbon-monoxide-poisoning_827ae14b-3d0f-4c3a-937a-e450a7eec716.md",
+ "title": "Carbon Monoxide Poisoning",
"docid": "827ae14b-3d0f-4c3a-937a-e450a7eec716",
- "breadcrumbs": [],
- "authors": [],
- "pageKeywords": [],
+ "breadcrumbs": [
+ "Brain",
+ "Diagnosis",
+ "Pathology-Based Diagnoses",
+ "Acquired Toxic/Metabolic/Degenerative Disorders",
+ "Toxic, Metabolic, Nutritional, Systemic Diseases With CNS Manifestations",
+ "Carbon Monoxide Poisoning"
+ ],
+ "authors": [
+ {
+ "key": "a25c450b-3d34-4f64-bba3-cc0834813df6",
+ "value": "Miral D. Jhaveri, MD, MBA"
+ }
+ ],
+ "pageKeywords": "Brain, Diagnosis, Pathology-Based Diagnoses, Acquired Toxic/Metabolic/Degenerative Disorders, Toxic, Metabolic, Nutritional, Systemic Diseases With CNS Manifestations, Carbon Monoxide Poisoning",
"reasons": [
"Linked (Diff Diag of Adult Hypoxic-Ischemic Injury)"
],
- "snippet": "",
- "linked_info": null,
- "is_cached": false
+ "snippet": "\n## KEY FACTS\n\n- ### Terminology\n \n \n - Anoxic-ischemic encephalopathy, usually with bilateral lesions, caused by inhalation of carbon monoxide (CO) gas\n- ### Imaging\n \n \n - Best diagnostic clue: Globi pallidi (GP) T2/FLAIR hyperintensity\n - T1 MR: Both hypointensity in GP (likely necro...",
+ "linked_info": {
+ "anatomy": {
+ "exists": false,
+ "links": []
+ },
+ "differential": {
+ "exists": true,
+ "links": [
+ {
+ "title": "Adult Hypoxic-Ischemic Injury",
+ "docid": "91ac293f-161c-4b3b-81e5-740f831eaa5d"
+ },
+ {
+ "title": "Drug Abuse",
+ "docid": "48859403-0b26-44d8-ba74-e0919e4c3147"
+ },
+ {
+ "title": "Wilson Disease",
+ "docid": "b89eef10-ea47-4ca9-a3b7-b8aeeca86802"
+ },
+ {
+ "title": "Japanese Encephalitis",
+ "docid": "7e3dd11d-21c7-468a-a227-b363d595bbce"
+ },
+ {
+ "title": "Arteriolosclerosis",
+ "docid": "07e561a5-0554-4867-b811-448c36890ee3"
+ },
+ {
+ "title": "Creutzfeldt-Jakob Disease (CJD)",
+ "docid": "30a88a01-b24d-476d-a933-48aabcdb6f95"
+ }
+ ]
+ }
+ },
+ "is_cached": true
},
{
- "path": "missing_30a88a01-b24d-476d-a933-48aabcdb6f95",
- "title": "Creutzfeldt-Jakob Disease",
+ "path": "docs_md/articles/creutzfeldt-jakob-disease-cjd_30a88a01-b24d-476d-a933-48aabcdb6f95.md",
+ "title": "Creutzfeldt-Jakob Disease (CJD)",
"docid": "30a88a01-b24d-476d-a933-48aabcdb6f95",
- "breadcrumbs": [],
- "authors": [],
- "pageKeywords": [],
+ "breadcrumbs": [
+ "Brain",
+ "Diagnosis",
+ "Pathology-Based Diagnoses",
+ "Acquired Toxic/Metabolic/Degenerative Disorders",
+ "Dementias and Degenerative Disorders",
+ "Creutzfeldt-Jakob Disease (CJD)"
+ ],
+ "authors": [
+ {
+ "key": "1fa14dfd-71ea-4960-908e-e720313bc63a",
+ "value": "Santhosh Gaddikeri, MD"
+ },
+ {
+ "key": "a25c450b-3d34-4f64-bba3-cc0834813df6",
+ "value": "Miral D. Jhaveri, MD, MBA"
+ }
+ ],
+ "pageKeywords": "Brain, Diagnosis, Pathology-Based Diagnoses, Acquired Toxic/Metabolic/Degenerative Disorders, Dementias and Degenerative Disorders, Creutzfeldt-Jakob Disease (CJD)",
"reasons": [
"Linked (Diff Diag of Adult Hypoxic-Ischemic Injury)"
],
- "snippet": "",
- "linked_info": null,
- "is_cached": false
