AHL
MCID: ACT032
MIFTS: 38

Acute Hemorrhagic Leukoencephalitis (AHL) malady

Categories: Rare diseases, Neuronal diseases

Aliases & Classifications for Acute Hemorrhagic Leukoencephalitis

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Aliases & Descriptions for Acute Hemorrhagic Leukoencephalitis:

Name: Acute Hemorrhagic Leukoencephalitis 11 48 13
Leukoencephalitis, Acute Hemorrhagic 39 68
Ahl 11 48
Acute Haemorrhagic Leucoencephalitis of Weston Hurst 11
 
Acute Necrotizing Hemorrhagic Leukoencephalitis 11
Acute Hemorrhagic Encephalomyelitis 11
Weston-Hurst Syndrome 11
Ahle 48

Classifications:



External Ids:

Disease Ontology11 DOID:10992
MeSH39 D004684
SNOMED-CT62 72986009
NCIt45 C84535

Summaries for Acute Hemorrhagic Leukoencephalitis

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NIH Rare Diseases:48 Acute hemorrhagic leukoencephalitis (ahl) is a very rare form of acute disseminated encephalomyelitis that usually results in death. it is characterized by a brief but intense attack of inflammation in the brain and spinal cord that damages the myelin -- the protective covering of the nerve fibers. it may also cause bleeding in the brain, leading to damage of the white matter. symptoms usually come on quickly, beginning with symptoms such as fever, neck stiffness, fatigue, headache, nausea vomiting, seizures, and coma. ahl has a very poor prognosis, with rapid deterioration and death usually occurring within days to one week after onset of symptoms because of severe inflammation in the brain. although the exact cause is unclear, ahl usually follows a viral infection, or less often, vaccination for measles, mumps, or rubella. some researchers think that an infection or vaccination can initiate an autoimmune process in the body thus leading to ahl. last updated: 8/19/2011

MalaCards based summary: Acute Hemorrhagic Leukoencephalitis, also known as leukoencephalitis, acute hemorrhagic, is related to ehlers-danlos syndrome, kyphoscoliotic form and childhood brainstem astrocytoma. An important gene associated with Acute Hemorrhagic Leukoencephalitis is CFI (Complement Factor I), and among its related pathways are Spinal Cord Injury and Glypican 1 network. Affiliated tissues include brain, spinal cord and cerebellum, and related mouse phenotypes are reproductive system and cardiovascular system.

Disease Ontology:11 A very rare form of acute disseminated encephalomyelitis, characterized by a brief but intense attack of inflammation and necrotizing vasculitis of venules and hemorrhage, and edema.

Related Diseases for Acute Hemorrhagic Leukoencephalitis

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Diseases related to Acute Hemorrhagic Leukoencephalitis via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 34)
idRelated DiseaseScoreTop Affiliating Genes
1ehlers-danlos syndrome, kyphoscoliotic form10.1AQP1, AQP4
2childhood brainstem astrocytoma10.1AQP1, AQP4, MBP
3niemann-pick disease10.1AQP1, AQP4, MBP
4crest syndrome10.1AQP4, MBP
5hand, foot and mouth disease10.0KDR, VEGFA
6herpes simplex10.0
7neurilemmoma of the pleura10.0KDR, NRP1, VEGFA
8placental choriocarcinoma10.0CFI, KDR, VEGFA
9laryngeal adenoid cystic carcinoma10.0FLT1, VEGFA
10multinodular goiter10.0IL1RN, KDR, VEGFA
11cone-rod dystrophy 1710.0KDR, VEGFA
12kidney osteogenic sarcoma10.0FLT1, KDR, VEGFA
13neuropathy with hearing impairment10.0FLT1, KDR, VEGFA
14relapsing-remitting multiple sclerosis9.9
15mitral valve stenosis9.9FLT1, KDR, VEGFA
16subdural empyema9.9FLT1, KDR, VEGFA
17histiocytoid hemangioma9.9AQP1, AQP4, VEGFA
18vulvitis9.9FLT1, KDR, VEGFA
19human granulocytic anaplasmosis9.9FLT1, KDR, VEGFA
20citrullinemia, type ii, neonatal-onset9.9FLT1, KDR, VEGFA
21hemolytic anemia9.9FLT1, KDR, VEGFA
22schwannoma of twelfth cranial nerve9.9AQP4, CD8A, MBP
23albinism, oculocutaneous, type ib9.9FLT1, KDR, NRP1, VEGFA
24vernal conjunctivitis9.8FLT1, KDR, OCLN, VEGFA
25brain injury9.8
26mumps9.8
27tuberculosis9.8
28myopathy9.8
29influenza9.8
30encephalitis9.8
31herpes simplex encephalitis9.8
32camptodactyly-arthropathy-coxa vara-pericarditis syndrome9.8CFI, FLT1, KDR, VEGFA
33thymus adenosquamous carcinoma9.7FLT1, KDR, VEGFA
34ovarian disease8.3AQP1, AQP4, CD8A, CFI, FASLG, FLT1

