Abstract

The EEGs of twenty-three patients and five asymptomatic siblings with Wilson's disease were correlated with their clinical states and biochemical studies related to liver function and copper metabolism. In uncomplicated cases of the disease the EEG may be normal or abnormal. When the EEG is abnormal there does not appear to be a clear correlation with a clinical or biochemical state. Severely abnormal EEGs are seen with severe complications of the disease and are appropriate to the complication. These EEGs show improvement parallel with clinical improvement. Asymptomatic siblings with the characteristic biochemical defect of copper metabolism may also show normal or abnormal EEGs, a feature which seems to have a familial tendency. The abnormal EEGs of asymptomatic siblings show improvement with treatment, supporting the argument for prophylaxis in such cases.

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