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A number of recently published studies provide evidence for disturbed copper metabolism in focal dystonia. Postmortem studies have identified significantly elevated copper and manganese levels in the globus pallidus and putamen of two patients with focal dystonia.1 Menkes protein was found to be reduced, whereas Wilson protein and ceruloplasmin were increased in the lentiform nuclei of the patients with focal dystonia.2 Disturbances of copper metabolism may not be restricted to the basal ganglia because reduction of Menkes mRNA and copper was also observed in leukocytes of patients with focal dystonia.3 Interestingly, there is a report of a family with autosomal-dominantly inherited whispering dysphonia in whom impaired copper metabolism is implicated: two siblings who were phenotypically very similar to other family members developed Wilson’s disease (WD).4
Both the autosomal-recessively inherited WD and the X- chromosomal inherited Menkes disease (MD) are due to mutations in structurally similar copper translocating P-type ATPases named ATP7B (WD) and ATP7A (MD). The …
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