Wilson's Disease (Hepatolenticular Degeneration)

Goldstein, Norman

doi:10.1001/archneur.1971.00480350025002

Cited by 12 publications

(1 citation statement)

References 19 publications

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“…In another study, initial neurological deterioration was observed in as high as 75% of patients following penicillamine whereas none deteriorated following zinc [13]. Many other studies, however, did not report deterioration following penicillamine [14,15,16,17,18,19]. In a systematic review of penicillamine and zinc therapy in WD, overall unfavorable outcome was reported in 14.6% patients; 9.6% deteriorated, 3.5% died and 1.5% remained unchanged.…”

Section: Discussionmentioning

confidence: 99%

Worsening of Wilson Disease following Penicillamine Therapy

Kalita¹,

Kumar²,

Chandra³

et al. 2013

Eur Neurol

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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.

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