[Medulloadrenal secretion and peripheral sympathomimetic receptors in familial dysautonomia]

A case of familial dysautonomia in a 30-year old woman with severe postural hypotension is reported. A study of adrenal medulla secretion showed that it was inadapted to the fall in blood pressure, with no increase in dopamine secretion. Insulin-induced hypoglycaemia had no effect on adrenal medulla secretion. Changes in blood pressure and heart rate were studied after administration of alpha- and beta-adrenergic drugs combined with fludrocortisone. The increase observed in blood pressure and heart rate suggests that peripheral catecholamine receptors are present in dysautonomic patients. The best treatment of postural hypotension in these patients seems to be a dihydroergotamine-fludrocortisone combination.