Abstract

Background: Upper limb dystonia is a frequent complication of Wilson's disease (WD). It can lead to poor quality of life and disability. Currently, no effective treatment for it exists. Therefore, we carried out a clinical trial to determine whether high frequency repetitive transcranial magnetic stimulation (rTMS) on the primary motor cortex alleviates upper limb dystonia in WD patients.Methods: This study was a single-center, double-blind, randomized clinical study, included 60 WD patients with upper limb dystonia from a research base of WD in Hefei, China. Participants were randomly divided into a treatment group (TG) and a control group (CG). The TG received rTMS at 10 Hz, while the CG received sham stimulation for 7 consecutive days. Participants were assessed at baseline, after the seventh treatment session, and at 2 and 4 weeks after the seventh treatment session. The primary outcomes included patients' objective muscle tension and stiffness as measured with the MyotonPRO device. The secondary results were scores on clinical scales assessing muscle spasm and motor symptoms, which included the Modified Ashworth Scale (MAS), Unified Wilson's Disease Rating Scale (UWDRS), Burke Fahn Marsden Scale (BFM), and the Activities of Daily Living (ADL) scale.Results: The analysis revealed that after 10 Hz rTMS, muscle tension (P < 0.01) and stiffness (P < 0.01) as measured by the MyotonPRO device decreased significantly in the TG compared to the CG. Moreover, clinically relevant scale scores, including the MAS (P < 0.01), UWDRS (P < 0.01), BFM (P < 0.01), and ADL (P < 0.01) were also significantly reduced.Conclusion: High-frequency rTMS over the primary motor cortex may be an effective complementary and alternative therapy to alleviating upper limb dystonia in WD patients.Clinical Trial Registration: http://www.chictr.org.cn/, identifier: ChiCTR2100046258.

Highlights

  • Wilson’s disease (WD) was first systematically described by Kinnear Wilson in 1912; it is an autosomal recessive neurological disease characterized by chronic and progressive perturbations in copper ion metabolism [1, 2]

  • We carried out a clinical trial to determine whether high frequency repetitive transcranial magnetic stimulation on the primary motor cortex alleviates upper limb dystonia in WD patients

  • The analysis revealed that after 10 Hz repetitive transcranial magnetic stimulation (rTMS), muscle tension (P < 0.01) and stiffness (P < 0.01) as measured by the MyotonPRO device decreased significantly in the treatment group (TG) compared to the control group (CG)

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Summary

Introduction

Wilson’s disease (WD) was first systematically described by Kinnear Wilson in 1912; it is an autosomal recessive neurological disease characterized by chronic and progressive perturbations in copper ion metabolism [1, 2]. Typical symptoms include juvenile Parkinson’s and dystonia; Upper limb dystonia is one of the most common symptoms [3]. No recognized standard objective evaluation of muscle tension or effective treatment methods are available. WD seriously affects patients’ quality of life and results in social and economic burdens. An urgent need exists for an objective evaluation system to monitor patients’ muscle tone as well as alternative therapies that relieve dystonia. Upper limb dystonia is a frequent complication of Wilson’s disease (WD). It can lead to poor quality of life and disability. We carried out a clinical trial to determine whether high frequency repetitive transcranial magnetic stimulation (rTMS) on the primary motor cortex alleviates upper limb dystonia in WD patients

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