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This commit is contained in:
Ross
@@ -15,6 +15,23 @@
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}
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}
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]
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]
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||||||
},
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},
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||||||
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{
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||||||
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"label": "'Hypotensive' (exact)",
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||||||
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"queries": [
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||||||
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{
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||||||
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"qval": "Hypotensive",
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||||||
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"mode": "exact",
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||||||
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"targets": [
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"Title",
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||||||
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"Content",
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||||||
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"Breadcrumbs",
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||||||
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"Keywords",
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||||||
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"Category",
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"Type"
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||||||
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]
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||||||
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}
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||||||
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]
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||||||
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},
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||||||
{
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{
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||||||
"label": "'polycystic live' (exact)",
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"label": "'polycystic live' (exact)",
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||||||
"queries": [
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"queries": [
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||||||
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|||||||
+18
@@ -0,0 +1,18 @@
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|||||||
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FROM python:3.10-slim
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||||||
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||||||
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WORKDIR /app
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||||||
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||||||
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# Install system dependencies if any are needed
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||||||
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RUN apt-get update && apt-get install -y --no-install-recommends \
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build-essential \
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||||||
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&& rm -rf /var/lib/apt/lists/*
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||||||
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COPY requirements.txt /app/
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RUN pip install --no-cache-dir -r requirements.txt
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COPY . /app
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||||||
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EXPOSE 8000
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||||||
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||||||
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CMD ["uvicorn", "scrapers.cache_server:app", "--host", "0.0.0.0", "--port", "8000"]
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||||||
@@ -0,0 +1,13 @@
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|||||||
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version: '3.8'
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||||||
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||||||
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services:
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||||||
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cache-server:
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||||||
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build: .
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||||||
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container_name: statdx-cache-server
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||||||
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ports:
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||||||
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- "8000:8000"
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||||||
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volumes:
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||||||
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- .:/app
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||||||
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environment:
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||||||
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- PYTHONUNBUFFERED=1
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||||||
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restart: unless-stopped
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||||||
@@ -535,5 +535,43 @@
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|||||||
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||||||
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||||||
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||||||
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||||||
|
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||||||
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||||||
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||||||
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||||||
|
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|
||||||
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||||||
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|
||||||
}
|
}
|
||||||
File diff suppressed because it is too large
Load Diff
+274
@@ -0,0 +1,274 @@
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|||||||
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---
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||||||
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title: "Abdominal Aorta and Visceral Vasculature Anatomy"
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docid: "341c0af5-bfe8-4755-a401-39b7b9f2d9b9"
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authors:
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||||||
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- key: "91e93745-f376-45a8-9b33-eae419cd3322"
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value: "T. Gregory Walker, MD, FSIR"
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breadcrumbs:
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||||||
|
-
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||||||
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name: "Vasculature"
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||||||
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slug: "vasculature"
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||||||
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treeNodeId: "6de1ee4d-afe9-419c-a868-d4074ec0fb7e"
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||||||
|
-
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||||||
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name: "Anatomy"
|
||||||
|
slug: "anatomy"
|
||||||
|
treeNodeId: "3bbfaa8b-2dbc-41eb-b3da-3bd83a9737c4"
|
||||||
|
-
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||||||
|
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
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||||||
|
|
||||||
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||||||
|
- 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
|
||||||
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- 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
|
||||||
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|
||||||
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|
||||||
|
- 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
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||||||
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- Continues along lesser curvature of stomach giving branches anteriorly and posteriorly; terminally anastomoses with right gastric artery
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||||||
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- Splenic artery: Largest celiac branch; tortuous course to left side to supply pancreas, spleen, and stomach
|
||||||
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- Pancreatic branches: Multiple branches, including dorsal pancreatic and pancreatica magna arteries, supply pancreatic body and tail
|
||||||
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- Splenic branches: Splenic artery divides in hilum of spleen into multiple branches
|
||||||
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- 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)
|
||||||
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- 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
|
||||||
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- Inferior pancreaticoduodenal arteries: Supplies duodenum (distal to bile duct), pancreas, and spleen
|
||||||
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- Anastomoses with superior pancreaticoduodenal arteries to form arterial arcade
|
||||||
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- Middle colic artery: Supplies proximal 2/3 of transverse colon up to splenic flexure
|
||||||
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- May have separate right and left branches that supply respective regions of transverse colon
|
||||||
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- Jejunal and ileal branches: Supply respectively named segments of small intestine
|
||||||
|
- Form anastomotic loops (arterial arcades), which give off vasa recta (straight arteries)
|
||||||
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- 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.*
|
||||||
|
|
||||||
+505
@@ -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"
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imageCount: 53
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lastUpdated: "02/01/23"
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pageDescription: "Abnormal Shape/Configuration of Corpus Callosum"
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pageKeywords: "Brain, Differential Diagnosis, Supratentorial Brain Parenchyma, Anatomically Based Differentials, Abnormal Shape/Configuration of Corpus Callosum"
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pageTitle: "Abnormal Shape/Configuration of Corpus Callosum | STATdx"
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enhancedTitle: "Abnormal Shape/Configuration of Corpus Callosum"
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type: "DDX"
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references: true
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breadcrumbs:
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- "Brain"
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|
- "Differential Diagnosis"
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|
- "Supratentorial Brain Parenchyma"
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- "Anatomically Based Differentials"
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- "Abnormal Shape/Configuration of Corpus Callosum"
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|
---
|
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|
## ESSENTIAL INFORMATION
|
||||||
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|
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- ### Key Differential Diagnosis Issues
|
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|
||||||
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|
||||||
|
- Clinical features to consider
|
||||||
