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Neurologic deterioration in a child with Wilson's disease on penicillamine therapy
Alexander Chandran Paul1, Sneha Varkki, Nisha B Yohannan
1Departments of Child Health, Christian Medical College and Hospital, Vellore 632 004, Tamil Nadu.
Insights
Penicillamine, a standard Wilson's disease treatment, can cause reversible extrapyramidal symptoms in children. This case highlights the need for careful monitoring during penicillamine therapy for pediatric Wilson's disease.
Area of Science:
- Pediatric Neurology
- Hepatology
- Pharmacology
Background:
- Wilson's disease is a genetic disorder of copper metabolism, primarily affecting the liver and brain.
- Penicillamine is a chelating agent widely used as a first-line treatment for Wilson's disease in pediatric patients.
Observation:
- An 8-year-old girl with Wilson's disease and liver involvement presented with new-onset extrapyramidal symptoms.
- These neurological symptoms emerged after the initiation of penicillamine therapy.
Findings:
- The extrapyramidal symptoms in the patient resolved completely within 20 hours of discontinuing penicillamine.
- Upon reintroduction of low-dose penicillamine, the symptoms reappeared within 24 hours, demonstrating a clear drug-induced effect.
Implications:
- This case underscores the potential for penicillamine to induce reversible extrapyramidal symptoms in pediatric Wilson's disease patients.
- Clinicians should consider drug-induced movement disorders when evaluating neurological changes in children treated with penicillamine.
- Careful dose titration and vigilant monitoring are crucial for managing Wilson's disease with penicillamine in children.
Abstract:
Penicillamine is the standard therapy for Wilson's disease in children. We report an 8-year-old-girl with liver disease due to Wilson's disease who developed extrapyramidal symptoms following administration of penicillamine. Symptoms resolved within 20 hours of stopping the drug but recurred within 24 hours when gradually increasing small doses were recommenced.