Graphical network of the top 20 diseases related to Acute Hemorrhagic Leukoencephalitis:



Diseases related to acute hemorrhagic leukoencephalitis

Symptoms & Phenotypes for Acute Hemorrhagic Leukoencephalitis

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MGI Mouse Phenotypes related to Acute Hemorrhagic Leukoencephalitis according to GeneCards Suite gene sharing:

41
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053898.3AQP1, FASLG, FLT1, IL1RN, MBP, OCLN
2MP:00053858.1AQP1, AQP4, FASLG, FLT1, IL1RN, KDR
3MP:00053788.0AQP1, AQP4, FLT1, IL1RN, KDR, MBP
4MP:00053767.4AQP1, AQP4, CFI, FASLG, FLT1, IL1RN
5MP:00053917.3AQP1, AQP4, FASLG, FLT1, KDR, MBP
6MP:00036316.9AQP1, AQP4, CD8A, FASLG, FLT1, KDR
7MP:00107686.9AQP1, AQP4, CD8A, FASLG, FLT1, IL1RN
8MP:00053876.9AQP4, CD8A, FASLG, FLT1, IL1RN, KDR
9MP:00053976.7AQP1, AQP4, CD8A, CFI, FASLG, FLT1

Drugs & Therapeutics for Acute Hemorrhagic Leukoencephalitis

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Interventional clinical trials:

Search ClinicalTrials, NIH Clinical Center for Acute Hemorrhagic Leukoencephalitis


Cochrane evidence based reviews: leukoencephalitis, acute hemorrhagic

Genetic Tests for Acute Hemorrhagic Leukoencephalitis

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Anatomical Context for Acute Hemorrhagic Leukoencephalitis

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MalaCards organs/tissues related to Acute Hemorrhagic Leukoencephalitis:

36
Brain, Spinal cord, Cerebellum

Publications for Acute Hemorrhagic Leukoencephalitis

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Articles related to Acute Hemorrhagic Leukoencephalitis:

