Background/Objective:Multiple system atrophy (MSA) is a highly debilitating, rare neurodegenerative disorder with two clinical motor variants (parkinsonian or MSA-P and cerebellar or MSA-C). There is a wide span of motor and non-motor symptoms (NMS) that progress over time. We studied the cohort from the Catalan Multiple System Atrophy Registry (CMSAR) to determine which symptoms are most likely to progress throughout a 2-year follow-up. Methods:We analyzed baseline, 12-month, and 24-month follow-up evaluations from the 80 cases recruited by the CMSAR. Evaluations included the UMSARS assessment, cognitive and neuropsychiatric evaluations, and a non-motor scale (NMSS-PD). Statistical analysis was done using a Generalized Estimated Equations (GEE) model. Results:Both UMSARS I and II sub-scores significantly increased at 12- and 24-month follow-ups (p < 0.001), with a median total score increase of 11 and 12.5 points, respectively. Items on UMSARS I that significantly worsened were mostly motor affecting daily activities. NMS, including urinary and sexual dysfunction, as well as sleep difficulties showed a significant progression on the NMSS-PD; however, other NMS such as postural hypotension, gastrointestinal, and mood dysfunction, although prevalent, did not show a clear progression on clinical scales. Conclusion:Within 24 months and as early as 12 months, MSA cases may experience significant motor worsening, affecting basic daily activities. NMS are prevalent; however, not all clinical scales register a clear progression of symptoms, perhaps suggesting that they are not sensitive enough for non-motor evaluation.

中文翻译：

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多系统萎缩中运动和非运动症状的进展：来自 Catalan-MSA 登记处的前瞻性研究

背景/目的：多系统萎缩 (MSA) 是一种高度衰弱、罕见的神经退行性疾病，具有两种临床运动变异（帕金森病或 MSA-P 和小脑或 MSA-C）。随着时间的推移，存在广泛的运动和非运动症状 (NMS)。我们研究了来自加泰罗尼亚多系统萎缩登记处 (CMSAR) 的队列，以确定哪些症状最有可能在 2 年的随访中进展。方法：我们分析了 CMSAR 招募的 80 例病例的基线、12 个月和 24 个月的随访评估。评估包括 UMSARS 评估、认知和神经精神评估以及非运动量表 (NMSS-PD)。使用广义估计方程（GEE）模型进行统计分析。结果：在 12 个月和 24 个月的随访中，UMSARS I 和 II 子分数均显着增加（p < 0.001），中位总分分别增加 11 和 12.5 分。UMSARS I 上显着恶化的项目主要是影响日常活动的运动。NMS，包括泌尿和性功能障碍，以及睡眠困难，在 NMSS-PD 上显示出显着进展；然而，其他 NMS，如体位性低血压、胃肠道和情绪功能障碍，虽然很普遍，但在临床量表上并未显示出明显的进展。结论：在 24 个月内和早在 12 个月内，MSA 病例可能会出现明显的运动恶化，影响基本的日常活动。NMS很普遍；然而，并非所有临床量表都记录了明显的症状进展，