肌萎缩侧索硬化症的区域进展间隔根据不同身体区域受累的时间而变化。
Amyotrophic lateral sclerosis regional progression intervals change according to time of involvement of different body regions.
作者信息
Manera Umberto, D'Ovidio Fabrizio, Cabras Sara, Torrieri Maria Claudia, Canosa Antonio, Vasta Rosario, Palumbo Francesca, Grassano Maurizio, De Marchi Fabiola, Mazzini Letizia, Mora Gabriele, Moglia Cristina, Calvo Andrea, Chiò Adriano
机构信息
ALS Centre, "Rita Levi Montalcini" Department of Neuroscience, University of Turin, Turin, Italy.
Neurology 1, AOU Città della Salute e della Scienza di Torino, Turin, Italy.
出版信息
Eur J Neurol. 2023 Apr;30(4):872-880. doi: 10.1111/ene.15674. Epub 2023 Jan 23.
BACKGROUND AND PURPOSE
The prediction of disease course is one of the main targets of amyotrophic lateral sclerosis (ALS) research, particularly considering its wide phenotypic heterogeneity. Despite many attempts to classify patients into prognostic categories according to the different spreading patterns at diagnosis, a precise regional progression rate and the time of involvement of each region has yet to be clarified. The aim of our study was to evaluate the functional decline in different body regions according to their time of involvement during disease course.
METHODS
In a population-based dataset of ALS patients, we analysed the functional decline in different body regions according to time and order of regional involvement. We calculated the regional progression intervals (RPIs) between initial involvement and severe functional impairment using the ALS Functional Rating Scale revised (ALSFRS-r) subscores for the bulbar, upper limb, lower limb and respiratory/thoracic regions. Time-to-event analyses, adjusted for age, sex, ALSFRS-r pre-slope (ΔALSFRS-R), cognitive status, and mutational status were performed.
RESULTS
The duration of RPI differed significantly among ALS phenotypes, with the RPI of the first region involved being significantly longer than the RPIs of regions involved later. Cox proportional hazard models showed that in fact a longer time between disease onset and initial regional involvement was related to a reduced duration of the RPI duration in each different body region (bulbar region: hazard ratio [HR] 1.11, 95% confidence interval [CI] 1.06-1.16, p < 0.001; upper limb region: HR 1.16, 95% CI 1.06-1.28, p = 0.002; lower limb region: HR 1.11, 95% CI 1.03-1.19, p = 0.009; respiratory/thoracic region: HR 1.10, 95% CI 1.06-1.14, p = 0.005).
CONCLUSIONS
We found that the progression of functional decline accelerates in regions involved later during disease course. Our findings can be useful in patient management and prognosis prediction.
背景与目的
疾病进程的预测是肌萎缩侧索硬化症（ALS）研究的主要目标之一，尤其是考虑到其广泛的表型异质性。尽管人们多次尝试根据诊断时不同的扩散模式将患者分类为不同的预后类别，但每个区域的确切进展速度以及受累时间仍有待明确。我们研究的目的是根据疾病进程中不同身体区域的受累时间来评估其功能衰退情况。
方法
在一个基于人群的ALS患者数据集中，我们根据区域受累的时间和顺序分析了不同身体区域的功能衰退情况。我们使用修订的ALS功能评定量表（ALSFRS-r）中延髓、上肢、下肢以及呼吸/胸部区域的子评分，计算了从最初受累到严重功能损害之间的区域进展间隔（RPI）。进行了事件发生时间分析，并对年龄、性别、ALSFRS-r预斜率（ΔALSFRS-R）、认知状态和突变状态进行了校正。
结果
RPI的持续时间在不同ALS表型之间存在显著差异，首个受累区域的RPI明显长于随后受累区域的RPI。Cox比例风险模型显示，事实上，疾病发作与最初区域受累之间的时间越长，每个不同身体区域的RPI持续时间就越短（延髓区域：风险比[HR] 1.11，95%置信区间[CI] 1.06 - 1.16，p < 0.001；上肢区域：HR 1.16，95% CI 1.06 - 1.28，p = 0.002；下肢区域：HR 1.11，95% CI 1.03 - 1.19，p = 0.009；呼吸/胸部区域：HR 1.10，95% CI 1.06 - 1.
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