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- 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) <img src='img/arrows/CS.png' alt='cyan solid arrow'/>. The CC will gradually thicken as it myelinates from posterior to anterior. Note that the entire pituitary gland normally shows T1 shortening <img src='img/arrows/CO.png' alt='cyan open arrow'/> 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) <img src='img/arrows/CS.png' alt='cyan solid arrow'/>. The CC will gradually thicken as it myelinates from posterior to anterior. Note that the entire pituitary gland normally shows T1 shortening <img src='img/arrows/CO.png' alt='cyan open arrow'/> 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) <img src='img/arrows/CS.png' alt='cyan solid arrow'/>. The CC will gradually thicken as it myelinates from posterior to anterior. Note that the entire pituitary gland normally shows T1 shortening <img src='img/arrows/CO.png' alt='cyan open arrow'/> 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) <img src='img/arrows/CS.png' alt='cyan solid arrow'/>. The CC will gradually thicken as it myelinates from posterior to anterior. Note that the entire pituitary gland normally shows T1 shortening <img src='img/arrows/CO.png' alt='cyan open arrow'/> 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/>. 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 <img src='img/arrows/CO.png' alt='cyan open arrow'/> with relatively little signal abnormality. Also note the angular margins <img src='img/arrows/CC.png' alt='cyan curved arrow'/> 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/>. This results in marked CC thinning.*
|
||||||
|
|
||||||
|
|
||||||
|
**Hypoxic-Ischemic Encephalopathy**
|
||||||
|
*Midline sagittal T1 MR in the same patient shows marked thinning of the CC <img src='img/arrows/CS.png' alt='cyan solid arrow'/> 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> 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 <img src='img/arrows/CO.png' alt='cyan open arrow'/>. Note the enlarged lateral <img src='img/arrows/CS.png' alt='cyan solid arrow'/>, 3rd <img src='img/arrows/BS.png' alt='black solid arrow'/>, & 4th <img src='img/arrows/BO.png' alt='black open arrow'/> ventricles as well as thin T2 hypointensity <img src='img/arrows/CC.png' alt='cyan curved arrow'/> along the brainstem, consistent with hemosiderin deposition.*
|
||||||
|
|
||||||
|
|
||||||
|
**Obstructive Hydrocephalus**
|
||||||
|
*Midline sagittal T2 MR in a neonate with posthemorrhagic hydrocephalus shows a stretched & thinned CC <img src='img/arrows/CO.png' alt='cyan open arrow'/>. Note the enlarged lateral <img src='img/arrows/CS.png' alt='cyan solid arrow'/>, 3rd <img src='img/arrows/BS.png' alt='black solid arrow'/>, & 4th <img src='img/arrows/BO.png' alt='black open arrow'/> ventricles as well as thin T2 hypointensity <img src='img/arrows/CC.png' alt='cyan curved arrow'/> 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> as well as numerous thin, pencil-like gyri (stenogyria) <img src='img/arrows/CO.png' alt='cyan open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Obstructive Hydrocephalus**
|
||||||
|
*Midline sagittal T1 MR in the same patient 1 year after shunting shows a thinned & dysmorphic CC <img src='img/arrows/CS.png' alt='cyan solid arrow'/> as well as numerous thin, pencil-like gyri (stenogyria) <img src='img/arrows/CO.png' alt='cyan open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Corpus Callosotomy**
|
||||||
|
*Coronal FLAIR MR shows changes of a left functional hemispherotomy with a WM disconnection <img src='img/arrows/CS.png' alt='cyan solid arrow'/> & insular decortication <img src='img/arrows/CO.png' alt='cyan open arrow'/>. 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> & insular decortication <img src='img/arrows/CO.png' alt='cyan open arrow'/>. 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> with persistent paramidline callosal tissue <img src='img/arrows/CC.png' alt='cyan curved arrow'/>, consistent with an isolated surgical callosotomy.*
|
||||||
|
|
||||||
|
|
||||||
|
**Corpus Callosotomy**
|
||||||
|
*Coronal T2 MR shows absence of the midline CC <img src='img/arrows/CS.png' alt='cyan solid arrow'/> with persistent paramidline callosal tissue <img src='img/arrows/CC.png' alt='cyan curved arrow'/>, 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> in the anterior body of the CC. Note the caudal migration of the cerebellum & brainstem <img src='img/arrows/CO.png' alt='cyan open arrow'/>, 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> 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 <img src='img/arrows/CC.png' alt='cyan curved arrow'/>, a high-riding 3rd ventricle <img src='img/arrows/CS.png' alt='cyan solid arrow'/>, & bilateral Probst bundles <img src='img/arrows/WS.png' alt='white solid arrow'/>. Also note the extensive periventricular gray matter (GM) heterotopia <img src='img/arrows/CO.png' alt='cyan open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Callosal Dysgenesis**
|
||||||
|
*Midline sagittal T1 MR in a 5-month-old with isolated callosal dysgenesis shows a very short & thin CC <img src='img/arrows/WO.png' alt='white open arrow'/> 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> & rostrum <img src='img/arrows/CO.png' alt='cyan open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Chiari 2 Malformation**
|
||||||
|
*Midline sagittal T2 MR in a child with a repaired myelomeningocele & Chiari 2 malformation (with beaked tectum <img src='img/arrows/CO.png' alt='cyan open arrow'/>, small 4th ventricle <img src='img/arrows/CC.png' alt='cyan curved arrow'/>, & scalloped clivus <img src='img/arrows/WS.png' alt='white solid arrow'/>) shows a thinned & dysmorphic CC <img src='img/arrows/CS.png' alt='cyan solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Chiari 2 Malformation**
|
||||||
|
*Midline sagittal T2 MR in a child with a repaired myelomeningocele & Chiari 2 malformation (with beaked tectum <img src='img/arrows/CO.png' alt='cyan open arrow'/>, small 4th ventricle <img src='img/arrows/CC.png' alt='cyan curved arrow'/>, & scalloped clivus <img src='img/arrows/WS.png' alt='white solid arrow'/>) shows a thinned & dysmorphic CC <img src='img/arrows/CS.png' alt='cyan solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Glioblastoma**
|
||||||
|
*Coronal T2 MR in a 10-year-old with glioblastoma IDH-wildtype shows mass-like infiltrative signal <img src='img/arrows/CS.png' alt='cyan solid arrow'/> 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 <img src='img/arrows/CO.png' alt='cyan open arrow'/>, 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 <img src='img/arrows/CO.png' alt='cyan open arrow'/>, 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> along the dorsal CC with associated absence of the splenium <img src='img/arrows/CO.png' alt='cyan open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**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 <img src='img/arrows/CO.png' alt='cyan open arrow'/> that communicates with a monoventricle <img src='img/arrows/CS.png' alt='cyan solid arrow'/>. 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 <img src='img/arrows/CO.png' alt='cyan open arrow'/> that communicates with a monoventricle <img src='img/arrows/CS.png' alt='cyan solid arrow'/>. 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> across the midline.*
|
||||||
|
|
||||||
|
|
||||||
|
**Holoprosencephaly**
|
||||||
|
*Midline sagittal T1 MR in a teenager with the middle interhemispheric variant of holoprosencephaly shows an intact CC anteriorly <img src='img/arrows/CO.png' alt='cyan open arrow'/> & posteriorly <img src='img/arrows/CC.png' alt='cyan curved arrow'/> but abnormal extension of GM <img src='img/arrows/CS.png' alt='cyan solid arrow'/> 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 <img src='img/arrows/CO.png' alt='cyan open arrow'/> & posteriorly <img src='img/arrows/CC.png' alt='cyan curved arrow'/> but abnormal extension of GM <img src='img/arrows/CS.png' alt='cyan solid arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> crossing the midline along the CC WM <img src='img/arrows/CS.png' alt='cyan solid arrow'/>. Also note the azygous internal carotid artery (ICA) <img src='img/arrows/CO.png' alt='cyan open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Holoprosencephaly**
|
||||||
|
*Coronal T2 MR in the same patient with syntelencephaly shows abnormal GM <img src='img/arrows/WS.png' alt='white solid arrow'/> crossing the midline along the CC WM <img src='img/arrows/CS.png' alt='cyan solid arrow'/>. Also note the azygous internal carotid artery (ICA) <img src='img/arrows/CO.png' alt='cyan open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Metachromatic Leukodystrophy**
|
||||||
|
*Coronal T2 MR in a 13-year-old girl with metachromatic leukodystrophy shows symmetric extensive WM signal abnormality <img src='img/arrows/CS.png' alt='cyan solid arrow'/> with preservation of the subcortical WM <img src='img/arrows/CC.png' alt='cyan curved arrow'/>. Note the marked thinning of the CC <img src='img/arrows/CO.png' alt='cyan open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**X-Linked Adrenoleukodystrophy**
|
||||||
|
*Axial FLAIR MR in a 14-year-old boy with X-linked adrenoleukodystrophy (ALD) shows symmetric increased FLAIR signal intensity <img src='img/arrows/CO.png' alt='cyan open arrow'/> that crosses the splenium <img src='img/arrows/CS.png' alt='cyan solid arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>, a common normal finding.*
|
||||||
|
|
||||||
|
|
||||||
|
**Normal Variant**
|
||||||
|
*Midline sagittal T1 MR shows a normal neonatal CC <img src='img/arrows/WS.png' alt='white solid arrow'/>, thin due to an age-appropriate lack of myelin. The cingulate gyrus <img src='img/arrows/WC.png' alt='white curved arrow'/> is normal.*
|
||||||
|
|
||||||
|
|
||||||
|
**Periventricular Leukomalacia**
|
||||||
|
*Midline sagittal T1 MR shows diffuse thinning of the posterior CC <img src='img/arrows/WS.png' alt='white solid arrow'/>, 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 <img src='img/arrows/BO.png' alt='black open arrow'/> at the site of a prior grade 4 hemorrhage. The posterior white matter loss correlates with the focal corpus callosum atrophy <img src='img/arrows/CS.png' alt='cyan solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Periventricular Leukomalacia**
|
||||||
|
*Axial T2 MR in the same child shows marked loss of the right periventricular parenchyma <img src='img/arrows/BO.png' alt='black open arrow'/> at the site of a prior grade 4 hemorrhage. The posterior white matter loss correlates with the focal corpus callosum atrophy <img src='img/arrows/CS.png' alt='cyan solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Periventricular Leukomalacia**
|
||||||
|
*Midline sagittal T1 MR shows marked callosal thinning <img src='img/arrows/WO.png' alt='white open arrow'/> in a child whose hydrocephalus follows unilateral grade 4 intraventricular hemorrhage. Note the more severe callosal volume loss posteriorly <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Periventricular Leukomalacia**
|
||||||
|
*Midline sagittal T1 MR shows marked callosal thinning <img src='img/arrows/WO.png' alt='white open arrow'/> in a child whose hydrocephalus follows unilateral grade 4 intraventricular hemorrhage. Note the more severe callosal volume loss posteriorly <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Chronic Cerebral Infarction**
|
||||||
|
*Midline sagittal T1 MR shows thinning <img src='img/arrows/WO.png' alt='white open arrow'/> 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 <img src='img/arrows/WO.png' alt='white open arrow'/> & marked thinning of the posterior CC <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**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 <img src='img/arrows/BO.png' alt='black open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Chemotherapy & Radiation Therapy**
|
||||||
|
*Coronal FLAIR MR shows thinning & gliosis of the CC <img src='img/arrows/WO.png' alt='white open arrow'/> & 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 <img src='img/arrows/WO.png' alt='white open arrow'/>, which had been the site of a prior surgical approach to this child's suprasellar tumor <img src='img/arrows/WS.png' alt='white solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Corpus Callosotomy**
|
||||||
|
*Paramidline sagittal T1 MR in a 7-year-old with intractable epilepsy shows near-complete absence of the CC <img src='img/arrows/CS.png' alt='cyan solid arrow'/> 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> but presence of a cingulate gyrus <img src='img/arrows/CO.png' alt='cyan open arrow'/>. 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>, which point toward the 3rd ventricle.*
|
||||||
|
|
||||||
|
|
||||||
|
**Callosal Agenesis**
|
||||||
|
*Axial T1 MR in a patient with callosal agenesis shows parallel lateral ventricles with colpocephaly <img src='img/arrows/BS.png' alt='black solid arrow'/>, resulting in a typical tear-drop shape.*
|
||||||
|
|
||||||
|
|
||||||
|
**Callosal Agenesis**
|
||||||
|
*Axial T1 MR in a patient with callosal agenesis shows parallel lateral ventricles with colpocephaly <img src='img/arrows/BS.png' alt='black solid arrow'/>, resulting in a typical tear-drop shape.*
|
||||||
|
|
||||||
|
|
||||||
|
**Primary Callosal Dysgenesis**
|
||||||
|
*Midline sagittal T1 MR shows only a residual genu <img src='img/arrows/WC.png' alt='white curved arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Chiari 2 Malformation**
|
||||||
|
*Midline sagittal T1 MR shows an abnormal CC with an absent rostrum, small deformed genu, thick body <img src='img/arrows/WO.png' alt='white open arrow'/>, & absent splenium in this child with a Chiari 2 malformation due to a myelomeningocele. Note the prominent massa intermedia <img src='img/arrows/WS.png' alt='white solid arrow'/>, inferiorly beaked tectum <img src='img/arrows/WC.png' alt='white curved arrow'/>, & caudally displaced elongated 4th ventricle with flattening of the fastigium <img src='img/arrows/CS.png' alt='cyan solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Chiari 2 Malformation**
|
||||||
|
