Treatment of choreoathetotic movements in chronic liver disease with a dopamine-blocking agent. Pathophysiologic implications.

Abstract

Acquired hepatocerebral degeneration (AHCD) is a well-defined clinical and neuropathological entity associated with various types of chronic liver disease, and usually related to portal-systemic shunting. Various movement disorders, including choreoathetosis, are commonly seen in AHCD. The frequent neuropathological finding of neuronal degeneration in the striatum (caudate nuclei and putamens) suggested that choreoathetotic movements in AHCD might be caused by dopamine hypersensitivity in striatal receptor sites and that treatment of such movement disorders might be effected by known striatal dopamine-blocking agents of proven benefit in similar movement disorders, e. g. Huntington’s chorea. A patient with documented post-necrotic cirrhosis and portal hypertension who had had a successful portacaval shunt developed signs of AHCD with choreoathetotic movements of the tongue, face, and mouth (linguo-facio-buccal dyskinesia); he was treated with haloperidol, a striatal dopamine-blocking agent, with prompt and sustained cessation of his dyskinesia. Evidence of a normal baseline central dopamine content and of central dopamine blockade after administration of haloperidol was obtained. The implications of these findings and of similar findings in other movement disorders regarding the role of neuro-transmitting agents in the normal individual and in various disease states are discussed, and therapeutic considerations are presented.