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- Title
Worsening of Wilson Disease following Penicillamine Therapy.
- Authors
Kalita, Jayantee; Kumar, Vijay; Chandra, Satish; Kumar, Bishwanath; Misra, Usha Kant
- Abstract
Background: Penicillamine is a standard therapy for Wilson disease (WD) but some patients have paradoxical worsening. Predictors of such deterioration have not been evaluated. This study documents frequency and predictors of deterioration following treatment in WD. Methods: 59 consecutive patients with neurologic WD and 4 asymptomatic siblings were prospectively evaluated. Their clinical, laboratory, ultrasound abdomen and cranial MRI findings with and without worsening were compared. Patients were treated with oral penicillamine and/or zinc and followed up at 1, 3 and 6 months or earlier if needed. Deterioration was defined by >10% worsening in baseline Burke-Fahn-Marsden score or appearance of new neurological sign. Results: Patients' median age was 13 years and 13 were females. 19 patients (30.2%) worsened following treatment; 10 within 1 month, 7 in 1-3 months, and 2 after 3 months of treatment. Deterioration was associated with drooling, leukopenia, thrombocytopenia, splenomegaly and evidence of chronic liver disease. None of the asymptomatic patients following zinc therapy deteriorated. Conclusions: In the deteriorating group, withdrawal of penicillamine resulted in improvement/stabilization in 11 patients, 2 improved by trientine therapy and 4 continued to deteriorate till 3 months. 30.2% patients with WD deteriorated following penicillamine, especially those with chronic liver disease, leukopenia and thrombocytopenia. © 2013 S. Karger AG, Basel
- Subjects
HEPATOLENTICULAR degeneration; PENICILLAMINE; ABDOMEN; MAGNETIC resonance imaging of the brain; DRUG side effects; NEUROLOGY; MAGNETIC resonance imaging
- Publication
European Neurology, 2014, Vol 71, Issue 3/4, p126
- ISSN
0014-3022
- Publication type
Article
- DOI
10.1159/000355276