Increased tissue copper and manganese content in the lentiform nucleus in primary adult-onset dystonia

Abstract

We analyzed trace metals in frozen brain tissue of several subcortical nuclei from 3 patients with primary adult-onset dystonia and 10 control subjects. Copper levels were significantly increased in the globus pallidus and putamen of patients with dystonia A slight increase in manganese content was identified in the putamen and thalamus of patients with dystonia. Our findings show for the first time an accumulation of trace metals in the lentiform nuclei in patients with primary dystonia, which may play a pathogenetic role in primary dystonia and may explain recent ultrasound and magnetic resonance imaging findings.