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KMID : 0391520050130010104
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Journal of the Korean Child Neurology Society
2005 Volume.13 No. 1 p.104 ~ p.108
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One Case of Childhood Adrenoleukodystrophy Involving the White Matter of the Frontal Lobe
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Lee Hyun-Suk
Kang Hoon-Chul
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Abstract
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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.
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KEYWORD
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Childhood Adrenoleukodystrophy, Frontal Lobe
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