title: Clinical efficacy of combined sodium dimercaptopropanesulfonate and zinc treatment in neurological Wilson's disease with D-penicillamine treatment failure.
abstract: OBJECTIVE
  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.affiliation: 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.
authors.forename: Dingbang
authors.initials: D
authors.lastname: Chen
authors.affiliation: 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.
authors.forename: Xiangxue
authors.initials: X
authors.lastname: Zhou
authors.affiliation: Department of Neurology, The First Affiliated Hospital, Zhengzhou University, Zhengzhou, China.
authors.forename: Haiman
authors.initials: H
authors.lastname: Hou
authors.affiliation: 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.
authors.forename: Li
authors.initials: L
authors.lastname: Feng
authors.affiliation: 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.
authors.forename: JunXiu
authors.initials: J
authors.lastname: Liu
authors.affiliation: 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.
authors.forename: Yinyin
authors.initials: Y
authors.lastname: Liang
authors.affiliation: Department of Neurology, The First People's Hospital of Zhongshan City, Zhongshan, China Nanfang Hospital, Southern Medical University, Guangzhou, China.
authors.forename: Xiaopu
authors.initials: X
authors.lastname: Lin
authors.affiliation: 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.
authors.forename: Jiwei
authors.initials: J
authors.lastname: Zhang
authors.affiliation: 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.
authors.forename: Chao
authors.initials: C
authors.lastname: Wu
authors.affiliation: 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.
authors.forename: Xiuling
authors.initials: X
authors.lastname: Liang
authors.affiliation: 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.
authors.forename: Zhong
authors.initials: Z
authors.lastname: Pei
authors.affiliation: Department of Neurology, First Affiliated Hospital, Sun Yat-Sen University, 58 2nd Zhongshan Road, Guangzhou 510080, China.
authors.forename: Xunhua
authors.initials: X
authors.lastname: Li
keywords: Wilson’s disease; copper; neurological deterioration; sodium dimercaptopropanesulfonate; zinc