+ "snippet": "\n## KEY FACTS\n\n- ### Terminology\n \n \n - Creutzfeldt-Jakob disease (CJD): Rapidly progressing, fatal, potentially transmissible dementia caused by prion\n- ### Imaging\n \n \n - Best imaging clue: Progressive DWI/FLAIR hyperintensity of basal ganglia (BG), thalamus, and cerebral cortex\n - Pr...",
+ "linked_info": {
+ "anatomy": {
+ "exists": true,
+ "links": [
+ {
+ "title": "Default Mode Network",
+ "docid": "a29f7551-d39d-4deb-933e-b8d2816168c3"
+ },
+ {
+ "title": "Basal Ganglia",
+ "docid": "a9de3815-ec59-4c78-adf0-94974065a7e3"
+ },
+ {
+ "title": "Thalamus",
+ "docid": "b7f0cd6b-4ba2-4ed4-8dd0-5ca56d9ae92b"
+ },
+ {
+ "title": "Language Overview",
+ "docid": "40f2ed79-0d31-4943-aaa2-7c3244a7e87b"
+ },
+ {
+ "title": "Functional Network Overview",
+ "docid": "ef0be4c8-3d36-4ca9-b4c5-f22f66d2b367"
+ },
+ {
+ "title": "Attention Control Network",
+ "docid": "a1bedda5-6478-40b2-98e7-6c5f5363b06f"
+ },
+ {
+ "title": "Visual Network",
+ "docid": "404625d9-3125-4923-9f9d-53d0f81c3542"
+ },
+ {
+ "title": "Limbic Network",
+ "docid": "e1a20b61-b2c1-44c5-ba04-59843855bfef"
+ },
+ {
+ "title": "Social Brain Anatomy",
+ "docid": "0352d34a-5966-494e-b9c3-c26bde257bca"
+ },
+ {
+ "title": "Gyral/Sulcal Anatomy",
+ "docid": "849da2a0-4a32-4a07-8f00-c69291e59434"
+ },
+ {
+ "title": "Gyral/Sulcal Anatomy",
+ "docid": "299a5990-1805-4018-85b5-191d8416385b"
+ },
+ {
+ "title": "Functional Network Overview",
+ "docid": "7b97f239-0f6f-4809-ac44-594cdf4842d5"
+ },
+ {
+ "title": "Brain",
+ "docid": "080771c2-02f3-408d-ad70-04a80d849500"
+ }
+ ]
+ },
+ "differential": {
+ "exists": true,
+ "links": [
+ {
+ "title": "Adult Hypoxic-Ischemic Injury",
+ "docid": "91ac293f-161c-4b3b-81e5-740f831eaa5d"
+ },
+ {
+ "title": "Osmotic Demyelination Syndrome",
+ "docid": "2f646ac5-9994-4bc0-a7ff-f0103334a366"
+ },
+ {
+ "title": "Other Causes of Dementia",
+ "docid": "aa75d198-88b8-45c2-8a35-750631009166"
+ },
+ {
+ "title": "Alzheimer disease",
+ "docid": "aa75d198-88b8-45c2-8a35-750631009166"
+ },
+ {
+ "title": "Dementia in motor neuron disease",
+ "docid": "cef8f538-52a0-4590-b14d-3578e9214330"
+ },
+ {
+ "title": "Frontotemporal dementia",
+ "docid": "596be24d-885f-40ed-832e-2de041f974f5"
+ },
+ {
+ "title": "Vascular Dementia",
+ "docid": "20864adf-5e46-4fbf-ba27-e8e30fa6506f"
+ },
+ {
+ "title": "Corticobasal Degeneration",
+ "docid": "99473ccd-173f-4ed5-9b92-e52d5630a8a9"
+ },
+ {
+ "title": "Wilson Disease",
+ "docid": "b89eef10-ea47-4ca9-a3b7-b8aeeca86802"
+ },
+ {
+ "title": "Arteriolosclerosis",
+ "docid": "07e561a5-0554-4867-b811-448c36890ee3"
+ },
+ {
+ "title": "Homonymous Hemianopsia",
+ "docid": "1cdca8f4-95f8-4d19-b28a-19a433d1a624"
+ }
+ ]
+ }
+ },
+ "is_cached": true
},
{
"path": "docs_md/articles/porencephalic-cyst_132b5c61-f762-46ad-853c-615f4117d010.md",
@@ -6165,27 +8083,14 @@
"is_cached": true
},
{
- "path": "missing_c75baee2-7a4c-4fd8-9b3f-cc662d0f1c18",
- "title": "Differential Diagnosis",
- "docid": "c75baee2-7a4c-4fd8-9b3f-cc662d0f1c18",
- "breadcrumbs": [],
- "authors": [],
- "pageKeywords": [],
- "reasons": [
- "Linked (Diff Diag of Chronic Cerebral Infarction)"
- ],
- "snippet": "",
- "linked_info": null,
- "is_cached": false
- },
- {
- "path": "docs_md/articles/abnormal-shape-configuration-of-corpus-callosum_238ca32d-6bc6-4f5a-81b1-6601dd605856.md",