*Axial T2 MR shows a prominent massa intermedia <img src='img/arrows/WC.png' alt='white curved arrow'/> & colpocephalic lateral ventricles with periventricular white matter deficiency in Chiari 2. The genu <img src='img/arrows/WO.png' alt='white open arrow'/> 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/>. Note the typical Chiari 2 features, including a small posterior fossa with caudal herniation of the brainstem & cerebellum, clival scalloping <img src='img/arrows/CC.png' alt='cyan curved arrow'/>, elongated 4th ventricle <img src='img/arrows/WS.png' alt='white solid arrow'/>, & beaked tectum <img src='img/arrows/BS.png' alt='black solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Glioblastoma**
|
||||||
|
*Coronal T1 C+ MR shows a classic "butterfly" glioblastoma multiforme of the corpus callosum <img src='img/arrows/WS.png' alt='white solid arrow'/>. 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>. 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 <img src='img/arrows/BS.png' alt='black solid arrow'/> 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/>, which corresponded to hyperdense areas on CT (not shown). Note the abnormally thickened CC <img src='img/arrows/CO.png' alt='cyan open arrow'/> that is infiltrated by a nonenhancing tumor.*
|
||||||
|
|
||||||
|
|
||||||
|
**Pericallosal Lipoma**
|
||||||
|
*Midline sagittal T1 MR shows a large pericallosal lipoma with severe dysgenesis of the CC <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Pericallosal Lipoma**
|
||||||
|
*Axial FLAIR MR shows a large midline lipoma. Two smaller lipomatous masses <img src='img/arrows/WO.png' alt='white open arrow'/> protrude into the lateral ventricles.*
|
||||||
|
|
||||||
|
|
||||||
|
**Pericallosal Lipoma**
|
||||||
|
*Axial FLAIR MR shows a large midline lipoma. Two smaller lipomatous masses <img src='img/arrows/WO.png' alt='white open arrow'/> protrude into the lateral ventricles.*
|
||||||
|
|
||||||
|
|
||||||
|
**Holoprosencephaly**
|
||||||
|
*Axial T1 MR in a patient with holoprosencephaly shows the lack of a midline fissure. WM <img src='img/arrows/WC.png' alt='white curved arrow'/> is in continuity across the midline. The small basal ganglia <img src='img/arrows/WO.png' alt='white open arrow'/> approximate each other. Note the monoventricle <img src='img/arrows/CS.png' alt='cyan solid arrow'/> communicating with a dorsal cyst <img src='img/arrows/CO.png' alt='cyan open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Holoprosencephaly**
|
||||||
|
*Midline sagittal T1 MR shows both WM & GM <img src='img/arrows/WO.png' alt='white open arrow'/> crossing midline anterior & posterior to the "dip" <img src='img/arrows/WC.png' alt='white curved arrow'/> 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 <img src='img/arrows/WO.png' alt='white open arrow'/> in the expected location of the splenium. GM also protrudes <img src='img/arrows/WC.png' alt='white curved arrow'/> into the ventricular system. The septum pellucidum is absent.*
|
||||||
|
|
||||||
+514
@@ -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"
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slug: "brain"
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treeNodeId: "0361042c-9c90-48e4-864e-a2e6428ee9c9"
|
||||||
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-
|
||||||
|
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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> suggesting an anterior circulation aneurysm, likely ACoA. The low density within the hyperdense hemorrhage <img src='img/arrows/WS.png' alt='white solid arrow'/> may represent the aneurysm.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial CTA MIP shows the ACoA aneurysm <img src='img/arrows/WS.png' alt='white solid arrow'/> within the interhemispheric fissure hemorrhage <img src='img/arrows/CS.png' alt='cyan solid arrow'/>. Anterior circulation aneurysms are most common, representing ~ 90% of aneurysms.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial NECT in a 52-year-old with severe headache shows SAH <img src='img/arrows/WS.png' alt='white solid arrow'/> in the basal cisterns, right sylvian fissure, and a right temporal parenchymal hematoma <img src='img/arrows/CS.png' alt='cyan solid arrow'/>. Hyperdensity is noted in the left sylvian fissure <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial CTA shows bilateral MCA aneurysms <img src='img/arrows/WS.png' alt='white solid arrow'/>. The right aneurysm is larger and mildly irregular and lies adjacent to the hemorrhage <img src='img/arrows/CS.png' alt='cyan solid arrow'/>. 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> throughout the basal cisterns. Early hydrocephalus is noted with dilatation of temporal horns <img src='img/arrows/WC.png' alt='white curved arrow'/>. 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial NECT in a 48-year-old with a possible stroke shows focal SAH in the parietal lobe <img src='img/arrows/WS.png' alt='white solid arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> within a hematoma <img src='img/arrows/CS.png' alt='cyan solid arrow'/> 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 <img src='img/arrows/WO.png' alt='white open arrow'/> in virtually all the visualized sulci. CSF-blood level <img src='img/arrows/WS.png' alt='white solid arrow'/> 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 <img src='img/arrows/WC.png' alt='white curved arrow'/> and sylvian fissures <img src='img/arrows/WS.png' alt='white solid arrow'/>. Note focal hemorrhage in the left inferomedial frontal lobe <img src='img/arrows/WO.png' alt='white open arrow'/>. This suggests an ACoA "culprit" aneurysm that ruptured superolaterally. Early severe obstructive hydrocephalus is also present.*
|
||||||
|
|
||||||
|
|
||||||
|
*More cephalad NECT shows SAH in the sulci <img src='img/arrows/WS.png' alt='white solid arrow'/>. Moderately severe hydrocephalus is present. Bilateral choroid plexus hemorrhage <img src='img/arrows/WO.png' alt='white open arrow'/> is also present.*
|
||||||
|
|
||||||
|
|
||||||
|
*CTA in the same patient shows an ACoA aneurysm <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*DSA in the same patient shows a large, multilobulated ACoA aneurysm <img src='img/arrows/BC.png' alt='black curved arrow'/> with a "tit" <img src='img/arrows/BS.png' alt='black solid arrow'/> 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 <img src='img/arrows/WO.png' alt='white open arrow'/> and normally suppressed CSF in the lateral ventricles with blood-fluid level <img src='img/arrows/WS.png' alt='white solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial GRE MR in the same patient shows blood in the sulci as "blooming" hypointensities <img src='img/arrows/BO.png' alt='black open arrow'/>. The blood-CSF level <img src='img/arrows/BS.png' alt='black solid arrow'/> in the lateral ventricles is also clearly seen.*
|
||||||
|
|
||||||
|
|
||||||
|
*Sagittal T1 MR shows typical findings of acute aSAH. Note "dirty" CSF <img src='img/arrows/WO.png' alt='white open arrow'/> that appears isointense with adjacent brain. The normal basilar artery flow void <img src='img/arrows/WS.png' alt='white solid arrow'/> is surrounded by the SAH.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial T1 MR in the same patient shows a nice contrast between the isointense (with brain) "dirty" CSF <img src='img/arrows/WO.png' alt='white open arrow'/> and the more normal-appearing, hypointense ("dark") CSF in the cistern <img src='img/arrows/WS.png' alt='white solid arrow'/> and temporal horns <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial T2 MR in the same patient shows that the hyperintense SAH is difficult to distinguish from the normal "bright" CSF <img src='img/arrows/WS.png' alt='white solid arrow'/>. The SAH <img src='img/arrows/WO.png' alt='white open arrow'/> is very slightly less hyperintense than the adjacent CSF.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial FLAIR MR in the same patient shows CSF in the suprasellar cistern <img src='img/arrows/WO.png' alt='white open arrow'/> is abnormally hyperintense. Sulcal-cisternal hyperintensity is also seen in the left perimesencephalic and superior cerebellar cisterns as well as the parietooccipital subarachnoid spaces <img src='img/arrows/WS.png' alt='white solid arrow'/>. Normal CSF suppresses on FLAIR.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial NECT shows the typical appearance of aSAH. Acute subarachnoid blood is seen as hyperdensity <img src='img/arrows/WS.png' alt='white solid arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> throughout the basal cisterns. Note the enlargement of both temporal horns of the lateral ventricles <img src='img/arrows/WO.png' alt='white open arrow'/>, consistent with early extraventricular obstructive hydrocephalus.*
|
||||||
|
|
||||||
|
|
||||||
|
*Coronal MIP CTA in the same patient shows a saccular aneurysm <img src='img/arrows/WO.png' alt='white open arrow'/> 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 <img src='img/arrows/WO.png' alt='white open arrow'/>. 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>. Note mild enlargement of the temporal horns, consistent with early obstructive hydrocephalus <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Sagittal NECT shows hemorrhage in the prepontine and suprasellar cisterns <img src='img/arrows/BS.png' alt='black solid arrow'/>. A small amount of hemorrhage is present in the perimesencephalic cistern <img src='img/arrows/WC.png' alt='white curved arrow'/> and occipital sulci <img src='img/arrows/WS.png' alt='white solid arrow'/>. 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial T1 MR shows the suprasellar cistern <img src='img/arrows/WC.png' alt='white curved arrow'/> and interpeduncular notch <img src='img/arrows/WS.png' alt='white solid arrow'/> 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 <img src='img/arrows/WC.png' alt='white curved arrow'/>, while blood in the dependent interpeduncular notch is much more hypointense <img src='img/arrows/WS.png' alt='white solid arrow'/>. A hematocrit level <img src='img/arrows/WO.png' alt='white open arrow'/> seems to be present within the subarachnoid space.*
|
||||||
|
|
||||||
|
|
||||||
|
*FLAIR MR shows mixed signal intensity in the suprasellar cistern <img src='img/arrows/WS.png' alt='white solid arrow'/>, while the surface sulci are hyperintense <img src='img/arrows/WC.png' alt='white curved arrow'/>. 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 <img src='img/arrows/WO.png' alt='white open arrow'/> involving the ACoA. The lesion was successfully coiled.*
|
||||||
|
|
||||||
@@ -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) <img src='img/arrows/BS.png' alt='black solid arrow'/> 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) <img src='img/arrows/BS.png' alt='black solid arrow'/> 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 <img src='img/arrows/CC.png' alt='cyan curved arrow'/> in the GP bilaterally. Note relatively normal-appearing WM.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial DWI MR in the same patient shows extensive symmetric hyperintensities involving the GP <img src='img/arrows/CS.png' alt='cyan solid arrow'/>, cerebral WM <img src='img/arrows/CC.png' alt='cyan curved arrow'/>, and corpus callosum <img src='img/arrows/CO.png' alt='cyan open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial ADC map in the same patient shows corresponding low signal in the GP <img src='img/arrows/CS.png' alt='cyan solid arrow'/>, cerebral WM <img src='img/arrows/CC.png' alt='cyan curved arrow'/>, and corpus callosum <img src='img/arrows/CO.png' alt='cyan open arrow'/> 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 <img src='img/arrows/CO.png' alt='cyan open arrow'/> in the GP.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial T1 MR in the same patient demonstrates central hypointensity and rim of hyperintensity <img src='img/arrows/CC.png' alt='cyan curved arrow'/> 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 <img src='img/arrows/CC.png' alt='cyan curved arrow'/> in the GP bilaterally. Note central low signal in the GP <img src='img/arrows/CS.png' alt='cyan solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial SWI image in the same patient shows central areas of low signal <img src='img/arrows/CO.png' alt='cyan open arrow'/> 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 <img src='img/arrows/CC.png' alt='cyan curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial T1 MR in the same patient shows low signal intensity lesions <img src='img/arrows/CS.png' alt='cyan solid arrow'/> 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 <img src='img/arrows/BS.png' alt='black solid arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*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 <img src='img/arrows/WC.png' alt='white curved arrow'/> and hippocampi <img src='img/arrows/WO.png' alt='white open arrow'/>, a less common finding.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial DWI MR in the same patient shows areas of restricted diffusion involving the posterior temporal cortex <img src='img/arrows/WC.png' alt='white curved arrow'/>, hippocampal tail, and insular cortex <img src='img/arrows/WS.png' alt='white solid arrow'/> 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 <img src='img/arrows/WO.png' alt='white open arrow'/> and posterior temporal cortex <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/>. The hyperintensity is likely related to blood products.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial T1 C+ FS MR in the same patient shows heterogeneous enhancement of the GP bilaterally <img src='img/arrows/CO.png' alt='cyan open arrow'/>. 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial T2 MR in the same patient shows symmetric, bilateral, CSF intensity lesions within the GP <img src='img/arrows/CC.png' alt='cyan curved arrow'/>. 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> and diffuse hyperintensity throughout the WM <img src='img/arrows/CO.png' alt='cyan open arrow'/> with sparing of the subcortical U fibers.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial T2 MR in the same patient shows bilateral diffuse hyperintensity throughout the WM <img src='img/arrows/CO.png' alt='cyan open arrow'/> 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.*
