Abstract

Background:

Multiple system atrophy (MSA) is a rare neurodegenerative disease characterized by Parkinsonism, ataxia, and autonomic nervous failure. Orthostatic hypotension (OH) is the main feature of central vascular autonomic failure in MSA.

Objective:

The study aimed elucidate the effects of OH on cognitive function, disease milestones, and survival.

Methods:

A total of 444 patients with clinically established MSA were enrolled. Mild and severe OH were defined as a decrease in systolic blood pressure (SBP)/diastolic blood pressure (DBP) >20/10 mmHg and SBP/DBP ≥30/15 mmHg, respectively.

Results:

In this study, 215 MSA patients presented without OH, 88 had mild OH, and 141 had severe OH. The proportion of MSA-C in the severe OH subgroup was significantly higher than that in the subgroup without OH (95/46 vs. 113/102, p = 0.021). The UMSARS I score and the frequency of supine hypertension (SH) in patients with OH were significantly higher than those in patients without OH (16.22 vs. 16.89 vs. 14.60, p < 0.001; 77/64 vs. 29/59 vs. 32/183, p < 0.001). Factors related to the severity of OH included sex (OR, 0.65; p = 0.031), onset age (OR, 0.98; p = 0.029), and SH (OR, 0.21; p < 0.001). The median survival time of patients with severe OH was significantly lower than that of patients without OH (6.79 vs. 8.13 years, p = 0.001). Consistently, Cox survival analysis found that compared with patients without OH, patients with severe OH had a significantly increased risk of death (OR, 2.22; p < 0.001).

Conclusion:

Our large cohort study of MSA provides additional evidence for the negative impact of severe OH on survival.

中文翻译：

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多系统萎缩症中的直立性低血压：相关因素和疾病预后

摘要

背景：

多系统萎缩症（MSA）是一种罕见的神经退行性疾病，其特征是帕金森症、共济失调和自主神经衰竭。直立性低血压（OH）是 MSA 中枢血管自主神经衰竭的主要特征。

客观的：

该研究旨在阐明 OH 对认知功能、疾病里程碑和生存的影响。

方法：

共有 444 名临床确诊的 MSA 患者入组。轻度和重度OH分别定义为收缩压(SBP)/舒张压(DBP)降低≥20/10mmHg和SBP/DBP≥30/15mmHg。

结果：

在这项研究中，215 名 MSA 患者没有出现 OH，88 名患有轻度 OH，141 名患有重度 OH。严重 OH 亚组中 MSA-C 的比例显着高于无 OH 亚组（95/46 vs. 113/102，p = 0.021）。合并 OH 患者的 UMSARS I 评分和卧位高血压（SH）频率均显着高于未合并 OH 的患者（16.22 vs. 16.89 vs. 14.60，p< a i=4> < 0.001；77/64 vs. 29/59 vs. 32/183，p < 0.001）。与 OH 严重程度相关的因素包括性别（OR，0.65；p = 0.031）、发病年龄（OR，0.98；p = 0.029）和 SH（OR，0.21；p < 0.001）。重度OH患者的中位生存时间显着低于无OH患者（6.79年与8.13年，p = 0.001）。一致的是，Cox 生存分析发现，与无 OH 的患者相比，重度 OH 患者的死亡风险显着增加（OR，2.22；p < 0.001）。

结论：

我们的 MSA 大型队列研究为严重 OH 对生存的负面影响提供了额外的证据。