+ "path": "docs_md/articles/abnormal-shape-configuration-of-corpus-callosum_c75baee2-7a4c-4fd8-9b3f-cc662d0f1c18.md",
"title": "Abnormal Shape/Configuration of Corpus Callosum",
- "docid": "238ca32d-6bc6-4f5a-81b1-6601dd605856",
+ "docid": "c75baee2-7a4c-4fd8-9b3f-cc662d0f1c18",
"breadcrumbs": [
- "Pediatrics",
- "Differential Diagnosis",
"Brain",
+ "Differential Diagnosis",
+ "Supratentorial Brain Parenchyma",
+ "Anatomically Based Differentials",
"Abnormal Shape/Configuration of Corpus Callosum"
],
"authors": [
@@ -6194,11 +8099,11 @@
"value": "Luke L. Linscott, MD"
}
],
- "pageKeywords": "Pediatrics, Differential Diagnosis, Brain, Abnormal Shape/Configuration of Corpus Callosum",
+ "pageKeywords": "Brain, Differential Diagnosis, Supratentorial Brain Parenchyma, Anatomically Based Differentials, Abnormal Shape/Configuration of Corpus Callosum",
"reasons": [
"Linked (Diff Diag of Chronic Cerebral Infarction)"
],
- "snippet": "\n# ESSENTIAL INFORMATION\n\n- ## Key Differential Diagnosis Issues\n \n \n - Clinical features to consider\n - Normal corpus callosum (CC) varies in thickness & shape\n - Associated anomalies portend worse prognosis\n - If not congenital anomaly, clinical history is crucial\n ...",
+ "snippet": "\n## ESSENTIAL INFORMATION\n\n- ### Key Differential Diagnosis Issues\n \n \n - Clinical features to consider\n - Normal corpus callosum (CC) varies in thickness & shape\n - Associated anomalies portend worse prognosis\n - If not congenital anomaly, clinical history is crucial...",
"linked_info": {
"anatomy": {
"exists": false,
diff --git a/scrapers/cache_server.py b/scrapers/cache_server.py
new file mode 100644
index 0000000..125cd22
--- /dev/null
+++ b/scrapers/cache_server.py
@@ -0,0 +1,190 @@
+#!/usr/bin/env python3
+import os
+import json
+import base64
+import hashlib
+from pathlib import Path
+from typing import Dict, Optional
+from fastapi import FastAPI, HTTPException
+from fastapi.middleware.cors import CORSMiddleware
+from pydantic import BaseModel
+
+# Import exact helpers from capture_passive_playwright_async
+from scrapers.capture_passive_playwright_async import (
+ sanitize,
+ guess_ext,
+ now_ts,
+ try_pretty_json,
+ save_binary,
+ save_text
+)
+
+app = FastAPI(title="STATdx Central Cache Server")
+
+# Allow CORS for options requests and standard requests
+app.add_middleware(
+ CORSMiddleware,
+ allow_origins=["*"],
+ allow_credentials=True,
+ allow_methods=["*"],
+ allow_headers=["*"],
+)
+
+OUTPUT_DIR = Path("xhr_captured_async")
+OUTPUT_DIR.mkdir(parents=True, exist_ok=True)
+INDEX_PATH = OUTPUT_DIR / "capture_index.jsonl"
+
+# Persistent in-memory index
+in_memory_index: Dict[str, list] = {}
+
+def load_existing_index():
+ if INDEX_PATH.exists():
+ print(f"Loading existing index from {INDEX_PATH}...")
+ try:
+ with open(INDEX_PATH, "r", encoding="utf-8") as f:
+ for line in f:
+ line = line.strip()
+ if not line:
+ continue
+ try:
+ entry = json.loads(line)
+ ch = entry.get("content_hash")
+ if ch:
+ in_memory_index.setdefault(ch, []).append(entry)
+ except Exception:
+ pass
+ print(f"Loaded {len(in_memory_index)} unique hashes into memory.")
