Мультимодальные вызванные потенциалы головного мозга у больных гепатолентикулярной дегенерацией

The complex multimodal evoked potentials study was conducted on 14 patients with hepatolenticular degeneration aged between 15 and 46. The analysis of brainstem auditory evoked potentials outcomes revealed that there was the conduction defect both at the brainstem and at the peripheral levels (as a decrease in peak amplitude of 1). The definite improvement of brainstem auditory evoked potentials outcomes was noticed on long-term copper eliminating therapy. P300 outcomes demonstrate the cognitive deficit even on long-term therapy. The obvious disorder of visual and sensory afferentation at brain level (a part from the brainstem to the cerebral cortex) was revealed. The complex multimodal evoked potentials study is a significant additional non-invasive technique that provides objective assessment of neurophysiological brain processes in patients with hepatolenticular disease.