AHL
MCID: ACT032
MIFTS: 32

Acute Hemorrhagic Leukoencephalitis (AHL)

Categories: Cardiovascular diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Acute Hemorrhagic Leukoencephalitis

MalaCards integrated aliases for Acute Hemorrhagic Leukoencephalitis:

Name: Acute Hemorrhagic Leukoencephalitis 56 12 52 15 17
Ahl 56 12 52
Leukoencephalitis, Acute Hemorrhagic 43 71
Acute Haemorrhagic Leucoencephalitis of Weston Hurst 12
Acute Necrotizing Hemorrhagic Leukoencephalitis 12
Acute Hemorrhagic Encephalomyelitis 12
Weston-Hurst Syndrome 12
Ahle 52

Classifications:



External Ids:

Disease Ontology 12 DOID:10992
OMIM 56 606752
MeSH 43 D004684
NCIt 49 C84535
SNOMED-CT 67 72986009
MedGen 41 C0014077
UMLS 71 C0014077

Summaries for Acute Hemorrhagic Leukoencephalitis

NIH Rare Diseases : 52 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 acute disseminated encephalomyelitis and usher syndrome. An important gene associated with Acute Hemorrhagic Leukoencephalitis is CFI (Complement Factor I). Affiliated tissues include brain, spinal cord and myeloid, and related phenotype is hearing/vestibular/ear.

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.

More information 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 84)
# Related Disease Score Top Affiliating Genes
1 acute disseminated encephalomyelitis 30.4 PNMA2 CDH23 AQP4
2 usher syndrome 30.2 USH1G FSCN2 CDH23
3 usher syndrome, type id 30.1 USH1G CDH23
4 branchiootic syndrome 1 29.9 USH1G CDH23
5 encephalitis 10.5
6 demyelinating disease 10.5
7 age-related hearing loss 10.5
8 ocular motor apraxia 10.4
9 herpes simplex 10.4
10 quadriplegia 10.4
11 cerebritis 10.4
12 myocarditis 10.4
13 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 10.4
14 bacterial infectious disease 10.3
15 ataxia and polyneuropathy, adult-onset 10.3
16 herpes simplex encephalitis 10.3
17 usher syndrome, type ih 10.3 USH1G CDH23
18 usher syndrome, type ij 10.2 USH1G CDH23
19 deafness, autosomal dominant 20 10.2 USH1G CDH23
20 deafness, autosomal dominant 25 10.2 SLC17A8 FSCN2
21 hearing loss, noise-induced 10.2
22 stenotrophomonas maltophilia infection 10.2
23 usher syndrome, type iid 10.2 USH1G CDH23
24 multiple sclerosis 10.2
25 hemiplegia 10.2
26 deafness, autosomal recessive 2 10.2 USH1G CDH23
27 deafness, autosomal recessive 23 10.2 USH1G CDH23
28 deafness, autosomal dominant 11 10.1 USH1G CDH23
29 usher syndrome, type ig 10.1 USH1G CDH23
30 usher syndrome, type if 10.1 USH1G CDH23
31 usher syndrome, type iiia 10.1 USH1G FSCN2 CDH23
32 usher syndrome, type iic 10.0 USH1G CDH23
33 papillomatosis, confluent and reticulated 10.0
34 retinitis pigmentosa 10.0
35 retinitis pigmentosa-deafness syndrome 10.0
36 lymphoplasmacytic lymphoma 10.0
37 sensorineural hearing loss 10.0
38 neuroretinitis 10.0
39 listeriosis 10.0
40 typhoid fever 10.0
41 severe acute respiratory syndrome 10.0
42 retinitis 10.0
43 amyloidosis 10.0
44 al amyloidosis 10.0
45 anaplastic oligoastrocytoma 10.0
46 overgrowth syndrome 10.0
47 thrombophilia due to thrombin defect 10.0
48 goodpasture syndrome 10.0
49 kearns-sayre syndrome 10.0
50 leukemia, acute myeloid 10.0

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:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hearing/vestibular/ear MP:0005377 9.02 AQP4 CDH23 FSCN2 SLC17A8 USH1G

Drugs & Therapeutics for Acute Hemorrhagic Leukoencephalitis

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Management of Acute Disseminated Encephalomyelitis in Neurology Unit of Assiut University Children Hospital Unknown status NCT03284801

Search 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:

40
Brain, Spinal Cord, Myeloid, T Cells, Cerebellum, Endothelial

Publications for Acute Hemorrhagic Leukoencephalitis

Articles related to Acute Hemorrhagic Leukoencephalitis:

