title: Neuropsychiatric manifestations of defect in mitochondrial beta oxidation response to riboflavin.
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.
authors.affiliation: University of Texas Health Science Center, Department of Neurology, Houston.
authors.forename: W J
authors.initials: WJ
authors.lastname: Triggs
authors.forename: C R
authors.initials: CR
authors.lastname: Roe
authors.forename: W J
authors.initials: WJ
authors.lastname: Rhead
authors.forename: S K
authors.initials: SK
authors.lastname: Hanson
authors.forename: S N
authors.initials: SN
authors.lastname: Lin
authors.forename: L J
authors.initials: LJ
authors.lastname: Willmore
mesh_terms: D000143:Acids / Q000652:urine; D000328:Adult; D003128:Coma / Q000139:chemically induced* / Q000201:enzymology; D044927:Electron-Transferring Flavoproteins; D017029:Epilepsy, Complex Partial / Q000188:drug therapy* / Q000201:enzymology; D044943:Fatty Acid Desaturases / Q000172:deficiency* / Q000502:physiology; D005260:Female; D005420:Flavoproteins / Q000502:physiology*; D006801:Humans; D007506:Iron-Sulfur Proteins; D008928:Mitochondria / Q000187:drug effects / Q000201:enzymology*; D009097:Multienzyme Complexes / Q000172:deficiency* / Q000502:physiology; D009460:Neurologic Examination; D009483:Neuropsychological Tests; D000587:Oxidoreductases Acting on CH-NH Group Donors; D012256:Riboflavin / Q000008:administration & dosage*; D014635:Valproic Acid / Q000008:administration & dosage / Q000009:adverse effects*
chemical_list: D000143:Acids; D044927:Electron-Transferring Flavoproteins; D005420:Flavoproteins; D007506:Iron-Sulfur Proteins; D009097:Multienzyme Complexes; D014635:Valproic Acid; D044943:Fatty Acid Desaturases; D000587:Oxidoreductases Acting on CH-NH Group Donors; C023120:electron-transferring-flavoprotein dehydrogenase; D012256:Riboflavin