MCID: ACT032
MIFTS: 42

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 57 12 53 15
Ahl 57 12 53
Leukoencephalitis, Acute Hemorrhagic 44 73
Acute Haemorrhagic Leucoencephalitis of Weston Hurst 12
Acute Necrotizing Hemorrhagic Leukoencephalitis 12
Acute Hemorrhagic Encephalomyelitis 12
Weston-Hurst Syndrome 12
Ahle 53

Classifications:



External Ids:

OMIM 57 606752
Disease Ontology 12 DOID:10992
MeSH 44 D004684
NCIt 50 C84535
SNOMED-CT 68 72986009
MedGen 42 C0014077
UMLS 73 C0014077

Summaries for Acute Hemorrhagic Leukoencephalitis

NIH Rare Diseases : 53 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.  

MalaCards based summary : Acute Hemorrhagic Leukoencephalitis, also known as ahl, is related to inappropriate adh syndrome and intracranial hypertension, idiopathic. 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 homeostasis/metabolism

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 56)
# Related Disease Score Top Affiliating Genes
1 inappropriate adh syndrome 10.5 AQP1 AQP4
2 intracranial hypertension, idiopathic 10.5 AQP1 AQP4
3 subependymoma 10.5 AQP1 AQP4
4 balo concentric sclerosis 10.5 AQP4 MBP
5 dysferlinopathy 10.4 AQP1 AQP4
6 acute disseminated encephalomyelitis 10.4 AQP4 MBP
7 capillary hemangioma 10.2 KDR VEGFA
8 skin hemangioma 10.2 KDR VEGFA
9 central pontine myelinolysis 10.2 AQP1 AQP4 MBP
10 transverse myelitis 10.2 AQP4 MBP
11 neuromyelitis optica 10.2 AQP1 AQP4 MBP
12 radiation proctitis 10.2 KDR VEGFA
13 internuclear ophthalmoplegia 10.2 AQP4 MBP
14 kuhnt-junius degeneration 10.1 KDR VEGFA
15 herpes simplex 10.1
16 degeneration of macula and posterior pole 10.1 CFI KDR VEGFA
17 multiple sclerosis 10.0
18 relapsing-remitting multiple sclerosis 10.0
19 influenza 10.0
20 angiomatous meningioma 10.0 KDR NRP1 VEGFA
21 glomeruloid hemangioma 10.0 FLT1 VEGFA
22 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 10.0
23 anterior spinal artery syndrome 10.0 MBP SERPINC1
24 microcystic meningioma 9.9 FLT1 VEGFA
25 brain injury 9.9
26 mumps 9.9
27 myopathy 9.9
28 encephalitis 9.9
29 type i 9.9
30 glioblastoma 9.9
31 herpes simplex encephalitis 9.9
32 lipodermatosclerosis 9.9 FLT1 VEGFA
33 leukostasis 9.8 KDR VEGFA
34 cancer-associated retinopathy 9.8 FLT1 VEGFA
35 brain edema 9.8 AQP1 AQP4 VEGFA
36 hydrarthrosis 9.8 AQP1 IL1RN
37 angiokeratoma circumscriptum 9.7 FLT1 KDR VEGFA
38 epithelioid hemangioendothelioma 9.7 FLT1 KDR VEGFA
39 nervous system disease 9.7 AQP4 MBP VEGFA
40 meningococcemia 9.7 IL1RN SERPINC1
41 vascular cancer 9.7 FLT1 KDR VEGFA
42 breast carcinoma in situ 9.7 FLT1 KDR VEGFA
43 corneal neovascularization 9.7 FLT1 KDR VEGFA
44 placental insufficiency 9.7 FLT1 KDR VEGFA
45 angiosarcoma 9.7 FLT1 KDR VEGFA
46 microvascular complications of diabetes 5 9.7 FLT1 KDR VEGFA
47 peripartum cardiomyopathy 9.7 FLT1 VEGFA
48 intracranial hypertension 9.6 AQP1 AQP4 SERPINC1
49 hellp syndrome 9.5 CFI FLT1 SERPINC1
50 retinal vascular occlusion 9.5 SERPINC1 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:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.17 AQP1 AQP4 CFI FASLG FLT1 IL1RN
2 homeostasis/metabolism MP:0005376 10.14 AQP1 AQP4 CFI FASLG FLT1 IL1RN
3 cardiovascular system MP:0005385 10.13 AQP1 AQP4 FASLG FLT1 IL1RN KDR
4 growth/size/body region MP:0005378 10.11 AQP1 AQP4 FLT1 IL1RN KDR MBP
5 immune system MP:0005387 10.07 AQP4 FASLG FLT1 IL1RN KDR MBP
6 mortality/aging MP:0010768 10.02 IL1RN KDR MBP NRP1 SERPINC1 VEGFA
7 nervous system MP:0003631 9.96 NRP1 OCLN SERPINC1 VEGFA AQP1 AQP4
8 renal/urinary system MP:0005367 9.63 AQP1 AQP4 CFI FASLG SERPINC1 VEGFA
9 reproductive system MP:0005389 9.56 AQP1 FASLG FLT1 IL1RN MBP OCLN
10 vision/eye MP:0005391 9.28 SERPINC1 VEGFA AQP1 AQP4 FASLG FLT1