|
||||||
|
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---
|
||||||
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title: "Central Skull Base"
|
||||||
|
docid: "8887bf04-6027-42cb-9a34-3e15c6cc27ff"
|
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|
authors:
|
||||||
|
- key: "a25c450b-3d34-4f64-bba3-cc0834813df6"
|
||||||
|
value: "Miral D. Jhaveri, MD, MBA"
|
||||||
|
- key: "94f835c8-fa13-4e8a-995b-53048e6b0605"
|
||||||
|
value: "Philip R. Chapman, MD"
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|
breadcrumbs:
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-
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||||||
|
name: "Head and Neck"
|
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|
slug: "head-and-neck"
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treeNodeId: "5c1f8e17-7acd-48d8-9d55-f9f8c2cad850"
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-
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|
name: "Anatomy"
|
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slug: "anatomy"
|
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treeNodeId: "5deb3a75-762a-49d7-8d1c-dffda4a1b190"
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-
|
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|
name: "Skull Base"
|
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|
slug: "skull-base"
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treeNodeId: "d613fd1d-ce43-4be4-85b1-508426706997"
|
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-
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||||||
|
name: "Central Skull Base"
|
||||||
|
slug: "central-skull-base"
|
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treeNodeId: null
|
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|
category: "Head and Neck"
|
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|
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.*
|
||||||
|
|
||||||
+386
@@ -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 <img src='img/arrows/CC.png' alt='cyan curved arrow'/>. Note subtle lack of FLAIR suppression <img src='img/arrows/WS.png' alt='white solid arrow'/> 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 <img src='img/arrows/CC.png' alt='cyan curved arrow'/>. Note subtle lack of FLAIR suppression <img src='img/arrows/WS.png' alt='white solid arrow'/> 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> and more subtle enhancement in the right hemisphere <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial SWI MIP MR shows cortical superficial siderosis <img src='img/arrows/WC.png' alt='white curved arrow'/> and innumerable blooming "black dots" representing cortical microbleeds <img src='img/arrows/BS.png' alt='black solid arrow'/>. 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 <img src='img/arrows/BS.png' alt='black solid arrow'/> within the sulci and patchy cortical and subcortical hyperintensities <img src='img/arrows/CS.png' alt='cyan solid arrow'/>. There was no DWI restriction (not shown).*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial T1 C+ MR in the same patient shows multifocal leptomeningeal enhancement bilaterally <img src='img/arrows/CC.png' alt='cyan curved arrow'/>. 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 <img src='img/arrows/CO.png' alt='cyan open arrow'/> 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 <img src='img/arrows/CC.png' alt='cyan curved arrow'/> 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 <img src='img/arrows/CC.png' alt='cyan curved arrow'/>. Numerous other patchy white matter hyperintensities <img src='img/arrows/CS.png' alt='cyan solid arrow'/> are present in both hemispheres.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial T2* GRE shows multiple blooming "black dots" in the adjacent cortex <img src='img/arrows/BS.png' alt='black solid arrow'/>. 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 <img src='img/arrows/WC.png' alt='white curved arrow'/>, but some subtle, adjacent sulcal enhancement <img src='img/arrows/WO.png' alt='white open arrow'/> is present.*
|
||||||
|
|
||||||
|
|
||||||
|
*Close-up view of axial T1 C+ FS MR "black blood" study shows diffuse leptomeningeal enhancement <img src='img/arrows/WO.png' alt='white open arrow'/> with striking vessel wall enhancement <img src='img/arrows/WC.png' alt='white curved arrow'/>. 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> in the left hemisphere.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial FLAIR MR in the same patient shows striking cortical and subcortical hyperintensity in the left hemisphere <img src='img/arrows/CC.png' alt='cyan curved arrow'/>. Note FLAIR signal in the left paramedian sulci <img src='img/arrows/WO.png' alt='white open arrow'/>. More subtle parenchymal and <img src='img/arrows/CS.png' alt='cyan solid arrow'/> sulcal abnormalities <img src='img/arrows/BO.png' alt='black open arrow'/> 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> and more focal enhancement <img src='img/arrows/CC.png' alt='cyan curved arrow'/> related to a recent hemorrhage. SWI confirmed multiple microhemorrhages.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial T1 C+ MR in the same patient shows the leptomeningeal enhancement <img src='img/arrows/CS.png' alt='cyan solid arrow'/> in the right parietal lobe.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial C+ VWI shows vessel wall enhancement <img src='img/arrows/CS.png' alt='cyan solid arrow'/> and leptomeningeal enhancement <img src='img/arrows/CO.png' alt='cyan open arrow'/> 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.*
|
||||||
|
|
||||||
@@ -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)
|
||||||
|
1. [Domingues R et al: Diagnostic evaluation for nontraumatic intracerebral hemorrhage. Neurol Clin. 33(2):315-328, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25907908%5Bpmid%5D)
|
||||||
|
1. [Esiri M et al: Cerebral amyloid angiopathy, subcortical white matter disease and dementia: literature review and study in OPTIMA. Brain Pathol. 25(1):51-62, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25521177%5Bpmid%5D)
|
||||||
|
1. [Murray ME et al: Clinicopathologic and 11C-Pittsburgh compound B implications of Thal amyloid phase across the Alzheimer's disease spectrum. Brain. 138(Pt 5):1370-81, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25805643%5Bpmid%5D)
|
||||||
|
1. [Nayate AP et al: Use of standardized uptake value ratios decreases interreader variability of [18F] Florbetapir PET brain scan interpretation. AJNR Am J Neuroradiol. 36(7):1237-44, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25767185%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, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25523590%5Bpmid%5D)
|
||||||
|
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)
|
||||||
|
1. [Shoamanesh A et al: Interrelationship of superficial siderosis and microbleeds in cerebral amyloid angiopathy. Neurology. 83(20):1838-43, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25320098%5Bpmid%5D)
|
||||||
|
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)
|
||||||
|
1. [Greenberg SM et al: Microbleeds versus macrobleeds: evidence for distinct entities. Stroke. 40(7):2382-6, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19443797%5Bpmid%5D)
|
||||||
|
1. [Mittal S et al: Susceptibility-weighted imaging: technical aspects and clinical applications, part 2. AJNR Am J Neuroradiol. 30(2):232-52, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19131406%5Bpmid%5D)
|
||||||
|
1. [Nandigam RN et al: MR imaging detection of cerebral microbleeds: effect of susceptibility-weighted imaging, section thickness, and field strength. AJNR Am J Neuroradiol. 30(2):338-43, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19001544%5Bpmid%5D)
|
||||||
|
1. [Santhosh K et al: Susceptibility weighted imaging: a new tool in magnetic resonance imaging of stroke. Clin Radiol. 2009 Jan;64(1):74-83. Epub 2008 Aug 21. Review. Erratum in: Clin Radiol. 64(6):653, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19070701%5Bpmid%5D)
|
||||||
|
1. [Soontornniyomkij V et al: Cerebral microinfarcts associated with severe cerebral beta-amyloid angiopathy. Brain Pathol. 20(2):459-67, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19725828%5Bpmid%5D)
|
||||||
|
1. [Tschampa HJ et al: MRI in amyloid beta--related brain angiitis. Neurology. 73(3):247, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19620616%5Bpmid%5D)
|
||||||
|
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)
|
||||||
|
1. [Chalela JA et al: Multiple cerebral microbleeds: MRI marker of a diffuse hemorrhage-prone state. J Neuroimaging. 14(1):54-7, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14748209%5Bpmid%5D)
|
||||||
|
1. [Eng JA et al: Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Ann Neurol. 55(2):250-6, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14755729%5Bpmid%5D)
|
||||||
|
1. [Georgiades CS et al: Amyloidosis: review and CT manifestations. Radiographics. 24(2):405-16, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15026589%5Bpmid%5D)
|
||||||
|
1. [Tian J et al: Relationships between arteriosclerosis, cerebral amyloid angiopathy and myelin loss from cerebral cortical white matter in Alzheimer's disease. Neuropathol Appl Neurobiol. 30(1):46-56, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14720176%5Bpmid%5D)
|
||||||
|
1. [Gandhi D et al: CT and MR imaging of intracerebral amyloidoma: case report and review of the literature. AJNR Am J Neuroradiol. 24(3):519-22, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12637308%5Bpmid%5D)
|
||||||
|
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)
|
||||||
|
1. [Gallucci M et al: Neuroradiological findings in two cases of isolated amyloidoma of the central nervous system. Neuroradiology. 44(4):333-7, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=11914811%5Bpmid%5D)
|
||||||
|
1. [Pfeifer LA et al: Cerebral amyloid angiopathy and cognitive function: the HAAS autopsy study. Neurology. 58(11):1629-34, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12058090%5Bpmid%5D)
|
||||||
|
1. [Yamada M: Risk factors for cerebral amyloid angiopathy in the elderly. Ann N Y Acad Sci. 977:37-44, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12480732%5Bpmid%5D)
|
||||||
|
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)
|
||||||
|
1. [Greenberg SM et al: MRI detection of new hemorrhages: potential marker of progression in cerebral amyloid angiopathy. Neurology. 53(5):1135-8, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10496283%5Bpmid%5D)
|
||||||
|
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)
|
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|||||||
|
---
|
||||||
|
title: "CHANTER Syndrome"
|
||||||
|
docid: "f14c06ed-2452-49b2-b82d-a3572040da08"
|
||||||
|
authors:
|
||||||
|
- 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"
|
||||||
|
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 <img src='img/arrows/CC.png' alt='cyan curved arrow'/> in both cerebellar hemispheres with mass effect on the 4th ventricle <img src='img/arrows/CS.png' alt='cyan solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial NECT in a 59-year old with substance use disorder presenting with altered mental status shows symmetric edema <img src='img/arrows/CC.png' alt='cyan curved arrow'/> in both cerebellar hemispheres with mass effect on the 4th ventricle <img src='img/arrows/CS.png' alt='cyan solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial NECT in a 59-year old with substance use disorder presenting with altered mental status shows symmetric edema <img src='img/arrows/CC.png' alt='cyan curved arrow'/> in both cerebellar hemispheres with mass effect on the 4th ventricle <img src='img/arrows/CS.png' alt='cyan solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> with FLAIR hyperintensity <img src='img/arrows/CO.png' alt='cyan open arrow'/> in both cerebellar hemispheres.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial DWI (top) and FLAIR (bottom) images in a patient with fentanyl overdose show restricted diffusion <img src='img/arrows/CS.png' alt='cyan solid arrow'/> with FLAIR hyperintensity <img src='img/arrows/CO.png' alt='cyan open arrow'/> in both cerebellar hemispheres.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial DWI in the same patient shows foci of restricted diffusion <img src='img/arrows/CC.png' alt='cyan curved arrow'/> 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 <img src='img/arrows/CC.png' alt='cyan curved arrow'/> 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.*
|
||||||
|
|
||||||
+447
@@ -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"
|
||||||
|
-
|
||||||
|
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|
||||||
|
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 <img src='img/arrows/WS.png' alt='white solid arrow'/> and superolateral vector of spread into the middle ear <img src='img/arrows/WO.png' alt='white open arrow'/> of a jugular paraganglioma.*
|
||||||
|
|
||||||
|
|
||||||
|
**Paraganglioma (Jugular)**
|
||||||
|
*Axial bone CT shows characteristic permeative-destructive bone changes <img src='img/arrows/WS.png' alt='white solid arrow'/> and superolateral vector of spread into the middle ear <img src='img/arrows/WO.png' alt='white open arrow'/> of a jugular paraganglioma.*
|
||||||
|
|
||||||
|
|
||||||
|
**Paraganglioma (Jugular)**
|
||||||
|
