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Neuropsychiatric manifestations of defect in mitochondrial beta oxidation response to riboflavin.
  1. W J Triggs,
  2. C R Roe,
  3. W J Rhead,
  4. S K Hanson,
  5. S N Lin,
  6. L J Willmore
  1. University of Texas Health Science Center, Department of Neurology, Houston.


    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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