Recurrent acute necrotizing encephalopathy in a Korean child: the first non-Caucasian case.

Abstract:

:Acute necrotizing encephalopathy is characterized by fever, seizures, acute encephalopathy, and rapid progression to coma. It is usually associated with viral illness and shows characteristic brain magnetic resonance imaging features, including symmetrical involvement of bilateral thalami, brain stem, white matter, and cerebellum. After the first report of recurrent or familial cases in 2003, similar cases were found exclusively in American and European regions. The association with Ran-binding protein 2 gene was identified in 75% of familial or recurrent cases. This report describes a previous healthy 22-month-old boy who recurrently manifested typical clinical and radiological characteristics of acute necrotizing encephalopathy. His neurological outcome worsened with repeated episodes. There was no family history of acute necrotizing encephalopathy and no mutation in the coding region of Ran-binding protein 2 (RANBP2) gene. This is the first reported case of recurrent acute necrotizing encephalopathy in a non-Caucasian family.

journal_name

J Child Neurol

authors

Lee JH,Lee M,Lee J

doi

10.1177/0883073811435240

subject

Has Abstract

pub_date

2012-10-01 00:00:00

pages

1343-7

issue

10

eissn

0883-0738

issn

1708-8283

pii

0883073811435240

journal_volume

27

pub_type

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