Neuropsychological Aspects of Wilson's Disease

Abstract

A consecutive series of 34 patients with confirmed diagnoses of Wilson's disease (WD) was administered complete neuropsychological examinations upon admission to a university medical center for routine laboratory tests. Twenty-five patients with neurological and/or hepatic symptoms (symptomatics) revealed frequent and severe motor deficits and infrequent and mild cognitive deficits in contrast to nine patients with genetic findings of Wilson's disease but no symptomatic findings (asymptomatics). Somato-sensory tests were normal in all. One of the most intriguing findings was the absence of a significant correlation between the level of copper toxicity and the degree, nature, and frequency of associated neurological deficits in both symptomatic and asymtomatic patients. Fifty per cent of the present sample received psychiatric treatment, including hospitalization, for schizophrenia, depression, anxiety, and related disorders prior to confirmed diagnosis of WD. The present findings provide additional evidence that patients with the initial presenting psychological symptoms may be easily misdiagnosed and mistreated if the possibilities of Wilson's disease are not ruled out first.

Key Words:

• Wilson's disease
• hepatolenticular degeneration
• neuropsychological
• psychiatric
• copper toxicity
• lenticular nuclei