+ except Exception as e:
+ print(f"Error loading index: {e}")
+
+# Load index at import/startup
+load_existing_index()
+
+class HashCheckRequest(BaseModel):
+ content_hash: str
+
+class CapturePayload(BaseModel):
+ url: str
+ resource_type: str
+ status: int
+ timestamp: Optional[str] = None
+ headers: Dict[str, str]
+ body_base64: str
+ content_type: str
+
+@app.post("/api/check-hash")
+async def check_hash(payload: HashCheckRequest):
+ exists = payload.content_hash in in_memory_index
+ return {"exists": exists}
+
+@app.post("/api/capture")
+async def capture(payload: CapturePayload):
+ try:
+ # Decode body
+ body_bytes = base64.b64decode(payload.body_base64)
+ content_hash = hashlib.sha256(body_bytes).hexdigest()
+
+ # Check deduplication
+ if content_hash in in_memory_index:
+ # We already have this exact content saved
+ # Just return and do not write duplicate files
+ return {"status": "skipped", "reason": "content_hash already cached"}
+
+ url = payload.url
+ ts = payload.timestamp or now_ts()
+ safe = sanitize(url.replace('https://', '').replace('http://', ''))
+ h = hashlib.sha1((url + ts).encode('utf-8')).hexdigest()[:8]
+
+ body_path = None
+ excerpt = None
+ ctype = payload.content_type.lower()
+ rtype = payload.resource_type.lower()
+
+ # Handle text content
+ if 'application/json' in ctype or ctype.startswith('text/') or 'javascript' in ctype:
+ try:
+ txt = body_bytes.decode('utf-8')
+ except Exception:
+ txt = body_bytes.decode('latin-1', errors='replace')
+
+ pretty = try_pretty_json(txt)
+ if pretty is not None:
+ use_text = pretty
+ excerpt = pretty[:800]
+ ext = 'json'
+ else:
+ use_text = txt
+ excerpt = txt[:800]
+ ext = 'txt'
+
+ content_sha1 = hashlib.sha1(use_text.encode('utf-8')).hexdigest()
+ fname = f"{safe}_{content_sha1}.{ext}"
+ body_path = str(OUTPUT_DIR / fname)
+
+ if not os.path.exists(body_path):
+ save_text(OUTPUT_DIR / fname, use_text)
+ else:
+ # Handle binary content
+ ext = guess_ext(ctype)
+ content_sha1 = hashlib.sha1(body_bytes).hexdigest()
+ fname = f"{safe}_{content_sha1}.{ext}"
+
+ if ctype.startswith('image/'):
+ subdir = OUTPUT_DIR / 'images'
+ subdir.mkdir(parents=True, exist_ok=True)
+ body_path = str(subdir / fname)
+ if not os.path.exists(body_path):
+ save_binary(subdir / fname, body_bytes)
+ else:
+ subdir = OUTPUT_DIR / 'assets'
+ subdir.mkdir(parents=True, exist_ok=True)
+ body_path = str(subdir / fname)
+ if not os.path.exists(body_path):
+ save_binary(subdir / fname, body_bytes)
+
+ # Meta file creation
+ meta_base = content_hash or h
+ new_meta_name = f"{safe}_{meta_base}.meta.json"
+ new_meta_path = OUTPUT_DIR / new_meta_name
+
+ meta = {
+ 'url': url,
+ 'resource_type': rtype,
+ 'status': payload.status,
+ 'timestamp': ts,
+ 'response_headers': payload.headers,
+ 'response_body_file': body_path,
+ 'response_excerpt': excerpt,
+ 'content_hash': content_hash,
+ }
+
+ save_text(new_meta_path, json.dumps(meta, ensure_ascii=False, indent=2))
+
+ # Write to log index
+ entry = {
+ 'url': url,
+ 'resource_type': rtype,
+ 'timestamp': ts,
+ 'body_file': body_path,
+ 'meta_file': str(new_meta_path),
+ 'excerpt': excerpt,
+ 'content_hash': content_hash
+ }
+
+ with open(INDEX_PATH, 'a', encoding='utf-8') as idx:
+ idx.write(json.dumps(entry, ensure_ascii=False) + '\n')
+
+ # Update in-memory index
+ in_memory_index.setdefault(content_hash, []).append(entry)
+
+ print(f"SAVED: {url} -> {body_path}")
+ return {"status": "saved", "path": body_path}
+
+ except Exception as e:
+ import traceback
+ traceback.print_exc()
+ raise HTTPException(status_code=500, detail=str(e))
+
+if __name__ == "__main__":
+ import uvicorn
+ uvicorn.run("scrapers.cache_server:app", host="0.0.0.0", port=8000, reload=True)
diff --git a/scrapers/statdx_passive_capture.user.js b/scrapers/statdx_passive_capture.user.js
new file mode 100644
index 0000000..b058ee0
--- /dev/null
+++ b/scrapers/statdx_passive_capture.user.js
@@ -0,0 +1,375 @@
+// ==UserScript==
+// @name STATdx Passive Capture Link
+// @namespace http://tampermonkey.net/
+// @version 1.1
+// @description Captures STATdx content (JSON/HTML/images) passively and sends it to the central cache server.
