Dystonia in Wilson's disease

The frequency and type of dystonic movements, as well as brain abnormalitie s, as depicted with magnetic resonance imaging (MRI), which might correlate with dystonia, were studied in 27 consecutive patients with a neurologic f orm of Wilson's disease (WD) and optimized treatment. Dystonia was found in 10 patients (37%), being generalized in half of them, while two patients h ad segmental, two patients multifocal dystonia, and one patient bilateral f oot dystonia. Dystonia was a presenting sign in four patients and developed later in the course of the disease in six patients, despite the administer ed therapy for WD. Putamen was the only structure significantly more freque ntly lesioned in dystonic (80%) in comparison to WD patients without dyston ia (24%), suggesting a relation between abnormalities in this brain region and dystonic movements in WD. (C) 2001 Movement Disorder Society.