ANE
MCID: ACT232
MIFTS: 28

Acute Necrotizing Encephalopathy (ANE)

Categories: Genetic diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Acute Necrotizing Encephalopathy

MalaCards integrated aliases for Acute Necrotizing Encephalopathy:

Name: Acute Necrotizing Encephalopathy 53 72
Encephalitis, Acute Necrotizing 72
Acute Necrotizing Encephalitis 53
Ane 53

Classifications:



External Ids:

UMLS 72 C0338418 C1855020

Summaries for Acute Necrotizing Encephalopathy

NIH Rare Diseases : 53 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 TNF? antagonists.

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

Diseases in the Acute Necrotizing Encephalopathy family:

Acute Necrotizing Encephalopathy Type 1 Familial Acute Necrotizing Encephalopathy

Diseases related to Acute Necrotizing Encephalopathy via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 13090)
# Related Disease Score Top Affiliating Genes
1 acute necrotizing encephalopathy type 1 12.8
2 acute necrotizing encephalitis 12.7
3 familial acute necrotizing encephalopathy 12.6
4 hair-an syndrome 12.3
5 rare hemorrhagic disorder due to an acquired platelet anomaly 12.2
6 obsolete: male infertility with impaired virilization due to an acquired testicular defect associated with an environmental toxin 12.2
7 hemophagocytic syndrome associated with an infection 12.2
8 systemic mastocytosis with an associated clonal hematologic non-mast cell lineage disease 12.2
9 rare hemorrhagic disorder due to an acquired coagulation factor defect 12.1
10 sickle cell disease associated with an other hemoglobin anomaly 12.1
11 obsolete: common variable immunodeficiency due to an intrinsic b cell defect 12.1
12 hemolytic anemia due to an erythrocyte nucleotide metabolism disorder 12.1
13 obsolete: common variable immunodeficiency due to an intrinsic t cell defect 12.1
14 sucking/swallowing disorder associated with an identified syndrome 12.0
15 particular clinical situation or non rare disorder with an orphan designation 12.0
16 rare thrombotic disorder due to an acquired platelet anomaly 12.0
17 rare thrombotic disorder due to an acquired coagulation factors defect 12.0
18 systemic inflammatory disease associated with an acquired peripheral neuropathy 12.0
19 hematological disease associated with an acquired peripheral neuropathy 12.0
20 solid tumor associated with an acquired peripheral neuropathy 12.0
21 silver-russell syndrome due to an imprinting defect of 11p15 12.0
22 obsolete: polydactyly of an index finger, unilateral 12.0
23 obsolete: polydactyly of an index finger, bilateral 12.0
24 rare female infertility due to an implantation defect 12.0
25 female infertility due to an implantation defect of genetic origin 12.0
26 rare female infertility due to an anomaly of ovarian function of genetic origin 12.0
27 rare female infertility due to an anomaly of ovarian function 12.0
28 rare female infertility due to an adrenal disorder 12.0
29 obsolete: channelopathy due to an inwardly rectifying potassium channel defect 12.0
30 obsolete: channelopathy due to an epithelial sodium channel defect 12.0
31 obsolete: pituitary deficiency secondary to an anevrysm 12.0
32 obsolete: spinocerebellar degenerescence and spastic paraparesis with an oculomotor anomaly 12.0
33 obsolete: autoimmune neurological channelopathy due to an acetylcholine receptor subunits defect 12.0
34 obsolete: male infertility with normal virilization due to an acquired testicular defect associated with mycoplasma infection 12.0
35 obsolete: male infertility with normal virilization due to an acquired testicular defect 12.0
36 obsolete: male infertility with normal virilization due to an acquired testicular defect associated with drug 12.0
37 obsolete: male infertility with normal virilization due to an acquired testicular defect associated with radiation 12.0
38 obsolete: male infertility with normal virilization due to an acquired testicular defect associated with environmental toxin 12.0
39 obsolete: male infertility with impaired virilization due to an hypothalamic or pituitary disorder 12.0
40 obsolete: male infertility with impaired virilization due to an acquired testicular defect associated with autoimmunity 12.0
41 obsolete: male infertility with impaired virilization due to an acquired testicular defect associated with a granulomatous disease 12.0
42 obsolete: male infertility with impaired virilization due to a testicular disorder associated with an immune disorder 12.0
43 obsolete: male infertility with impaired virilization due to an acquired testicular defect 12.0
44 obsolete: male infertility with impaired virilization due to an acquired testicular defect drug-related 12.0
45 obsolete: male infertility with impaired virilization due to an acquired testicular defect associated with trauma 12.0
46 rare constitutional hemolytic anemia due to an enzyme disorder 12.0
47 congenital intestinal disease due to an enzymatic defect 12.0
48 alopecia, neurologic defects, and endocrinopathy syndrome 12.0
49 encephalopathy, acute, infection-induced 4 11.9
50 schizophrenia 11.8

