Neurophysiological evidence of effective therapy in patients with Wilson's disease

Introduction: Wilson's disease is a rare autosomal recessive disorder of hepatic copper transport leading to mainly hepatic or extrapyramidal motor clinical symptoms. Involvement of other central nervous pathways is detectable by multimodal evoked potentials. A continuous drug therapy and regular controls are necessary to prevent progression of the disease which potentially could be fatal. Evoked potentials may be useful as early indicators of insufficient treatment. Materials: In nine patients with a neurologic form of Wilson's disease undergoing long-term treatment, the visual, acoustic, somatosensory (median…