Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder of motor neurons in the brain and spinal cord. Accumulation of misfolded proteins is central to the pathogenesis of ALS and the glymphatic system is emerging as a potential therapeutic target to reduce proteinopathy. Using diffusion tensor imaging analysis along the perivascular spaces (DTI-ALPS) to assess glymphatic function, we performed a longitudinal analysis of glymphatic function in ALS and compared it to a disorder in the motor neuron disease spectrum, primary lateral sclerosis (PLS). From a cohort of 45 participants from the Calgary site in the CALSNIC study (Canadian ALS Neuroimaging Consortium), including 18 ALS, 5 PLS and 22 control participants, DTI-ALPS was analysed and correlated to clinical features (age, sex, disease presentation, disease severity and progression rate) and white matter hyperintensity burden. This included longitudinal measurements at three time points, 4 months apart. The DTI-ALPS index was reduced in ALS participants compared with PLS and control participants across all three time points. There was no association with clinical factors; however, the index tended to decline with advancing age. Our study suggests heterogeneity in glymphatic dysfunction in motor neuron diseases that may be related to the underlying pathogenesis.

中文翻译：

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肌萎缩侧索硬化症和原发性侧索硬化症淋巴功能的纵向分析


肌萎缩侧索硬化症 （ALS） 是一种大脑和脊髓运动神经元的神经退行性疾病。错误折叠蛋白的积累是 ALS 发病机制的核心，淋巴系统正在成为减少蛋白病变的潜在治疗靶点。使用沿血管周围间隙的弥散张量成像分析 （DTI-ALPS） 来评估淋巴功能，我们对 ALS 的淋巴功能进行了纵向分析，并将其与运动神经元疾病谱中的一种疾病原发性侧索硬化症 （PLS） 进行了比较。从 CALSNIC 研究（加拿大 ALS 神经影像学联盟）卡尔加里站点的 45 名参与者队列中，包括 18 名 ALS、5 名 PLS 和 22 名对照参与者，对 DTI-ALPS 进行了分析并与临床特征（年龄、性别、疾病表现、疾病严重程度和进展率）和白质高信号负担相关联。这包括在三个时间点进行的纵向测量，间隔 4 个月。与 PLS 和对照组参与者相比，ALS 参与者的 DTI-ALPS 指数在所有三个时间点均降低。与临床因素无关;然而，该指数往往随着年龄的增长而下降。我们的研究表明，运动神经元疾病中淋巴功能障碍的异质性可能与潜在的发病机制有关。