[Electrophysiological impairment profile of patients with Wilson's disease].
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.
Hermann
W
W
Klinik und Poliklinik für Neurologie, Universität Leipzig. hermw@medizin.uni-leipzig.de
Villmann
T
T
Wagner
A
A
D001342:Autonomic Nervous System Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D001480:Basal Ganglia Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology*; D002540:Cerebral Cortex / Q000187:drug effects / Q000503:physiopathology; D002614:Chelating Agents / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004558:Electric Stimulation; D005116:Extrapyramidal Tracts / Q000187:drug effects / Q000503:physiopathology; D005712:Galvanic Skin Response / Q000187:drug effects / Q000502:physiology; D006527:Hepatolenticular Degeneration / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology*; D006801:Humans; D009422:Nervous System Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology*; D009460:Neurologic Examination; D010398:Penicillic Acid / Q000031:analogs & derivatives* / Q000627:therapeutic use; D010525:Peripheral Nerves / Q000187:drug effects / Q000503:physiopathology; D011930:Reaction Time / Q000187:drug effects / Q000502:physiology; D014266:Trientine / Q000627:therapeutic use
D002614:Chelating Agents; C028100:penicillamide; D010398:Penicillic Acid; D014266:Trientine