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Adrenergic dysfunction in hereditary adult-onset leukodystrophy

Clinical Neuropharmacology Section (Drs. Brown and Polinsky and Ms. Dailey) and the Human Motor Control Section (Dr. Schwankhaus), Medical Neurology Branch; the Neuroepidemiology Branch (Dr. Eldridge); and the Neuroimmunology Branch (Dr. McFarland), National Institute of Neurological and Communicative Disorders and Stroke; and the Medical Genetics Program (Ms. Schlesinger), Clinical Center, National Institutes of Health, Bethesda, MD.

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 noradrenergic neurons. Absence of the epinephrine response to insulin-induced hypoglycemia indicated that autonomic neuropathy was attended by severe adrenal medullary dysfunction.

Address correspondence and reprint requests to Dr. Brown, NIH, Building 10, Room 5N236, Bethesda, MD 20892.

Presented in part at the thirty-eighth annual meeting of the American Academy of Neurology, New Orleans, LA, April 1986.

Received August 25, 1986. Accepted for publication in final form December 3, 1986.

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