*Axial T1 C+ FS MR of a jugular paraganglioma <img src='img/arrows/WS.png' alt='white solid arrow'/> shows typical avid contrast enhancement and high-velocity flow voids <img src='img/arrows/WC.png' alt='white curved arrow'/> 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 <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Nasopharyngeal Carcinoma**
|
||||||
|
*Axial CECT of nasopharyngeal carcinoma (NPC) shows a bulky mass <img src='img/arrows/WS.png' alt='white solid arrow'/> centered in the lateral pharyngeal recess, extending anteriorly to the nasal cavity. Posterolaterally, the mass involves the carotid space (CS) <img src='img/arrows/WO.png' alt='white open arrow'/>, which contains CNIX-XII at this level. Note bulky contralateral retropharyngeal adenopathy <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Nasopharyngeal Carcinoma**
|
||||||
|
*Axial T2 FS MR better maps the extent of the NPC <img src='img/arrows/WS.png' alt='white solid arrow'/>, including involvement of the ipsilateral CS by primary tumor <img src='img/arrows/WO.png' alt='white open arrow'/> and of the contralateral CS by retropharyngeal adenopathy <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Squamous Cell Carcinoma, Nodes, Internal Jugular**
|
||||||
|
*Axial CECT reveals a right tonsillar mass extending anteriorly into the tongue base <img src='img/arrows/WC.png' alt='white curved arrow'/>. Internal jugular nodes <img src='img/arrows/WS.png' alt='white solid arrow'/> appear infiltrating and with extranodal extension invading the CS. Note the normal CS <img src='img/arrows/WO.png' alt='white open arrow'/> 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 <img src='img/arrows/WO.png' alt='white open arrow'/>, sternocleidomastoid muscle <img src='img/arrows/WC.png' alt='white curved arrow'/>, and the CS <img src='img/arrows/WS.png' alt='white solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**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 <img src='img/arrows/WS.png' alt='white solid arrow'/> corresponding to the region of lower cranial nerve nuclei. Note diffusion bright thrombus within the right V4 vertebral artery <img src='img/arrows/WO.png' alt='white open arrow'/>. Additional infarction is present in the right cerebellum <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**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 <img src='img/arrows/WS.png' alt='white solid arrow'/> and inferomedial cerebellum <img src='img/arrows/WO.png' alt='white open arrow'/>. Note abnormal increased signal within the tortuous right V4 vertebral artery <img src='img/arrows/WC.png' alt='white curved arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>, carotid canal <img src='img/arrows/WO.png' alt='white open arrow'/>, and inner table of mastoid <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Metastasis, Skull Base**
|
||||||
|
*Axial T1 SPGR C+ shows an enhancing skull base metastatic deposit <img src='img/arrows/WS.png' alt='white solid arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> with prominent internal cystic area <img src='img/arrows/WO.png' alt='white open arrow'/>. Note marked effacement of the cerebellomedullary cistern <img src='img/arrows/WC.png' alt='white curved arrow'/>, 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> bilaterally.*
|
||||||
|
|
||||||
|
|
||||||
|
**Squamous Cell Carcinoma, Palatine Tonsil**
|
||||||
|
*Axial CECT demonstrates an invasive right palatine tonsil SCCa primary <img src='img/arrows/WS.png' alt='white solid arrow'/> with extensive extranodal disease in the right CS <img src='img/arrows/WO.png' alt='white open arrow'/>, parotid space <img src='img/arrows/WC.png' alt='white curved arrow'/>, and perivertebral space <img src='img/arrows/BS.png' alt='black solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Squamous Cell Carcinoma, Palatine Tonsil**
|
||||||
|
*Axial CECT shows a right palatine tonsil SCCa primary tumor <img src='img/arrows/WS.png' alt='white solid arrow'/> spreading posterolaterally into the oropharyngeal CS <img src='img/arrows/WO.png' alt='white open arrow'/> where CNIX-XII reside. Note also the massive extranodal SCCa in the right posterior cervical space <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Schwannoma, Jugular Foramen**
|
||||||
|
*Axial T1 C+ FS MR shows the typical appearance of a schwannoma <img src='img/arrows/WS.png' alt='white solid arrow'/> with a bilobed configuration and "waist" at the expanded jugular foramen <img src='img/arrows/WO.png' alt='white open arrow'/>. Note mass effect upon the upper CS <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Schwannoma, Jugular Foramen**
|
||||||
|
*Coronal T1 C+ FS MR of a jugular foramen schwannoma <img src='img/arrows/WS.png' alt='white solid arrow'/> shows the characteristic vector of spread along the CNIX-XI bundle into the expanded jugular foramen <img src='img/arrows/WO.png' alt='white open arrow'/>. Note benign remodeling of the jugular tubercle <img src='img/arrows/WC.png' alt='white curved arrow'/> with maintained dark cortical margin due to the slow-growing mass.*
|
||||||
|
|
||||||
|
|
||||||
|
**Cavernous Malformation, Medulla**
|
||||||
|
*Axial SWI shows a left dorsal medullary cavernous malformation <img src='img/arrows/WS.png' alt='white solid arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> and true lumen <img src='img/arrows/WO.png' alt='white open arrow'/> but no flow limitation.*
|
||||||
|
|
||||||
|
|
||||||
|
**Paraganglioma (Vagal)**
|
||||||
|
*Axial T1 C+ FS MR of a vagal paraganglioma shows an enhancing CS mass <img src='img/arrows/WS.png' alt='white solid arrow'/> 2 cm below skull base displacing the ICA anteromedially <img src='img/arrows/WO.png' alt='white open arrow'/>. Note multiple high-velocity flow voids <img src='img/arrows/WC.png' alt='white curved arrow'/> at the periphery, typical of paraganglioma.*
|
||||||
|
|
||||||
|
|
||||||
|
**Paraganglioma (Vagal)**
|
||||||
|
*Coronal 68-Ga DOTANOC PET/CT shows marked uptake in a large vagal paraganglioma <img src='img/arrows/WS.png' alt='white solid arrow'/> and a smaller carotid body tumor <img src='img/arrows/WO.png' alt='white open arrow'/>. 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>, which enhances heterogeneously with internal nonenhancing cysts <img src='img/arrows/WO.png' alt='white open arrow'/>. The mass splays the ICA <img src='img/arrows/WC.png' alt='white curved arrow'/> away from the internal jugular vein <img src='img/arrows/BS.png' alt='black solid arrow'/>, suggestive of vagal schwannoma.*
|
||||||
|
|
||||||
|
|
||||||
|
**Ependymoma, Basal Cistern**
|
||||||
|
*Axial T2 MR shows a large ependymoma <img src='img/arrows/WS.png' alt='white solid arrow'/> extending from the 4th ventricle lateral aperture into the cerebellomedullary cistern. Note mass effect upon the cerebellum and medulla with cerebellar vasogenic edema <img src='img/arrows/WC.png' alt='white curved arrow'/>. Dark signal within the mass was due to hemorrhage.*
|
||||||
|
|
||||||
|
|
||||||
|
**Multiple Sclerosis, Medulla**
|
||||||
|
*Axial T2 MR shows a lateral medullary focus <img src='img/arrows/WS.png' alt='white solid arrow'/> of multiple sclerosis. The CNIX-XI bundle <img src='img/arrows/WO.png' alt='white open arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> that extends through the jugular foramen <img src='img/arrows/WO.png' alt='white open arrow'/> and into the upper CS <img src='img/arrows/WC.png' alt='white curved arrow'/>. Note the extraaxial location of this avidly enhancing mass, which demonstrates conspicuous dural tails of enhancement <img src='img/arrows/BS.png' alt='black solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Chondrosarcoma, Skull Base**
|
||||||
|
*Axial bone CT shows a chondrosarcoma <img src='img/arrows/WS.png' alt='white solid arrow'/> centered at the petrooccipital fissure with expansile, erosive bone changes, including erosion into the jugular foramen <img src='img/arrows/WO.png' alt='white open arrow'/>. Scant matrix mineralization <img src='img/arrows/WC.png' alt='white curved arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> within an enlarged hypoglossal canal. The tumor encroaches upon the superolateral jugular foramen <img src='img/arrows/WO.png' alt='white open arrow'/>. Note multiple intramural cysts, commonly seen with larger schwannomas.*
|
||||||
|
|
||||||
|
|
||||||
|
### Additional Images
|
||||||
|
|
||||||
|
|
||||||
|
**Paraganglioma (Jugular)**
|
||||||
|
*Axial bone CT reveals a jugular foramen permeative-destructive lesion <img src='img/arrows/WS.png' alt='white solid arrow'/> with soft tissue evident in the middle ear cavity <img src='img/arrows/WO.png' alt='white open arrow'/>. Note opposite normal corticated jugular foramen bony margins <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Paraganglioma (Jugular)**
|
||||||
|
*Coronal T1 C+ FS MR shows the inferior portion of a large jugular paraganglioma involving the basal cistern <img src='img/arrows/WS.png' alt='white solid arrow'/>, area of hypoglossal canal <img src='img/arrows/WO.png' alt='white open arrow'/>, and nasopharyngeal CS <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Paraganglioma (Jugular)**
|
||||||
|
*Coronal T1 C+ FS MR depicts enhancing jugular paraganglioma filling the left jugular foramen, extending superolaterally into middle ear cavity <img src='img/arrows/WO.png' alt='white open arrow'/> and medially into the hypoglossal canal <img src='img/arrows/WS.png' alt='white solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Paraganglioma (Jugular)**
|
||||||
|
*Axial bone CT shows characteristic permeative-destructive bone changes <img src='img/arrows/WS.png' alt='white solid arrow'/> and superolateral vector of spread into the middle ear <img src='img/arrows/WO.png' alt='white open arrow'/> of a jugular paraganglioma. In contrast, note the crisp cortical margins of the normal right jugular foramen <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Paraganglioma (Jugular)**
|
||||||
|
*Axial T1 C+ FS MR of a jugular paraganglioma <img src='img/arrows/WS.png' alt='white solid arrow'/> shows typical avid contrast enhancement and high-velocity flow voids <img src='img/arrows/WC.png' alt='white curved arrow'/> seen with paragangliomas in the head and neck. The mass is centered at the jugular foramen abutting the ICA <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Nasopharyngeal Carcinoma**
|
||||||
|
*Axial CECT of nasopharyngeal carcinoma shows a bulky mass <img src='img/arrows/WS.png' alt='white solid arrow'/> centered in the lateral nasopharyngeal recess. This mass has invaded the nasopharyngeal carotid space <img src='img/arrows/WO.png' alt='white open arrow'/>, which contains CNIX-XII at this level. Note the maintained CS tissue planes <img src='img/arrows/WC.png' alt='white curved arrow'/> on the contralateral side for comparison.*
|
||||||
|
|
||||||
|
|
||||||
|
**Nasopharyngeal Carcinoma**
|
||||||
|
*Axial T2 FS MR shows invasive nasopharyngeal carcinoma <img src='img/arrows/WS.png' alt='white solid arrow'/> and retropharyngeal adenopathy <img src='img/arrows/WO.png' alt='white open arrow'/> that exert mass effect upon the effaced CS <img src='img/arrows/WC.png' alt='white curved arrow'/>. Note mastoid effusion due to obstructed eustachian tube.*
|
||||||
|
|
||||||
|
|
||||||
|
**Nasopharyngeal Carcinoma**
|
||||||
|
*Axial CECT shows an invasive carcinoma in the right lateral nasopharyngeal recess <img src='img/arrows/WS.png' alt='white solid arrow'/>. Notice that the CS (containing CNIX-XII at this level) has lost its distinct soft tissue planes <img src='img/arrows/BO.png' alt='black open arrow'/> as a result of tumor invasion.*
|
||||||
|
|
||||||
|
|
||||||
|
**Nasopharyngeal Carcinoma**
|
||||||
|
*Axial CECT demonstrates the inferior aspect of a nasopharyngeal carcinoma <img src='img/arrows/WS.png' alt='white solid arrow'/> with invasion of the CS <img src='img/arrows/BO.png' alt='black open arrow'/> deep to the styloid process <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Cerebral Ischemia-Infarction, Acute, Posterior Inferior Cerebellar Artery**
|
||||||
|
*Axial DWI MR shows high signal (reduced diffusivity) <img src='img/arrows/WS.png' alt='white solid arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>. Notice the metastatic tumor also involves the meninges along the adjacent posterior fossa <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Brainstem Tumors, Pediatric**
|
||||||
|
*Axial T2 FS MR of a pilocytic astrocytoma (WHO 1) shows a heterogeneously hyperintense mass <img src='img/arrows/WS.png' alt='white solid arrow'/> centered in the left posterolateral medulla with cystic component <img src='img/arrows/WO.png' alt='white open arrow'/>. Note obliteration of the left cerebellomedullary cistern <img src='img/arrows/WC.png' alt='white curved arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>. Notice the thickened high signal proximal CNIX-XI cranial nerve bundle <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Brainstem Tumors, Pediatric**
|
||||||
|
*Axial T2WI MR reveals a small high-signal posterolateral medullary glioma <img src='img/arrows/WS.png' alt='white solid arrow'/> affecting the area of cranial nerve nuclei XI-XII.*
|
||||||
|
|
||||||
|
|
||||||
|
**Schwannoma, Jugular Foramen**
|
||||||
|
*Axial T1 C+ MR shows the typical appearance of a schwannoma <img src='img/arrows/WS.png' alt='white solid arrow'/>. Areas of nonenhancement correspond with intramural cysts <img src='img/arrows/WC.png' alt='white curved arrow'/>. Note the vector of spread along the CNIX-XI bundle into the expanded jugular foramen <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Schwannoma, Jugular Foramen**
|
||||||
|
*Axial bone CT shows typical bone changes of a schwannoma. The smooth jugular foramen enlargement with thin, sclerotic margins <img src='img/arrows/WS.png' alt='white solid arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> emerging from the left jugular foramen <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Schwannoma, Jugular Foramen**
