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:

(showing 84, show less)
# 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
51 aphasia 10.0
52 thrombosis 10.0
53 mumps 10.0
54 common cold 10.0
55 locked-in syndrome 10.0
56 mycoplasma pneumoniae pneumonia 10.0
57 polyneuropathy 10.0
58 status epilepticus 10.0
59 gastroenteritis 10.0
60 relapsing-remitting multiple sclerosis 10.0
61 glioblastoma multiforme 10.0
62 myopathy 10.0
63 acquired immunodeficiency syndrome 10.0
64 viral encephalitis 10.0
65 cerebrovascular disease 10.0
66 influenza 10.0
67 subacute delirium 10.0
68 chickenpox 10.0
69 myeloid leukemia 10.0
70 intracranial hypertension 10.0
71 miliary tuberculosis 10.0
72 allergic encephalomyelitis 10.0
73 glioma 10.0
74 brain injury 10.0
75 headache 10.0
76 glial tumor 10.0
77 usher syndrome, type i 10.0 USH1G SLC17A8 CDH23
78 usher syndrome, type ic 10.0 USH1G SLC17A8 CDH23
79 autosomal dominant nonsyndromic deafness 10.0 USH1G SLC17A8 CDH23
80 nonsyndromic deafness 10.0 USH1G SLC17A8 CDH23
81 deafness, autosomal recessive 12 9.9 USH1G CDH23
82 neurosarcoidosis 9.9 PNMA2 AQP4
83 encephalopathy 9.9
84 intracranial arteriosclerosis 9.9 PNMA2 AQP4

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 (showing 1, show less)
# 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:

(showing 1, show less)
# 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:

(showing 90, show less)
# 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
51
Pediatric acute hemorrhagic leukoencephalitis: report of a surviving patient and review. 61
11810602 2002
52
Successful recovery after high-dose intravenous methylprednisolone in acute hemorrhagic leukoencephalitis. 61
11697528 2001
53
Cerebral phaeohyphomycosis caused by a dematiaceous scopulariopsis species. 61
11594508 2001
54
Full recovery after acute hemorrhagic leukoencephalitis (Hurst's disease). 61
11200696 2000
55
Case 1-1999: Acute hemorrhagic leukoencephalitis. 61
10577082 1999
56
Case 1-1999: Acute hemorrhagic leukoencephalitis. 61
10577083 1999
57
Case 1-1999: Acute hemorrhagic leukoencephalitis. 61
10577084 1999
58
Fulminant form of acute disseminated encephalomyelitis: successful treatment with hypothermia. 61
10426155 1999
59
Acute hemorrhagic leukoencephalitis in patients with acute myeloid leukemia in hematologic complete remission. 61
10189394 1999
60
Neuroradiology case of the day. Unilateral acute hemorrhagic leukoencephalitis (AHL) complicated by venous thrombosis. 61
9460130 1998
61
Acute hemorrhagic leukoencephalitis during treatment for disseminated tuberculosis in a patient with AIDS. 61
9487459 1997
62
Acute hemorrhagic leukoencephalitis: recovery and reversal of magnetic resonance imaging findings in a child. 61
9373802 1997
63
[Acute hemorrhagic leukoencephalitis in a woman with Goodpasture syndrome]. 61
9290028 1997
64
[Acute Weston Hurst necrotizing hemorrhagic leukoencephalitis]. 61
9205699 1996
65
Acute hemorrhagic leukoencephalitis: a cause of acute brainstem dysfunction. 61
8052903 1994
66
Tubular inclusions in macrophages in the brain of a patient with acute hemorrhagic leukoencephalitis (Weston-Hurst syndrome). 61
8191625 1994
67
Intensive care management of acute hemorrhagic leukoencephalitis with favorable neurologic outcome. 61
1468137 1992
68
Acute hemorrhagic leukoencephalitis. A successful recovery. 61
1929904 1991
69
Acute hemorrhagic leukoencephalitis. 61
1844176 1991
70
Acute hemorrhagic leukoencephalitis. 61
1746242 1991
71
Neuropathology of two fatal cases of measles in the 1988-1989 Houston epidemic. 61
2340030 1990
72
[Acute hemorrhagic leukoencephalitis is often misdiagnosed as herpes encephalitis]. 61
2593761 1989
73
[MR tomography of acute hemorrhagic leukoencephalitis in childhood]. 61
2539632 1989
74
[Acute Borrelia burgdorferi encephalitis (Lyme disease) in an Algerian child]. 61
2798000 1989
75
Intracranial pressure and outcome in adult encephalitis. 61
3351587 1988
76
Histopathology of the central nervous system in the acquired immunodeficiency syndrome. 61
3596583 1987
77
An autopsied case of acute hemorrhagic leukoencephalitis. 61
3773347 1986
78
Acute hemorrhagic leukoencephalitis: diagnosis by computed tomography. 61
6742799 1984
79
Computed tomography in acute hemorrhagic leukoencephalitis. 61
6721456 1984
80
Computerized tomography as a diagnostic aid in acute hemorrhagic leukoencephalitis. 61
6847149 1983
81
Postinfectious leukoencephalitis complicating Mycoplasma pneumoniae infection. 61
6824440 1983
82
[Acute hemorrhagic leukoencephalitis]. 61
7060171 1982
83
Acute hemorrhagic leukoencephalitis: report of three cases and review of the literature. 61
1105376 1975
84
[The neurosurgical forms of Hurst's acute hemorrhagic leukoencephalitis]. 61
1228485 1975
85
[Hurst's acute hemorrhagic leukoencephalitis and sclerosing plaques]. 61
5378489 1969
86
Acute hemorrhagic leukoencephalitis. Report of 2 cases. 61
6016398 1967
87
Acute hemorrhagic leukoencephalitis of herpes simplex-virus type. Histological and electronmicroscopical findings. 61
4290137 1967
88
[CONTRIBUTION TO THE STUDY OF ACUTE HEMORRHAGIC LEUKOENCEPHALITIS (OF HURST)]. 61
14162222 1963
89
ACUTE HEMORRHAGIC LEUKOENCEPHALITIS; A CLINICAL AND ELECTRON-MICROSCOPIC REPORT OF 2 PATIENTS TREATED WITH SURGICAL DECOMPRESSION. 61
14058421 1963
90
[Acute hemorrhagic leukoencephalitis of Hurst (anatomoclinical study of a case)]. 61
13703563 1960

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:

(showing 1, show less)
# 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:

(showing 5, show less)
# 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
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11 DGIdb
17 EFO
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19 FMA
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33 ICD10 via Orphanet
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35 IUPHAR
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44 MESH via Orphanet
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48 NCI
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50 NDF-RT
53 NINDS
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57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
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72 UMLS via Orphanet
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