Neuropsychiatric manifestations of defect in mitochondrial beta oxidation response to riboflavin.
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.
Triggs
W J
WJ
University of Texas Health Science Center, Department of Neurology, Houston.
Roe
C R
CR
Rhead
W J
WJ
Hanson
S K
SK
Lin
S N
SN
Willmore
L J
LJ
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*
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