Computational Neurobiology Laboratory, Salk Institute for Biological Studies, La Jolla, CA, USA.
Neurology. 2013 Aug 6;81(6):589-98. doi: 10.1212/WNL.0b013e31829e6f72. Epub 2013 Jul 24.
Musician's dystonia (MD) is a focal adult-onset dystonia most commonly involving the hand. It has much greater relative prevalence than non-musician's focal hand dystonias, exhibits task specificity at the level of specific musical passages, and is a particularly difficult form of dystonia to treat. For most MD patients, the diagnosis confirms the end of their music performance careers. Research on treatments and pathophysiology is contingent upon measures of motor function abnormalities. In this review, we comprehensively survey the literature to identify the rating scales used in MD and the distribution of their use. We also summarize the extent to which the scales have been evaluated for their clinical utility, including reliability, validity, sensitivity, specificity to MD, and practicality for a clinical setting. Out of 135 publications, almost half (62) included no quantitative measures of motor function. The remaining 73 studies used a variety of choices from among 10 major rating scales. Most used subjective scales involving either patient or clinician ratings. Only 25% (18) of the studies used objective scales. None of the scales has been completely and rigorously evaluated for clinical utility. Whether studies involved treatments or pathophysiologic assays, there was a heterogeneous choice of rating scales used with no clear standard. As a result, the collective interpretive value of those studies is limited because the results are confounded by measurement effects. We suggest that the development and widespread adoption of a new clinically useful rating scale is critical for accelerating basic and clinical research in MD.
音乐家肌张力障碍(MD)是一种常见的成人发病的局灶性肌张力障碍,最常累及手部。它的相对发病率远高于非音乐家的局灶性手部肌张力障碍,在特定音乐段落的水平上表现出任务特异性,并且是一种特别难以治疗的肌张力障碍形式。对于大多数 MD 患者来说,诊断结果确认了他们音乐表演生涯的结束。针对治疗方法和病理生理学的研究取决于运动功能异常的测量。在这篇综述中,我们全面调查了文献,以确定用于 MD 的评定量表及其使用分布。我们还总结了这些量表在临床实用性方面的评估程度,包括可靠性、有效性、对 MD 的敏感性、特异性和临床实用性。在 135 篇出版物中,近一半(62 篇)没有包含运动功能的定量测量。其余 73 项研究使用了 10 种主要评定量表中的各种选择。大多数使用了涉及患者或临床医生评分的主观量表。只有 25%(18 项)的研究使用了客观量表。没有一种量表在临床实用性方面得到了全面和严格的评估。无论研究涉及治疗方法还是病理生理测定,评定量表的选择都是多种多样的,没有明确的标准。因此,由于测量效果的影响,这些研究的综合解释价值受到限制。我们建议,开发和广泛采用一种新的临床有用的评定量表对于加速 MD 的基础和临床研究至关重要。