MCID: ACT032
MIFTS: 43

Acute Hemorrhagic Leukoencephalitis

Categories: Rare diseases, Neuronal diseases

Aliases & Classifications for Acute Hemorrhagic Leukoencephalitis

MalaCards integrated aliases for Acute Hemorrhagic Leukoencephalitis:

Name: Acute Hemorrhagic Leukoencephalitis 53 12 49 14
Ahl 53 12 49
Leukoencephalitis, Acute Hemorrhagic 41 69
Acute Haemorrhagic Leucoencephalitis of Weston Hurst 12
Acute Necrotizing Hemorrhagic Leukoencephalitis 12
Acute Hemorrhagic Encephalomyelitis 12
Weston-Hurst Syndrome 12
Ahle 49

Classifications:



External Ids:

OMIM 53 606752
Disease Ontology 12 DOID:10992
MeSH 41 D004684
NCIt 46 C84535
SNOMED-CT 64 72986009
MedGen 39 C0014077
UMLS 69 C0014077

Summaries for Acute Hemorrhagic Leukoencephalitis

NIH Rare Diseases : 49 Acute hemorrhagic leukoencephalitis (AHLE) is a very rare form of acute disseminated encephalomyelitis that frequently 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 and may include fever, neck stiffness, fatigue, headache, nausea, vomiting, seizures, and coma. AHLE has a very poor prognosis, with rapid deterioration and death usually occurring within days to one week after onset of symptoms. Although the exact cause is unclear, AHLE usually follows a viral or bacterial infection, or less often, vaccination for measles or rabies. Some researchers think that an infection or vaccination can initiate an autoimmune process in the body thus leading to AHLE. While favorable outcomes are uncommon, timely treatment with immunosuppressant medications and plasma exchange may lead to an improved outlook.   Last updated: 1/11/2018

MalaCards based summary : Acute Hemorrhagic Leukoencephalitis, also known as ahl, is related to inappropriate adh syndrome and dysferlinopathy. An important gene associated with Acute Hemorrhagic Leukoencephalitis is CFI (Complement Factor I), and among its related pathways/superpathways are MAPK signaling pathway and Ras signaling pathway. Affiliated tissues include brain, spinal cord and cerebellum, and related phenotypes are hematopoietic system and growth/size/body region

Disease Ontology : 12 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.

Description from OMIM: 606752

Related Diseases for Acute Hemorrhagic Leukoencephalitis

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

(show top 50) (show all 59)
# Related Disease Score Top Affiliating Genes
1 inappropriate adh syndrome 10.3 AQP1 AQP4
2 dysferlinopathy 10.3 AQP1 AQP4
3 subependymoma 10.3 AQP1 AQP4
4 intracranial hypertension, idiopathic 10.3 AQP1 AQP4
5 balo concentric sclerosis 10.2 AQP4 MBP
6 acute disseminated encephalomyelitis 10.2 AQP4 MBP
7 placenta accreta 10.2 FLT1 KDR
8 transverse myelitis 10.2 AQP4 MBP
9 hydrarthrosis 10.1 AQP1 IL1RN
10 herpes simplex 10.1
11 internuclear ophthalmoplegia 10.1 AQP4 MBP
12 central pontine myelinolysis 10.1 AQP1 AQP4 MBP
13 neuromyelitis optica 10.1 AQP1 AQP4 MBP
14 multiple sclerosis 10.0
15 relapsing-remitting multiple sclerosis 10.0
16 influenza 10.0
17 myelitis 10.0 AQP4 MBP
18 skin hemangioma 10.0 KDR VEGFA
19 radiation proctitis 10.0 KDR VEGFA
20 traumatic brain injury 10.0 AQP4 MBP
21 kuhnt-junius degeneration 10.0 KDR VEGFA
22 mycobacterium tuberculosis 1 9.9
23 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 9.9
24 brain injury 9.9
25 mumps 9.9
26 myopathy 9.9
27 encephalitis 9.9
28 glioblastoma 9.9
29 herpes simplex encephalitis 9.9
30 glomeruloid hemangioma 9.9 FLT1 VEGFA
31 coats disease 9.9 KDR VEGFA
32 degeneration of macula and posterior pole 9.9 CFI KDR VEGFA
33 angiomatous meningioma 9.9 KDR NRP1 VEGFA
34 leukostasis 9.8 KDR VEGFA
35 microcystic meningioma 9.8 FLT1 VEGFA
36 cancer-associated retinopathy 9.8 FLT1 VEGFA
37 lipodermatosclerosis 9.8 FLT1 VEGFA
38 neuroretinitis 9.8 AQP4 VEGFA
39 kidney cancer 9.8 AQP1 KDR VEGFA
40 peripartum cardiomyopathy 9.8 FLT1 VEGFA
41 spinal cord disease 9.7 AQP4 MBP
42 brain edema 9.7 AQP1 AQP4 VEGFA
43 angiokeratoma circumscriptum 9.7 FLT1 KDR VEGFA
44 capillary hemangioma 9.7 FLT1 KDR VEGFA
45 epithelioid hemangioendothelioma 9.7 FLT1 KDR VEGFA
46 vascular cancer 9.7 FLT1 KDR VEGFA
47 lymphangioma 9.7 KDR VEGFA
48 corneal neovascularization 9.7 FLT1 KDR VEGFA
49 breast carcinoma in situ 9.7 FLT1 KDR VEGFA
50 placental insufficiency 9.7 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