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

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 The International Database for Leigh Syndrome Recruiting NCT03137355

Search NIH Clinical Center for Acute Necrotizing Encephalopathy

Genetic Tests for Acute Necrotizing Encephalopathy

Anatomical Context for Acute Necrotizing Encephalopathy

MalaCards organs/tissues related to Acute Necrotizing Encephalopathy:

41
Brain, Liver, Cerebellum, Spinal Cord, Thalamus, Pons

Publications for Acute Necrotizing Encephalopathy

Articles related to Acute Necrotizing Encephalopathy:

(show top 50) (show all 211)
# Title Authors PMID Year
1
Detailed clinical course of fatal acute encephalopathy in children. 38
31337523 2019
2
Elderly-onset acute necrotizing encephalopathy mimicking severe heat stroke: a case report and review of the literature. 38
31304037 2019
3
Acute Necrotizing Encephalopathy. 38
31253217 2019
4
Neuropsychological outcomes of childhood acute necrotizing encephalopathy. 38
31376945 2019
5
Acute Necrotizing Encephalopathy in Children: a Long Way to Go. 38
31099193 2019
6
Temporal Course of Imaging and Laboratory Findings in a Fulminant Case of Acute Necrotizing Encephalopathy of Childhood. 38
31178236 2019
7
Neuroinvasion of influenza A/H3N2: a fatal case in an immunocompetent adult. 38
30397824 2019
8
Favorable Outcomes With Early Interleukin 6 Receptor Blockade in Severe Acute Necrotizing Encephalopathy of Childhood. 38
31201070 2019
9
Acute necrotizing encephalopathy of childhood. 38
31085896 2019
10
Acute necrotizing encephalopathy of childhood associated with human herpes virus 6 in Croatia. 38
30374582 2019
11
Acute necrotizing encephalopathy and a carnitine palmitoyltransferase 2 variant in an adult. 38
30470651 2019
12
Adenovirus-Associated Central Nervous System Disease in Children. 38
30413311 2019
13
Diagnosis and Management of Acute Encephalitis in Children. 38
30232787 2019
14
Steroids for familial acute necrotizing encephalopathy: A future investment? 38
30241006 2018
15
RANBP2 Mutation in Clinically Undiagnosed Acute Necrotizing Encephalopathy. 38
29687329 2018
16
Influenza-Associated Acute Necrotizing Encephalopathy in Siblings. 38
29741717 2018
17
Teaching NeuroImages: Radiographic evolution in an adult case of acute necrotizing encephalopathy. 38
30061348 2018
18
Evoked potential studies for predicting functional recovery in a case of acute necrotizing encephalopathy. 38
29744063 2018
19
Acute Hemorrhagic Encephalitis Responding to Combined Decompressive Craniectomy, Intravenous Immunoglobulin, and Corticosteroid Therapies: Association with Novel RANBP2 Variant. 38
29593631 2018
20
Acute necrotizing encephalopathy in an adult with influenza A infection. 38
29720877 2018
21
Teaching NeuroImages: Acute necrotizing encephalopathy of childhood: Neuroimaging findings. 38
29311376 2018
22
Influenza-associated Encephalitis/Encephalopathy Identified by the Australian Childhood Encephalitis Study 2013-2015. 38
28654561 2017
23
[Advances in clinical and imaging studies of acute necrotizing encephalopathy]. 38
29141322 2017
24
Deep cerebral venous thrombosis mimicking influenza-associated acute necrotizing encephalopathy: a case report. 38
