penicillamide and Basal-Ganglia-Diseases

In addition to hepatic and extrapyramidal motor clinical symptoms, Wilson's disease patients also exhibit subclinical disorders of other central nervous pathways. In this study, an impairment profile is described by means of eight electrophysiological tests (EAEP, MSEP, TSEP, T-VEP, MEP, EEG, heart frequency variability, and SSR) for 37 patients (28 with neurological, nine with tnon-neurological form) undergoing long-term drug therapy. The occurrence in 64.3% of a delayed wave III and/or IPL III-V prolongation in patients with the neurological form makes pathological FAEP the most common form of the disorder, followed by disorders in MSEP, TSEP, MEP, and T-VEP. Patients with the non-neurological form usually have normal values, although latency prolongations occur in isolated cases. The range of evoked potential findings is characterised primarily by latency prolongations, i.e. a demyelinising impairment type, and significant losses of potential hardly occur (except in the MEP). The electrophysiological impairment profile does not include EEG changes or vegetative disorders.

Topics: Autonomic Nervous System Diseases; Basal Ganglia Diseases; Cerebral Cortex; Chelating Agents; Diagnosis, Differential; Electric Stimulation; Extrapyramidal Tracts; Galvanic Skin Response; Hepatolenticular Degeneration; Humans; Nervous System Diseases; Neurologic Examination; Penicillic Acid; Peripheral Nerves; Reaction Time; Trientine