AHL
MCID: ACT032
MIFTS: 38

Acute Hemorrhagic Leukoencephalitis (AHL) malady

Categories: Rare diseases, Neuronal diseases

Aliases & Classifications for Acute Hemorrhagic Leukoencephalitis

Aliases & Descriptions for Acute Hemorrhagic Leukoencephalitis:

Name: Acute Hemorrhagic Leukoencephalitis 12 50 14
Leukoencephalitis, Acute Hemorrhagic 42 69
Ahl 12 50
Acute Haemorrhagic Leucoencephalitis of Weston Hurst 12
Acute Necrotizing Hemorrhagic Leukoencephalitis 12
Acute Hemorrhagic Encephalomyelitis 12
Weston-Hurst Syndrome 12
Ahle 50

Classifications:



External Ids:

Disease Ontology 12 DOID:10992
MeSH 42 D004684
NCIt 47 C84535
SNOMED-CT 64 72986009
UMLS 69 C0014077

Summaries for Acute Hemorrhagic Leukoencephalitis

NIH Rare Diseases : 50 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/superpathways are Ras signaling pathway and TRAF 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.

Related Diseases for Acute Hemorrhagic Leukoencephalitis

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

(show all 34)
id Related Disease Score Top Affiliating Genes
1 ehlers-danlos syndrome, kyphoscoliotic form 10.1 AQP1 AQP4
2 childhood brainstem astrocytoma 10.1 AQP1 AQP4 MBP
3 niemann-pick disease 10.1 AQP1 AQP4 MBP
4 crest syndrome 10.1 AQP4 MBP
5 hand, foot and mouth disease 10.0 KDR VEGFA
6 herpes simplex 10.0
7 neurilemmoma of the pleura 10.0 KDR NRP1 VEGFA
8 placental choriocarcinoma 10.0 CFI KDR VEGFA
9 laryngeal adenoid cystic carcinoma 10.0 FLT1 VEGFA
10 multinodular goiter 10.0 IL1RN KDR VEGFA
11 cone-rod dystrophy 17 10.0 KDR VEGFA
12 kidney osteogenic sarcoma 10.0 FLT1 KDR VEGFA
13 neuropathy with hearing impairment 10.0 FLT1 KDR VEGFA
14 relapsing-remitting multiple sclerosis 9.9
15 mitral valve stenosis 9.9 FLT1 KDR VEGFA
16 subdural empyema 9.9 FLT1 KDR VEGFA
17 histiocytoid hemangioma 9.9 AQP1 AQP4 VEGFA
18 vulvitis 9.9 FLT1 KDR VEGFA
19 human granulocytic anaplasmosis 9.9 FLT1 KDR VEGFA
20 citrullinemia, type ii, neonatal-onset 9.9 FLT1 KDR VEGFA
21 hemolytic anemia 9.9 FLT1 KDR VEGFA
22 schwannoma of twelfth cranial nerve 9.9 AQP4 CD8A MBP
23 albinism, oculocutaneous, type ib 9.9 FLT1 KDR NRP1 VEGFA
24 vernal conjunctivitis 9.8 FLT1 KDR OCLN VEGFA
25 brain injury 9.8
26 mumps 9.8
27 tuberculosis 9.8
28 myopathy 9.8
29 influenza 9.8
30 encephalitis 9.8
31 herpes simplex encephalitis 9.8
32 camptodactyly-arthropathy-coxa vara-pericarditis syndrome 9.8 CFI FLT1 KDR VEGFA
33 thymus adenosquamous carcinoma 9.7 FLT1 KDR VEGFA
34 ovarian disease 8.3 AQP1 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

MGI Mouse Phenotypes related to Acute Hemorrhagic Leukoencephalitis:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.14 KDR MBP NRP1 VEGFA AQP1 AQP4
2 growth/size/body region MP:0005378 10.09 AQP4 FLT1 IL1RN KDR MBP NRP1
3 homeostasis/metabolism MP:0005376 10.07 AQP1 AQP4 CFI FASLG FLT1 IL1RN
4 cardiovascular system MP:0005385 10.06 AQP1 AQP4 FASLG FLT1 IL1RN KDR
5 immune system MP:0005387 10.02 AQP4 CD8A FASLG FLT1 IL1RN KDR
6 mortality/aging MP:0010768 9.96 AQP1 AQP4 CD8A FASLG FLT1 IL1RN
7 nervous system MP:0003631 9.85 MBP NRP1 OCLN VEGFA AQP1 AQP4
8 reproductive system MP:0005389 9.5 AQP1 FASLG FLT1 IL1RN MBP OCLN
9 vision/eye MP:0005391 9.23 AQP1 AQP4 FASLG FLT1 KDR MBP

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:

39
Brain, Spinal Cord, Cerebellum

Publications for Acute Hemorrhagic Leukoencephalitis

Articles related to Acute Hemorrhagic Leukoencephalitis:

(show all 49)
id Title Authors Year
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

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)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
12.45 FASLG FLT1 KDR VEGFA
2
Show member pathways
12.36 FASLG FLT1 KDR NRP1 VEGFA
3 12.29 FASLG FLT1 KDR VEGFA
4
Show member pathways
12.08 FLT1 KDR NRP1 VEGFA
5 11.92 AQP4 IL1RN VEGFA
6
Show member pathways
11.92 FLT1 KDR NRP1 VEGFA
7 11.75 AQP1 AQP4 MBP
8
Show member pathways
11.71 FLT1 KDR VEGFA
9 11.36 FLT1 KDR RANBP2 VEGFA
10
Show member pathways
11.22 FLT1 KDR NRP1 VEGFA
11 10.96 FLT1 KDR VEGFA
12 10.94 FLT1 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:

id Name GO ID Score Top Affiliating Genes
1 plasma membrane GO:0005886 9.96 AQP1 AQP4 CD8A FASLG FLT1 IL1RN
2 extracellular space GO:0005615 9.85 CFI FASLG FLT1 IL1RN NRP1 VEGFA
3 integral component of plasma membrane GO:0005887 9.8 AQP1 AQP4 CD8A FASLG FLT1 KDR
4 extracellular region GO:0005576 9.76 CD8A CFI FASLG FLT1 IL1RN KDR
5 membrane GO:0016020 9.44 AQP1 AQP4 CD8A CFI FASLG FLT1
6 sorting endosome GO:0097443 8.96 KDR NRP1

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

(show all 27)
id Name GO ID Score Top Affiliating Genes
1 angiogenesis GO:0001525 9.83 FLT1 KDR NRP1 VEGFA
2 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.78 CD8A FLT1 KDR
3 positive regulation of endothelial cell proliferation GO:0001938 9.71 KDR NRP1 VEGFA
4 positive regulation of endothelial cell migration GO:0010595 9.65 KDR NRP1 VEGFA
5 branching involved in blood vessel morphogenesis GO:0001569 9.63 NRP1 VEGFA
6 positive regulation of focal adhesion assembly GO:0051894 9.63 KDR VEGFA
7 artery morphogenesis GO:0048844 9.62 NRP1 VEGFA
8 camera-type eye morphogenesis GO:0048593 9.62 AQP1 VEGFA
9 positive regulation of angiogenesis GO:0045766 9.62 AQP1 FLT1 KDR VEGFA
10 water transport GO:0006833 9.61 AQP1 AQP4
11 positive regulation of vascular endothelial growth factor receptor signaling pathway GO:0030949 9.6 FLT1 VEGFA
12 positive regulation of positive chemotaxis GO:0050927 9.59 KDR VEGFA
13 endothelial cell chemotaxis GO:0035767 9.58 NRP1 VEGFA
14 negative regulation of heterotypic cell-cell adhesion GO:0034115 9.58 IL1RN MBP
15 multicellular organismal water homeostasis GO:0050891 9.57 AQP1 AQP4
16 commissural neuron axon guidance GO:0071679 9.56 NRP1 VEGFA
17 coronary artery morphogenesis GO:0060982 9.55 NRP1 VEGFA
18 positive regulation of axon extension involved in axon guidance GO:0048842 9.51 NRP1 VEGFA
19 positive regulation of endothelial cell chemotaxis by VEGF-activated vascular endothelial growth factor receptor signaling pathway GO:0038033 9.49 KDR VEGFA
20 motor neuron migration GO:0097475 9.46 NRP1 VEGFA
21 vascular endothelial growth factor receptor signaling pathway GO:0048010 9.46 FLT1 KDR NRP1 VEGFA
22 regulation of retinal ganglion cell axon guidance GO:0090259 9.43 NRP1 VEGFA
23 cell migration involved in sprouting angiogenesis GO:0002042 9.43 KDR NRP1 VEGFA
24 positive regulation of retinal ganglion cell axon guidance GO:1902336 9.4 NRP1 VEGFA
25 VEGF-activated neuropilin signaling pathway GO:0038190 9.37 NRP1 VEGFA
26 vascular endothelial growth factor signaling pathway GO:0038084 9.13 FLT1 KDR NRP1
27 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:

id Name GO ID Score Top Affiliating Genes
1 water channel activity GO:0015250 9.26 AQP1 AQP4
2 water transmembrane transporter activity GO:0005372 9.16 AQP1 AQP4
3 growth factor binding GO:0019838 9.13 FLT1 KDR NRP1
4 vascular endothelial growth factor-activated receptor activity GO:0005021 8.8 FLT1 KDR NRP1
5 protein binding GO:0005515 10.13 AQP1 AQP4 CD8A CFI FASLG FLT1

Sources for Acute Hemorrhagic Leukoencephalitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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