Dingbang Chen1, Xiangxue Zhou1, Haiman Hou2, Li Feng1, JunXiu Liu1, Yinyin Liang1, Xiaopu Lin3, Jiwei Zhang1, Chao Wu1, Xiuling Liang1, Zhong Pei1, Xunhua Li4. 1. Department of Neurology, National Key Clinical Department and Key Discipline of Neurology, Guangdong Key Laboratory for Diagnosis and Treatment of Major Neurological Disease, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, China. 2. Department of Neurology, The First Affiliated Hospital, Zhengzhou University, Zhengzhou, China. 3. Department of Neurology, The First People's Hospital of Zhongshan City, Zhongshan, China Nanfang Hospital, Southern Medical University, Guangzhou, China. 4. Department of Neurology, First Affiliated Hospital, Sun Yat-Sen University, 58 2nd Zhongshan Road, Guangzhou 510080, China.
Abstract
OBJECTIVES: There are limited pharmacological treatments for patients with neurological Wilson's disease (WD) and a history of copper-chelating treatment failure. METHODS: We retrospectively evaluated the clinical records of 38 patients with WD who were treated with sodium dimercaptopropanesulfonate (DMPS) and zinc (group 1) or zinc alone (group 2). All patients had a history of neurological deterioration during their previous treatment with D-penicillamine (DPA). RESULTS: Twenty-one patients were treated with intravenous DMPS for 4 weeks, followed by zinc gluconate for 6 months, and the treatment protocol was repeated twice. Relative to the baseline, repeated DMPS therapy and zinc maintenance therapy decreased neurological scores continuously (p < 0.01). Sixteen patients (76.2%) demonstrated neurological improvements after 1 year of therapy and four patients (19.0%) exhibited neurological deterioration at the follow-up session. In addition, 17 patients were treated with zinc monotherapy for 12 months. Two patients (11.8%) demonstrated neurological improvements and five patients (29.4%) exhibited neurological deterioration. Compared with the patients in group 2, a greater improvement ratio (p < 0.01) and lower deterioration ratio (p < 0.01) were observed in the patients in group 1 after 1 year of therapy. CONCLUSIONS: Our findings indicate that the safety and efficacy of combined treatment of DMPS and zinc is superior to those of zinc monotherapy in patients with neurological WD with a history of DPA treatment failure.
OBJECTIVES: There are limited pharmacological treatments for patients with neurological Wilson's disease (WD) and a history of copper-chelating treatment failure. METHODS: We retrospectively evaluated the clinical records of 38 patients with WD who were treated with sodium dimercaptopropanesulfonate (DMPS) and zinc (group 1) or zinc alone (group 2). All patients had a history of neurological deterioration during their previous treatment with D-penicillamine (DPA). RESULTS: Twenty-one patients were treated with intravenous DMPS for 4 weeks, followed by zinc gluconate for 6 months, and the treatment protocol was repeated twice. Relative to the baseline, repeated DMPS therapy and zinc maintenance therapy decreased neurological scores continuously (p < 0.01). Sixteen patients (76.2%) demonstrated neurological improvements after 1 year of therapy and four patients (19.0%) exhibited neurological deterioration at the follow-up session. In addition, 17 patients were treated with zinc monotherapy for 12 months. Two patients (11.8%) demonstrated neurological improvements and five patients (29.4%) exhibited neurological deterioration. Compared with the patients in group 2, a greater improvement ratio (p < 0.01) and lower deterioration ratio (p < 0.01) were observed in the patients in group 1 after 1 year of therapy. CONCLUSIONS: Our findings indicate that the safety and efficacy of combined treatment of DMPS and zinc is superior to those of zinc monotherapy in patients with neurological WD with a history of DPA treatment failure.
Authors: Karl Heinz Weiss; Daniel Nils Gotthardt; Daniela Klemm; Uta Merle; Daniela Ferenci-Foerster; Mark Schaefer; Peter Ferenci; Wolfgang Stremmel Journal: Gastroenterology Date: 2010-12-24 Impact factor: 22.682
Authors: George J Brewer; Fred Askari; Matthew T Lorincz; Martha Carlson; Michael Schilsky; Karen J Kluin; Peter Hedera; Paolo Moretti; John K Fink; Roberta Tankanow; Robert B Dick; Julia Sitterly Journal: Arch Neurol Date: 2006-04
Authors: George J Brewer; Fred Askari; Robert B Dick; Julia Sitterly; John K Fink; Martha Carlson; Karen J Kluin; Matthew T Lorincz Journal: Transl Res Date: 2009-06-06 Impact factor: 7.012