(show all 49)
idTitleAuthorsYear
1
Acute Hemorrhagic Leukoencephalitis in Children: A Case Report. (27217610)
2016
2
Commentary: Acute Hemorrhagic Leukoencephalitis (Weston-Hurst Syndrome) in a Patient with Relapse-Remitting Multiple Sclerosis. (27252706)
2016
3
Acute hemorrhagic leukoencephalitis (Weston-Hurst syndrome) in a patient with relapse-remitting multiple sclerosis. (26376717)
2015
4
Acute hemorrhagic leukoencephalitis associated with autoimmune myopathy. (25422709)
2014
5
Autopsy-proven Acute Hemorrhagic Leukoencephalitis in an Elderly Patient. (24384347)
2014
6
First case report of acute hemorrhagic leukoencephalitis following Plasmodium vivax infection. (23508437)
2013
7
Mutations of complement factor I and potential mechanisms of neuroinflammation in acute hemorrhagic leukoencephalitis. (22926405)
2013
8
Value of susceptibility-weighted imaging in acute hemorrhagic leukoencephalitis. (22465779)
2012
9
Serial computed tomography and magnetic resonance imaging findings of biphasic acute hemorrhagic leukoencephalitis localized to the brain stem and cerebellum. (21519996)
2011
10
Acute hemorrhagic leukoencephalitis with severe brainstem and spinal cord involvement: MRI features with neuropathological confirmation. (21448963)
2011
11
Atypical acute hemorrhagic leukoencephalitis (Hurst's disease) presenting with focal hemorrhagic brainstem lesion. (19847676)
2010
12
Acute hemorrhagic leukoencephalitis: a critical entity for forensic pathologists to recognize. (20010289)
2010
13
Acute hemorrhagic leukoencephalitis and hypoxic brain injury associated with H1N1 influenza. (20558397)
2010
14
Acute hemorrhagic leukoencephalitis with atypical features. (19145402)
2009
15
Teaching NeuroImages: Acute hemorrhagic leukoencephalitis after mumps. (19917982)
2009
16
Treatment leading to dramatic recovery in acute hemorrhagic leukoencephalitis. (17608318)
2007
17
Use of therapeutic plasma exchange in the management of acute hemorrhagic leukoencephalitis: a case report and review of the literature. (17524086)
2007
18
The importance of EEG and variability of MRI findings in acute hemorrhagic leukoencephalitis. (17038020)
2006
19
Acute hemorrhagic leukoencephalitis: neuroimaging features and neuropathologic diagnosis. (16163044)
2005
20
Acute hemorrhagic leukoencephalitis manifesting as intracerebral hemorrhage associated with herpes simplex virus type I. (15917264)
2005
21
Acute hemorrhagic leukoencephalitis (Hurst's disease) linked to Epstein-Barr virus infection. (15791481)
2005
22
Serial MR imaging findings of acute hemorrhagic leukoencephalitis: a case report. (16155148)
2005
23
Acute hemorrhagic leukoencephalitis mimicking herpes simplex encephalitis: case report. (15122448)
2004
24
Acute hemorrhagic leukoencephalitis vs ADEM: FLAIR MRI and neuropathology findings. (12601124)
2003
25
Possible acute hemorrhagic leukoencephalitis manifesting as intracerebral hemorrhage on computed tomography--case report. (12206493)
2002
26
Acute hemorrhagic leukoencephalitis in male sibs. (11840490)
2002
27
Pediatric acute hemorrhagic leukoencephalitis: report of a surviving patient and review. (11810602)
2002
28
Detection of infectious agents in brain of patients with acute hemorrhagic leukoencephalitis. (12402170)
2002
29
Successful recovery after high-dose intravenous methylprednisolone in acute hemorrhagic leukoencephalitis. (11697528)
2001
30
Full recovery after acute hemorrhagic leukoencephalitis (Hurst's disease). (11200696)
2000
31
Case 1-1999: Acute hemorrhagic leukoencephalitis. (10577083)
1999
32
Case 1-1999: Acute hemorrhagic leukoencephalitis. (10577082)
1999
33
Case 1-1999: Acute hemorrhagic leukoencephalitis. (10577084)
1999
34
Acute hemorrhagic leukoencephalitis: recovery and reversal of magnetic resonance imaging findings in a child. (9373802)
1997
35
Acute hemorrhagic leukoencephalitis during treatment for disseminated tuberculosis in a patient with AIDS. (9487459)
1997
36
Acute hemorrhagic leukoencephalitis: a cause of acute brainstem dysfunction. (8052903)
1994
37
Tubular inclusions in macrophages in the brain of a patient with acute hemorrhagic leukoencephalitis (Weston-Hurst syndrome). (8191625)
1994
38
Intensive care management of acute hemorrhagic leukoencephalitis with favorable neurologic outcome. (1468137)
1992
39
Acute hemorrhagic leukoencephalitis. (1844176)
1991
40
Acute hemorrhagic leukoencephalitis. (1746242)
1991
41
Acute hemorrhagic leukoencephalitis. A successful recovery. (1929904)
1991
42
An autopsied case of acute hemorrhagic leukoencephalitis. (3773347)
1986
43
Computed tomography in acute hemorrhagic leukoencephalitis. (6721456)
1984
44
Acute hemorrhagic leukoencephalitis: diagnosis by computed tomography. (6742799)
1984
45
Computerized tomography as a diagnostic aid in acute hemorrhagic leukoencephalitis. (6847149)
1983
46
Acute hemorrhagic leukoencephalitis: report of three cases and review of the literature. (1105376)
1975
47
Acute hemorrhagic leukoencephalitis. Report of 2 cases. (6016398)
1967
48
Acute hemorrhagic leukoencephalitis of herpes simplex-virus type. Histological and electronmicroscopical findings. (4290137)
1967
49
ACUTE HEMORRHAGIC LEUKOENCEPHALITIS; A CLINICAL AND ELECTRON-MICROSCOPIC REPORT OF 2 PATIENTS TREATED WITH SURGICAL DECOMPRESSION. (14058421)
1963

Variations for Acute Hemorrhagic Leukoencephalitis

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Expression for genes affiliated with Acute Hemorrhagic Leukoencephalitis

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Search GEO for disease gene expression data for Acute Hemorrhagic Leukoencephalitis.

