基于 60 个月的纵向随访的伴有认知和行为改变的肌萎缩侧索硬化症的预后。
Prognosis of amyotrophic lateral sclerosis with cognitive and behavioural changes based on a sixty-month longitudinal follow-up.
机构信息
Neurology Department, Peking University Third Hospital, Beijing, China.
Beijing Municipal Key Laboratory of Biomarker and Translational Research in Neurodegenerative Diseases, Beijing, China.
出版信息
PLoS One. 2021 Aug 11;16(8):e0253279. doi: 10.1371/journal.pone.0253279. eCollection 2021.
OBJECTIVE
Approximately 50% of amyotrophic lateral sclerosis (ALS) patients have cognitive and behavioural dysfunction in varying degrees and forms. Previous studies have shown that cognitive and behavioural changes may indicate a poor prognosis, and cognitive function gradually deteriorates over the course of disease, but the results of different studies have been inconsistent. In addition, there are relatively limited long-term follow-up studies tracking death as an endpoint. The purpose of this study was to investigate the clinical prognostic characteristics of ALS patients with cognitive behavioural changes through long-term follow-up in a cohort.
METHODS
A total of 87 ALS patients from 2014 to 2015 in the Third Hospital of Peking University were selected and divided into a pure ALS group, an ALS with behavioural variant of frontotemporal dementia (ALS-bvFTD) group, and an ALS with cognitive and behaviour changes group. All patients were followed up for 60 months. The main end point was death and tracheotomy.
RESULTS
There was no significant difference in survival curve between pure ALS and ALS with cognitive and behavioural change group, but the survival time of ALS-bvFTD group was significantly lower than the other two groups (P < 0.001). For those who was followed up to the endpoint, the survival time of the ALS-bvFTD group was significantly shorter than that of the pure ALS group (t = 5.33, p < 0.001) or the ALS with cognitive and behaviour changes group (t = 4.25, p < 0.001). The progression rate of ALS Functional Rating Scale-Revised (FRS-R) scores from recruitment to endpoint was significantly faster in the ALS-bvFTD group than in the pure ALS group (z = 2.68, p = 0.01) or the ALS with cognitive and behavioural changes group (z = 2.75, p = 0.01). There was no significant difference in survival time (t = 0.52, P = 0.60) or FRS-R score progression rate (z = 0.31, p = 0.76) between the pure ALS group and the ALS with cognitive and behavioural changes group. The total Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen (ECAS) score was positively correlated with survival time (r = 0.38, p = 0.01).
CONCLUSION
ALS-bvFTD patients have shorter survival time. The total ECAS score may be correlated with survival time.
目的
约 50%的肌萎缩侧索硬化症（ALS）患者存在不同程度和形式的认知和行为功能障碍。既往研究表明，认知和行为改变可能预示预后不良，且认知功能在疾病过程中逐渐恶化，但不同研究的结果并不一致。此外，以死亡为终点的长期随访研究相对较少。本研究旨在通过队列研究对伴有认知行为改变的 ALS 患者的临床预后特征进行长期随访。
方法
选取 2014 年至 2015 年北京大学第三医院的 87 例 ALS 患者，分为单纯 ALS 组、ALS 伴额颞叶痴呆行为变异型（ALS-bvFTD）组和 ALS 伴认知和行为改变组。所有患者均随访 60 个月。主要终点为死亡和气管切开术。
结果
单纯 ALS 组和 ALS 伴认知和行为改变组的生存曲线无显著差异，但 ALS-bvFTD 组的生存时间明显短于其他两组（P<0.001）。对于随访至终点的患者，ALS-bvFTD 组的生存时间明显短于单纯 ALS 组（t=5.33，P<0.001）或 ALS 伴认知和行为改变组（t=4.25，P<0.001）。从入组到终点，ALS 功能评定量表修订版（FRS-R）评分的进展率在 ALS-bvFTD 组明显快于单纯 ALS 组（z=2.68，P=0.01）或 ALS 伴认知和行为改变组（z=2.75，P=0.01）。单纯 ALS 组与 ALS 伴认知和行为改变组的生存时间（t=0.52，P=0.60）或 FRS-R 评分进展率（z=0.31，P=0.76）无显著差异。总爱丁堡认知和行为 ALS 筛查量表（ECAS）评分与生存时间呈正相关（r=0.38，P=0.01）。
结论
ALS-bvFTD 患者的生存时间较短。总 ECAS 评分可能与生存时间相关。
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