Penicillamine-related neurologic syndrome in a child affected by Wilson disease with hepatic presentation

S. Porzio, R. Iorio, P. Vajro, P. Pensati and A. Vegnente
Department of Pediatrics, Universita Federico II, Naples, Italy.

OBJECTIVES: To describe a case of penicillamine-related neurologic symptoms in a 9-year-old boy affected by asymptomatic Wilson disease with hepatic presentation; to compare this case with similar cases in adults; and to discuss the role of zinc therapy as an alternative treatment for patients who have an adverse reaction to penicillamine therapy. SETTING: Referral hospital. MAIN OUTCOME MEASURE: The occurrence of a neurologic syndrome that severely impaired a child's usual daily activities and his health-related quality of life after the institution of penicillamine therapy. RESULTS: Initial penicillamine therapy was chronologically related to the development of progressive neurologic deterioration in the absence of other causes of neurologic syndrome. The discontinuation of penicillamine therapy and the initiation of zinc therapy were followed by a prompt disappearance of neurologic symptoms and a return to neurologic baseline status. CONCLUSIONS: Penicillamine therapy, even in children affected by Wilson disease with hepatic presentation alone and without neurologic disease at the beginning of treatment, may trigger neurologic symptoms. Zinc therapy may be a satisfactory alternative.