Prell Tino, Vlad Benjamin, Gaur Nayana, Stubendorff Beatrice, Grosskreutz Julian
Department of Geriatrics, Halle University Hospital, Jena, Germany.
Department of Neurology, Jena University Hospital, Jena, Germany.
Front Neurosci. 2021 Oct 29;15:656456. doi: 10.3389/fnins.2021.656456. eCollection 2021.
The pathogenesis of the fatal neurodegenerative condition amyotrophic lateral sclerosis (ALS) remains to be fully understood. Blood-brain barrier damage (BBBD) has been implicated as an exacerbating factor in several neurodegenerative conditions, including ALS. Therefore, this cross-sectional study used the novel D50 progression model to assess the clinical relevance of BBBD within a cohort of individuals with either ALS ( = 160) or ALS mimicking conditions ( = 31). Routine laboratory parameters in cerebrospinal fluid (CSF) and blood were measured, and the ratio of CSF to serum albumin levels (Qalb) was used as a proxy measure of BBBD. In the univariate analyses, Qalb levels correlated weakly with disease aggressiveness (as indicated by individual D50 values) and physical function (as measured by ALS Functional Rating Scale). However, after adjustment for cofactors in the elastic net regularization, only having limb-onset disease was associated with BBBD. The results reported here emphasize the clinical heterogeneity of ALS and the need for additional longitudinal and multi-modal studies to fully clarify the extent and effect of BBBD in ALS.
致命的神经退行性疾病肌萎缩侧索硬化症（ALS）的发病机制仍有待充分了解。血脑屏障损伤（BBBD）被认为是包括ALS在内的几种神经退行性疾病的一个加重因素。因此，这项横断面研究使用了新的D50进展模型，以评估在一组ALS患者（n = 160）或疑似ALS疾病患者（n = 31）中BBBD的临床相关性。测量了脑脊液（CSF）和血液中的常规实验室参数，并将CSF与血清白蛋白水平之比（Qalb）用作BBBD的替代指标。在单变量分析中，Qalb水平与疾病侵袭性（由个体D50值表示）和身体功能（由ALS功能评定量表测量）弱相关。然而，在弹性网络正则化中对协变量进行调整后，只有肢体起病的疾病与BBBD相关。此处报告的结果强调了ALS的临床异质性，以及需要进行更多的纵向和多模式研究，以充分阐明BBBD在ALS中的程度和影响。
