Document Detail


Clinical and MRI characteristics of acute encephalopathy in congenital adrenal hyperplasia.
MedLine Citation:
PMID:  21496829     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Acute encephalopathy in childhood is frequently associated with common infections, especially in East Asia. Various types have been identified although many cases remain unclassified. Congenital adrenal hyperplasia (CAH) is an autosomal recessive disease presenting impairment of cortisol biosynthesis. We report three CAH children with acute infection-related encephalopathy. They exhibited disturbed consciousness or seizures, which did not improve after glucocorticoid administration, accompanied by clinical and laboratory findings of adrenal insufficiency. Brain MRI disclosed various patterns of white matter lesions, suggesting different types of acute encephalopathy such as clinically mild encephalitis/encephalopathy with a reversible splenial lesion or hemiconvulsion-hemiplegia syndrome. Acute encephalopathy should be considered and brain MRI immediately performed when impairment of consciousness does not improve after intravenous glucocorticoid administration in CAH patients. Further research is required to elucidate the epidemiology and pathogenic mechanisms of acute encephalopathy in CAH.
Authors:
Sooyoung Lee; Masafumi Sanefuji; Kenji Watanabe; Ayumi Uematsu; Hiroyuki Torisu; Haruhisa Baba; Ryutaro Kira; Yui Takada; Yoshito Ishizaki; Mitsuo Toyoshima; Fumio Aragaki; Daisuke Hata; Toshiro Hara
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-4-13
Journal Detail:
Title:  Journal of the neurological sciences     Volume:  -     ISSN:  1878-5883     ISO Abbreviation:  -     Publication Date:  2011 Apr 
Date Detail:
Created Date:  2011-4-18     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0375403     Medline TA:  J Neurol Sci     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2011 Elsevier B.V. All rights reserved.
Affiliation:
Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
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