+// @author Antigravity
+// @match https://app.statdx.com/*
+// @grant GM_xmlhttpRequest
+// @connect localhost
+// @connect *
+// @run-at document-start
+// ==/UserScript==
+
+(function() {
+ 'use strict';
+
+ // CHANGE THIS URL to the IP address or hostname of your central cache server if running on remote machines
+ // e.g., "http://192.168.1.50:8000"
+ const CACHE_SERVER_URL = "http://localhost:8000";
+
+ const capturedUrls = new Set();
+
+ function shouldCapture(url, contentType) {
+ try {
+ if (!url) return false;
+ const lowerUrl = url.toLowerCase();
+ const lowerContentType = (contentType || "").toLowerCase();
+
+ // Always capture known document content / summary endpoints
+ if (lowerUrl.includes('/document/content/') || lowerUrl.includes('/document/summary/')) {
+ return true;
+ }
+
+ // Check if contentType indicates target resource types
+ if (lowerContentType.includes('application/json') ||
+ lowerContentType.startsWith('text/') ||
+ lowerContentType.includes('javascript') ||
+ lowerContentType.startsWith('image/')) {
+ return true;
+ }
+ } catch (e) {}
+ return false;
+ }
+
+ // Helper: Compute SHA-256 hash of an ArrayBuffer using Web Crypto API
+ async function getSha256(buffer) {
+ const hashBuffer = await crypto.subtle.digest('SHA-256', buffer);
+ const hashArray = Array.from(new Uint8Array(hashBuffer));
+ return hashArray.map(b => b.toString(16).padStart(2, '0')).join('');
+ }
+
+ // Helper: Convert ArrayBuffer to Base64 string
+ function arrayBufferToBase64(buffer) {
+ let binary = '';
+ const bytes = new Uint8Array(buffer);
+ const len = bytes.byteLength;
+ for (let i = 0; i < len; i++) {
+ binary += String.fromCharCode(bytes[i]);
+ }
+ return window.btoa(binary);
+ }
+
+ // Helper: Parse response headers string to dict
+ function parseHeaders(headersString) {
+ const headers = {};
+ try {
+ if (!headersString) return headers;
+ const lines = headersString.split('\r\n');
+ lines.forEach(line => {
+ const parts = line.split(':');
+ if (parts.length >= 2) {
+ const key = parts[0].trim().toLowerCase();
+ const value = parts.slice(1).join(':').trim();
+ headers[key] = value;
+ }
+ });
+ } catch (e) {}
+ return headers;
+ }
+
+ // Helper: Get Headers dict from fetch Headers object
+ function getHeadersDict(headers) {
+ const dict = {};
+ try {
+ if (!headers) return dict;
+ if (typeof headers.forEach === 'function') {
+ headers.forEach((val, key) => {
+ dict[key.toLowerCase()] = val;
+ });
+ } else {
+ for (const [key, val] of Object.entries(headers)) {
+ dict[key.toLowerCase()] = val;
+ }
+ }
+ } catch (e) {}
+ return dict;
+ }
+
+ // Helper: Extract buffer from XHR
+ async function getXhrBuffer(xhr) {
+ try {
+ if (xhr.responseType === 'arraybuffer') {
+ return xhr.response;
+ }
+ if (xhr.responseType === 'blob') {
+ return await xhr.response.arrayBuffer();
+ }
+ if (typeof xhr.response === 'string' || !xhr.responseType || xhr.responseType === 'text') {
+ const text = xhr.responseText || xhr.response;
+ if (text) {
+ return new TextEncoder().encode(text).buffer;
+ }
+ }
+ } catch (e) {}
+ return null;
+ }
+
+ // Server request: check if server already has hash
+ function checkServerHash(contentHash) {
+ return new Promise((resolve) => {
+ try {
+ GM_xmlhttpRequest({
+ method: "POST",
+ url: `${CACHE_SERVER_URL}/api/check-hash`,
+ headers: { "Content-Type": "application/json" },
+ data: JSON.stringify({ content_hash: contentHash }),
+ onload: function(res) {
+ if (res.status === 200) {
+ try {
+ const data = JSON.parse(res.responseText);
+ resolve(data.exists);
+ } catch (e) {
+ resolve(false);
+ }
+ } else {
+ resolve(false);
+ }
+ },
+ onerror: function() {
+ resolve(false);
+ }
+ });
+ } catch (e) {
+ resolve(false);
+ }
+ });
+ }
+
+ // Server request: upload capture payload
+ function sendToServer(payload) {
+ return new Promise((resolve, reject) => {
+ try {
+ GM_xmlhttpRequest({
+ method: "POST",
+ url: `${CACHE_SERVER_URL}/api/capture`,
+ headers: { "Content-Type": "application/json" },
+ data: JSON.stringify(payload),
+ onload: function(res) {
+ if (res.status === 200) {
+ resolve(res.responseText);
+ } else {
+ reject(new Error(`Server error`));
+ }
+ },
+ onerror: function(err) {
