MCID: ACT032
MIFTS: 38

Acute Hemorrhagic Leukoencephalitis 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 prostate leiomyosarcoma and subpulmonary stenosis. 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 top 50)    (show all 55)
idRelated DiseaseScoreTop Affiliating Genes
1prostate leiomyosarcoma10.4AQP1, IL1RN
2subpulmonary stenosis10.4AQP1, AQP4
3dystrophinopathies10.4AQP1, AQP4
4cogan-reese syndrome10.3AQP4, MBP
5ovarian papillary cystadenoma10.3AQP4, MBP
6hepatic osteogenic sarcoma10.1AQP1, AQP4, MBP
7newborn respiratory distress syndrome10.1AQP1, AQP4, MBP
8bile duct cystadenoma10.1AQP4, MBP
9silicosiderosis10.1KDR, VEGFA
10herpes simplex10.0
11neurilemmoma10.0AQP4, CD8A, MBP
12malignant gastric germ cell tumor10.0KDR, VEGFA
13myelitis10.0AQP4, CD8A, MBP
14arthrogryposis, ectodermal dysplasia, cleft lip/palate, and developmental delay10.0KDR, VEGFA
15angiomyolipoma10.0AQP4, CD8A, MBP
16epidermolysis bullosa10.0KDR, VEGFA
17iliac vein thrombophlebitis10.0KDR, VEGFA
18relapsing-remitting multiple sclerosis9.9
19acute dacryocystitis9.9KDR, VEGFA
20calcaneonavicular coalition9.9KDR, VEGFA
21diverticulitis9.9CD8A, FASLG
22hereditary renal cell carcinoma9.9OCLN, VEGFA
23hyperinsulinism9.9CFI, KDR, VEGFA
24gastric small cell carcinoma9.9KDR, NRP1, VEGFA
25ocular melanoma9.8KDR, VEGFA
26brain injury9.8
27mumps9.8
28tuberculosis9.8
29myopathy9.8
30influenza9.8
31encephalitis9.8
32herpes simplex encephalitis9.8
33spinal cord astrocytoma9.8FLT1, VEGFA
34vaginal squamous tumor9.8CD8A, IL1RN
35cystadenoma9.7AQP1, KDR, VEGFA
36spinal muscular atrophy-dandy-walker malformation-cataracts syndrome9.7FLT1, VEGFA
37atrophic rhinitis9.7AQP1, AQP4, VEGFA
38gemistocytic astrocytoma9.7CD8A, MBP, VEGFA
39perivascular epithelioid cell tumor9.7KDR, VEGFA
40intestinal benign neoplasm9.6FLT1, VEGFA
41endobronchial lipoma9.6AQP4, VEGFA
42acute lymphoblastic leukemia congenital sporadic aniridia9.6FLT1, VEGFA
43bladder urachal carcinoma9.6FLT1, KDR, VEGFA
44epidural abscess9.6FLT1, KDR, VEGFA
45lipoatrophy with diabetes, leukomelanodermic papules, liver steatosis, and hypertrophic cardiomyopathy9.6FLT1, KDR, VEGFA
46chikungunya9.6FLT1, KDR, VEGFA
47apocrine adenoma9.5FLT1, KDR, VEGFA
48hematopoietic stem cell transplantation9.5FLT1, KDR, VEGFA
49citrullinemia, adult-onset type ii9.5FLT1, KDR, VEGFA
50third cranial nerve disease9.4CFI, FLT1, KDR, VEGFA