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:

41
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
Diffuse glioblastoma resembling acute hemorrhagic leukoencephalitis. ( 29184769 )
2017
2
Acute hemorrhagic leukoencephalitis after seasonal influenza vaccination. ( 29134580 )
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
Autopsy-proven Acute Hemorrhagic Leukoencephalitis in an Elderly Patient. ( 24384347 )
2014
7
Acute hemorrhagic leukoencephalitis associated with autoimmune myopathy. ( 25422709 )
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
Atypical acute hemorrhagic leukoencephalitis (Hurst's disease) presenting with focal hemorrhagic brainstem lesion. ( 19847676 )
2010
14
Acute hemorrhagic leukoencephalitis: a critical entity for forensic pathologists to recognize. ( 20010289 )
2010
15
Acute hemorrhagic leukoencephalitis and hypoxic brain injury associated with H1N1 influenza. ( 20558397 )
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
Acute hemorrhagic leukoencephalitis manifesting as intracerebral hemorrhage associated with herpes simplex virus type I. ( 15917264 )
2005
22
Acute hemorrhagic leukoencephalitis: neuroimaging features and neuropathologic diagnosis. ( 16163044 )
2005
23
Serial MR imaging findings of acute hemorrhagic leukoencephalitis: a case report. ( 16155148 )
2005
24
Acute hemorrhagic leukoencephalitis (Hurst's disease) linked to Epstein-Barr virus infection. ( 15791481 )
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
Possible acute hemorrhagic leukoencephalitis manifesting as intracerebral hemorrhage on computed tomography--case report. ( 12206493 )
2002
28
Acute hemorrhagic leukoencephalitis in male sibs. ( 11840490 )
2002
29
Detection of infectious agents in brain of patients with acute hemorrhagic leukoencephalitis. ( 12402170 )
2002
30
Pediatric acute hemorrhagic leukoencephalitis: report of a surviving patient and review. ( 11810602 )
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. ( 10577082 )
1999
34
Case 1-1999: Acute hemorrhagic leukoencephalitis. ( 10577083 )
1999
35
Case 1-1999: Acute hemorrhagic leukoencephalitis. ( 10577084 )
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
Tubular inclusions in macrophages in the brain of a patient with acute hemorrhagic leukoencephalitis (Weston-Hurst syndrome). ( 8191625 )
1994
39
Acute hemorrhagic leukoencephalitis: a cause of acute brainstem dysfunction. ( 8052903 )
1994
40
Intensive care management of acute hemorrhagic leukoencephalitis with favorable neurologic outcome. ( 1468137 )
1992
41
Acute hemorrhagic leukoencephalitis. A successful recovery. ( 1929904 )
1991
42
Acute hemorrhagic leukoencephalitis. ( 1844176 )
1991
43
Acute hemorrhagic leukoencephalitis. ( 1746242 )
1991
44
An autopsied case of acute hemorrhagic leukoencephalitis. ( 3773347 )
1986
45
Acute hemorrhagic leukoencephalitis: diagnosis by computed tomography. ( 6742799 )
1984
46
Computed tomography in acute hemorrhagic leukoencephalitis. ( 6721456 )
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 14)
# Super pathways Score Top Affiliating Genes
1 12.5 FASLG FLT1 KDR VEGFA
2
Show member pathways
12.45 FASLG FLT1 KDR VEGFA
3
Show member pathways
12.13 FLT1 KDR NRP1 VEGFA
4
Show member pathways
11.99 FLT1 KDR NRP1 VEGFA
5 11.91 AQP4 IL1RN VEGFA
6 11.79 FLT1 KDR RANBP2 VEGFA
7 11.75 AQP1 AQP4 MBP
8
Show member pathways
11.71 FLT1 KDR VEGFA
9
Show member pathways
11.22 FLT1 KDR NRP1 VEGFA
10 11.17 FLT1 KDR VEGFA
11 11.03 FLT1 KDR VEGFA
12 10.85 FLT1 SERPINC1 VEGFA
13 10.71 FLT1 KDR VEGFA
14
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 plasma membrane GO:0005886 9.96 AQP1 AQP4 FASLG FLT1 IL1RN KDR
2 extracellular space GO:0005615 9.7 CFI FASLG FLT1 IL1RN NRP1 SERPINC1
3 extracellular region GO:0005576 9.56 CFI FASLG FLT1 IL1RN KDR NRP1
4 receptor complex GO:0043235 9.54 FLT1 KDR NRP1
5 sorting endosome GO:0097443 8.62 KDR NRP1
6 membrane GO:0016020 10.1 AQP1 AQP4 CFI FASLG FLT1 KDR

