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Neuropsychiatric manifestations of defect in mitochondrial beta oxidation response to riboflavin

Triggs WJ, Roe CR, Rhead WJ, Hanson SK, Lin SN, Willmore LJ

Journal of neurology, neurosurgery, and psychiatry, 1992

18 citations

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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