Adrenergic dysfunction in hereditary adult-onset leukodystrophy

We studied the pressor response to norepinephrine infusion in patients with an autosomal dominant adult-onset leukodystrophy. We also examined cardiovascular and catecholamine responses to insulin-induced hypoglycemia. A parallel shift to the left of the norepinephrine dose response curve, in conjunction with low baseline plasma norepinephrine levels, was consistent with denervation supersensitivity, suggesting a distal lesion of sympathetic nor-adrenergic neurons. Absence of the epinephrine response to insulin-induced hypoglycemia indicated that autonomic neuropathy was attended by severe adrenal medullary dysfunction.