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Journal of Neurology, Neurosurgery and Psychiatry 1992-Mar

Neuropsychiatric manifestations of defect in mitochondrial beta oxidation response to riboflavin.

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W J Triggs
C R Roe
W J Rhead
S K Hanson
S N Lin
L J Willmore

Keywords

Abstract

A 29 year old woman is described with severe hyperemesis gravidarum, atypical migraine, numerous admissions to hospital for psychiatric illness, non-epileptic seizures, and valproate-induced coma. Metabolic studies and measurement of [9,10(n)-3H]palmitate oxidation by cultured fibroblasts suggested a multiple acyl-CoA dehydrogenation disorder. Treatment with riboflavin abolished headaches and abnormal behaviour and normalised the plasma free carnitine level. Subtle defects in mitochondrial beta oxidation may be a treatable cause of disordered behaviour in adults.

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