+ reject(err);
+ }
+ });
+ } catch (e) {
+ reject(e);
+ }
+ });
+ }
+
+ // Core processing function
+ async function processBuffer(url, resourceType, status, buffer, contentType, headers) {
+ try {
+ if (!shouldCapture(url, contentType)) {
+ return;
+ }
+
+ const contentHash = await getSha256(buffer);
+
+ // Deduplication check: check server cache
+ const exists = await checkServerHash(contentHash);
+ if (exists) {
+ return;
+ }
+
+ // Convert to base64 and send
+ const bodyBase64 = arrayBufferToBase64(buffer);
+ const payload = {
+ url: url,
+ resource_type: resourceType,
+ status: status,
+ timestamp: new Date().toISOString(),
+ headers: headers || {},
+ body_base64: bodyBase64,
+ content_type: contentType
+ };
+
+ await sendToServer(payload);
+ } catch (e) {
+ // Fail completely silently to prevent stack trace leaks
+ }
+ }
+
+ // Helper: Masquerade overridden functions as native code (toString spoofing)
+ function masqueradeAsNative(customFn, originalFn) {
+ try {
+ Object.defineProperty(customFn, 'toString', {
+ value: function() {
+ return `function ${originalFn.name || 'fetch'}() { [native code] }`;
+ },
+ writable: false,
+ enumerable: false,
+ configurable: true
+ });
+ } catch (e) {}
+ }
+
+ // --- INTERCEPT FETCH ---
+ const originalFetch = window.fetch;
+ const myFetch = async function(...args) {
+ const response = await originalFetch.apply(this, args);
+ try {
+ const url = response.url;
+ const contentType = response.headers.get("content-type") || "";
+
+ if (shouldCapture(url, contentType)) {
+ const clone = response.clone();
+ (async () => {
+ try {
+ const buffer = await clone.arrayBuffer();
+ const headers = getHeadersDict(clone.headers);
+ await processBuffer(url, "fetch", clone.status, buffer, contentType, headers);
+ } catch (e) {}
+ })();
+ }
+ } catch (e) {}
+ return response;
+ };
+
+ masqueradeAsNative(myFetch, originalFetch);
+ try {
+ Object.defineProperty(window, 'fetch', {
+ value: myFetch,
+ writable: true,
+ configurable: true,
+ enumerable: true
+ });
+ } catch (e) {
+ window.fetch = myFetch;
+ }
+
+ // --- INTERCEPT XHR ---
+ const originalOpen = XMLHttpRequest.prototype.open;
+ const originalSend = XMLHttpRequest.prototype.send;
+
+ const myOpen = function(method, url) {
+ try {
+ this._url = url;
+ } catch (e) {}
+ return originalOpen.apply(this, arguments);
+ };
+
+ masqueradeAsNative(myOpen, originalOpen);
+ try {
+ Object.defineProperty(XMLHttpRequest.prototype, 'open', {
+ value: myOpen,
+ writable: true,
+ configurable: true,
+ enumerable: true
+ });
+ } catch (e) {
+ XMLHttpRequest.prototype.open = myOpen;
+ }
+
+ const mySend = function() {
+ try {
+ this.addEventListener('load', function() {
+ try {
+ const url = this._url;
+ const contentType = this.getResponseHeader("content-type") || "";
+ if (shouldCapture(url, contentType)) {
+ const xhr = this;
+ (async () => {
+ try {
+ const buffer = await getXhrBuffer(xhr);
+ if (buffer) {
+ const headers = parseHeaders(xhr.getAllResponseHeaders());
+ await processBuffer(url, "xhr", xhr.status, buffer, contentType, headers);
+ }
+ } catch (e) {}
+ })();
+ }
+ } catch (e) {}
+ });
+ } catch (e) {}
+ return originalSend.apply(this, arguments);
+ };
+
+ masqueradeAsNative(mySend, originalSend);
+ try {
+ Object.defineProperty(XMLHttpRequest.prototype, 'send', {
+ value: mySend,
+ writable: true,
+ configurable: true,
+ enumerable: true
+ });
+ } catch (e) {
+ XMLHttpRequest.prototype.send = mySend;
+ }
+
+ // --- INTERCEPT STATIC IMAGES IN THE DOM ---
+ function captureImageByUrl(url) {
+ try {
+ GM_xmlhttpRequest({
+ method: "GET",
+ url: url,
+ responseType: "arraybuffer",
+ onload: async function(details) {
+ try {
+ if (details.status !== 200) return;
+ const buffer = details.response;
+ if (!buffer) return;
+
+ const contentType = details.responseHeaders.match(/content-type:\s*(.*)/i)?.[1] || "image/png";
+ const headers = parseHeaders(details.responseHeaders);
+ await processBuffer(url, "image", details.status, buffer, contentType, headers);
+ } catch (e) {}
+ }
+ });
+ } catch (e) {}
+ }
+
+ function scanImages() {
+ try {
+ const images = document.querySelectorAll('img');
+ images.forEach(img => {
+ const src = img.src;
+ if (!src) return;
+
+ // Focus on STATdx / Elsevier images and skip local or non-related URLs
+ const isTarget = src.includes('statdx') || src.includes('elsevier') || src.startsWith('/');
+ if (isTarget && !capturedUrls.has(src)) {
+ capturedUrls.add(src);
+ captureImageByUrl(src);
+ }
+ });
+ } catch (e) {}
+ }
+
+ // Set up MutationObserver to dynamically capture images added by page scripts
+ try {
+ const observer = new MutationObserver((mutations) => {
+ scanImages();
+ });
+
+ // Wait for DOM to be ready to observe
+ if (document.body) {
+ scanImages();
+ observer.observe(document.body, { childList: true, subtree: true });
+ } else {
+ document.addEventListener('DOMContentLoaded', () => {
+ scanImages();
+ observer.observe(document.body, { childList: true, subtree: true });
+ });
+ }
+ } catch (e) {}
+
+ // Periodic sweep as a backup
+ setInterval(scanImages, 4000);
+
+})();
diff --git a/tools/search_md.py b/tools/search_md.py
index 5266c34..2099623 100644
--- a/tools/search_md.py
+++ b/tools/search_md.py
@@ -693,6 +693,68 @@ def write_output(results: List[Dict[str, Any]], out_path: str, fmt: str = 'json'
w.writerow(['path', 'title', 'docid', 'breadcrumbs', 'authors', 'pageKeywords'])
for r in results:
w.writerow([r['path'], r['title'], r.get('docid') or '', json.dumps(r.get('breadcrumbs') or []), json.dumps(r.get('authors') or []), r.get('pageKeywords') or ''])
+ elif fmt == 'combined_json':
+ combined_docs = []
+ for r in results:
+ path = r.get('path')
+ title = r.get('title', 'Untitled')
+ docid = r.get('docid', '')
+
+ content = ""
+ if path and os.path.exists(path):
+ try:
+ with open(path, 'r', encoding='utf-8') as f:
+ content = f.read()
+ except Exception as e:
+ content = f"Error reading file {path}: {e}"
+ else:
+ content = f"File not found: {path}"
+
+ # Format using XML tags as a clear separator that LLMs understand
+ doc_str = f'\n{content.strip()}\n'
+ combined_docs.append(doc_str)
+
+ combined_document = "\n\n".join(combined_docs)
+
+ output_data = {
+ "combined_document": combined_document,
+ "articles": [
+ {
+ "title": r.get('title'),
+ "docid": r.get('docid'),
+ "path": r.get('path')
+ }
+ for r in results
+ ]
+ }
+
+ with open(out_path, 'w', encoding='utf-8') as f:
+ json.dump(output_data, f, indent=2, ensure_ascii=False)
+ elif fmt == 'combined_md':
+ combined_docs = []
+ for r in results:
+ path = r.get('path')
+ title = r.get('title', 'Untitled')
+ docid = r.get('docid', '')
+
+ content = ""
+ if path and os.path.exists(path):
+ try:
+ with open(path, 'r', encoding='utf-8') as f:
+ content = f.read()
+ except Exception as e:
+ content = f"Error reading file {path}: {e}"
+ else:
+ content = f"File not found: {path}"
+
+ # Format using XML tags as a clear separator that LLMs understand
+ doc_str = f'\n{content.strip()}\n'
+ combined_docs.append(doc_str)
+
+ combined_document = "\n\n".join(combined_docs)
+
+ with open(out_path, 'w', encoding='utf-8') as f:
+ f.write(combined_document)
def main(argv=None):
@@ -701,7 +763,7 @@ def main(argv=None):
p.add_argument('--query', required=True, help='Query. Can be key:value or a raw search string')
p.add_argument('--mode', choices=['exact', 'fuzzy', 'stemming'], default='exact', help='Search matching mode')
p.add_argument('--out', default='results.json', help='Output file')
- p.add_argument('--format', choices=['json','csv'], default='json')
+ p.add_argument('--format', choices=['json', 'csv', 'combined_json', 'combined_md'], default='json')
p.add_argument('--copy-to', help='Optional: copy matched markdown files to this dir')
p.add_argument("--copy-to-clear", default=True, action="store_true", help="Overwrite files when copying to --copy-to")
args = p.parse_args(argv)
diff --git a/tools/search_md_gui.py b/tools/search_md_gui.py
index 4c97341..4c08b22 100644
--- a/tools/search_md_gui.py
+++ b/tools/search_md_gui.py
@@ -365,7 +365,7 @@ def search_page() -> None: # build UI
with ui.row().classes('w-full gap-4 items-start p-4'):
root_input = ui.input(value='docs_md/articles', label='Root directory').classes('w-64').props('outlined dense')