(show top 50) (show all 90)
# Title Authors PMID Year
1
Acute hemorrhagic leukoencephalitis in male sibs. 61 56
11840490 2002
2
Acute hemorrhagic leukoencephalitis of Weston Hurst secondary to herpes encephalitis presenting as status epilepticus: A case report and review of literature. 61
31239199 2019
3
Fulminant acute disseminated encephalomyelitis in children. 61
30522797 2019
4
Intravenous immunoglobulin for acute hemorrhagic leukoencephalitis refractory to plasmapheresis. 61
30656033 2019
5
Acute hemorrhagic leukoencephalitis after seasonal influenza vaccination. 61
29134580 2018
6
Diffuse glioblastoma resembling acute hemorrhagic leukoencephalitis. 61
29184769 2017
7
Sudden Death of a Young Man by Acute Hemorrhagic Leukoencephalitis. 61
31239997 2017
8
Acute Hemorrhagic Leukoencephalitis in Children: A Case Report. 61
27217610 2016
9
Acute disseminated encephalomyelitis presenting as a brainstem encephalitis. 61
26903073 2016
10
Mycoplasma pneumoniae, a trigger for Weston Hurst syndrome. 61
26819961 2016
11
Commentary: Acute Hemorrhagic Leukoencephalitis (Weston-Hurst Syndrome) in a Patient with Relapse-Remitting Multiple Sclerosis. 61
27252706 2016
12
Hemorrhagic Longitudinally Extensive Transverse Myelitis. 61
27847660 2016
13
Fulminant Demyelinating Diseases of the Central Nervous System. 61
26595866 2015
14
Acute hemorrhagic leukoencephalitis (Weston-Hurst syndrome) in a patient with relapse-remitting multiple sclerosis. 61
26376717 2015
15
Acute hemorrhagic leukoencephalitis associated with autoimmune myopathy. 61
25422709 2014
16
Early and widespread injury of astrocytes in the absence of demyelination in acute haemorrhagic leukoencephalitis. 61
24887055 2014
17
The diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review. 61
24524923 2014
18
Autopsy-proven Acute Hemorrhagic Leukoencephalitis in an Elderly Patient. 61
24384347 2014
19
Fulminant form of acute disseminated encephalomyelitis in a child treated with mild hypothermia. 61
24330300 2013
20
Fulminant demyelinating diseases. 61
23983890 2013
21
First case report of acute hemorrhagic leukoencephalitis following Plasmodium vivax infection. 61
23508437 2013
22
Mutations of complement factor I and potential mechanisms of neuroinflammation in acute hemorrhagic leukoencephalitis. 61
22926405 2013
23
Value of susceptibility-weighted imaging in acute hemorrhagic leukoencephalitis. 61
22465779 2012
24
Preserved vascular integrity and enhanced survival following neuropilin-1 inhibition in a mouse model of CD8 T cell-initiated CNS vascular permeability. 61
22985494 2012
25
Neuroimaging in postinfectious demyelination and nutritional disorders of the central nervous system. 61
22032502 2011
26
Serial computed tomography and magnetic resonance imaging findings of biphasic acute hemorrhagic leukoencephalitis localized to the brain stem and cerebellum. 61
21519996 2011
27
Acute hemorrhagic leukoencephalitis with severe brainstem and spinal cord involvement: MRI features with neuropathological confirmation. 61
21448963 2011
28
Acute hemorrhagic leukoencephalitis and hypoxic brain injury associated with H1N1 influenza. 61
20558397 2010
29
Update on investigation and management of postinfectious encephalitis. 61
20442573 2010
30
Acute hemorrhagic leukoencephalitis: a critical entity for forensic pathologists to recognize. 61
20010289 2010
31
Atypical acute hemorrhagic leukoencephalitis (Hurst's disease) presenting with focal hemorrhagic brainstem lesion. 61
19847676 2010
32
CD8 T cell-initiated vascular endothelial growth factor expression promotes central nervous system vascular permeability under neuroinflammatory conditions. 61
20008293 2010
33
Teaching NeuroImages: Acute hemorrhagic leukoencephalitis after mumps. 61
19917982 2009
34
Post-infectious encephalitis in adults: diagnosis and management. 61
19368974 2009
35
Acute hemorrhagic leukoencephalitis with atypical features. 61
19145402 2009
36
Induction of blood brain barrier tight junction protein alterations by CD8 T cells. 61
18725947 2008
37
[Hurst acute haemorrhagic leukoencephalitis: MRI findings]. 61
17966249 2007
38
Use of therapeutic plasma exchange in the management of acute hemorrhagic leukoencephalitis: a case report and review of the literature. 61
17524086 2007
39
Treatment leading to dramatic recovery in acute hemorrhagic leukoencephalitis. 61
17608318 2007
40
The importance of EEG and variability of MRI findings in acute hemorrhagic leukoencephalitis. 61
17038020 2006
41
Serial MR imaging findings of acute hemorrhagic leukoencephalitis: a case report. 61
16155148 2005
42
Acute hemorrhagic leukoencephalitis: neuroimaging features and neuropathologic diagnosis. 61
16163044 2005
43
Acute hemorrhagic leukoencephalitis manifesting as intracerebral hemorrhage associated with herpes simplex virus type I. 61
15917264 2005
44
Acute hemorrhagic leukoencephalitis (Hurst's disease) linked to Epstein-Barr virus infection. 61
15791481 2005
45
Treatment of refractory status epilepticus with inhalational anesthetic agents isoflurane and desflurane. 61
15313843 2004
46
Acute hemorrhagic leukoencephalitis mimicking herpes simplex encephalitis: case report. 61
15122448 2004
47
Acute hemorrhagic leukoencephalitis vs ADEM: FLAIR MRI and neuropathology findings. 61
12601124 2003
48
Detection of infectious agents in brain of patients with acute hemorrhagic leukoencephalitis. 61
12402170 2002
49
[Weston Hurst acute haemorrhagic leukoencephalitis. Neuropathological study of one case]. 61
12235562 2002
50
Possible acute hemorrhagic leukoencephalitis manifesting as intracerebral hemorrhage on computed tomography--case report. 61
12206493 2002

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:

# Name GO ID Score Top Affiliating Genes
1 stereocilium GO:0032420 8.62 FSCN2 CDH23

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

# Name GO ID Score Top Affiliating Genes
1 sensory perception of sound GO:0007605 9.33 USH1G SLC17A8 CDH23
2 photoreceptor cell maintenance GO:0045494 9.32 USH1G CDH23
3 inner ear receptor cell stereocilium organization GO:0060122 9.26 USH1G CDH23
4 sensory perception of light stimulus GO:0050953 8.96 USH1G CDH23
5 equilibrioception GO:0050957 8.62 USH1G CDH23

Sources for Acute Hemorrhagic Leukoencephalitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
Content
Loading form....