MCID: ACT032
MIFTS: 43

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 10 45 12
Leukoencephalitis, Acute Hemorrhagic 36 65
Ahl 10 45
Acute Haemorrhagic Leucoencephalitis of Weston Hurst 10
 
Acute Necrotizing Hemorrhagic Leukoencephalitis 10
Acute Hemorrhagic Encephalomyelitis 10
Weston-Hurst Syndrome 10
Ahle 45

Classifications:



External Ids:

Disease Ontology10 DOID:10992
MeSH36 D004684
SNOMED-CT59 72986009
NCIt42 C84535
UMLS65 C0014077

Summaries for Acute Hemorrhagic Leukoencephalitis

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NIH Rare Diseases:45 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 gastric 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 IL-15 Signaling Pathways and their Primary Biological Effects in Different Immune Cell Types. Affiliated tissues include brain, spinal cord and breast, and related mouse phenotypes are digestive/alimentary and muscle.

Disease Ontology:10 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 68)
idRelated DiseaseScoreTop Affiliating Genes
1gastric leiomyosarcoma10.6AQP1, IL1RN
2subpulmonary stenosis10.5AQP1, AQP4
3dystonia/parkinsonism, hypermanganesemia, polycythemia, and chronic liver disease10.5AQP1, AQP4
4porokeratosis 3, disseminated superficial actinic10.5IL1RN, TNFRSF1A
5treponema infection10.5AQP4, MBP
6cogan syndrome10.4AQP4, MBP
7opiate dependence10.3IL1RN, TNFRSF1B
8metanephric adenoma10.3AQP4, MBP
9telangiectasis10.3TNFRSF1A, TNFRSF1B
10acral lentiginous melanoma10.3AQP1, AQP4, MBP
11causalgia10.3AQP4, MBP
12neutropenia10.2AQP1, AQP4, MBP
13lemierre's syndrome10.2CD8A, TNFRSF1B
14allergic cutaneous vasculitis10.2TNFRSF1A, TNFRSF1B
15bartholin's gland adenoma10.2AQP4, MBP
16precursor b lymphoblastic lymphoma/leukemia10.2TNFRSF1A, TNFRSF1B
17patent ductus arteriosus10.2IL1RN, TNFRSF1B
18wells-jankovic syndrome10.2IL1RN, TNFRSF1A
19herpes simplex10.2
20gonadal dysgenesis10.2AQP1, AQP4, TNFRSF1A
21nemaline myopathy10.2AQP4, CD8A, MBP
22polyglucosan body disease, adult form10.1IL1RN, TNFRSF1A
23malignant pleural mesothelioma10.1CD8A, FASLG
24meningocele10.1KDR, VEGFA
25female breast central part cancer10.1CD8A, IL1RN, TNFRSF1B
26cryoglobulinemia10.1CD8A, IL1RN, TNFRSF1B
27jejunal somatostatinoma10.1AQP4, MBP, TNFRSF1A
28bacterial exanthem10.1CD8A, IL1RN, TNFRSF1B
29glycoproteinosis10.0CD8A, IL1RN, TNFRSF1B
30rectal disease10.0MBP, TNFRSF1A, TNFRSF1B
31gastric neuroendocrine neoplasm10.0KDR, VEGFA
32rhabdoid cancer10.0CD8A, TNFRSF1A, TNFRSF1B
33psoriasis susceptibility 110.0IL1RN, TNFRSF1A, TNFRSF1B
34inherited metabolic disorder10.0KDR, NRP1, VEGFA
35conduct disorder10.0KDR, VEGFA
36connective tissue cancer10.0CFI, KDR, VEGFA
37brain injury9.9
38mumps9.9
39relapsing-remitting multiple sclerosis9.9
40tuberculosis9.9
41myopathy9.9
42influenza9.9
43encephalitis9.9
44herpes simplex encephalitis9.9
45right bundle branch block9.9FLT1, VEGFA
46common bile duct neoplasm9.8FLT1, VEGFA
47refractory plasma cell neoplasm9.8IL1RN, VEGFA
48spinal canal intradural extramedullary neoplasm9.8CFI, IL1RN, VEGFA
49vasomotor rhinitis9.7AQP1, AQP4, VEGFA
50bladder urachal squamous cell carcinoma9.7FLT1, KDR, VEGFA

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



Diseases related to acute hemorrhagic leukoencephalitis

Symptoms for Acute Hemorrhagic Leukoencephalitis

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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:

33
Brain, Spinal cord, Breast, Endothelial, Prostate, B lymphoblasts, T cells

Animal Models for Acute Hemorrhagic Leukoencephalitis or affiliated genes

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MGI Mouse Phenotypes related to Acute Hemorrhagic Leukoencephalitis:

38 (show all 18)
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053817.7AQP4, FASLG, OCLN, TNFRSF1A, TNFRSF1B, VEGFA
2MP:00053697.6FLT1, KDR, NRP1, OCLN, TNFRSF1A, TNFRSF1B
3MP:00107717.5CD8A, FASLG, IL1RN, KDR, TNFRSF1A, TNFRSF1B
4MP:00053887.5AQP1, AQP4, FASLG, KDR, TNFRSF1A, TNFRSF1B
5MP:00053677.5AQP1, AQP4, CFI, FASLG, TNFRSF1A, TNFRSF1B
6MP:00053897.3AQP1, FASLG, FLT1, IL1RN, MBP, OCLN
7MP:00053907.2FASLG, IL1RN, KDR, OCLN, TNFRSF1A, TNFRSF1B
8MP:00053797.2AQP1, AQP4, CD8A, FASLG, OCLN, TNFRSF1A
9MP:00053866.9AQP1, AQP4, FLT1, KDR, MBP, OCLN
10MP:00053846.7FASLG, FLT1, IL1RN, KDR, MBP, NRP1
11MP:00053786.4AQP1, AQP4, FLT1, IL1RN, KDR, NRP1
12MP:00053916.4AQP1, AQP4, FASLG, FLT1, KDR, MBP
13MP:00053856.0AQP1, AQP4, FASLG, FLT1, IL1RN, KDR
14MP:00036315.7AQP1, AQP4, CD8A, FASLG, FLT1, KDR
15MP:00053765.6AQP1, AQP4, CFI, FASLG, FLT1, IL1RN
16MP:00107685.4AQP1, AQP4, CD8A, FASLG, FLT1, IL1RN
17MP:00053875.2AQP4, CD8A, FASLG, FLT1, IL1RN, KDR
18MP:00053974.8AQP1, AQP4, CD8A, CFI, FASLG, FLT1

Publications for Acute Hemorrhagic Leukoencephalitis

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Articles related to Acute Hemorrhagic Leukoencephalitis:

(show all 48)
idTitleAuthorsYear
1
Acute Hemorrhagic Leukoencephalitis in Children: A Case Report. (27217610)
2016
2
Acute hemorrhagic leukoencephalitis (Weston-Hurst syndrome) in a patient with relapse-remitting multiple sclerosis. (26376717)
2015
3
Acute hemorrhagic leukoencephalitis associated with autoimmune myopathy. (25422709)
2014
4
Autopsy-proven Acute Hemorrhagic Leukoencephalitis in an Elderly Patient. (24384347)
2014
5
Mutations of complement factor I and potential mechanisms of neuroinflammation in acute hemorrhagic leukoencephalitis. (22926405)
2013
6
First case report of acute hemorrhagic leukoencephalitis following Plasmodium vivax infection. (23508437)
2013
7
Value of susceptibility-weighted imaging in acute hemorrhagic leukoencephalitis. (22465779)
2012
8
Serial computed tomography and magnetic resonance imaging findings of biphasic acute hemorrhagic leukoencephalitis localized to the brain stem and cerebellum. (21519996)
2011
9
Acute hemorrhagic leukoencephalitis with severe brainstem and spinal cord involvement: MRI features with neuropathological confirmation. (21448963)
2011
10
Acute hemorrhagic leukoencephalitis: a critical entity for forensic pathologists to recognize. (20010289)
2010
11
Atypical acute hemorrhagic leukoencephalitis (Hurst's disease) presenting with focal hemorrhagic brainstem lesion. (19847676)
2010
12
Acute hemorrhagic leukoencephalitis and hypoxic brain injury associated with H1N1 influenza. (20558397)
2010
13
Acute hemorrhagic leukoencephalitis with atypical features. (19145402)
2009
14
Teaching NeuroImages: Acute hemorrhagic leukoencephalitis after mumps. (19917982)
2009
15
Use of therapeutic plasma exchange in the management of acute hemorrhagic leukoencephalitis: a case report and review of the literature. (17524086)
2007
16
Treatment leading to dramatic recovery in acute hemorrhagic leukoencephalitis. (17608318)
2007
17
The importance of EEG and variability of MRI findings in acute hemorrhagic leukoencephalitis. (17038020)
2006
18
Acute hemorrhagic leukoencephalitis manifesting as intracerebral hemorrhage associated with herpes simplex virus type I. (15917264)
2005
19
Serial MR imaging findings of acute hemorrhagic leukoencephalitis: a case report. (16155148)
2005
20
Acute hemorrhagic leukoencephalitis (Hurst's disease) linked to Epstein-Barr virus infection. (15791481)
2005
21
Acute hemorrhagic leukoencephalitis: neuroimaging features and neuropathologic diagnosis. (16163044)
2005
22
Acute hemorrhagic leukoencephalitis mimicking herpes simplex encephalitis: case report. (15122448)
2004
23
Acute hemorrhagic leukoencephalitis vs ADEM: FLAIR MRI and neuropathology findings. (12601124)
2003
24
Acute hemorrhagic leukoencephalitis in male sibs. (11840490)
2002
25
Possible acute hemorrhagic leukoencephalitis manifesting as intracerebral hemorrhage on computed tomography--case report. (12206493)
2002
26
Detection of infectious agents in brain of patients with acute hemorrhagic leukoencephalitis. (12402170)
2002
27
Pediatric acute hemorrhagic leukoencephalitis: report of a surviving patient and review. (11810602)
2002
28
Successful recovery after high-dose intravenous methylprednisolone in acute hemorrhagic leukoencephalitis. (11697528)
2001
29
Full recovery after acute hemorrhagic leukoencephalitis (Hurst's disease). (11200696)
2000
30
Case 1-1999: Acute hemorrhagic leukoencephalitis. (10577083)
1999
31
Case 1-1999: Acute hemorrhagic leukoencephalitis. (10577082)
1999
32
Case 1-1999: Acute hemorrhagic leukoencephalitis. (10577084)
1999
33
Acute hemorrhagic leukoencephalitis during treatment for disseminated tuberculosis in a patient with AIDS. (9487459)
1997
34
Acute hemorrhagic leukoencephalitis: recovery and reversal of magnetic resonance imaging findings in a child. (9373802)
1997
35
Tubular inclusions in macrophages in the brain of a patient with acute hemorrhagic leukoencephalitis (Weston-Hurst syndrome). (8191625)
1994
36
Acute hemorrhagic leukoencephalitis: a cause of acute brainstem dysfunction. (8052903)
1994
37
Intensive care management of acute hemorrhagic leukoencephalitis with favorable neurologic outcome. (1468137)
1992
38
Acute hemorrhagic leukoencephalitis. (1844176)
1991
39
Acute hemorrhagic leukoencephalitis. (1746242)
1991
40
Acute hemorrhagic leukoencephalitis. A successful recovery. (1929904)
1991
41
An autopsied case of acute hemorrhagic leukoencephalitis. (3773347)
1986
42
Acute hemorrhagic leukoencephalitis: diagnosis by computed tomography. (6742799)
1984
43
Computed tomography in acute hemorrhagic leukoencephalitis. (6721456)
1984
44
Computerized tomography as a diagnostic aid in acute hemorrhagic leukoencephalitis. (6847149)
1983
45
Acute hemorrhagic leukoencephalitis: report of three cases and review of the literature. (1105376)
1975
46
Acute hemorrhagic leukoencephalitis. Report of 2 cases. (6016398)
1967
47
Acute hemorrhagic leukoencephalitis of herpes simplex-virus type. Histological and electronmicroscopical findings. (4290137)
1967
48
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 17)
idSuper pathwaysScoreTop Affiliating Genes
19.4AQP1, AQP4, MBP
2
Show member pathways
8.9FASLG, TNFRSF1A, TNFRSF1B
3
Show member pathways
8.9FASLG, TNFRSF1A, TNFRSF1B
48.8FLT1, KDR, VEGFA
58.8FLT1, KDR, VEGFA
6
Show member pathways
8.8FLT1, KDR, VEGFA
78.8FLT1, KDR, VEGFA
8
Show member pathways
8.8FLT1, KDR, VEGFA
9
Show member pathways
8.8FLT1, KDR, VEGFA
10
Show member pathways
8.7FASLG, RANBP2, TNFRSF1A, TNFRSF1B
11
Show member pathways
8.6FLT1, KDR, NRP1, VEGFA
12
Show member pathways
8.6FLT1, KDR, NRP1, VEGFA
13
Show member pathways
8.6FLT1, KDR, NRP1, VEGFA
148.6FLT1, KDR, RANBP2, VEGFA
158.2FASLG, FLT1, KDR, VEGFA
16
Show member pathways
7.9FASLG, FLT1, KDR, NRP1, TNFRSF1A, TNFRSF1B
17
Show member pathways
7.7AQP1, AQP4, FLT1, KDR, TNFRSF1A, VEGFA

