MCID: ACT232
MIFTS: 21

Acute Necrotizing Encephalopathy

Categories: Rare diseases, Neuronal diseases, Genetic diseases

Aliases & Classifications for Acute Necrotizing Encephalopathy

MalaCards integrated aliases for Acute Necrotizing Encephalopathy:

Name: Acute Necrotizing Encephalopathy 49 28 69
Encephalitis, Acute Necrotizing 69
Acute Necrotizing Encephalitis 49
Ane 49

Classifications:



External Ids:

UMLS 69 C1855020

Summaries for Acute Necrotizing Encephalopathy

NIH Rare Diseases : 49 Acute necrotizing encephalopathy (ANE) is a rare disease characterized by brain damage (encephalopathy) that usually follows an acute febrile disease, mostly viral infections.. Most of the reported cases are from previously healthy Japanese and Taiwanese children, but it is now known that the disease may affect anybody in the world.  The symptoms of the viral infection (fever, respiratory infection, and gastroenteritis, among others) are followed by seizures, disturbance of consciousness that may rapidly progress to a coma, liver problems, and neurological deficits. The disease is caused by both environmental factors and genetic factors. Usually, ANE develops secondary to viral infections, among which the influenza A, influenza B, and the human herpes virus 6, are the most common. ANE can be familial or sporadic, but both forms are very similar to each other. Most familial cases are caused by mutations in the RANBP2 gene, and are known as “infection-induced acute encephalopathy 3 (IIAE3)”.  Although the clinical course and the prognosis of ANE are diverse, the characteristic that is specific to the disease is the “multifocal symmetric brain lesions affecting the thalami, brain stem, cerebral white matter, and cerebellum” which can be seen on computed tomography (CT) or magnetic resonance imaging (MRI) exams. A picture of the brain may help you better visualize the areas of the brain affected. The best treatment of ANE is still under investigation but may include corticosteroids and anticytokine therapies, including TNFα antagonists.  Last updated: 2/23/2017

MalaCards based summary : Acute Necrotizing Encephalopathy, also known as encephalitis, acute necrotizing, is related to acute necrotizing encephalopathy type 1 and acute necrotizing encephalitis. An important gene associated with Acute Necrotizing Encephalopathy is RANBP2 (RAN Binding Protein 2). Affiliated tissues include brain, liver and cerebellum.

Related Diseases for Acute Necrotizing Encephalopathy

Graphical network of the top 20 diseases related to Acute Necrotizing Encephalopathy:



Diseases related to Acute Necrotizing Encephalopathy

Symptoms & Phenotypes for Acute Necrotizing Encephalopathy

Drugs & Therapeutics for Acute Necrotizing Encephalopathy

Search Clinical Trials , NIH Clinical Center for Acute Necrotizing Encephalopathy

Genetic Tests for Acute Necrotizing Encephalopathy

Genetic tests related to Acute Necrotizing Encephalopathy:

# Genetic test Affiliating Genes
1 Acute Necrotizing Encephalopathy 28

Anatomical Context for Acute Necrotizing Encephalopathy

MalaCards organs/tissues related to Acute Necrotizing Encephalopathy:

38
Brain, Liver, Cerebellum

Publications for Acute Necrotizing Encephalopathy

Articles related to Acute Necrotizing Encephalopathy:

(show all 31)
# Title Authors Year
1
Teaching NeuroImages: Acute necrotizing encephalopathy of childhood: Neuroimaging findings. ( 29311376 )
2018
2
Acute Necrotizing Encephalopathy of Childhood (ANEC): A Case Report. ( 28277560 )
2017
3
Acute Necrotizing Encephalopathy of Childhood Secondary to Dengue Infection: A Case Report from Pakistan. ( 28904577 )
2017
4
Acute necrotizing encephalopathy causing human bocavirus. ( 28059631 )
2017
5
Deep cerebral venous thrombosis mimicking influenza-associated acute necrotizing encephalopathy: a case report. ( 28950885 )
2017
6
Familial acute necrotizing encephalopathy with RANBP2 mutation: The first report in Northeast Asia. ( 28336122 )
2017
7
Influenza B-associated acute necrotizing encephalopathy of childhood: a report from North America. ( 26627900 )
2016
8
Familial acute necrotizing encephalopathy without RANBP2 mutation: Poor outcome. ( 27882739 )
2016
9
Specific HLA genotypes confer susceptibility to acute necrotizing encephalopathy. ( 27467284 )
2016
10
Bilateral Thalamic Lesions in Acute Necrotizing Encephalopathy Due to H1N1 Infection. ( 27647154 )
2016
11
Influenza A (H1N1) Infection Associated Acute Necrotizing Encephalopathy in a Child With Periodic Lateralized Epileptiform Discharges. ( 27898637 )
2016
12
Brain damage resembling acute necrotizing encephalopathy as a specific manifestation of haemophagocytic lymphohistiocytosis - induced by hypersensitivity. ( 27576518 )
2016
13
Influenza Virus-Associated Fatal Acute Necrotizing Encephalopathy: Role of Nonpermissive Viral Infection? ( 27812294 )
2016
14
Variable clinical course in acute necrotizing encephalopathy and identification of a novel RANBP2 mutation. ( 26923722 )
2016
15
RANBP2 mutation in an Indian child with recurrent acute necrotizing encephalopathy. ( 27591117 )
2016
16
Acute necrotizing encephalopathy in an adult. ( 25973284 )
2015
17
RANBP2 mutation and acute necrotizing encephalopathy: 2 cases and a literature review of the expanding clinico-radiological phenotype. ( 25522933 )
2015
18
An autopsy case of elderly-onset acute necrotizing encephalopathy secondary to influenza. ( 25982501 )
2015
19
Only a Touch of the Flu? The Simultaneous Manifestation of Acute Necrotizing Encephalopathy in Two Consanguineous Patients. ( 26110162 )
2015
20
Acute necrotizing encephalopathy: an underrecognized clinicoradiologic disorder. ( 25873770 )
2015
21
Fatal Acute Necrotizing Encephalopathy: Clinical Presentation and Imaging Findings. ( 25937389 )
2015
22
Acute necrotizing encephalopathy secondary to sepsis. ( 25827707 )
2014
23
Acute necrotizing encephalopathy (ANE1): rare autosomal-dominant disorder presenting as acute transverse myelitis. ( 23329376 )
2013
24
Acute necrotizing encephalopathy secondary to diphtheria, tetanus toxoid and whole-cell pertussis vaccination: diffusion-weighted imaging and proton MR spectroscopy findings. ( 20119724 )
2010
25
Genetic basis for acute necrotizing encephalopathy of childhood. ( 19807769 )
2010
26
Recurrent acute necrotizing encephalopathy following influenza A in a genetically predisposed family. ( 19811512 )
2010
27
Infection-triggered familial or recurrent cases of acute necrotizing encephalopathy caused by mutations in a component of the nuclear pore, RANBP2. ( 19118815 )
2009
28
[Infection-triggered familial or recurrent acute necrotizing encephalopathy]. ( 19643689 )
2009
29
Acute necrotizing encephalopathy associated with hemophagocytic syndrome. ( 16638510 )
2006
30
Autosomal dominant acute necrotizing encephalopathy maps to 2q12.1-2q13. ( 14755735 )
2004
31
Autosomal dominant acute necrotizing encephalopathy. ( 12874403 )
2003

Variations for Acute Necrotizing Encephalopathy

Expression for Acute Necrotizing Encephalopathy

Search GEO for disease gene expression data for Acute Necrotizing Encephalopathy.

Pathways for Acute Necrotizing Encephalopathy

GO Terms for Acute Necrotizing Encephalopathy

Sources for Acute Necrotizing Encephalopathy

3 CDC
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10 dbSNP
11 DGIdb
16 ExPASy
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27 GO
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29 HGMD
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31 HPO
32 ICD10
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50 NINDS
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58 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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