Clinical features from OMIM:

606752

MGI Mouse Phenotypes related to Acute Hemorrhagic Leukoencephalitis:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.07 VEGFA NRP1 FLT1 AQP1 IL1RN AQP4
2 growth/size/body region MP:0005378 10.06 NRP1 OCLN VEGFA MBP FLT1 AQP1
3 cardiovascular system MP:0005385 10.03 VEGFA FASLG NRP1 FLT1 AQP1 IL1RN
4 homeostasis/metabolism MP:0005376 10.02 OCLN VEGFA MBP FASLG FLT1 AQP1
5 immune system MP:0005387 9.91 OCLN VEGFA MBP FASLG NRP1 FLT1
6 nervous system MP:0003631 9.81 OCLN VEGFA MBP FASLG NRP1 FLT1
7 reproductive system MP:0005389 9.5 OCLN VEGFA MBP FASLG FLT1 AQP1
8 vision/eye MP:0005391 9.23 VEGFA MBP FASLG NRP1 FLT1 AQP1

Drugs & Therapeutics for Acute Hemorrhagic Leukoencephalitis

Search Clinical Trials , NIH Clinical Center for Acute Hemorrhagic Leukoencephalitis

Cochrane evidence based reviews: leukoencephalitis, acute hemorrhagic

Genetic Tests for Acute Hemorrhagic Leukoencephalitis

Anatomical Context for Acute Hemorrhagic Leukoencephalitis

MalaCards organs/tissues related to Acute Hemorrhagic Leukoencephalitis:

38
Brain, Spinal Cord, Cerebellum

Publications for Acute Hemorrhagic Leukoencephalitis

Articles related to Acute Hemorrhagic Leukoencephalitis:

(show top 50) (show all 51)
# Title Authors Year
1
Acute hemorrhagic leukoencephalitis after seasonal influenza vaccination. ( 29134580 )
2017
2
Diffuse glioblastoma resembling acute hemorrhagic leukoencephalitis. ( 29184769 )
2017
3
Acute Hemorrhagic Leukoencephalitis in Children: A Case Report. ( 27217610 )
2016
4
Commentary: Acute Hemorrhagic Leukoencephalitis (Weston-Hurst Syndrome) in a Patient with Relapse-Remitting Multiple Sclerosis. ( 27252706 )
2016
5
Acute hemorrhagic leukoencephalitis (Weston-Hurst syndrome) in a patient with relapse-remitting multiple sclerosis. ( 26376717 )
2015
6
Acute hemorrhagic leukoencephalitis associated with autoimmune myopathy. ( 25422709 )
2014
7
Autopsy-proven Acute Hemorrhagic Leukoencephalitis in an Elderly Patient. ( 24384347 )
2014
8
First case report of acute hemorrhagic leukoencephalitis following Plasmodium vivax infection. ( 23508437 )
2013
9
Mutations of complement factor I and potential mechanisms of neuroinflammation in acute hemorrhagic leukoencephalitis. ( 22926405 )
2013
10
Value of susceptibility-weighted imaging in acute hemorrhagic leukoencephalitis. ( 22465779 )
2012
11
Acute hemorrhagic leukoencephalitis with severe brainstem and spinal cord involvement: MRI features with neuropathological confirmation. ( 21448963 )
2011
12
Serial computed tomography and magnetic resonance imaging findings of biphasic acute hemorrhagic leukoencephalitis localized to the brain stem and cerebellum. ( 21519996 )
2011
13
Acute hemorrhagic leukoencephalitis and hypoxic brain injury associated with H1N1 influenza. ( 20558397 )
2010
14
Atypical acute hemorrhagic leukoencephalitis (Hurst's disease) presenting with focal hemorrhagic brainstem lesion. ( 19847676 )
2010
15
Acute hemorrhagic leukoencephalitis: a critical entity for forensic pathologists to recognize. ( 20010289 )
2010
16
Teaching NeuroImages: Acute hemorrhagic leukoencephalitis after mumps. ( 19917982 )
2009
17
Acute hemorrhagic leukoencephalitis with atypical features. ( 19145402 )
2009
18
Treatment leading to dramatic recovery in acute hemorrhagic leukoencephalitis. ( 17608318 )
2007
19
Use of therapeutic plasma exchange in the management of acute hemorrhagic leukoencephalitis: a case report and review of the literature. ( 17524086 )
2007
20
The importance of EEG and variability of MRI findings in acute hemorrhagic leukoencephalitis. ( 17038020 )
2006
21
Serial MR imaging findings of acute hemorrhagic leukoencephalitis: a case report. ( 16155148 )
2005
22
Acute hemorrhagic leukoencephalitis (Hurst's disease) linked to Epstein-Barr virus infection. ( 15791481 )
2005
23
Acute hemorrhagic leukoencephalitis: neuroimaging features and neuropathologic diagnosis. ( 16163044 )
2005
24
Acute hemorrhagic leukoencephalitis manifesting as intracerebral hemorrhage associated with herpes simplex virus type I. ( 15917264 )
2005
25
Acute hemorrhagic leukoencephalitis mimicking herpes simplex encephalitis: case report. ( 15122448 )
2004
26
Acute hemorrhagic leukoencephalitis vs ADEM: FLAIR MRI and neuropathology findings. ( 12601124 )
2003
27
Detection of infectious agents in brain of patients with acute hemorrhagic leukoencephalitis. ( 12402170 )
2002
28
Possible acute hemorrhagic leukoencephalitis manifesting as intracerebral hemorrhage on computed tomography--case report. ( 12206493 )
2002
29
Pediatric acute hemorrhagic leukoencephalitis: report of a surviving patient and review. ( 11810602 )
2002
30
Acute hemorrhagic leukoencephalitis in male sibs. ( 11840490 )
2002
31
Successful recovery after high-dose intravenous methylprednisolone in acute hemorrhagic leukoencephalitis. ( 11697528 )
2001
32
Full recovery after acute hemorrhagic leukoencephalitis (Hurst's disease). ( 11200696 )
2000
33
Case 1-1999: Acute hemorrhagic leukoencephalitis. ( 10577084 )
1999
34
Case 1-1999: Acute hemorrhagic leukoencephalitis. ( 10577083 )
1999
35
Case 1-1999: Acute hemorrhagic leukoencephalitis. ( 10577082 )
1999
36
Acute hemorrhagic leukoencephalitis: recovery and reversal of magnetic resonance imaging findings in a child. ( 9373802 )
1997
37
Acute hemorrhagic leukoencephalitis during treatment for disseminated tuberculosis in a patient with AIDS. ( 9487459 )
1997
38
Acute hemorrhagic leukoencephalitis: a cause of acute brainstem dysfunction. ( 8052903 )
1994
39
Tubular inclusions in macrophages in the brain of a patient with acute hemorrhagic leukoencephalitis (Weston-Hurst syndrome). ( 8191625 )
1994
40
Intensive care management of acute hemorrhagic leukoencephalitis with favorable neurologic outcome. ( 1468137 )
1992
41
Acute hemorrhagic leukoencephalitis. ( 1746242 )
1991
42
Acute hemorrhagic leukoencephalitis. A successful recovery. ( 1929904 )
1991
43
Acute hemorrhagic leukoencephalitis. ( 1844176 )
1991
44
An autopsied case of acute hemorrhagic leukoencephalitis. ( 3773347 )
1986
45
Computed tomography in acute hemorrhagic leukoencephalitis. ( 6721456 )
1984
46
Acute hemorrhagic leukoencephalitis: diagnosis by computed tomography. ( 6742799 )
1984
47
Computerized tomography as a diagnostic aid in acute hemorrhagic leukoencephalitis. ( 6847149 )
1983
48
Acute hemorrhagic leukoencephalitis: report of three cases and review of the literature. ( 1105376 )
1975
49
Acute hemorrhagic leukoencephalitis. Report of 2 cases. ( 6016398 )
1967
50
Acute hemorrhagic leukoencephalitis of herpes simplex-virus type. Histological and electronmicroscopical findings. ( 4290137 )
1967

