Neuropsychiatric manifestations of defect in mitochondrial beta oxidation response to riboflavin

J Neurol Neurosurg Psychiatry. 1992 Mar;55(3):209-11. doi: 10.1136/jnnp.55.3.209.

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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Acids / urine
  • Adult
  • Coma / chemically induced*
  • Coma / enzymology
  • Electron-Transferring Flavoproteins
  • Epilepsy, Complex Partial / drug therapy*
  • Epilepsy, Complex Partial / enzymology
  • Fatty Acid Desaturases / deficiency*
  • Fatty Acid Desaturases / physiology
  • Female
  • Flavoproteins / physiology*
  • Humans
  • Iron-Sulfur Proteins*
  • Mitochondria / drug effects
  • Mitochondria / enzymology*
  • Multienzyme Complexes / deficiency*
  • Multienzyme Complexes / physiology
  • Neurologic Examination
  • Neuropsychological Tests*
  • Oxidoreductases Acting on CH-NH Group Donors*
  • Riboflavin / administration & dosage*
  • Valproic Acid / administration & dosage
  • Valproic Acid / adverse effects*

Substances

  • Acids
  • Electron-Transferring Flavoproteins
  • Flavoproteins
  • Iron-Sulfur Proteins
  • Multienzyme Complexes
  • Valproic Acid
  • Fatty Acid Desaturases
  • Oxidoreductases Acting on CH-NH Group Donors
  • electron-transferring-flavoprotein dehydrogenase
  • Riboflavin