327 lines
19 KiB
Markdown
327 lines
19 KiB
Markdown
---
|
||
title: "AHLE"
|
||
docid: "0ec0bca6-abee-4931-a6ed-43541b626261"
|
||
authors:
|
||
- key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
|
||
value: "Anne G. Osborn, MD, FACR"
|
||
breadcrumbs:
|
||
-
|
||
name: "Brain"
|
||
slug: "brain"
|
||
treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a"
|
||
-
|
||
name: "Diagnosis"
|
||
slug: "diagnosis"
|
||
treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8"
|
||
-
|
||
name: "Pathology-Based Diagnoses"
|
||
slug: "pathology-based-diagnoses"
|
||
treeNodeId: "d9d3a8ed-f21b-4831-8c77-591a3500ef77"
|
||
-
|
||
name: "Infectious, Inflammatory, and Demyelinating Disease"
|
||
slug: "infectious-inflammatory-and-demyel-"
|
||
treeNodeId: "7210f860-fe5f-4a2d-81cc-4fe06c769607"
|
||
-
|
||
name: "Inflammatory and Demyelinating Disease"
|
||
slug: "inflammatory-and-demyelinating-dis-"
|
||
treeNodeId: "62ab4dc3-dbf6-45a9-8532-f0e962aa62dc"
|
||
-
|
||
name: "AHLE"
|
||
slug: "ahle"
|
||
treeNodeId: null
|
||
category: "Brain"
|
||
documentVersionId: "05d076c7-6110-4f03-952a-c22726d85e4d"
|
||
imageCount: 12
|
||
lastUpdated: "08/05/20"
|
||
pageDescription: "AHLE"
|
||
pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, AHLE"
|
||
pageTitle: "AHLE | STATdx"
|
||
enhancedTitle: "AHLE"
|
||
type: "DX"
|
||
references: true
|
||
breadcrumbs:
|
||
- "Brain"
|
||
- "Diagnosis"
|
||
- "Pathology-Based Diagnoses"
|
||
- "Infectious, Inflammatory, and Demyelinating Disease"
|
||
- "Inflammatory and Demyelinating Disease"
|
||
- "AHLE"
|
||
---
|
||
# KEY FACTS
|
||
|
||
- ## Terminology
|
||
|
||
|
||
- Hyperacute, fulminant hemorrhagic perivascular demyelinating disorder
|
||
- Usually viral or immunization related
|
||
- ## Imaging
|
||
|
||
|
||
- Best imaging: MR with T2* GRE, SWI
|
||
- CT: May be normal if only microbleeds present
|
||
- MR
|
||
- Multifocal scattered or confluent WM hyperintensities on T2/FLAIR
|
||
- T2* shows multifocal blooming microbleeds in WM (often striking sparing of cortex)
|
||
- Most striking in corpus callosum
|
||
- Less common: Large, lobar, confluent hemorrhages
|
||
- SWI significantly more sensitive than GRE
|
||
- ## Top Differential Diagnoses
|
||
|
||
|
||
- Acute disseminated encephalomyelitis (ADEM)
|
||
- Multiple sclerosis
|
||
- Acute necrotizing encephalopathy
|
||
- Other brain "microbleeds"
|
||
- Critical illness associated (e.g., ARDS ± on ECMO)
|
||
- Trauma (DAI), fat emboli, HUS/TTP
|
||
- Sepsis, vasculitis, hemorrhagic viral fevers
|
||
- High-altitude cerebral edema
|
||
- ## Clinical Issues
|
||
|
||
|
||
- Demographics
|
||
- AHLE represents ~ 2% of ADEM cases
|
||
- All ages but children, young adults most common
|
||
- Presentation and course
|
||
- Fever, then rapid neurologic deterioration
|
||
- 60-80% mortality without treatment
|
||
- ## Diagnostic Checklist
|
||
|
||
|
||
- T2* (GRE or SWI) MR in all febrile CNS illnesses with rapid clinical deterioration
|
||
|
||
# TERMINOLOGY
|
||
|
||
- ## Abbreviations
|
||
|
||
|
||
- Acute hemorrhagic leukoencephalitis (AHLE)
|
||
- Acute disseminated encephalomyelopathy (ADEM)
|
||
- ## Synonyms
|
||
|
||
|
||
- Acute hemorrhagic encephalomyelitis (AHEM)
|
||
- Weston-Hurst disease
|
||
- ## Definitions
|
||
|
||
|
||
- Hyperacute, fulminant, hemorrhagic perivascular demyelinating disorder
|
||
- Usually post viral or immunization related
|
||
- May be exceptionally severe, fulminant form of ADEM
|
||
|
||
# IMAGING
|
||
|
||
- ## General Features
|
||
|
||
|
||
- ### Best diagnostic clue
|
||
|
||
|
||
- T2* shows multifocal petechial white matter (WM) microhemorrhages with striking sparing of cortex
|
||
- Less common: Large, lobar, confluent hemorrhages
|
||
- ### Location
|
||
|
||
|
||
- WM
|
||
- Corpus callosum, subcortical WM (U fibers)
|
||
- Less common: Basal ganglia, midbrain, pons, cerebellum
|
||
- ### Size
|
||
|
||
|
||
- Punctate microbleeds > large, lobar hemorrhages
|
||
- ## CT Findings
|
||
|
||
|
||
- ### NECT
|
||
|
||
|
||
- May be normal if only microbleeds present
|
||
- ± WM edema with hypodensity
|
||
- ## MR Findings
|
||
|
||
|
||
- ### T1WI
|
||
|
||
|
||
- Often normal
|
||
- ### T2WI
|
||
|
||
|
||
- Multifocal scattered or confluent WM hyperintensities
|
||
- Most striking in corpus callosum
|
||
- May have reversible corpus callosum splenium lesion
|
||
- ### T2* GRE
|
||
|
||
|
||
- Multifocal blooming microbleeds
|
||
- SWI significantly more sensitive than GRE
|
||
- ## Imaging Recommendations
|
||
|
||
|
||
- ### Best imaging tool
|
||
|
||
|
||
- MR with T2* GRE, SWI
|
||
|
||
# DIFFERENTIAL DIAGNOSIS
|
||
|
||
- [Acute Disseminated Encephalomyelitis](/document/adem/a3fafeb7-5861-4364-beb8-c0e30220564e)
|
||
- AHLE may be most severe form of ADEM
|
||
- ADEM usually less fulminant
|
||
- ADEM lacks lobar or perivascular hemorrhages of AHLE
|
||
- [Multiple Sclerosis](/document/multiple-sclerosis/7892b2a2-f52a-4d7f-9858-a326f2b7ab04)
|
||
- Acute fulminant multiple sclerosis
|
||
- Lacks hemorrhage, high fever, marked leukocytosis
|
||
- ## Acute Necrotizing Encephalopathy
|
||
|
||
|
||
- Rare complication of acute viral infection, such as influenza A
|
||
- Children < 4 years most common
|
||
- Bilateral, symmetric lesions in thalami typical
|
||
- WM predominance rare
|
||
- ## Other Etiologies of Brain Microbleeds
|
||
|
||
|
||
- Diffuse, traumatic vascular injury
|
||
- Critical illness-associated (e.g., ARDS ± on ECMO)
|
||
- Sepsis, vasculitis
|
||
- Fat emboli
|
||
- HUS/TTP
|
||
- Hemorrhagic infections
|
||
- Viral (H1N1, coronavirus), malaria, rickettsia
|
||
- High-altitude cerebral edema
|
||
|
||
# PATHOLOGY
|
||
|
||
- ## Gross Pathologic & Surgical Features
|
||
|
||
|
||
- Predominantly involves WM of brain ± spinal cord
|
||
- May affect basal ganglia but usually spares cortical gray matter
|
||
- Focal confluent &/or multifocal petechial WM hemorrhages
|
||
- ## Microscopic Features
|
||
|
||
|
||
- Fibrinoid necrosis of vessel walls
|
||
- Perivascular demyelination, hemorrhages
|
||
|
||
# CLINICAL ISSUES
|
||
|
||
- ## Presentation
|
||
|
||
|
||
- ### Most common signs/symptoms
|
||
|
||
|
||
- Fever, then rapid neurologic deterioration
|
||
- Somnolence, impaired consciousness
|
||
- ### Other signs/symptoms
|
||
|
||
|
||
- Long-tract signs
|
||
- ## Demographics
|
||
|
||
|
||
- AHLE represents ~ 2% of ADEM cases
|
||
- All ages but children, young adults most common
|
||
- ## Natural History & Prognosis
|
||
|
||
|
||
- Rapid clinical deterioration; death within days typical course
|
||
- Mortality: 60-80%
|
||
- ## Treatment