|
||||||
|
*Axial T2 MR reveals a jugular foramen schwannoma <img src='img/arrows/WS.png' alt='white solid arrow'/>. Notice that the vector of spread of the lesion is along the CNXI-XI bundle <img src='img/arrows/WO.png' alt='white open arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> with a dark rim of hemosiderin from a remote bleed and fluid-fluid level <img src='img/arrows/WO.png' alt='white open arrow'/> from a more recent hemorrhage.*
|
||||||
|
|
||||||
|
|
||||||
|
**Cavernous Malformation, Medulla**
|
||||||
|
*Axial T1 C+ FS MR demonstrates a partially enhancing left medullary cavernous malformation <img src='img/arrows/WS.png' alt='white solid arrow'/> with associated developmental venous anomaly <img src='img/arrows/WO.png' alt='white open arrow'/>, 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 <img src='img/arrows/WO.png' alt='white open arrow'/>. Note the metastatic tumor has entered the ICAs <img src='img/arrows/WS.png' alt='white solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Dissection, Carotid Artery, Neck**
|
||||||
|
*Axial 3D TOF MRA shows a left cervical ICA dissecting pseudoaneurysm <img src='img/arrows/WS.png' alt='white solid arrow'/>. There is maintained flow-related enhancement in the true ICA lumen <img src='img/arrows/WO.png' alt='white open arrow'/> without flow-limiting stenosis.*
|
||||||
|
|
||||||
|
|
||||||
|
**Dissection, Carotid Artery, Neck**
|
||||||
|
*Axial MRA source image shows residual lumen of ICA dissection <img src='img/arrows/WS.png' alt='white solid arrow'/> in association with a pseudoaneurysm <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Paraganglioma (Vagal)**
|
||||||
|
*Coronal T1 C+ FS MR reveals an ovoid, avidly enhancing CS mass <img src='img/arrows/WS.png' alt='white solid arrow'/> with a few sporadic high-velocity flow voids <img src='img/arrows/WO.png' alt='white open arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> shows a circumscribed, avidly enhancing mass with internal nonenhancing cysts <img src='img/arrows/WC.png' alt='white curved arrow'/> 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 <img src='img/arrows/WO.png' alt='white open arrow'/>. The superior margin "points" <img src='img/arrows/WS.png' alt='white solid arrow'/> toward the jugular foramen.*
|
||||||
|
|
||||||
|
|
||||||
|
**Meningioma, Jugular Foramen**
|
||||||
|
*Coronal SPGR C+ shows enhancing meningioma <img src='img/arrows/WS.png' alt='white solid arrow'/> with extension through jugular foramen <img src='img/arrows/WO.png' alt='white open arrow'/> and hypoglossal canal <img src='img/arrows/WC.png' alt='white curved arrow'/> into the nasopharyngeal CS <img src='img/arrows/BS.png' alt='black solid arrow'/> (where CNIX-XII reside). Note intraosseous tumor and hyperostosis of the jugular tubercle <img src='img/arrows/BC.png' alt='black curved arrow'/>, 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>, involving both the lateral clival bone marrow <img src='img/arrows/WO.png' alt='white open arrow'/> and the nasopharyngeal CS <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**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 <img src='img/arrows/WS.png' alt='white solid arrow'/> and the jugular foramen <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Cerebral Ischemia-Infarction, Acute, Posterior Inferior Cerebellar Artery**
|
||||||
|
*Axial graphic shows the vagal nuclei in the dorsolateral medulla <img src='img/arrows/BS.png' alt='black solid arrow'/>. Note the proximity of the glossopharyngeal <img src='img/arrows/WS.png' alt='white solid arrow'/>, vagal <img src='img/arrows/WO.png' alt='white open arrow'/>, and spinal accessory <img src='img/arrows/WC.png' alt='white curved arrow'/> 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 <img src='img/arrows/BS.png' alt='black solid arrow'/>, vagus <img src='img/arrows/BO.png' alt='black open arrow'/>, spinal accessory <img src='img/arrows/BC.png' alt='black curved arrow'/>, and glossopharyngeal <img src='img/arrows/WO.png' alt='white open arrow'/> nerves. Nasopharyngeal carcinoma commonly invades the CS, causing complex cranial neuropathy involving these nerves.*
|
||||||
|
|
||||||
+575
@@ -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:
|
||||||
|
-
|
||||||
|
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: "Dementias and Degenerative Disorders"
|
||||||
|
slug: "dementias-and-degenerative-disorde-"
|
||||||
|
treeNodeId: "db2baff9-3d1d-4ed3-8f37-282a634e6a7f"
|
||||||
|
-
|
||||||
|
name: "Creutzfeldt-Jakob Disease (CJD)"
|
||||||
|
slug: "creutzfeldt-jakob-disease-cjd"
|
||||||
|
treeNodeId: null
|
||||||
|
category: "Brain"
|
||||||
|
documentVersionId: "1247b3bb-fbc2-445e-b385-89f43aa24601"
|
||||||
|
imageCount: 27
|
||||||
|
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"
|
||||||
|
references: true
|
||||||
|
ddx: true
|
||||||
|
tables: 1
|
||||||
|
anatomy:
|
||||||
|
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|
||||||
|
- "{'authors': 'Lubdha M. Shah, MD', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/a9de3815-ec59-4c78-adf0-94974065a7e3', 'category': 'Brain', 'compareUrl': '/compare/document/a9de3815-ec59-4c78-adf0-94974065a7e3/related-anatomy/treeNode?subContext=Basal Ganglia', 'documentId': 'a9de3815-ec59-4c78-adf0-94974065a7e3', 'documentType': 'ANATOMY', 'documentUrl': '/document/basal-ganglia/a9de3815-ec59-4c78-adf0-94974065a7e3', 'enhancedTitle': 'Basal Ganglia', 'entryDate': '02/23/21', 'imageCount': 3, 'imageUrl': '/image/thumbnail/97e4f2fc-0aaa-4929-b396-2b01350715b7?size=174&quality=85', 'inCompareCart': False, 'rank': 2, 'referenceCount': 6, 'showCompareButton': False, 'title': 'Basal Ganglia'}"
|
||||||
|
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|
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- "{'authors': 'Jeffrey S. Anderson, MD, PhD', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/0352d34a-5966-494e-b9c3-c26bde257bca', 'category': 'Brain', 'compareUrl': '/compare/document/0352d34a-5966-494e-b9c3-c26bde257bca/related-anatomy/treeNode?subContext=Social Brain Anatomy', 'documentId': '0352d34a-5966-494e-b9c3-c26bde257bca', 'documentType': 'ANATOMY', 'documentUrl': '/document/social-brain-anatomy/0352d34a-5966-494e-b9c3-c26bde257bca', 'enhancedTitle': 'Social Brain Anatomy', 'entryDate': '02/23/21', 'imageCount': 2, 'imageUrl': '/image/thumbnail/34a59189-2473-4319-bc72-abe41236496e?size=174&quality=85', 'inCompareCart': False, 'rank': 9, 'referenceCount': 19, 'showCompareButton': False, 'title': 'Social Brain Anatomy'}"
|
||||||
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|
||||||
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|
||||||
|
- "{'authors': 'Jeffrey S. Anderson, MD, PhD', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/7b97f239-0f6f-4809-ac44-594cdf4842d5', 'category': 'Brain', 'compareUrl': '/compare/document/7b97f239-0f6f-4809-ac44-594cdf4842d5/related-anatomy/treeNode?subContext=Functional Network Overview', 'documentId': '7b97f239-0f6f-4809-ac44-594cdf4842d5', 'documentType': 'ANATOMY', 'documentUrl': '/document/functional-network-overview/7b97f239-0f6f-4809-ac44-594cdf4842d5', 'enhancedTitle': 'Functional Network Overview', 'entryDate': '02/23/21', 'imageCount': 2, 'imageUrl': '/image/thumbnail/c60f307b-e1ac-4987-8c19-b81747902e8f?size=174&quality=85', 'inCompareCart': False, 'rank': 12, 'referenceCount': 10, 'showCompareButton': False, 'title': 'Functional Network Overview'}"
|
||||||
|
- "{'authors': 'Paula J. Woodward, MD, FSRU', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/080771c2-02f3-408d-ad70-04a80d849500', 'category': 'Ultrasound', 'compareUrl': '/compare/document/080771c2-02f3-408d-ad70-04a80d849500/related-anatomy/treeNode?subContext=Brain', 'documentId': '080771c2-02f3-408d-ad70-04a80d849500', 'documentType': 'ANATOMY', 'documentUrl': '/document/brain/080771c2-02f3-408d-ad70-04a80d849500', 'enhancedTitle': 'Brain', 'entryDate': '12/20/17', 'imageCount': 77, 'imageUrl': '/image/thumbnail/244b60fe-e15f-4ffb-8a39-cdbfe64fb3c4?size=174&quality=85', 'inCompareCart': False, 'rank': 13, 'referenceCount': 0, 'showCompareButton': False, 'title': 'Brain'}"
|
||||||
|
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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> with signal drop on ADC <img src='img/arrows/CC.png' alt='cyan curved arrow'/>. Findings consistent with probable CJD.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial DWI shows symmetric hyperintensity in bilateral caudate & putamen <img src='img/arrows/CS.png' alt='cyan solid arrow'/>. Symmetric hyperintense signal involves bilateral pulvinar & dorsomedial thalamic nuclei, indicating hockey stick sign <img src='img/arrows/CC.png' alt='cyan curved arrow'/>. Also note asymmetric cortical hyperintensity (cortical ribbon sign) involving bilateral frontal, parietal, occipital lobes <img src='img/arrows/CO.png' alt='cyan open arrow'/>, & 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial FLAIR MR in the same patient shows hyperintense signal involving bilateral pulvinar & dorsomedial nuclei of thalami <img src='img/arrows/CO.png' alt='cyan open arrow'/>. 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 <img src='img/arrows/CC.png' alt='cyan curved arrow'/> & putamina <img src='img/arrows/CS.png' alt='cyan solid arrow'/>. 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
### 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>, 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/>, 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 <img src='img/arrows/CO.png' alt='cyan open arrow'/>. 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> & asymmetric cortical restricted diffusion (cortical ribbon sign) in bilateral (right > > left) hemispheres <img src='img/arrows/CO.png' alt='cyan open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial FLAIR MR in the same patient demonstrates hyperintense signal in bilateral BG <img src='img/arrows/CS.png' alt='cyan solid arrow'/> & cortex <img src='img/arrows/CO.png' alt='cyan open arrow'/>. CSF was positive for 14-3-3 protein indicating probable sCJD.*
|
||||||
|
|
||||||
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|
|||||||
|
---
|
||||||
|
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 <img src='img/arrows/WS.png' alt='white solid arrow'/>, and both abducens nerves <img src='img/arrows/BO.png' alt='black open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Metastases**
|
||||||
|
*Axial T1 C+ MR in a patient with disseminated malignant glial neoplasm shows diffuse enhancing metastases covering the brain, CPA/IACs <img src='img/arrows/WS.png' alt='white solid arrow'/>, and both abducens nerves <img src='img/arrows/BO.png' alt='black open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Metastases**
|
||||||
|
*Axial T1 C+ MR in a patient with disseminated malignant glial neoplasm shows diffuse enhancing metastases covering the brain, CPA/IACs <img src='img/arrows/WS.png' alt='white solid arrow'/>, and both abducens nerves <img src='img/arrows/BO.png' alt='black open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Neurofibromatosis Type 2**
|
||||||
|
*Coronal T1 C+ FS MR of a patient with NF2 shows bilateral enhancing masses consistent with CNVIII schwannomas <img src='img/arrows/WS.png' alt='white solid arrow'/>. There is a schwannoma traversing the left jugular foramen <img src='img/arrows/WO.png' alt='white open arrow'/>, most likely CNX. Superiorly, an additional mass <img src='img/arrows/WC.png' alt='white curved arrow'/> most likely represents a schwannoma of the left CNV. Note normal right CNV <img src='img/arrows/CS.png' alt='cyan solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**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 <img src='img/arrows/WS.png' alt='white solid arrow'/>. Note scalp plexiform neurofibroma <img src='img/arrows/WO.png' alt='white open arrow'/>. (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 <img src='img/arrows/WS.png' alt='white solid arrow'/>, 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>, including the intracanalicular segment <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Multiple Sclerosis**
|
||||||
|
*Coronal T1 C+ FS MR in a patient with multiple sclerosis and left trigeminal neuralgia shows enhancing left CNV <img src='img/arrows/WS.png' alt='white solid arrow'/>. Compare to normal nonenhancing right side <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Bell Palsy**
|
||||||
|
*Axial T1 C+ FS MR with magnified view shows variant case with enhancing "fundal tuft" in IAC <img src='img/arrows/WS.png' alt='white solid arrow'/> and enhancing labyrinthine segment <img src='img/arrows/WO.png' alt='white open arrow'/> leading to enhancing geniculate ganglion <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Herpes Zoster**
|
||||||
|
*Axial T1 C+ FS MR shows enhancing CNVII in IAC <img src='img/arrows/WC.png' alt='white curved arrow'/>, tympanic segment <img src='img/arrows/WS.png' alt='white solid arrow'/> along with enhancement of the extracranial soft tissues involving the left ear <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**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 <img src='img/arrows/WS.png' alt='white solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Lyme Disease**
|
||||||
|
*Axial T1 C+ FS MR shows abnormal enhancement of the right facial nerve at the canalicular <img src='img/arrows/WS.png' alt='white solid arrow'/>, labyrinthine <img src='img/arrows/CS.png' alt='cyan solid arrow'/>, geniculate <img src='img/arrows/WO.png' alt='white open arrow'/>, and tympanic <img src='img/arrows/WC.png' alt='white curved arrow'/> segments. There is also abnormal enhancement of the cisternal segments of the bilateral abducens nerves <img src='img/arrows/CO.png' alt='cyan open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Lymphoma**
