Department of Neurology, Charité University Medicine Berlin, Berlin, Germany.
Center for Neurodegenerative Diseases (CEMAND), Department of Medicine, Surgery and Dentistry "Scuola Medica Salernitana", University of Salerno, Baronissi, SA, Italy.
Parkinsonism Relat Disord. 2019 Sep;66:9-15. doi: 10.1016/j.parkreldis.2019.02.020. Epub 2019 Feb 16.
In isolated, sporadic dystonia, it has been occasionally reported that some patients might undergo symptom remission. However, the exact clinical characteristics of patients with remission remain understudied. Given the important prognostic and pathophysiological implications of dystonic remission, we here provide a systematic review of the literature and a meta-analysis to assess demographic and clinical features associated with this phenomenon. We also provide a list of operational criteria to better define dystonic remission. Using PubMed and Embase, we conducted a systematic literature search in March 2018. 626 records were screened, 31 studies comprising data of 2551 cases with reports predominantly from patients with cervical dystonia (n = 1319) or blepharospasm/Meige syndrome (n = 704) were included in qualitative analysis. Five studies reporting remission in cervical dystonia were eligible for meta-analysis. Complete remission was reported in 11.8% and partial remission for 4.4% of cases. Remission rates were higher in cervical dystonia than in blepharospasm/Meige (e.g. complete remission 15.4% vs. 5.8% respectively). Remission occurred on average 4.5 years after onset of dystonic symptoms. However, the majority of patients (63.8%) relapsed. Meta-analysis for cervical dystonia showed that patients with remission were significantly younger at symptom onset than patients without remission (mean difference -7.13 years [95% CI: 10.58, -3.68], p < 0.0001). Based on our findings, we propose that the degree, the conditions associated with the onset, and the duration of remission are key factors to be considered in a unifying definition of dystonic remission.
在孤立性、散发性肌张力障碍中，偶尔有报道称一些患者可能会出现症状缓解。然而，缓解患者的确切临床特征仍有待研究。鉴于肌张力障碍缓解具有重要的预后和病理生理学意义，我们在此对文献进行了系统回顾和荟萃分析，以评估与该现象相关的人口统计学和临床特征。我们还提供了一系列操作性标准，以更好地定义肌张力障碍缓解。我们于 2018 年 3 月使用 PubMed 和 Embase 进行了系统文献检索。筛选了 626 条记录，纳入了 31 项研究的数据，这些研究共纳入了 2551 例病例，主要来自颈肌张力障碍（n=1319）或眼睑痉挛/Meige 综合征（n=704）患者的报告。有 5 项研究报告了颈肌张力障碍的缓解情况，符合荟萃分析的纳入标准。完全缓解的比例为 11.8%，部分缓解的比例为 4.4%。颈肌张力障碍的缓解率高于眼睑痉挛/Meige 综合征（例如，完全缓解分别为 15.4%和 5.8%）。缓解平均发生在肌张力障碍症状出现后 4.5 年。然而，大多数患者（63.8%）会复发。对颈肌张力障碍的荟萃分析显示，缓解患者的症状起始年龄明显小于无缓解患者（平均差异-7.13 岁[95%CI：10.58，-3.68]，p<0.0001）。基于我们的发现，我们提出缓解的程度、与缓解相关的发病条件和缓解持续时间是肌张力障碍缓解统一定义中需要考虑的关键因素。
