Late form with psychiatric presentation of Wilson's disease, with pseudo-compulsive stereotyped movements. Neuro-radiological correlations
Garnier, H.; Diederich, N.; Pilloy, W.; Lenaerts, M.; Dooms, G.; Metz, H.
Revue Neurologique (Paris) 153(2): 124-128
1997
ISSN/ISBN: 0035-3787 PMID: 9296124 Document Number: 470995
Wilson's disease rarely starts after the third decade and may present with misleading psychiatric signs. We observed a 39-year-old white male who developed hysterical behaviour followed by frank delusional psychosis. Secondary neurological symptoms like astasia and dyarthria were misinterpreted as drug-induced. Despite a treatment with D-penicillamine and zinc sulfate there was further deterioration with anarthria and pseudo-compulsive stereotypies. These latter signs cleared after five months, whereas astasia and abasia remained the same and MRI imaging showed further deterioration characterized by marked bilateral putaminal 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 in other diseases of the basal ganglia.