基于人群的队列研究中 ALS 各临床阶段的认知障碍。
Cognitive impairment across ALS clinical stages in a population-based cohort.
机构信息
From the ALS Center (A. Chiò, C.M., A. Canosa, U.M., R.V., M. Brunetti, M. Barberis, J.P.Z., L.P., B.I., A. Calvo), "Rita Levi Montalcini" Department of Neuroscience, University of Torino; Azienda Ospedaliero-Universitaria Città della Salute e della Scienza of Torino (A. Chiò, C.M., A. Calvo), Turin; Institute of Cognitive Sciences and Technologies (A. Chiò), National Research Council, Rome; Department of Health Sciences Interdisciplinary Research Center of Autoimmune Diseases (L.C., S.D.), "Amedeo Avogadro" University of Eastern Piedmont; ALS Center (E.B., M.F.S., V.S., L.M.), Department of Neurology, Azienda Ospedaliera Universitaria Maggiore della Carità, Novara; and Istituti Clinici Scientifici Maugeri (G.M.), IRCCS Milano, Milan, Italy.
出版信息
Neurology. 2019 Sep 3;93(10):e984-e994. doi: 10.1212/WNL.0000000000008063. Epub 2019 Aug 13.
OBJECTIVE
To assess the association of the degree of severity of motor impairment to that of cognitive impairment in a large cohort of patients with amyotrophic lateral sclerosis (ALS).
METHODS
This is a population-based cross-sectional study on patients with ALS incident in Piemonte, Italy, between 2007 and 2015. Cognitive status was classified according to the revised ALS-FTD Consensus Criteria. The King system and the Milano Torino Staging system (MiToS) were used for defining the severity of motor impairment.
RESULTS
Of the 797 patients included in the study, 163 (20.5%) had ALS-frontotemporal dementia (FTD), 38 (4.8%) cognitive and behavioral impairment (ALScbi), 132 (16.6%) cognitive impairment (ALSci), 63 (7.9%) behavioral impairment (ALSbi), 16 (2.0%) nonexecutive impairment, and 385 (48.2%) were cognitively normal. According to King staging, the frequency of cases with ALS-FTD progressively increased from 16.5% in stage 1-44.4% in stage 4; conversely, the frequency of ALSci, ALSbi, and ALScbi increased from King stage 1 to King stage 3 and decreased thereafter. A similar pattern was observed with the MiToS staging. ALS-FTD was more frequent in patients with bulbar involvement at time of cognitive testing. Patients with expansion (n = 61) showed more severe cognitive impairment with increasing King and MiToS stages.
CONCLUSION
Our findings suggest that ALS motor and cognitive components may worsen in parallel, and that cognitive impairment becomes more pronounced when bulbar function is involved. Our data support the hypothesis that ALS pathology disseminates in a regional ordered sequence, through a cortico-efferent spreading model.
目的
在一个大型肌萎缩侧索硬化症（ALS）患者队列中，评估运动障碍严重程度与认知障碍严重程度之间的相关性。
方法
这是一项基于人群的横断性研究，研究对象为 2007 年至 2015 年间在意大利皮埃蒙特新诊断的 ALS 患者。认知状态根据修订后的 ALS-FTD 共识标准进行分类。King 系统和米兰-都灵分期系统（MiToS）用于定义运动障碍的严重程度。
结果
在纳入研究的 797 例患者中，163 例（20.5%）患有肌萎缩侧索硬化症-额颞叶痴呆（ALS-FTD），38 例（4.8%）患有认知和行为障碍（ALScbi），132 例（16.6%）患有认知障碍（ALSci），63 例（7.9%）患有行为障碍（ALSbi），16 例（2.0%）患有非执行功能障碍，385 例（48.2%）认知正常。根据 King 分期，ALS-FTD 的发生率从 1 期的 16.5%逐渐增加到 4 期的 44.4%；相反，ALSci、ALSbi 和 ALScbi 的发生率从 King 1 期增加到 King 3 期，然后降低。MiToS 分期也观察到类似的模式。在认知测试时，伴有球部受累的患者更常发生 ALS-FTD。携带扩展（n = 61）的患者，随着 King 和 MiToS 分期的增加，认知障碍更为严重。
结论
我们的研究结果表明，ALS 的运动和认知成分可能会同时恶化，当球部功能受累时，认知障碍会更加明显。我们的数据支持 ALS 病理学通过皮质传出扩散模型呈区域性有序传播的假说。
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