Carbohydrate metabolism inmotorneuronedisease

SUMMARY Eight patients with classical motor neurone disease, and eight control patients withneurological disease and matched for degree ofwasting, were studied. Both groups had abnormalglucose tolerance, but the patients with motor neurone disease had a significantly lower insulin response both to oral glucose loading and to intravenous tolbutamide. Theseresults suggest that inpatients with motor neurone disease there is an impaired synthesis or release ofinsulin due to isletcelldamage.Blood pyruvate andlactate, andcerebrospinalfluid pyruvate, lactate, andcitrate, did not differ significantly fromthe control group. Bloodcitrate levels were significantly higher in patientswith motor neurone disease comparedwith the controls. Triglyceride levels were raised in patientswith motor neurone diseasecompared to thecontrolpatients. This may besecondary to theincreasedcitrate levels. Ahigh incidence of abnormal glucose tolerance tobothoral andintravenous testing has beenreportedin patients with motor neurone disease (Matthews,1958;