A LATE FORM OF WILSONS-DISEASE, WITH PSYC HIATRIC FIRST ASPECT AND PSEUDO-COMPULSIVE STEREOTYPIES NEURORADIOLOGICAL CORRELATIONS

Wilson's disease rarely starts after the third decade and may present with misleading psychiatric signs. We observed a 39-year-old white mal e who developed hysterical behaviour followed by frank delusional psyc hosis. Secondary neurological symptoms like astasia and dyarthria were misinterpreted as drug-induced. Despite a treatment with D-penicillam ine and zinc sulfate there was further deterioration with anarthria an d pseudo-compulsive stereotypies. These latter signs cleared after fiv e months, whereas astasia and abasia remained the same and MRI imaging showed further deterioration characterized by marked bilateral putami nal cavitation. SPECT imaging could not predict the clinical evolution . Our case emphasizes that Wilson's disease can have variable initial presentations, and confirms the relationship between pseudo-compulsive stereotypies and bilateral lenticular lesions, as already described i n other diseases of the basal ganglia.