Clinical and MRI characteristics of acute encephalopathy in congenital adrenal hyperplasia

JOURNAL OF THE NEUROLOGICAL SCIENCES

卷:306

Article

Sanefuji, Masafumi¹  Watanabe, Kenji²  Uematsu, Ayumi³  Toyoshima, Mitsuo²  Aragaki, Fumio⁴  Hata, Daisuke⁵ 

[1] Kyushu Univ, Grad Sch Med Sci, Dept Pediat, Higashi Ku, Fukuoka 8128582, Japan

[2] Kagoshima Univ, Grad Sch Med & Dent Sci, Dept Pediat, Kagoshima 890, Japan

[3] Shizuoka Childrens Hosp, Dept Endocrinol & Metab, Shizuoka, Japan

[4] Kurashiki Cent Hosp, Dept Pediat, Kurashiki, Okayama, Japan

[5] Kitano Hosp, Dept Pediat, Osaka, Japan

关键词: child;    congenital adrenal hyperplasia;    encephalitis;    encephalopathy;    MRI;    white matter;   

DOI  :  10.1016/j.jns.2011.03.037

来源: Elsevier

【 摘 要 】

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. (C) 2011 Elsevier B.V. All rights reserved.

【 授权许可】

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