ASYMMETRIC DYSTONIA WITH FRONTAL WHITE-MATTER LESIONS IN WILSONS-DISEASE

Asymmetrical manifestation of dystonia was investigated in 26 patients with Wilson's disease with modified dystonia rating scale and correla ted with the asymmetricity of CT and/or MRI findings. Ten patients (ei ght men and two women) had dystonia. The age of disease onset ranged f rom 11 to 30 years with a mean of 18.6 +/- 5.2 years. The duration of the illness was from 2 to 15 pears (mean 7.0; S.D. 3.6). Sis patients had subcortical white matter lesions in the frontal and/or parietal lo bes, as well as lesions in the basal ganglia. Five patients, who had a symmetrical white matter lesions in the frontal lobes and symmetrical lesions in the basal ganglia and the thalamus, developed more severe c ontralateral dystonia. The other four patients with symmetric lesions in the basal ganglia and the thalamus had nearly-symmetrical dystonia. One patient with symmetrical lesions in the frontal lobes and the bas al ganglia also had symmetrical manifestation of dystonia. We suggest that the interruption of the loop between the thalamus and premotor co rtex by subcortical white matter lesions may enhance contralateral dys tonia.