28950885 2017
25
Familial acute necrotizing encephalopathy with RANBP2 mutation: The first report in Northeast Asia. 38
28336122 2017
26
Acute Necrotizing Encephalopathy of Childhood Secondary to Dengue Infection: A Case Report from Pakistan. 38
28904577 2017
27
Acute necrotizing encephalopathy causing human bocavirus. 38
28059631 2017
28
"Symptomatic" infection-associated acute encephalopathy in children with underlying neurological disorders. 38
27780632 2017
29
Acute Necrotizing Encephalopathy of Childhood (ANEC): A Case Report. 38
28277560 2017
30
Bilateral Thalamic Lesions in Acute Necrotizing Encephalopathy Due to H1N1 Infection. 38
27647154 2016
31
Influenza A (H1N1) Infection Associated Acute Necrotizing Encephalopathy in a Child With Periodic Lateralized Epileptiform Discharges. 38
27898637 2016
32
Familial acute necrotizing encephalopathy without RANBP2 mutation: Poor outcome. 38
27882739 2016
33
RANBP2 mutation in an Indian child with recurrent acute necrotizing encephalopathy. 38
27591117 2016
34
Variable clinical course in acute necrotizing encephalopathy and identification of a novel RANBP2 mutation. 38
26923722 2016
35
Influenza B-associated acute necrotizing encephalopathy of childhood: a report from North America. 38
26627900 2016
36
Specific HLA genotypes confer susceptibility to acute necrotizing encephalopathy. 38
27467284 2016
37
Brain damage resembling acute necrotizing encephalopathy as a specific manifestation of haemophagocytic lymphohistiocytosis - induced by hypersensitivity. 38
27576518 2016
38
Acute Postviral Encephalopathy: Pathologic and Radiologic Correlation in an Atypical Case. 38
28503611 2016
39
Influenza Virus-Associated Fatal Acute Necrotizing Encephalopathy: Role of Nonpermissive Viral Infection? 38
27812294 2016
40
An autopsy case of elderly-onset acute necrotizing encephalopathy secondary to influenza. 38
25982501 2015
41
Fatal Acute Necrotizing Encephalopathy: Clinical Presentation and Imaging Findings. 38
25937389 2015
42
Only a Touch of the Flu? The Simultaneous Manifestation of Acute Necrotizing Encephalopathy in Two Consanguineous Patients. 38
26110162 2015
43
H1N1 triggered recurrent acute necrotizing encephalopathy in a family with a T653I mutation in the RANBP2 gene. 38
25170550 2015
44
A severity score for acute necrotizing encephalopathy. 38
24931733 2015
45
RANBP2 mutation and acute necrotizing encephalopathy: 2 cases and a literature review of the expanding clinico-radiological phenotype. 38
25522933 2015
46
Brain magnetic resonance imaging in acute phase of pandemic influenza A (H1N1) 2009--associated encephalopathy in children. 38
25290723 2015
47
[A clinical study on high-dose erythropoietin therapy for acute encephalopathy or encephalitis]. 38
25803909 2015
48
Acute necrotizing encephalopathy in an adult. 38
25973284 2015
49
Acute necrotizing encephalopathy: an underrecognized clinicoradiologic disorder. 38
25873770 2015
50
Hypoxic-ischemic encephalopathy mimicking acute necrotizing encephalopathy. 38
25447929 2015

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
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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