Health & Medical Neurological Conditions

Recognition and Treatment of Neurologic Wilson's Disease

´╗┐Recognition and Treatment of Neurologic Wilson's Disease

Clinical Manifestations


Wilson's disease presents with hepatic, neurologic, and psychiatric symptoms in roughly equal proportions, with the psychiatric presentation likely underappreciated. Walshe, who introduced penicillamine, trientine, and tetrathiomolybdate (TM) therapy for Wilson's disease, stated that "no two patients are ever the same, even in a sibship," and that there is no such thing as a typical picture of Wilson's disease. Early in life those carrying Wilson's disease mutations are presymptomatic, but accumulating copper, which invariably causes subclinical liver disease. Between early childhood and the fifth or sixth decade of life, with a peak incidence of around 17 years of age, the patient presents with hepatic, neurologic, and/or psychiatric manifestations. It is extremely rare for Wilson's disease to present after age 35, but late-onset cases continue to be reported. Those with a primarily hepatic presentation generally have an earlier age at presentation, 11 to 15 years of age, than those with a primarily neurologic presentation, mean age at onset of from 15 to 21 years of age.



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