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January 1953

EFFECT OF DIMERCAPROL (BAL) IN HEPATOLENTICULAR DEGENERATION: Report of a Case, with Clinical and Electroencephalographic Study

Author Affiliations


From the Department of Neurology of the Rothschild-Hadassah-University Hospital, and the Hebrew University-Hadassah Medical School.

AMA Arch NeurPsych. 1953;69(1):84-90. doi:10.1001/archneurpsyc.1953.02320250090010

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 observed2a 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

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