Correlation between neurological impairment and liver status in Wilson’s disease

Abstract

Background: The most widely recognized aspect of the neuro-hepatic relation is hepatic encephalopathy, in which neurotransmission in the brain is altered. Of course, there are many conditions that affect both the liver and the nervous system, Wilson’s disease being one of the best known. The aim was to characterize the neurological manifestations of Wilson’s diseases in terms of symptom type and degree of neurological impairment and correlate these features with degree of abnormalities in copper metabolism, and hepatic status. Material and methods: 15 patients diagnosed with Wilson’s disease were characterized by examination in terms of symptoms including consciousness, activities of daily living as reported by the patient. The neurological manifestations were analyzed in relation to copper abnormalities and liver status. Results: Most patients (52.9%) exhibited tremor and ataxia, whereas 9.3% were dystonic, and 7.3% had Parkinsonism. Discrete signs were observed in 19.6% of patients. A positive correlation between neurological impairment and higher level of free cooper was observed (Pearson r=0.71). Poor correlation was identified between neurological impairment and hepatic disturbances. Conclusions: The neurological manifestations of Wilson’s disease did not appear to be correlated with hepatic status. These results draw our attention to the symptomatic variability of Wilson’s disease, and an individualized approach to each patient is essential.