Peroxisomal acyl CoA oxidase deficiency.

Suzuki, Yasuyuki; Iai, Mizue; Kamei, Atsushi; Tanabe, Yuzo; Chida, Shoichi; Yamaguchi, Seiji; Zhang, Zhongyi; Takemoto, Yasuhiko et al. · J Pediatr · 2002

case_report · Level V

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Abstract

Three Japanese patients with peroxisomal acyl coenzyme A oxidase deficiency who manifested psychomotor retardation and regression during the late infantile period showed characteristic patterns of demyelination in the ponto- medullary corticospinal tracts and in the cerebellar and cerebral white matter. Molecular investigations revealed 2 novel missense mutations, M278V and G178C.

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