EFFECT OF DIMERCAPROL (BAL) IN HEPATOLENTICULAR DEGENERATION

Abstract

OBSERVATIONS on the development of Parkinsonian syndromes in persons exposed to toxic substances, in particular to heavy metals, have stimulated interesting investigations concerning the pathogenesis of these extrapyramidal diseases. In cases of hepatolenticular degeneration, for example, an increased content of copper in the liver and brain tissues has been demonstrated.¹ Recently, also, an increased excretion of copper² and amino acids has been found in the urine of patients with this disease. Since it had been observed^2a that dimercaprol U. S. P. increased the urinary output of copper in a patient with hepatolenticular degeneration, Cumings³ administered dimercaprol to four patients with this disease. In three of these patients a significant clinical improvement occurred. Denny-Brown and Porter⁴ have recently observed the beneficial effect of dimercaprol in five cases of the disease. The purpose of this communication is to report the clinical and electroencephalographic observations made on an