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:00053897.9AQP1, FASLG, FLT1, IL1RN, MBP, OCLN
2MP:00053857.7AQP1, AQP4, FASLG, FLT1, IL1RN, KDR
3MP:00053767.0AQP1, AQP4, CFI, FASLG, FLT1, IL1RN
4MP:00036316.9AQP1, AQP4, CD8A, FASLG, FLT1, KDR
5MP:00107686.9AQP1, AQP4, CD8A, FASLG, FLT1, IL1RN
6MP:00053876.9AQP4, CD8A, FASLG, FLT1, IL1RN, KDR
7MP:00053916.9AQP1, AQP4, FASLG, FLT1, KDR, MBP
8MP: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
Commentary: Acute Hemorrhagic Leukoencephalitis (Weston-Hurst Syndrome) in a Patient with Relapse-Remitting Multiple Sclerosis. (27252706)
2016
2
Acute Hemorrhagic Leukoencephalitis in Children: A Case Report. (27217610)
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
Mutations of complement factor I and potential mechanisms of neuroinflammation in acute hemorrhagic leukoencephalitis. (22926405)
2013
7
First case report of acute hemorrhagic leukoencephalitis following Plasmodium vivax infection. (23508437)
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
Acute hemorrhagic leukoencephalitis: a critical entity for forensic pathologists to recognize. (20010289)
2010
12
Atypical acute hemorrhagic leukoencephalitis (Hurst's disease) presenting with focal hemorrhagic brainstem lesion. (19847676)
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 manifesting as intracerebral hemorrhage associated with herpes simplex virus type I. (15917264)
2005
20
Serial MR imaging findings of acute hemorrhagic leukoencephalitis: a case report. (16155148)
2005
21
Acute hemorrhagic leukoencephalitis (Hurst's disease) linked to Epstein-Barr virus infection. (15791481)
2005
22
Acute hemorrhagic leukoencephalitis: neuroimaging features and neuropathologic diagnosis. (16163044)
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
Detection of infectious agents in brain of patients with acute hemorrhagic leukoencephalitis. (12402170)
2002
26
Acute hemorrhagic leukoencephalitis in male sibs. (11840490)
2002
27
Possible acute hemorrhagic leukoencephalitis manifesting as intracerebral hemorrhage on computed tomography--case report. (12206493)
2002
28
Pediatric acute hemorrhagic leukoencephalitis: report of a surviving patient and review. (11810602)
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 during treatment for disseminated tuberculosis in a patient with AIDS. (9487459)
1997
35
Acute hemorrhagic leukoencephalitis: recovery and reversal of magnetic resonance imaging findings in a child. (9373802)
1997
36
Tubular inclusions in macrophages in the brain of a patient with acute hemorrhagic leukoencephalitis (Weston-Hurst syndrome). (8191625)
1994
37
Acute hemorrhagic leukoencephalitis: a cause of acute brainstem dysfunction. (8052903)
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
Acute hemorrhagic leukoencephalitis: diagnosis by computed tomography. (6742799)
1984
44
Computed tomography in acute hemorrhagic leukoencephalitis. (6721456)
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 15)
idSuper pathwaysScoreTop Affiliating Genes
19.4AQP1, AQP4, MBP
29.0FLT1, VEGFA
38.8AQP4, IL1RN, VEGFA
48.7FLT1, KDR, VEGFA
5
Show member pathways
8.7FLT1, KDR, VEGFA
6
Show member pathways
8.7FLT1, KDR, VEGFA
7
Show member pathways
8.7FLT1, KDR, VEGFA
88.7FLT1, KDR, VEGFA
98.7FLT1, KDR, VEGFA
108.5FLT1, KDR, RANBP2, VEGFA
11
Show member pathways
8.5FLT1, KDR, NRP1, VEGFA
12
Show member pathways
8.5FLT1, KDR, NRP1, VEGFA
13
Show member pathways
8.2FASLG, FLT1, KDR, VEGFA
148.2FASLG, FLT1, KDR, VEGFA
15
Show member pathways
7.9FASLG, 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
2integral component of plasma membraneGO:00058878.7AQP1, AQP4, CD8A, FASLG, FLT1, KDR
3extracellular spaceGO:00056158.4CFI, FASLG, FLT1, IL1RN, NRP1, VEGFA
4plasma membraneGO:00058866.6AQP1, AQP4, CD8A, FASLG, FLT1, IL1RN

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

(show all 25)
idNameGO IDScoreTop Affiliating Genes
1cellular water homeostasisGO:000999210.4AQP1, AQP4
2glycerol transportGO:001579310.4AQP1, AQP4
3multicellular organismal water homeostasisGO:005089110.4AQP1, AQP4
4artery morphogenesisGO:004884410.1NRP1, VEGFA
5commissural neuron axon guidanceGO:007167910.1NRP1, VEGFA
6coronary artery morphogenesisGO:006098210.1NRP1, VEGFA
7endothelial cell chemotaxisGO:003576710.1NRP1, VEGFA
8positive regulation of axon extension involved in axon guidanceGO:004884210.0NRP1, VEGFA
9positive regulation of endothelial cell chemotaxis by VEGF-activated vascular endothelial growth factor receptor signaling pathwayGO:003803310.0KDR, VEGFA
10positive regulation of retinal ganglion cell axon guidanceGO:190233610.0NRP1, VEGFA
11regulation of retinal ganglion cell axon guidanceGO:009025910.0NRP1, VEGFA
12positive regulation of focal adhesion assemblyGO:005189410.0KDR, VEGFA
13positive regulation of positive chemotaxisGO:00509279.9KDR, VEGFA
14cell migration involved in sprouting angiogenesisGO:00020429.8KDR, NRP1, VEGFA
15water transportGO:00068339.7AQP1, AQP4
16VEGF-activated neuropilin signaling pathwayGO:00381909.7NRP1, VEGFA
17positive regulation of endothelial cell migrationGO:00105959.7KDR, NRP1, VEGFA
18positive regulation of endothelial cell proliferationGO:00019389.7KDR, NRP1, VEGFA
19transmembrane receptor protein tyrosine kinase signaling pathwayGO:00071699.7CD8A, FLT1, KDR
20positive regulation of vascular endothelial growth factor receptor signaling pathwayGO:00309499.6FLT1, VEGFA
21immune responseGO:00069559.5CD8A, FASLG, IL1RN, MBP
22angiogenesisGO:00015259.2FLT1, KDR, NRP1, VEGFA
23cellular response to vascular endothelial growth factor stimulusGO:00359249.2FLT1, KDR, NRP1, VEGFA
24positive regulation of angiogenesisGO:00457669.1AQP1, FLT1, KDR, VEGFA
25vascular endothelial growth factor receptor signaling pathwayGO:00480108.8FLT1, KDR, NRP1, VEGFA

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

idNameGO IDScoreTop Affiliating Genes
1coreceptor activityGO:001502610.3CD8A, NRP1
2glycerol channel activityGO:001525410.2AQP1, AQP4
3water channel activityGO:00152509.9AQP1, AQP4
4water transmembrane transporter activityGO:00053729.7AQP1, AQP4
5growth factor bindingGO:00198389.7FLT1, KDR, NRP1
6vascular endothelial growth factor-activated receptor activityGO:00050219.6FLT1, KDR, NRP1

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