GO Terms for genes affiliated with Acute Hemorrhagic Leukoencephalitis

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

(show all 15)
idNameGO IDScoreTop Affiliating Genes
1glycerol transportGO:001579310.0AQP1, AQP4
2regulation of retinal ganglion cell axon guidanceGO:009025910.0NRP1, VEGFA
3VEGF-activated neuropilin signaling pathwayGO:003819010.0NRP1, VEGFA
4commissural neuron axon guidanceGO:007167910.0NRP1, VEGFA
5vascular endothelial growth factor signaling pathwayGO:00380849.9NRP1, VEGFA
6endothelial cell chemotaxisGO:00357679.9NRP1, VEGFA
7positive regulation of endothelial cell chemotaxis by VEGF-activated vascular endothelial growth factor receptor signaling pathwayGO:00380339.9KDR, VEGFA
8patterning of blood vesselsGO:00015699.8NRP1, VEGFA
9coronary artery morphogenesisGO:00609829.8NRP1, VEGFA
10artery morphogenesisGO:00488449.7NRP1, VEGFA
11cell migration involved in sprouting angiogenesisGO:00020429.7KDR, NRP1, VEGFA
12cell surface receptor signaling pathwayGO:00071669.1CD8A, FASLG, TNFRSF1A
13angiogenesisGO:00015259.1KDR, NRP1, VEGFA
14cellular response to vascular endothelial growth factor stimulusGO:00359248.6FLT1, NRP1, VEGFA
15positive regulation of angiogenesisGO:00457668.5AQP1, FLT1, KDR, VEGFA

Sources for Acute Hemorrhagic Leukoencephalitis

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2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet