MCID: ACT032
MIFTS: 37

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 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 vagina leiomyosarcoma and efemp2-related cutis laxa. 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 top 50) (show all 55)
id Related Disease Score Top Affiliating Genes
1 vagina leiomyosarcoma 10.5 AQP1 IL1RN
2 efemp2-related cutis laxa 10.5 AQP1 AQP4
3 microcephaly, cerebellar hypoplasia, and cardiac conduction defect syndrome 10.5 AQP1 AQP4
4 sudden sensorineural hearing loss 10.4 AQP1 AQP4
5 cogan-reese syndrome 10.4 AQP4 MBP
6 senile reticular retinal degeneration 10.3 CFI IL1RN
7 testicular leydig cell tumor 10.3 FLT1 KDR
8 tricho onychic dysplasia 10.3 AQP4 MBP
9 mixed cell adenoma 10.2 AQP4 MBP
10 hypersplenism 10.2 AQP1 AQP4 MBP
11 newborn respiratory distress syndrome 10.1 AQP1 AQP4 MBP
12 tracheal stenosis 10.1 AQP4 MBP
13 extragonadal germ cell cancer 10.1 KDR VEGFA
14 iliac vein thrombophlebitis 10.1 KDR VEGFA
15 vesiculobullous skin disease 10.0 KDR VEGFA
16 herpes simplex 10.0
17 ulcerative colitis 10.0 AQP4 MBP
18 segmental odontomaxillary dysplasia 10.0 KDR VEGFA
19 relapsing-remitting multiple sclerosis 10.0
20 factitious disorder 9.9 KDR VEGFA
21 salivary gland adenoid cystic carcinoma 9.9 FLT1 VEGFA
22 arthrogryposis, ectodermal dysplasia, cleft lip/palate, and developmental delay 9.9 KDR VEGFA
23 placental choriocarcinoma 9.9 CFI KDR VEGFA
24 posterior foramen magnum meningioma 9.9 KDR NRP1 VEGFA
25 phlegmonous dacryocystitis 9.9 KDR VEGFA
26 peroxisomal biogenesis disorders 9.8 FLT1 VEGFA
27 diabetic peripheral angiopathy 9.8 AQP4 VEGFA
28 acitretin/etretinate embryopathy 9.8 FLT1 VEGFA
29 ulnar neuropathy 9.8 FLT1 VEGFA
30 brain injury 9.8
31 mumps 9.8
32 tuberculosis 9.8
33 myopathy 9.8
34 influenza 9.8
35 encephalitis 9.8
36 herpes simplex encephalitis 9.8
37 lipoic acid synthetase deficiency 9.8 FLT1 VEGFA
38 cellular schwannoma 9.7 AQP4 MBP
39 calciphylaxis 9.7 AQP1 AQP4 VEGFA
40 kidney pelvis papillary carcinoma 9.6 FLT1 KDR VEGFA
41 coloboma of superior eyelid 9.6 FLT1 KDR VEGFA
42 benign familial neonatal epilepsy 9.6 KDR VEGFA
43 mitral valve stenosis 9.6 FLT1 KDR VEGFA
44 subdural empyema 9.6 FLT1 KDR VEGFA
45 hidradenoma 9.6 FLT1 KDR VEGFA
46 chikungunya 9.6 FLT1 KDR VEGFA
47 prostate carcinoma in situ 9.6 AQP4 MBP VEGFA
48 citrullinemia, adult-onset type ii 9.6 FLT1 KDR VEGFA
49 mantle cell lymphoma 9.5 FASLG KDR VEGFA
50 ovarian brenner tumor 9.4 AQP1 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

MGI Mouse Phenotypes related to Acute Hemorrhagic Leukoencephalitis:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.07 AQP1 AQP4 CFI FASLG FLT1 IL1RN
2 growth/size/body region MP:0005378 10.06 AQP1 AQP4 FLT1 IL1RN KDR MBP
3 cardiovascular system MP:0005385 10.03 AQP1 AQP4 FASLG FLT1 IL1RN KDR
4 homeostasis/metabolism MP:0005376 10.02 CFI FASLG FLT1 IL1RN KDR MBP
5 immune system MP:0005387 9.91 AQP4 FASLG FLT1 IL1RN KDR MBP
6 nervous system MP:0003631 9.81 AQP1 AQP4 FASLG FLT1 KDR MBP
7 reproductive system MP:0005389 9.5 VEGFA AQP1 FASLG FLT1 IL1RN MBP
8 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
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
Acute hemorrhagic leukoencephalitis with severe brainstem and spinal cord involvement: MRI features with neuropathological confirmation. ( 21448963 )
2011
10
Serial computed tomography and magnetic resonance imaging findings of biphasic acute hemorrhagic leukoencephalitis localized to the brain stem and cerebellum. ( 21519996 )
2011
11
Acute hemorrhagic leukoencephalitis and hypoxic brain injury associated with H1N1 influenza. ( 20558397 )
2010
12
Atypical acute hemorrhagic leukoencephalitis (Hurst's disease) presenting with focal hemorrhagic brainstem lesion. ( 19847676 )
2010
13
Acute hemorrhagic leukoencephalitis: a critical entity for forensic pathologists to recognize. ( 20010289 )
2010
14
Teaching NeuroImages: Acute hemorrhagic leukoencephalitis after mumps. ( 19917982 )
2009
15
Acute hemorrhagic leukoencephalitis with atypical features. ( 19145402 )
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
Serial MR imaging findings of acute hemorrhagic leukoencephalitis: a case report. ( 16155148 )
2005
20
Acute hemorrhagic leukoencephalitis: neuroimaging features and neuropathologic diagnosis. ( 16163044 )
2005
21
Acute hemorrhagic leukoencephalitis manifesting as intracerebral hemorrhage associated with herpes simplex virus type I. ( 15917264 )
2005
22
Acute hemorrhagic leukoencephalitis (Hurst's disease) linked to Epstein-Barr virus infection. ( 15791481 )
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
Pediatric acute hemorrhagic leukoencephalitis: report of a surviving patient and review. ( 11810602 )
2002
27
Acute hemorrhagic leukoencephalitis in male sibs. ( 11840490 )
2002
28
Possible acute hemorrhagic leukoencephalitis manifesting as intracerebral hemorrhage on computed tomography--case report. ( 12206493 )
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. ( 10577084 )
1999
33
Case 1-1999: Acute hemorrhagic leukoencephalitis. ( 10577082 )
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. ( 1746242 )
1991
40
Acute hemorrhagic leukoencephalitis. ( 1844176 )
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 of herpes simplex-virus type. Histological and electronmicroscopical findings. ( 4290137 )
1967
48
Acute hemorrhagic leukoencephalitis. Report of 2 cases. ( 6016398 )
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

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 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)
id Name GO ID Score Top Affiliating Genes
1 angiogenesis GO:0001525 9.84 FLT1 KDR NRP1 VEGFA
2 positive regulation of endothelial cell proliferation GO:0001938 9.73 KDR NRP1 VEGFA
3 positive regulation of cell migration GO:0030335 9.69 FLT1 KDR VEGFA
4 positive regulation of endothelial cell migration GO:0010595 9.67 KDR NRP1 VEGFA
5 positive regulation of angiogenesis GO:0045766 9.67 AQP1 FLT1 KDR VEGFA
6 branching involved in blood vessel morphogenesis GO:0001569 9.64 NRP1 VEGFA
7 artery morphogenesis GO:0048844 9.63 NRP1 VEGFA
8 camera-type eye morphogenesis GO:0048593 9.63 AQP1 VEGFA
9 water transport GO:0006833 9.62 AQP1 AQP4
10 positive regulation of vascular endothelial growth factor receptor signaling pathway GO:0030949 9.62 FLT1 VEGFA
11 positive regulation of positive chemotaxis GO:0050927 9.61 KDR VEGFA
12 negative regulation of heterotypic cell-cell adhesion GO:0034115 9.6 IL1RN MBP
13 endothelial cell chemotaxis GO:0035767 9.59 NRP1 VEGFA
14 coronary artery morphogenesis GO:0060982 9.58 NRP1 VEGFA
15 multicellular organismal water homeostasis GO:0050891 9.58 AQP1 AQP4
16 commissural neuron axon guidance GO:0071679 9.57 NRP1 VEGFA
17 positive regulation of axon extension involved in axon guidance GO:0048842 9.55 NRP1 VEGFA
18 positive regulation of endothelial cell chemotaxis by VEGF-activated vascular endothelial growth factor receptor signaling pathway GO:0038033 9.52 KDR VEGFA
19 regulation of retinal ganglion cell axon guidance GO:0090259 9.48 NRP1 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 positive regulation of retinal ganglion cell axon guidance GO:1902336 9.43 NRP1 VEGFA
23 cell migration involved in sprouting angiogenesis GO:0002042 9.43 KDR NRP1 VEGFA
24 VEGF-activated neuropilin signaling pathway GO:0038190 9.4 NRP1 VEGFA
25 positive regulation of focal adhesion assembly GO:0051894 9.32 KDR VEGFA
26 positive regulation of cell migration involved in sprouting angiogenesis GO:0090050 9.3 VEGFA
27 vascular endothelial growth factor signaling pathway GO:0038084 9.13 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:

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 10.1 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
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 HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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