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