|
||
|
||
|
||
- Aggressive IV steroids, immunoglobulin, plasmapheresis
|
||
|
||
# DIAGNOSTIC CHECKLIST
|
||
|
||
- ## Consider
|
||
|
||
|
||
- T2* (GRE or SWI) MR in all febrile CNS illnesses with rapid clinical deterioration
|
||
- ## Image Interpretation Pearls
|
||
|
||
|
||
- Corpus callosum, subcortical WM microbleeds with striking sparing of overlying cortex typical for AHLE
|
||
|
||
8f325204-5ce7-4da8-b816-cb545fa5b054
|
||
|
||
## References
|
||
|
||
# Selected References
|
||
|
||
1. [Mondia MWL et al: Acute hemorrhagic leukoencephalitis of Weston Hurst secondary to herpes encephalitis presenting as status epilepticus: a case report and review of literature. J Clin Neurosci. 67:265-70, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31239199%5Bpmid%5D)
|
||
1. [Yae Y et al: Fulminant acute disseminated encephalomyelitis in children. Brain Dev. 41(4):373-7, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30522797%5Bpmid%5D)
|
||
1. [Bonduelle T et al: Weston-Hurst syndrome with acute hemorrhagic cerebellitis. Clin Neurol Neurosurg. 173:118-9, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30121019%5Bpmid%5D)
|
||
1. [Fanou EM et al: Critical illness-associated cerebral microbleeds. Stroke. 48(4):1085-7, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28235962%5Bpmid%5D)
|
||
1. [Nabi S et al: Weston-Hurst syndrome: a rare fulminant form of acute disseminated encephalomyelitis (ADEM). BMJ Case Rep. 2016, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27797801%5Bpmid%5D)
|
||
1. [Jeganathan N et al: Acute hemorrhagic leukoencephalopathy associated with influenza A (H1N1) virus. Neurocrit Care. 19(2):218-21, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23943349%5Bpmid%5D)
|
||
|
||
|
||
## Images
|
||
|
||
|
||
### Selected Images
|
||
|
||
|
||
*Close-up view of autopsied brain in a patient with acute hemorrhagic leukoencephalitis (AHLE) shows innumerable tiny microbleeds in the subcortical and deep white matter (WM) <img src='img/arrows/BO.png'/> and corpus callosum <img src='img/arrows/BS.png'/>. Note the overlying cortex is almost completely spared <img src='img/arrows/BC.png'/>. (Courtesy E. Rushing, MD.)*
|
||
|
||
|
||
*Close-up view of autopsied brain in a patient with acute hemorrhagic leukoencephalitis (AHLE) shows innumerable tiny microbleeds in the subcortical and deep white matter (WM) <img src='img/arrows/BO.png'/> and corpus callosum <img src='img/arrows/BS.png'/>. Note the overlying cortex is almost completely spared <img src='img/arrows/BC.png'/>. (Courtesy E. Rushing, MD.)*
|
||
|
||
|
||
*Close-up view of autopsied brain in a patient with acute hemorrhagic leukoencephalitis (AHLE) shows innumerable tiny microbleeds in the subcortical and deep white matter (WM) <img src='img/arrows/BO.png'/> and corpus callosum <img src='img/arrows/BS.png'/>. Note the overlying cortex is almost completely spared <img src='img/arrows/BC.png'/>. (Courtesy E. Rushing, MD.)*
|
||
|
||
|
||
*Axial FLAIR MR in a 28-year-old man with rapidly declining mental status after a flu-like illness shows patchy hyperintensities in the corpus callosum <img src='img/arrows/WC.png'/> and deep/subcortical WM <img src='img/arrows/WS.png'/>.*
|
||
|
||
|
||