Variations for Acute Hemorrhagic Leukoencephalitis

Expression for Acute Hemorrhagic Leukoencephalitis

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

Pathways for Acute Hemorrhagic Leukoencephalitis

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

(show all 15)
# Super pathways Score Top Affiliating Genes
1 12.49 FASLG FLT1 KDR VEGFA
2
Show member pathways
12.45 FASLG FLT1 KDR VEGFA
3
Show member pathways
12.36 FASLG FLT1 KDR NRP1 VEGFA
4
Show member pathways
12.13 FLT1 KDR NRP1 VEGFA
5
Show member pathways
11.96 FLT1 KDR NRP1 VEGFA
6 11.92 AQP4 IL1RN VEGFA
7 11.75 AQP1 AQP4 MBP
8 11.75 FLT1 KDR RANBP2 VEGFA
9
Show member pathways
11.71 FLT1 KDR VEGFA
10
Show member pathways
11.22 FLT1 KDR NRP1 VEGFA
11 11.12 FLT1 KDR VEGFA
12 10.96 FLT1 VEGFA
13 10.96 FLT1 KDR VEGFA
14 10.71 FLT1 KDR VEGFA
15
Show member pathways
10 FLT1 KDR VEGFA

GO Terms for Acute Hemorrhagic Leukoencephalitis

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

# Name GO ID Score Top Affiliating Genes
1 membrane GO:0016020 9.73 AQP1 AQP4 CFI FASLG FLT1 KDR
2 extracellular region GO:0005576 9.7 CFI FASLG FLT1 IL1RN KDR NRP1
3 sorting endosome GO:0097443 8.62 KDR NRP1

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

(show all 28)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.93 FASLG FLT1 KDR VEGFA
2 angiogenesis GO:0001525 9.83 FLT1 KDR NRP1 VEGFA
3 positive regulation of endothelial cell proliferation GO:0001938 9.72 KDR NRP1 VEGFA
4 positive regulation of endothelial cell migration GO:0010595 9.67 KDR NRP1 VEGFA
5 renal water homeostasis GO:0003091 9.64 AQP1 AQP4
6 branching involved in blood vessel morphogenesis GO:0001569 9.63 NRP1 VEGFA
7 artery morphogenesis GO:0048844 9.62 NRP1 VEGFA
8 water transport GO:0006833 9.62 AQP1 AQP4
9 positive regulation of vascular endothelial growth factor receptor signaling pathway GO:0030949 9.61 FLT1 VEGFA
10 positive regulation of cell migration involved in sprouting angiogenesis GO:0090050 9.61 NRP1 VEGFA
11 positive regulation of positive chemotaxis GO:0050927 9.6 KDR VEGFA
12 negative regulation of heterotypic cell-cell adhesion GO:0034115 9.59 IL1RN MBP
13 coronary artery morphogenesis GO:0060982 9.58 NRP1 VEGFA
14 commissural neuron axon guidance GO:0071679 9.58 NRP1 VEGFA
15 endothelial cell chemotaxis GO:0035767 9.57 NRP1 VEGFA
16 multicellular organismal water homeostasis GO:0050891 9.56 AQP1 AQP4
17 vascular endothelial growth factor receptor signaling pathway GO:0048010 9.56 FLT1 KDR NRP1 VEGFA
18 positive regulation of axon extension involved in axon guidance GO:0048842 9.54 NRP1 VEGFA
19 positive regulation of focal adhesion assembly GO:0051894 9.54 KDR NRP1 VEGFA
20 positive regulation of endothelial cell chemotaxis by VEGF-activated vascular endothelial growth factor receptor signaling pathway GO:0038033 9.51 KDR VEGFA
21 cell migration involved in sprouting angiogenesis GO:0002042 9.5 KDR NRP1 VEGFA
22 regulation of retinal ganglion cell axon guidance GO:0090259 9.48 NRP1 VEGFA
23 motor neuron migration GO:0097475 9.46 NRP1 VEGFA
24 VEGF-activated neuropilin signaling pathway GO:0038190 9.43 NRP1 VEGFA
25 positive regulation of retinal ganglion cell axon guidance GO:1902336 9.4 NRP1 VEGFA
26 positive regulation of angiogenesis GO:0045766 9.35 AQP1 FLT1 KDR NRP1 VEGFA
27 vascular endothelial growth factor signaling pathway GO:0038084 9.33 FLT1 KDR NRP1
28 cellular response to vascular endothelial growth factor stimulus GO:0035924 8.92 FLT1 KDR NRP1 VEGFA

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

# Name GO ID Score Top Affiliating Genes
1 channel activity GO:0015267 9.37 AQP1 AQP4
2 water channel activity GO:0015250 9.26 AQP1 AQP4
3 water transmembrane transporter activity GO:0005372 9.16 AQP1 AQP4
4 growth factor binding GO:0019838 9.13 FLT1 KDR NRP1
5 vascular endothelial growth factor-activated receptor activity GO:0005021 8.8 FLT1 KDR NRP1
6 protein binding GO:0005515 10.1 AQP1 AQP4 CFI FASLG FLT1 IL1RN

Sources for Acute Hemorrhagic Leukoencephalitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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