|
||||||
|
*Axial T1 C+ FS MR in a patient with known systemic lymphoma and right 3rd nerve palsy shows thickened, enhancing right oculomotor nerve <img src='img/arrows/WS.png' alt='white solid arrow'/> and intraconal retrobulbar enhancing tumor <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Neurosarcoid**
|
||||||
|
*Axial T1 C+ MR shows enhancement of both thickened optic nerves extending from the optic canal to the optic chiasm <img src='img/arrows/WS.png' alt='white solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Opportunistic Infection, AIDS**
|
||||||
|
*Axial T1 C+ MR in a patient with HIV/AIDS who presented with confusion and seizures shows tubercular meningitis <img src='img/arrows/WS.png' alt='white solid arrow'/> that thickens and encases the right trigeminal nerve <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**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 <img src='img/arrows/WS.png' alt='white solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
### Additional Images
|
||||||
|
|
||||||
|
|
||||||
|
**Metastases**
|
||||||
|
*Axial T1 C+ FS MR in a patient with known metastatic colon cancer shows IAC enhancement <img src='img/arrows/WS.png' alt='white solid arrow'/> extending through the cochlear aperture, across the modiolus and into the membranous labyrinth <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Optic Neuritis**
|
||||||
|
*Axial T1 C+ FS MR shows diffusely enhancing intraorbital portion of left optic nerve <img src='img/arrows/WS.png' alt='white solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Herpes Zoster**
|
||||||
|
*Axial T1 C+ MR in a patient with Herpes zoster, right-sided trigeminal neuralgia, shows cisternal CNV enhancement <img src='img/arrows/WS.png' alt='white solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**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 <img src='img/arrows/BS.png' alt='black solid arrow'/> 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 <img src='img/arrows/WC.png' alt='white curved arrow'/>. Note also dural thickening <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Neurofibromatosis Type 2**
|
||||||
|
*Axial T1 C+ MR shows bilateral vestibular schwannomas with classic "ice cream on cone" appearance <img src='img/arrows/WS.png' alt='white solid arrow'/>. Note arachnoid cyst associated with left lesion <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Lyme Disease**
|
||||||
|
*Axial T1 C+ FS MR shows enhancement in left IAC involving both CNVII and CNVIII <img src='img/arrows/WS.png' alt='white solid arrow'/>. Note pial enhancement along the pons <img src='img/arrows/WO.png' alt='white open arrow'/>, extending along CNVI from its brainstem exit to Dorello canal <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Metastases**
|
||||||
|
*Axial T1 C+ FS MR shows thickened, enhancing V2 in a patient with adenoid cystic carcinoma with perineural tumor spread in pterygopalatine fossa <img src='img/arrows/WC.png' alt='white curved arrow'/>, extending along the foramen rotundum <img src='img/arrows/WS.png' alt='white solid arrow'/> into the Meckel cave <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Neurofibromatosis Type 2**
|
||||||
|
*Coronal T1 C+ FS MR in a patient with known NF2 shows trigeminal schwannomas in both Meckel caves <img src='img/arrows/WS.png' alt='white solid arrow'/>, as well as multiple schwannomas involving cervical spinal nerve roots <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**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 <img src='img/arrows/WS.png' alt='white solid arrow'/> and bilateral brachium pontis <img src='img/arrows/WO.png' alt='white open arrow'/>. 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>. The right optic nerve is noticeably enlarged and enhancing <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**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 <img src='img/arrows/WS.png' alt='white solid arrow'/>, with extension posteriorly into the enlarged optic canal <img src='img/arrows/WO.png' alt='white open arrow'/>, 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>.*
|
||||||
|
|
||||||
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|
|||||||
|
---
|
||||||
|
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 <img src='img/arrows/WS.png' alt='white solid arrow'/> by an anterior inferior cerebellar artery (AICA) loop <img src='img/arrows/WO.png' alt='white open arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> by an anterior inferior cerebellar artery (AICA) loop <img src='img/arrows/WO.png' alt='white open arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> at the REZ <img src='img/arrows/WO.png' alt='white open arrow'/> by a tortuous loop of the vertebral artery <img src='img/arrows/WC.png' alt='white curved arrow'/>, 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>, 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> centered in the left CPA with extension into the internal auditory canal (IAC) <img src='img/arrows/WO.png' alt='white open arrow'/>. Note a conspicuous dural tail of enhancement <img src='img/arrows/WC.png' alt='white curved arrow'/> associated with this large meningioma.*
|
||||||
|
|
||||||
|
|
||||||
|
**Aneurysm in CPA-IAC**
|
||||||
|
*Axial T1 C+ FS MR of a right posterior inferior cerebellar artery (PICA) saccular aneurysm <img src='img/arrows/WS.png' alt='white solid arrow'/> shows a round, complex signal mass centered in the right CPA. Note a PICA flow void <img src='img/arrows/WO.png' alt='white open arrow'/> extending into the aneurysm with jet of contrast opacification <img src='img/arrows/WC.png' alt='white curved arrow'/> centrally and peripheral filling defects <img src='img/arrows/BS.png' alt='black solid arrow'/> of this partially thrombosed aneurysm.*
|
||||||
|
|
||||||
|
|
||||||
|
**Facial Nerve Schwannoma in CPA-IAC**
|
||||||
|
*Axial SPGR C+ MR of a left facial nerve schwannoma <img src='img/arrows/WS.png' alt='white solid arrow'/> shows an avidly enhancing CPA-IAC mass with a labyrinthine tail of enhancement <img src='img/arrows/WO.png' alt='white open arrow'/> extending to the geniculate ganglion <img src='img/arrows/WC.png' alt='white curved arrow'/>, 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>, which extends through the widened labyrinthine segment facial nerve canal <img src='img/arrows/WO.png' alt='white open arrow'/> into the IAC fundus <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Facial Nerve Perineural Tumor**
|
||||||
|
*Coronal T1 MR shows invasive parotid space malignancy <img src='img/arrows/WS.png' alt='white solid arrow'/> with perineural tumor spread cephalad through the stylomastoid foramen to involve the mastoid segment of the facial nerve <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Facial Nerve Venous Malformation ("Hemangioma") in T-Bone**
|
||||||
|
*Axial bone CT shows a CNVII venous malformation <img src='img/arrows/WS.png' alt='white solid arrow'/> centered at the geniculate fossa with characteristic "honeycomb" matrix. The lesion extends toward the labyrinthine segment <img src='img/arrows/WC.png' alt='white curved arrow'/> of CNVII.*
|
||||||
|
|
||||||
|
|
||||||
|
**Acute Cerebral Ischemia-Infarction**
|
||||||
|
*Axial DWI MR shows an acute left pontine infarction <img src='img/arrows/WS.png' alt='white solid arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> of a demyelinating plaque <img src='img/arrows/WO.png' alt='white open arrow'/> 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 <img src='img/arrows/BS.png' alt='black solid arrow'/> in the left CPA with large intranidal aneurysm <img src='img/arrows/WO.png' alt='white open arrow'/>. Note early opacification of the left transverse sinus <img src='img/arrows/WC.png' alt='white curved arrow'/> due to arterial-to-venous shunting as well as intraventricular hemorrhage <img src='img/arrows/WS.png' alt='white solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Arachnoid Cyst in CPA**
|
||||||
|
*Axial T2 FS MR shows a right CPA arachnoid cyst <img src='img/arrows/WS.png' alt='white solid arrow'/>, which exerts mild mass effect upon the cisternal segments of the vestibulocochlear and facial nerves <img src='img/arrows/WO.png' alt='white open arrow'/>. 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> with focus of internal low signal intensity from punctate intralesional calcification <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
### 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> by an ectatic vertebral artery <img src='img/arrows/WO.png' alt='white open arrow'/>. Note the point of neurovascular impingement <img src='img/arrows/WC.png' alt='white curved arrow'/> against the adjacent cerebellar flocculus <img src='img/arrows/BO.png' alt='black open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Vascular Loop Syndrome Affecting CNVII**
|
||||||
|
*Coronal T2 MR shows an ectatic vertebral artery <img src='img/arrows/WO.png' alt='white open arrow'/> "lifting" the posterior inferior cerebellar artery into the REZ of the facial nerve <img src='img/arrows/WS.png' alt='white solid arrow'/>. Note compression of the lateral pons.*
|
||||||
|
|
||||||
|
|
||||||
|
**Vascular Loop Syndrome Affecting CNVII**
|
||||||
|
*Axial T2 MR shows a markedly asymmetric left vertebral artery <img src='img/arrows/WO.png' alt='white open arrow'/> lifting the PICA <img src='img/arrows/WS.png' alt='white solid arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> and lobulated mass effect on the lateral margin of the brachium pontis <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Epidermoid Cyst in CPA**
|
||||||
|
*Axial T2 MR shows a right CPA cistern epidermoid cyst <img src='img/arrows/WS.png' alt='white solid arrow'/> scalloping the cerebellar contour and bowing the cisternal facial nerve anteriorly <img src='img/arrows/WO.png' alt='white open arrow'/>. The REZ <img src='img/arrows/WC.png' alt='white curved arrow'/> 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) <img src='img/arrows/WS.png' alt='white solid arrow'/> "on cone" (IAC component) <img src='img/arrows/WO.png' alt='white open arrow'/>. Note enhancing dural tails <img src='img/arrows/WC.png' alt='white curved arrow'/>, 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 <img src='img/arrows/WO.png' alt='white open arrow'/> with a "CSF-vascular cleft" <img src='img/arrows/WS.png' alt='white solid arrow'/> between it and the adjacent brachium pontis-pons. Note the normal REZ of the contralateral left CNVII <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Aneurysm in CPA-IAC**
|
||||||
|
*Axial T2 FS MR shows a giant right vetebral artery aneurysm <img src='img/arrows/WS.png' alt='white solid arrow'/> with complex signal intensity. The aneurysm obliterates the pontomedullary junction at the region of the right CNVII REZ <img src='img/arrows/WO.png' alt='white open arrow'/>. The same tortuous right vertebral artery <img src='img/arrows/WC.png' alt='white curved arrow'/> also effaces the left CNVII REZ region.*
|
||||||
|
|
||||||
|
|
||||||
|
**Aneurysm in CPA-IAC**
|
||||||
|
*Axial T1 MR shows complex signal associated with a vertebral artery aneurysm <img src='img/arrows/WS.png' alt='white solid arrow'/>. 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>, labyrinthine segment <img src='img/arrows/WO.png' alt='white open arrow'/>, and geniculate ganglion <img src='img/arrows/WC.png' alt='white curved arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> and labyrinthine segment <img src='img/arrows/WO.png' alt='white open arrow'/> facial nerve canal. Compared to the normal contralateral facial nerve canal <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Facial Nerve Schwannoma in T-Bone**
|
||||||
|
*Axial T1 C+ MR shows an aggressive schwannoma involving the tympanic segment <img src='img/arrows/WS.png' alt='white solid arrow'/>, posterior genu <img src='img/arrows/WO.png' alt='white open arrow'/>, and descending mastoid segment of the right facial nerve. Note several nonenhancing intramural cysts <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Facial Nerve Schwannoma in T-Bone**
|
||||||
|
*Axial T1 C+ MR demonstrates a mastoid segment facial nerve schwannoma <img src='img/arrows/WS.png' alt='white solid arrow'/>. Notice that the enhancing tumor has dehisced the posterior wall of the external auditory canal <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**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 <img src='img/arrows/WS.png' alt='white solid arrow'/>. Note the normal appearance of the contralateral CNVII descending mastoid segment <img src='img/arrows/WO.png' alt='white open arrow'/>. 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> within enlarged geniculate fossa. Note the black central dot of low signal intensity <img src='img/arrows/WO.png' alt='white open arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> in the left lateral pons near the REZ of the facial nerve <img src='img/arrows/WO.png' alt='white open arrow'/>. Demyelinating lesions accounting for HFS are often subtle and may not always be seen.*
|
||||||
|
|
||||||
|
|
||||||
|
**Multiple Sclerosis**
|
||||||
|
*Axial T2 MR shows a multiple sclerosis plaque <img src='img/arrows/WS.png' alt='white solid arrow'/> situated in the lateral right pons near the REZ of the facial nerve. Note a 2nd more subtle plaque <img src='img/arrows/WO.png' alt='white open arrow'/> in the left cerebellum.*
|
||||||
|
|
||||||
|
|
||||||
|
**Arteriovenous Malformation**
|
||||||
|
*Axial T2 MR shows a left cerebellopontine cistern arteriovenous malformation nidus <img src='img/arrows/WO.png' alt='white open arrow'/> with a large posterior draining vein <img src='img/arrows/WC.png' alt='white curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
**Arachnoid Cyst in CPA**
|
||||||
|
*Axial T2 MR through the CPA cistern reveals a CSF intensity arachnoid cyst on the left <img src='img/arrows/WS.png' alt='white solid arrow'/>. The arachnoid cyst flattens the cerebellar hemisphere and bows the facial and vestibulocochlear nerves <img src='img/arrows/BO.png' alt='black open arrow'/> anteromedially.*
|
||||||
|
|
||||||
|
|
||||||
|
**Arachnoid Cyst in CPA**
|
||||||
|
*Axial T2 MR through the CPA cistern reveals a CSF intensity arachnoid cyst on the left <img src='img/arrows/WS.png' alt='white solid arrow'/>. The arachnoid cyst flattens the cerebellar hemisphere and bows the facial and vestibulocochlear nerves <img src='img/arrows/BO.png' alt='black open arrow'/> anteromedially.*
|
||||||
|
|
||||||
@@ -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
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lastUpdated: "02/06/24"
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pageDescription: "Herpes Encephalitis Type 1"
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pageKeywords: "Pediatrics, Diagnosis, Pediatric Neuroradiology, Brain, Pathology-Based Diagnoses, Infectious Disease, Acquired Infections, Herpes Encephalitis Type 1"
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pageTitle: "Herpes Encephalitis Type 1 | STATdx"
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|
enhancedTitle: "Herpes Encephalitis Type 1"
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type: "DX"
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references: true
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breadcrumbs:
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- "Pediatrics"
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|
- "Diagnosis"
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- "Pediatric Neuroradiology"
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- "Brain"
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- "Pathology-Based Diagnoses"
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- "Infectious Disease"
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- "Acquired Infections"
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- "Herpes Encephalitis Type 1"
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---
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## KEY FACTS
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|
||||||
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- ### Terminology
|
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|
||||||
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- **HSV1** brain infection
|
||||||
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- Typically reactivation in immunocompetent patients
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||||||
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- **Different from neonatal (HSV2)**herpes encephalitis
|
||||||
|
- ### Imaging
|
||||||
|
|
||||||
|
|
||||||
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- T2/FLAIR hyperintensity in **mesial temporal, subfrontal, insular, and cingular cortex** with DWI restriction
|
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|
- Typically **bilateral** disease **but asymmetric**
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|
- **Hemorrhage** typically subacute/**late**
|
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|
- 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
|
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|
- **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)
|
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|
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)
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|
|
||||||
|
|
||||||
|
## 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.*
|
||||||
|
|
||||||
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|
||||||
|
*Coronal T1 C+ MR in a subacute case of HSV1 herpes encephalitis shows striking bilateral but asymmetric enhancement in the mesial <img src='img/arrows/BO.png' alt='black open arrow'/> more than lateral temporal lobes <img src='img/arrows/BS.png' alt='black solid arrow'/> and insular cortex <img src='img/arrows/WS.png' alt='white solid arrow'/>. Subtle lesions are seen in the cingulate gyri <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Coronal T1 C+ MR in a subacute case of HSV1 herpes encephalitis shows striking bilateral but asymmetric enhancement in the mesial <img src='img/arrows/BO.png' alt='black open arrow'/> more than lateral temporal lobes <img src='img/arrows/BS.png' alt='black solid arrow'/> and insular cortex <img src='img/arrows/WS.png' alt='white solid arrow'/>. Subtle lesions are seen in the cingulate gyri <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial DWI MR in a patient with acute herpes encephalitis shows diffusion restriction in the right mesial <img src='img/arrows/BO.png' alt='black open arrow'/> and lateral temporal <img src='img/arrows/BS.png' alt='black solid arrow'/> lobes. Subtle diffusion restriction of the left hippocampus <img src='img/arrows/BC.png' alt='black curved arrow'/> is also seen.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial DWI MR in a patient with acute herpes encephalitis shows diffusion restriction in the right mesial <img src='img/arrows/BO.png' alt='black open arrow'/> and lateral temporal <img src='img/arrows/BS.png' alt='black solid arrow'/> lobes. Subtle diffusion restriction of the left hippocampus <img src='img/arrows/BC.png' alt='black curved arrow'/> is also seen.*
|
||||||
|
|
||||||
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|
||||||
|
*Axial FLAIR MR at a higher level in the same patient shows diffuse cortical swelling and hyperintensity in the right superior temporal lobe <img src='img/arrows/BS.png' alt='black solid arrow'/>, right insular cortex <img src='img/arrows/BO.png' alt='black open arrow'/>, and bilateral cingulate gyri <img src='img/arrows/BC.png' alt='black curved arrow'/>. 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 <img src='img/arrows/BS.png' alt='black solid arrow'/>, right insular cortex <img src='img/arrows/BO.png' alt='black open arrow'/>, and bilateral cingulate gyri <img src='img/arrows/BC.png' alt='black curved arrow'/>. 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 <img src='img/arrows/BC.png' alt='black curved arrow'/>, hippocampus <img src='img/arrows/BS.png' alt='black solid arrow'/>, uncus <img src='img/arrows/BO.png' alt='black open arrow'/>, and (unusually) anterior temporal pole <img src='img/arrows/CS.png' alt='cyan solid arrow'/> and lateral temporal lobe <img src='img/arrows/CC.png' alt='cyan curved arrow'/>. Note mild right amygdalar <img src='img/arrows/WC.png' alt='white curved arrow'/>, uncal <img src='img/arrows/WO.png' alt='white open arrow'/> involvement.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial FLAIR MR in child with HSV-1 herpes encephalitis shows typical left temporal lobe hyperintensity, swelling of amygdala <img src='img/arrows/BC.png' alt='black curved arrow'/>, hippocampus <img src='img/arrows/BS.png' alt='black solid arrow'/>, uncus <img src='img/arrows/BO.png' alt='black open arrow'/>, and (unusually) anterior temporal pole <img src='img/arrows/CS.png' alt='cyan solid arrow'/> and lateral temporal lobe <img src='img/arrows/CC.png' alt='cyan curved arrow'/>. Note mild right amygdalar <img src='img/arrows/WC.png' alt='white curved arrow'/>, uncal <img src='img/arrows/WO.png' alt='white open arrow'/> involvement.*
|
||||||
|
|
||||||
|
|
||||||
|
*Sagittal T1 MR in patient 2 weeks after initial presentation of herpes encephalitis shows volume loss in temporal lobe <img src='img/arrows/BO.png' alt='black open arrow'/>, linear gyriform T1 hyperintensity <img src='img/arrows/BS.png' alt='black solid arrow'/>, characteristic of subacute cortical hemorrhage.*
|
||||||
|
|
||||||
|
|
||||||
|
*Sagittal T1 MR in patient 2 weeks after initial presentation of herpes encephalitis shows volume loss in temporal lobe <img src='img/arrows/BO.png' alt='black open arrow'/>, linear gyriform T1 hyperintensity <img src='img/arrows/BS.png' alt='black solid arrow'/>, characteristic of subacute cortical hemorrhage.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial T1 C+ MR in the subacute stage of herpes encephalitis shows bilateral but asymmetric hypointensities <img src='img/arrows/WS.png' alt='white solid arrow'/> in the insular regions. There is associated pial enhancement in the left insular region <img src='img/arrows/WC.png' alt='white curved arrow'/>. Pial, leptomeningeal, diffuse, ring-like enhancement patterns have been reported in herpes encephalitis.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial SWI MR in the same patient shows patchy hypointense hemorrhage <img src='img/arrows/BS.png' alt='black solid arrow'/> 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 <img src='img/arrows/WC.png' alt='white curved arrow'/> in the right medial temporal lobe.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial T2 MR in the same patient shows signal abnormality <img src='img/arrows/WC.png' alt='white curved arrow'/> 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 <img src='img/arrows/CO.png' alt='cyan open arrow'/> in an older adult woman with fever, confusion, decreased consciousness, and seizure.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial CECT in the same patient shows the involvement of insular cortex <img src='img/arrows/CO.png' alt='cyan open arrow'/>. 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 <img src='img/arrows/CC.png' alt='cyan curved arrow'/> 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 <img src='img/arrows/CC.png' alt='cyan curved arrow'/> 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial DWI MR in the same patient shows restricted diffusion in both mesial temporal lobes <img src='img/arrows/CS.png' alt='cyan solid arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial T1 MR in a case of herpes encephalitis imaged several days after symptom onset shows gyral hyperintensity <img src='img/arrows/CS.png' alt='cyan solid arrow'/>, 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/>, 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 <img src='img/arrows/CS.png' alt='cyan solid arrow'/> and subtle enhancement in the right temporal lobe cortex <img src='img/arrows/CO.png' alt='cyan open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial T1 C+ MR in the same patient shows striking gyriform enhancement in the left temporal lobe <img src='img/arrows/CS.png' alt='cyan solid arrow'/> and subtle enhancement in the right temporal lobe cortex <img src='img/arrows/CO.png' alt='cyan open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Coronal FLAIR MR shows classic bilateral, asymmetric involvement of the medial temporal lobes <img src='img/arrows/WS.png' alt='white solid arrow'/> and right insula <img src='img/arrows/WC.png' alt='white curved arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> and right insula <img src='img/arrows/WC.png' alt='white curved arrow'/> 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 <img src='img/arrows/CC.png' alt='cyan curved arrow'/> 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 <img src='img/arrows/CC.png' alt='cyan curved arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> and anterior cingulate gyrus <img src='img/arrows/WC.png' alt='white curved arrow'/>. 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> and anterior cingulate gyrus <img src='img/arrows/WC.png' alt='white curved arrow'/>. 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 <img src='img/arrows/WS.png' alt='white solid arrow'/>. Subtle enhancement of the cingulate gyri <img src='img/arrows/WC.png' alt='white curved arrow'/> is also noted. Also note some right temporal lobe abnormal cortical enhancement <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial T1 C+ MR in the same patient shows bilateral, asymmetric enhancement of the insular cortex <img src='img/arrows/WS.png' alt='white solid arrow'/>. Subtle enhancement of the cingulate gyri <img src='img/arrows/WC.png' alt='white curved arrow'/> is also noted. Also note some right temporal lobe abnormal cortical enhancement <img src='img/arrows/WO.png' alt='white open arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial T2 MR in an older adult with fever and confusion shows diffuse swelling and hyperintensity in the right mesial <img src='img/arrows/WS.png' alt='white solid arrow'/> and lateral <img src='img/arrows/WC.png' alt='white curved arrow'/> temporal lobe and orbitofrontal gyrus <img src='img/arrows/CC.png' alt='cyan curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial T2 MR in an older adult with fever and confusion shows diffuse swelling and hyperintensity in the right mesial <img src='img/arrows/WS.png' alt='white solid arrow'/> and lateral <img src='img/arrows/WC.png' alt='white curved arrow'/> temporal lobe and orbitofrontal gyrus <img src='img/arrows/CC.png' alt='cyan curved arrow'/>.*
|
||||||
|
|
||||||
|
|
||||||
|
*Axial FLAIR MR in the same patient shows marked edema and hyperintensity in the right mesial <img src='img/arrows/WS.png' alt='white solid arrow'/> and lateral <img src='img/arrows/WC.png' alt='white curved arrow'/> 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 <img src='img/arrows/WS.png' alt='white solid arrow'/> and lateral <img src='img/arrows/WC.png' alt='white curved arrow'/> 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 <img src='img/arrows/BO.png' alt='black open arrow'/> 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 <img src='img/arrows/BO.png' alt='black open arrow'/> 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.*
|
||||||
|
|
||||||
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Reference in New Issue
Block a user