*Axial FLAIR MR in a 28-year-old man with rapidly declining mental status after a flu-like illness shows patchy hyperintensities in the corpus callosum <img src='img/arrows/WC.png'/> and deep/subcortical WM <img src='img/arrows/WS.png'/>.*
|
||
|
||
|
||
*Axial T2* GRE MR in the same patient shows multiple tiny hypointensities in the corpus callosum <img src='img/arrows/BC.png'/> and deep/subcortical WM <img src='img/arrows/BS.png'/>. The cortex is largely spared.*
|
||
|
||
|
||
*Axial T2* GRE MR in the same patient shows multiple tiny hypointensities in the corpus callosum <img src='img/arrows/BC.png'/> and deep/subcortical WM <img src='img/arrows/BS.png'/>. The cortex is largely spared.*
|
||
|
||
|
||
*Axial T2* SWI MR MIP shows the innumerable microbleeds in the corpus callosum <img src='img/arrows/CC.png'/> with diffuse involvement of the hemispheric WM <img src='img/arrows/CS.png'/> in this patient with AHLE.*
|
||
|
||
|
||
*Axial T2* SWI MR MIP shows the innumerable microbleeds in the corpus callosum <img src='img/arrows/CC.png'/> with diffuse involvement of the hemispheric WM <img src='img/arrows/CS.png'/> in this patient with AHLE.*
|
||
|
||
|
||
### Additional Images
|
||
|
||
|
||
*Axial FLAIR MR in a 25-year-old man with fever and rapidly decreasing mental status shows no definite abnormalities.*
|
||
|
||
|
||
*Axial FLAIR MR in a 25-year-old man with fever and rapidly decreasing mental status shows no definite abnormalities.*
|
||
|
||
|
||
*Axial T2* GRE MR in the same patient shows a few punctate "blooming" foci in the corpus callosum genu <img src='img/arrows/WO.png'/> and splenium <img src='img/arrows/WS.png'/>. The remainder of the WM appears normal.*
|
||
|
||
|
||
*Axial T2* GRE MR in the same patient shows a few punctate "blooming" foci in the corpus callosum genu <img src='img/arrows/WO.png'/> and splenium <img src='img/arrows/WS.png'/>. The remainder of the WM appears normal.*
|
||
|
||
|
||
*Axial SWI MR n the same patient shows innumerable tiny "blooming" microbleeds in the corpus callosum <img src='img/arrows/BO.png'/> and subcortical and deep WM <img src='img/arrows/BS.png'/>. The cortex is largely spared.*
|
||
|
||
|
||
*More cephalad axial T2* SWI MR shows innumerable tiny "blooming" foci in the WM, especially in the corpus callosum <img src='img/arrows/BC.png'/>. These imaging findings are characteristic of AHLE.*
|
||
|
||
|
||
*Autopsy shows 2 areas of gross hemorrhagic necrosis <img src='img/arrows/BS.png'/>. These findings and clinical history of prior flu-like illness with rapidly progressive fatal clinical course are characteristic of AHLE. (Courtesy R. Hewlett, MD).*
|
||
|
||
|
||
*Axial T2* GRE MR in a patient with rapid decline after a flu-like illness shows a large left frontal hemorrhage <img src='img/arrows/WO.png'/> with numerous "blooming" foci in multiple WM lesions <img src='img/arrows/BS.png'/>. Diagnosis was AHLE. (Courtesy R. Ramakantan, MD).*
|
||
|
||
|
||
*Axial NECT in a patient with AHLE shows patchy hemorrhages in the corpus callosum splenium <img src='img/arrows/WS.png'/>.*
|
||
|
||
|
||
*Axial T2* SWI MR MIP in the same patient shows the microbleeds are heavily concentrated in the corpus callosum <img src='img/arrows/BC.png'/> and hemispheric WM <img src='img/arrows/BS.png'/>. Note involvement of the internal capsules and relative sparing of the basal ganglia and cortex.*
|
||
|