Department of Neurology, Tokyo Metropolitan Neurological Hospital (TMNH), 2-6-1 Musashidai, Fuchu, Tokyo, 183-0042, Japan.
Department of Clinical Trial, Clinical Research Support Center, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
J Neurol. 2024 Oct;271(10):6715-6723. doi: 10.1007/s00415-024-12623-7. Epub 2024 Aug 19.
The prognostic impact of dysphagia in multiple system atrophy (MSA) remains controversial. This study aimed to investigate the relationship between dysphagia severity and survival in MSA and to elucidate whether this impact differs between MSA-cerebellar ataxia (MSA-C) and MSA-parkinsonism (MSA-P).
This retrospective study included 297 patients with MSA: 251 met criteria for clinically established MSA and 46 for clinically probable MSA. Among them, 171 had MSA-C and 126 had MSA-P. We evaluated symptomatic dysphagia within 3 years of onset and quantified dysphagia severity using the Hyodo score (0 to 12) through fibreoptic endoscopic evaluation of swallowing (FEES) and clinical features, including autonomic dysfunction and vocal cord paralysis. Patients were followed up until death or tracheostomy, and survival factors were analysed using the log-rank test and multivariate Cox proportional hazards model.
Ninety patients developed symptomatic dysphagia within 3 years of onset, and 75 were evaluated for dysphagia severity using FEES. Survival from onset was shorter in patients with dysphagia within 3 years compared to those without (median: 4.2 years vs. 7.3 years; p < 0.001). Symptomatic dysphagia within 3 years of onset was an independent predictor of shorter survival in the multivariate Cox analysis. While the Hyodo score was higher in MSA-P than in MSA-C patients (p = 0.048), the Hyodo score was associated with survival in both MSA-C and MSA-P patients (log-rank p < 0.001 and p = 0.046, respectively).
Symptomatic dysphagia within 3 years of onset predicts shorter survival in MSA-C and MSA-P patients.
在多系统萎缩症（MSA）中，吞咽困难的预后影响仍存在争议。本研究旨在探讨 MSA 中吞咽困难严重程度与生存之间的关系，并阐明这种影响在 MSA-小脑共济失调（MSA-C）和 MSA-帕金森病（MSA-P）之间是否存在差异。
本回顾性研究纳入了 297 例 MSA 患者：251 例符合临床确诊 MSA 的标准，46 例符合临床可能 MSA 的标准。其中，171 例为 MSA-C，126 例为 MSA-P。我们评估了发病后 3 年内的症状性吞咽困难，并通过纤维内镜吞咽评估（FEES）和包括自主神经功能障碍和声带麻痹在内的临床特征，使用 Hyodo 评分（0 至 12）量化吞咽困难的严重程度。对患者进行随访，直至死亡或行气管切开术，并使用对数秩检验和多变量 Cox 比例风险模型分析生存因素。
90 例患者在发病后 3 年内出现症状性吞咽困难，75 例患者接受了 FEES 评估吞咽困难的严重程度。与无吞咽困难的患者相比，在发病后 3 年内出现吞咽困难的患者的生存时间更短（中位：4.2 年 vs. 7.3 年；p<0.001）。多变量 Cox 分析显示，发病后 3 年内出现症状性吞咽困难是生存时间更短的独立预测因素。虽然 MSA-P 患者的 Hyodo 评分高于 MSA-C 患者（p=0.048），但 Hyodo 评分与 MSA-C 和 MSA-P 患者的生存均相关（对数秩检验 p<0.001 和 p=0.046）。
发病后 3 年内出现症状性吞咽困难可预测 MSA-C 和 MSA-P 患者的生存时间更短。
