J Korean Child Neurol Soc.  2005 May;13(1):104-108.

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

• Lee HS
• Kang HC

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.

Keyword

Childhood adrenoleukodystrophy; Frontal lobe

MeSH Terms

Adrenoleukodystrophy*
Brain
Demyelinating Diseases
Frontal Lobe*
Humans
Magnetic Resonance Imaging
Male
Plasma