RT Journal Article
SR Electronic
T1 Neuropsychiatric manifestations of defect in mitochondrial beta oxidation response to riboflavin.
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP 209
OP 211
DO 10.1136/jnnp.55.3.209
VO 55
IS 3
A1 Triggs, W J
A1 Roe, C R
A1 Rhead, W J
A1 Hanson, S K
A1 Lin, S N
A1 Willmore, L J
YR 1992
UL http://jnnp.bmj.com/content/55/3/209.abstract
AB 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.