key_select = ui.select(['global', 'breadcrumbs', 'authors', 'pageKeywords', 'category', 'title', 'enhancedTitle', 'type', 'content'], value='global', label='Query key').classes('w-64').props('outlined dense')
- fmt_select = ui.select(['json', 'csv'], value='json', label='Output format').classes('w-40').props('outlined dense')
+ fmt_select = ui.select(['json', 'csv', 'combined_json', 'combined_md'], value='json', label='Output format').classes('w-40').props('outlined dense')
out_input = ui.input(value='results.json', label='Output file (server-side)').classes('w-64').props('outlined dense')
copy_input = ui.input(value='out', label='Copy matched files to (optional)').classes('w-64').props('outlined dense')
copy_clear = ui.checkbox('Clear destination before copy', value=False).classes('self-center text-slate-300')
@@ -379,6 +379,8 @@ def search_page() -> None: # build UI
with action_bar:
with ui.row().classes('gap-2'):
ui.button('Copy Selected Files', icon='content_copy', on_click=lambda: asyncio.create_task(copy_selected())).classes('bg-indigo-600 hover:bg-indigo-700 text-white font-medium px-4')
+ ui.button('Export Combined JSON', icon='library_books', on_click=lambda: asyncio.create_task(export_combined_selected())).classes('bg-teal-600 hover:bg-teal-700 text-white font-medium px-4')
+ ui.button('Export Combined MD', icon='article', on_click=lambda: asyncio.create_task(export_combined_md())).classes('bg-cyan-600 hover:bg-cyan-700 text-white font-medium px-4')
ui.button('Export Selected', icon='file_download', on_click=lambda: asyncio.create_task(export_selected())).classes('bg-emerald-600 hover:bg-emerald-700 text-white font-medium px-4')
ui.button('Deselect All', icon='close', on_click=lambda: table.selected.clear()).classes('bg-slate-700 hover:bg-slate-600 text-white font-medium px-4')
@@ -910,6 +912,8 @@ def search_page() -> None: # build UI
table.rows = results
table.selected.clear()
+ table.selected.extend(results)
+ table.update()
# write output if requested
if out_input.value:
@@ -967,6 +971,58 @@ def search_page() -> None: # build UI
status.set_text(status.text + f' Copy failed: {e}')
ui.notify(f'Copy failed: {e}', color='negative')
+ async def export_combined_selected():
+ with table.client:
+ if not table.selected:
+ ui.notify('No records selected', color='warning')
+ return
+
+ dest_dir = copy_input.value.strip() or 'out'
+ filename = os.path.basename(out_input.value or 'results.json')
+ if not filename.lower().endswith('.json'):
+ filename = os.path.splitext(filename)[0] + '.json'
+
+ outp = os.path.join(dest_dir, filename)
+ status.set_text(f'Exporting {len(table.selected)} records combined to {outp}...')
+
+ try:
+ os.makedirs(dest_dir, exist_ok=True)
+ await anyio.to_thread.run_sync(search_md.write_output, table.selected, outp, 'combined_json')
+ status.set_text(f'Exported {len(table.selected)} records combined to {outp}.')
+ ui.notify('Combined export successful', color='positive')
+ except Exception as e:
+ status.set_text(f'Combined export failed: {e}')
+ ui.notify(f'Combined export failed: {e}', color='negative')
+
+ async def export_combined_md():
+ with table.client:
+ if not table.selected:
+ ui.notify('No records selected', color='warning')
+ return
+
+ dest_dir = copy_input.value.strip() or 'out'
+
+ active_queries = [q for q in queries if q['qval'].strip()]
+ if active_queries:
+ qvals = [q['qval'].strip() for q in active_queries]
+ slug = "_".join([re.sub(r'[^a-zA-Z0-9_-]', '_', qv.lower()) for qv in qvals])
+ slug = re.sub(r'_+', '_', slug).strip('_')
+ filename = f"{slug}_combined.md"
+ else:
+ filename = "combined.md"
+
+ outp = os.path.join(dest_dir, filename)
+ status.set_text(f'Exporting {len(table.selected)} records combined to {outp}...')
+
+ try:
+ os.makedirs(dest_dir, exist_ok=True)
+ await anyio.to_thread.run_sync(search_md.write_output, table.selected, outp, 'combined_md')
+ status.set_text(f'Exported {len(table.selected)} records combined to {outp}.')
+ ui.notify('Combined markdown export successful', color='positive')
+ except Exception as e:
+ status.set_text(f'Combined markdown export failed: {e}')
+ ui.notify(f'Combined markdown export failed: {e}', color='negative')
+
async def export_selected():
with table.client:
if not table.selected: