The diagnostic value of multi-modality evoked potentials in Wilson's disease

19 patients with a neurological manifestation of Wilson's disease (WD), most of them with long-term disease (> 10 yr), underwent clinical, psychometrical and electrophysiological evaluation in order to test whether there is a correlation of psychometrical and evoked potential (EP) abnormalities with a semiquantitative clinical score ranging from no (0) to severe (3) symptoms. The following EPs were recorded: acoustically evoked event-related (ERPs), somato-sensory (SSEPs), visually (VEPs) and brainstem evoked potentials (BSEPs). Results were compared with the data of an age- and sex-matched control group. 89% of the patients revealed clinical signs of basal ganglia dysfunction, 11% oculomotor or cerebellar symptoms, but none of the individuals had any clinical hint for visual pathway affection. Psychometrically, 100% had mood disturbances without definite intellectual decline. In EP-recordings, 100% showed ERP, 58.0% SSEP and 53% VEP and BSEP abnormalities. VEP and BSEP measurements did not correlate with the clinical score. There was only a weak correlation of SSEP (right-side)- and ERP-amplitude reduction with the clinical total score. The only significant correlation was found between the clinical total score and the time dependent psychometrical tests. Thus, there is a fairly high percentage of subclinical cerebral impairment in Wilson patients with long-term disease detectable by ERP-recordings which do, however, not correlate with the clinical status of the patients.