ross/statdx
1
0
Fork 0
Code Issues Pull Requests Actions Packages Projects Releases Wiki Activity
Files
master
statdx/docs_md/articles/ahle_0ec0bca6-abee-4931-a6ed-43541b626261.md
T
Ross 1e36a4957c .
2025-10-20 08:40:33 +01:00

19 KiB
Raw Permalink Blame History

title, docid, authors, breadcrumbs, category, documentVersionId, imageCount, lastUpdated, pageDescription, pageKeywords, pageTitle, enhancedTitle, type, references, breadcrumbs
title docid authors breadcrumbs category documentVersionId imageCount lastUpdated pageDescription pageKeywords pageTitle enhancedTitle type references breadcrumbs
AHLE 0ec0bca6-abee-4931-a6ed-43541b626261
key value
5cff4116-3654-4b3a-bb75-5ebe0b8c9850 Anne G. Osborn, MD, FACR
name slug treeNodeId
Brain brain 6d8829f1-14d7-45af-8675-255189aa526a
name slug treeNodeId
Diagnosis diagnosis 51c00394-446e-4a38-94af-d3b1d14d34e8
name slug treeNodeId
Pathology-Based Diagnoses pathology-based-diagnoses d9d3a8ed-f21b-4831-8c77-591a3500ef77
name slug treeNodeId
Infectious, Inflammatory, and Demyelinating Disease infectious-inflammatory-and-demyel- 7210f860-fe5f-4a2d-81cc-4fe06c769607
name slug treeNodeId
Inflammatory and Demyelinating Disease inflammatory-and-demyelinating-dis- 62ab4dc3-dbf6-45a9-8532-f0e962aa62dc
name slug treeNodeId
AHLE ahle null
Brain 05d076c7-6110-4f03-952a-c22726d85e4d 12 08/05/20 AHLE Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, AHLE AHLE | STATdx AHLE DX true
Brain
Diagnosis
Pathology-Based Diagnoses
Infectious, Inflammatory, and Demyelinating Disease
Inflammatory and Demyelinating Disease
AHLE

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

    • AHLE may be most severe form of ADEM
    • ADEM usually less fulminant
    • ADEM lacks lobar or perivascular hemorrhages of AHLE

  • Multiple Sclerosis

    • 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
  2. Yae Y et al: Fulminant acute disseminated encephalomyelitis in children. Brain Dev. 41(4):373-7, 2019
  3. Bonduelle T et al: Weston-Hurst syndrome with acute hemorrhagic cerebellitis. Clin Neurol Neurosurg. 173:118-9, 2018
  4. Fanou EM et al: Critical illness-associated cerebral microbleeds. Stroke. 48(4):1085-7, 2017
  5. Nabi S et al: Weston-Hurst syndrome: a rare fulminant form of acute disseminated encephalomyelitis (ADEM). BMJ Case Rep. 2016, 2016
  6. Jeganathan N et al: Acute hemorrhagic leukoencephalopathy associated with influenza A (H1N1) virus. Neurocrit Care. 19(2):218-21, 2013

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) and corpus callosum . Note the overlying cortex is almost completely spared . (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) and corpus callosum . Note the overlying cortex is almost completely spared . (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) and corpus callosum . Note the overlying cortex is almost completely spared . (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) and corpus callosum . Note the overlying cortex is almost completely spared . (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) and corpus callosum . Note the overlying cortex is almost completely spared . (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) and corpus callosum . Note the overlying cortex is almost completely spared . (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 and deep/subcortical WM . 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 and deep/subcortical WM .

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 and deep/subcortical WM . 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 and deep/subcortical WM .

Axial T2* GRE MR in the same patient shows multiple tiny hypointensities in the corpus callosum and deep/subcortical WM . The cortex is largely spared. Axial T2 GRE MR in the same patient shows multiple tiny hypointensities in the corpus callosum and deep/subcortical WM . The cortex is largely spared.*

Axial T2* GRE MR in the same patient shows multiple tiny hypointensities in the corpus callosum and deep/subcortical WM . The cortex is largely spared. Axial T2 GRE MR in the same patient shows multiple tiny hypointensities in the corpus callosum and deep/subcortical WM . The cortex is largely spared.*

Axial T2* SWI MR MIP shows the innumerable microbleeds in the corpus callosum with diffuse involvement of the hemispheric WM in this patient with AHLE. Axial T2 SWI MR MIP shows the innumerable microbleeds in the corpus callosum with diffuse involvement of the hemispheric WM in this patient with AHLE.*

Axial T2* SWI MR MIP shows the innumerable microbleeds in the corpus callosum with diffuse involvement of the hemispheric WM in this patient with AHLE. Axial T2 SWI MR MIP shows the innumerable microbleeds in the corpus callosum with diffuse involvement of the hemispheric WM 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 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 and splenium . 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 and splenium . 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 and splenium . 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 and splenium . The remainder of the WM appears normal.*

Axial SWI MR n the same patient shows innumerable tiny "blooming" microbleeds in the corpus callosum and subcortical and deep WM . The cortex is largely spared. Axial SWI MR n the same patient shows innumerable tiny "blooming" microbleeds in the corpus callosum and subcortical and deep WM . The cortex is largely spared.

More cephalad axial T2* SWI MR shows innumerable tiny "blooming" foci in the WM, especially in the corpus callosum . These imaging findings are characteristic of AHLE. More cephalad axial T2 SWI MR shows innumerable tiny "blooming" foci in the WM, especially in the corpus callosum . These imaging findings are characteristic of AHLE.*

Autopsy shows 2 areas of gross hemorrhagic necrosis . These findings and clinical history of prior flu-like illness with rapidly progressive fatal clinical course are characteristic of AHLE. (Courtesy R. Hewlett, MD). Autopsy shows 2 areas of gross hemorrhagic necrosis . 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 with numerous "blooming" foci in multiple WM lesions . Diagnosis was AHLE. (Courtesy R. Ramakantan, MD). Axial T2 GRE MR in a patient with rapid decline after a flu-like illness shows a large left frontal hemorrhage with numerous "blooming" foci in multiple WM lesions . Diagnosis was AHLE. (Courtesy R. Ramakantan, MD).*

Axial NECT in a patient with AHLE shows patchy hemorrhages in the corpus callosum splenium . Axial NECT in a patient with AHLE shows patchy hemorrhages in the corpus callosum splenium .

Axial T2* SWI MR MIP in the same patient shows the microbleeds are heavily concentrated in the corpus callosum and hemispheric WM . Note involvement of the internal capsules and relative sparing of the basal ganglia and cortex. Axial T2 SWI MR MIP in the same patient shows the microbleeds are heavily concentrated in the corpus callosum and hemispheric WM . Note involvement of the internal capsules and relative sparing of the basal ganglia and cortex.*

Reference in New Issue View Git Blame Copy Permalink