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.