H.S. Pall

XENOBIOTIC METABOLISM IN MOTORNEURON DISEASE

Debrisoquine, carbocysteine, and paracetamol were selected as safe drugs to investigate the ability of the livers microsomal system to oxidise carbon, oxidise sulphur, and conjugate sulphate in patients with motor neuron disease (MND), other hospital patients, and healthy volunteers. Subjects with poor sulphur-oxidising and sulphur-conjugating activities were heavily over-represented in the MND group.

Movement disorder associated with abnormal copper metabolism and decreased blood antioxidants.

as important in extrapyramidal disorders.3 The normal defence against such oxidation is provided by a range of antioxidants of which glutathione is an important intracellular one.4 We describe a patient with a dystonic movement disorder who was found to have low serum copper, increased liver copper and decreased erythrocyte glutathione levels. We suggest that the combination of the copper and glutathione abnormalities may have resulted in dystonia when either on its own may have been insufficient to cause symptoms. A 15 year old Indian schoolboy presented with a 3 year history suggestive of intellectual deterioration which improved spontaneously while undergoing investigation and a 1 year history of persistent and progressive abnormal movements and posture. During this year he had lost 6kg in weight. He was not taking any drugs and in particular had never taken phenothiazines or other antidopaminergic medication. There were no abnormal vocalisations nor any obsessive compulsive behaviour. There