Department of Clinical Neuroscience, Brighton and Sussex Medical School, Brighton, UK.
Hurstwood Park Neurological Centre, Brighton and Sussex University Hospitals, Brighton, UK.
J Neurol Neurosurg Psychiatry. 2017 May;88(5):402-411. doi: 10.1136/jnnp-2016-314956. Epub 2017 Mar 1.
To undertake a systematic review and meta-analysis of studies that investigated prognostic factors and survival in patients with progressive supranuclear palsy (PSP) and multiple system atrophy (MSA).
Publications of at least 10 patients with a likely or confirmed diagnosis of PSP or MSA were eligible for inclusion. Methodological quality was rated using a modified version of the Quality in Prognostic Studies tool. For frequently examined prognostic factors, HRs derived by univariate and multivariate analysis were pooled in separate subgroups; other results were synthesised narratively and HRs could not be reported here.
Thirty-seven studies presenting findings on 6193 patients (1911 PSP, 4282 MSA) fulfilled the inclusion criteria. We identified the following variables as unfavourable predictors of survival. In PSP, PSP-Richardson's phenotype (univariate HR 2.53; 95% CI 1.69 to 3.78), early dysphagia and early cognitive symptoms. In MSA, severe dysautonomia and early development of combined autonomic and motor features but not MSA phenotype (multivariate HR 1.22; 95% CI 0.83 to 1.80).In PSP and MSA, survival was predicted by early falls (multivariate HR 2.32; 95% CI 1.94 to 2.77), the Neuroprotection and Natural History in Parkinson Plus Syndromes Parkinson Plus Score and the Clinical Global Impression Disease Severity Score but not sex (multivariate HR 0.93; 95% CI 0.67 to 1.28). There was conflicting evidence regarding the prognostic effect of age at onset and stridor.
Several clinical variables were strongly associated with shorter survival in PSP and MSA. Results on most prognostic factors were consistent across methodologically diverse studies; however, the lack of commonality of prognostic factors investigated is a significant limitation.
对探讨进行性核上性麻痹（PSP）和多系统萎缩（MSA）患者预后因素和生存情况的研究进行系统回顾和荟萃分析。
至少纳入 10 例可能或确诊为 PSP 或 MSA 的患者的研究符合纳入标准。使用改良版预后研究质量工具对方法学质量进行评分。对于经常检查的预后因素，通过单变量和多变量分析得出的 HR 在单独的亚组中进行汇总；其他结果则以叙述性方式进行综合，这里无法报告 HR。
37 项研究共纳入 6193 例患者（PSP 患者 1911 例，MSA 患者 4282 例），满足纳入标准。我们确定了以下变量为生存的不利预测因素。在 PSP 中，PSP-Richardson 表型（单变量 HR 2.53；95%CI 1.69 至 3.78）、早期吞咽困难和早期认知症状。在 MSA 中，严重的自主神经功能障碍和早期出现自主神经和运动功能合并特征，但不是 MSA 表型（多变量 HR 1.22；95%CI 0.83 至 1.80）。在 PSP 和 MSA 中，早期跌倒（多变量 HR 2.32；95%CI 1.94 至 2.77）、神经保护和帕金森病及相关综合征自然史帕金森病及相关综合征评分和临床总体印象疾病严重程度评分预测了生存，但性别无此预测作用（多变量 HR 0.93；95%CI 0.67 至 1.28）。关于发病年龄和喘鸣对预后的影响，证据存在冲突。
几项临床变量与 PSP 和 MSA 患者的较短生存期密切相关。大多数预后因素的研究结果在方法学差异较大的研究中是一致的；然而，所研究的预后因素缺乏共同性是一个显著的局限性。