Pathways for genes affiliated with Acute Hemorrhagic Leukoencephalitis

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Pathways related to Acute Hemorrhagic Leukoencephalitis according to GeneCards Suite gene sharing:

(show all 14)
idSuper pathwaysScoreTop Affiliating Genes
19.4AQP1, AQP4, MBP
29.4FLT1, VEGFA
3
Show member pathways
9.1FLT1, KDR, VEGFA
49.1FLT1, KDR, VEGFA
5
Show member pathways
9.1FLT1, KDR, VEGFA
6
Show member pathways
9.1FLT1, KDR, VEGFA
79.1AQP4, IL1RN, VEGFA
8
Show member pathways
8.8FLT1, KDR, NRP1, VEGFA
9
Show member pathways
8.8FLT1, KDR, NRP1, VEGFA
10
Show member pathways
8.8FLT1, KDR, NRP1, VEGFA
118.8FLT1, KDR, RANBP2, VEGFA
12
Show member pathways
8.6FASLG, FLT1, KDR, VEGFA
138.6FASLG, FLT1, KDR, VEGFA
14
Show member pathways
8.3FASLG, FLT1, KDR, NRP1, VEGFA

GO Terms for genes affiliated with Acute Hemorrhagic Leukoencephalitis

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Cellular components related to Acute Hemorrhagic Leukoencephalitis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1sorting endosomeGO:00974439.9KDR, NRP1
2extracellular spaceGO:00056158.8CFI, FASLG, FLT1, IL1RN, NRP1, VEGFA
3integral component of plasma membraneGO:00058878.5AQP1, AQP4, CD8A, FASLG, FLT1, KDR
4extracellular regionGO:00055767.7CD8A, CFI, FASLG, FLT1, IL1RN, KDR
5plasma membraneGO:00058866.5AQP1, AQP4, CD8A, FASLG, FLT1, IL1RN
6membraneGO:00160205.9AQP1, AQP4, CD8A, CFI, FASLG, FLT1

Biological processes related to Acute Hemorrhagic Leukoencephalitis according to GeneCards Suite gene sharing:

(show all 27)
idNameGO IDScoreTop Affiliating Genes
1multicellular organismal water homeostasisGO:005089110.4AQP1, AQP4
2negative regulation of heterotypic cell-cell adhesionGO:003411510.4IL1RN, MBP
3artery morphogenesisGO:004884410.3NRP1, VEGFA
4branching involved in blood vessel morphogenesisGO:000156910.3NRP1, VEGFA
5commissural neuron axon guidanceGO:007167910.3NRP1, VEGFA
6coronary artery morphogenesisGO:006098210.3NRP1, VEGFA
7endothelial cell chemotaxisGO:003576710.3NRP1, VEGFA
8motor neuron migrationGO:009747510.3NRP1, VEGFA
9positive regulation of axon extension involved in axon guidanceGO:004884210.2NRP1, VEGFA
10camera-type eye morphogenesisGO:004859310.2AQP1, VEGFA
11positive regulation of retinal ganglion cell axon guidanceGO:190233610.2NRP1, VEGFA
12regulation of retinal ganglion cell axon guidanceGO:009025910.2NRP1, VEGFA
13positive regulation of endothelial cell chemotaxis by VEGF-activated vascular endothelial growth factor receptor signaling pathwayGO:003803310.2KDR, VEGFA
14positive regulation of focal adhesion assemblyGO:005189410.2KDR, VEGFA
15positive regulation of positive chemotaxisGO:005092710.2KDR, VEGFA
16positive regulation of vascular endothelial growth factor receptor signaling pathwayGO:003094910.0FLT1, VEGFA
17cell migration involved in sprouting angiogenesisGO:000204210.0KDR, NRP1, VEGFA
18VEGF-activated neuropilin signaling pathwayGO:00381909.9NRP1, VEGFA
19positive regulation of endothelial cell migrationGO:00105959.9KDR, NRP1, VEGFA
20positive regulation of endothelial cell proliferationGO:00019389.9KDR, NRP1, VEGFA
21vascular endothelial growth factor signaling pathwayGO:00380849.8FLT1, KDR, NRP1
22water transportGO:00068339.8AQP1, AQP4
23angiogenesisGO:00015259.5FLT1, KDR, NRP1, VEGFA
24cellular response to vascular endothelial growth factor stimulusGO:00359249.5FLT1, KDR, NRP1, VEGFA
25positive regulation of angiogenesisGO:00457669.5AQP1, FLT1, KDR, VEGFA
26transmembrane receptor protein tyrosine kinase signaling pathwayGO:00071699.4CD8A, FLT1, KDR
27vascular endothelial growth factor receptor signaling pathwayGO:00480109.2FLT1, KDR, NRP1, VEGFA

Molecular functions related to Acute Hemorrhagic Leukoencephalitis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1growth factor bindingGO:001983810.3FLT1, KDR, NRP1
2water channel activityGO:00152509.9AQP1, AQP4
3vascular endothelial growth factor-activated receptor activityGO:00050219.8FLT1, KDR, NRP1
4water transmembrane transporter activityGO:00053729.8AQP1, AQP4
5protein bindingGO:00055155.6AQP1, AQP4, CD8A, CFI, FASLG, FLT1

Sources for Acute Hemorrhagic Leukoencephalitis

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet