Evoked potentials in patients with non-neurological Wilson's disease

Abstract

In Wilson's disease neurological manifestations result from the damage in the basal ganglia, even if a widespread degeneration of the brain occurs. The few studies performed using evoked potentials with the aim of identifying subclinical dysfunction in the three major sensory pathways have never shown abnormalities in patients without neurological manifestations. To verify this observation we studied 12 patients suffering from Wilson's disease in a pre-neurological stage by using pattern visual evoked potentials (VEPs), somatosensory evoked potentials (SEPs) to median nerve stimulation and brainstem auditory evoked potentials (BAEPs). Four of these patients had not yet been treated with penicillamine or trientine (triethylenetetramine dihydrochloride), while the remaining 8 patients were on treatment for at least 1 year. In 3 patients of this second group and in 1 patient of the first group we observed a significant (3 SD over the mean) increase in P100 wave latency, while SEPs and BAEPs were found to be abnormal in only 1 patient, respectively.

A study of clinical, MRI and multimodality evoked potentials in neurologic Wilson disease

“…Our study revealed EP abnormality in 61% patients with neurologic WD; abnormal MEP in 35.7%, ABER in 61.5%, VEP in 57% and SEP in 30.7%. In earlier studies, MEP abnormality has been reported in 39-50%, SEP in 15-69%, VEP in 5.3-75% and ABER in 8.3-92% [8][9][10][11][12][13][14][15][16][17][18][19][20]. MEP abnormality in our study correlated with MRI involvement of motor pathway in four patients.…”

“…Moreover, WD presents with predominant extrapyramidal features, pyramidal dysfunction may be overshadowed by rigidity and movement disorders which can be evaluated by motor evoked potential (MEP). On Medline search with key words evoked potentials and WD, we could retrieve 45 articles; out of which multimodality evoked potentials were performed in 14 studies; majority included both hepatic and neurologic WD, six correlated evoked potential changes with magnetic resonance imaging findings and only two conducted MEP [8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23]. In this communication, we report multimodality evoked potential changes in patients with neurologic WD and correlate these with their clinical severity and MRI findings.…”

Zerebrales MRT und evozierte Potenziale bei Morbus Wilson

“…Es hat keine Befundkonstellation eine Spezifität, die im Einzelfall das Auftreten morphologischer bzw. Ein zumeist Fehlen pathologischer MRT-Befunde [18,21] und regelrechte elektrophysiologische Befunde bei neurologisch asymptomatischen Patienten unterstützt diesen Zusammenhang [1,10]. Auch in den Untersuchungen von Giagheddu et al [7] korrelieren abnorme MRT-Befunde nicht regelmäßig mit gestörten evozierten Potenzialen.…”