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

(show all 28)
# Name GO ID Score Top Affiliating Genes
1 angiogenesis GO:0001525 9.86 FLT1 KDR NRP1 VEGFA
2 positive regulation of endothelial cell proliferation GO:0001938 9.74 KDR NRP1 VEGFA
3 vasculogenesis GO:0001570 9.71 FLT1 KDR VEGFA
4 positive regulation of endothelial cell migration GO:0010595 9.69 KDR NRP1 VEGFA
5 branching involved in blood vessel morphogenesis GO:0001569 9.64 NRP1 VEGFA
6 sprouting angiogenesis GO:0002040 9.63 FLT1 NRP1
7 artery morphogenesis GO:0048844 9.63 NRP1 VEGFA
8 positive regulation of cell migration involved in sprouting angiogenesis GO:0090050 9.63 KDR NRP1 VEGFA
9 positive regulation of vascular endothelial growth factor receptor signaling pathway GO:0030949 9.62 FLT1 VEGFA
10 water transport GO:0006833 9.62 AQP1 AQP4
11 positive regulation of positive chemotaxis GO:0050927 9.61 KDR VEGFA
12 negative regulation of heterotypic cell-cell adhesion GO:0034115 9.61 IL1RN MBP
13 endothelial cell chemotaxis GO:0035767 9.6 NRP1 VEGFA
14 coronary artery morphogenesis GO:0060982 9.59 NRP1 VEGFA
15 multicellular organismal water homeostasis GO:0050891 9.58 AQP1 AQP4
16 commissural neuron axon guidance GO:0071679 9.58 NRP1 VEGFA
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 9.55 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 9.52 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 9.49 NRP1 VEGFA
23 motor neuron migration GO:0097475 9.48 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 heparin binding GO:0008201 9.58 NRP1 SERPINC1 VEGFA
2 channel activity GO:0015267 9.43 AQP1 AQP4
3 water channel activity GO:0015250 9.32 AQP1 AQP4
4 water transmembrane transporter activity GO:0005372 9.26 AQP1 AQP4
5 vascular endothelial growth factor binding GO:0038085 9.16 KDR NRP1
6 growth factor binding GO:0019838 9.13 FLT1 KDR NRP1
7 vascular endothelial growth factor-activated receptor activity GO:0005021 8.8 FLT1 KDR NRP1
8 protein binding GO:0005515 10.18 AQP1 AQP4 CFI FASLG FLT1 IL1RN

Sources for Acute Hemorrhagic Leukoencephalitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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