One Case of Childhood Adrenoleukodystrophy Involving the White Matter of the Frontal Lobe.

Lee,  Hyun Sug; Kang,  Hoon Chul

Case Report

Journal of the Korean Child Neurology Society 2005;13(1):104-108.
Published online May 30, 2005.

One Case of Childhood Adrenoleukodystrophy Involving the White Matter of the Frontal Lobe.

Department of Pediatrics, College of Medicine, Epilepsy Center, Sanggye Paik Hospital, Inje University, Seoul, Korea. hipo0207@sanggyepaik.ac.kr

Abstract

Childhood adrenoleukodystrophy is a progressive, demyelinating disease of the white matter. Although it is sometimes misdiagnosed as a psychiatric disorder, it can be reliably confirmed by magnetic resonance imaging(MRI) and biochemical study. We experienced a case of adrenoleukodystrophy in a ten-year-old boy, who initially presented a behavioral disorder and mild speech disability. His brain MRI was compatible with leukodystrophy but it was atypically involved in both of the frontal regions. Childhood adrenoleukodystrophy could be confirmed by the elevated level of plasma hexacosanoic acid (C26:0) and the ratio of C26:0 and tetracosanoic acid(C24:0) to hexaenoic acid (C22:0). We report the case with a brief review of literature.

Key Words: Childhood adrenoleukodystrophy, Frontal lobe

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