Computational Neuroimaging Group, Biomedical Sciences Institute, Trinity College Dublin, Ireland; Department of Psychiatry and Psychotherapy and Institute for Psychology, University of Regensburg, 93053 Regensburg, Germany.
Computational Neuroimaging Group, Biomedical Sciences Institute, Trinity College Dublin, Ireland.
Neurobiol Aging. 2022 Jan;109:78-87. doi: 10.1016/j.neurobiolaging.2021.04.031. Epub 2021 Oct 8.
Motor neuron diseases encompass a divergent group of conditions with considerable differences in clinical manifestations, survival, and genetic vulnerability. One of the key aspects of clinical heterogeneity is the preferential involvement of upper (UMN) and lower motor neurons (LMN). While longitudinal imaging patters are relatively well characterized in ALS, progressive cortical changes in UMN,- and LMN-predominant conditions are seldom evaluated. Accordingly, the objective of this study is the juxtaposition of longitudinal trajectories in 3 motor neuron phenotypes; a UMN-predominant syndrome (PLS), a mixed UMN-LMN condition (ALS), and a lower motor neuron condition (poliomyelitis survivors). A standardized imaging protocol was implemented in a prospective, multi-timepoint longitudinal study with a uniform follow-up interval of 4 months. Forty-five poliomyelitis survivors, 61 patients with amyotrophic lateral sclerosis (ALS), and 23 patients with primary lateral sclerosis (PLS) were included. Cortical thickness alterations were evaluated in a dual analysis pipeline, using standard cortical thickness analyses, and a z-score-based individualized approach. Our results indicate that PLS patients exhibit rapidly progressive cortical thinning primarily in motor regions; ALS patients show cortical atrophy in both motor and extra-motor regions, while poliomyelitis survivors exhibit cortical thickness gains in a number of cerebral regions. Our findings suggest that dynamic cortical changes in motor neuron diseases may depend on relative UMN and/or LMN involvement, and increased cortical thickness in LMN-predominant conditions may represent compensatory, adaptive processes.
运动神经元病包含一组具有明显临床表现、生存和遗传易感性差异的不同疾病。临床异质性的一个关键方面是上运动神经元（UMN）和下运动神经元（LMN）的优先受累。虽然 ALS 中纵向成像模式相对较好地描述，但 UMN 和 LMN 优势疾病的皮质进行性变化很少得到评估。因此，本研究的目的是比较 3 种运动神经元表型的纵向轨迹；一个 UMN 优势综合征（PLS）、一个混合 UMN-LMN 疾病（ALS）和一个下运动神经元疾病（脊髓灰质炎幸存者）。在一项前瞻性、多时间点纵向研究中实施了标准化成像方案，具有统一的 4 个月随访间隔。纳入了 45 名脊髓灰质炎幸存者、61 名肌萎缩侧索硬化症（ALS）患者和 23 名原发性侧索硬化症（PLS）患者。使用标准皮质厚度分析和基于 z 分数的个体化方法在双分析管道中评估皮质厚度变化。我们的结果表明，PLS 患者表现出快速进展的皮质变薄，主要发生在运动区域；ALS 患者在运动和非运动区域均出现皮质萎缩，而脊髓灰质炎幸存者在多个脑区表现出皮质厚度增加。我们的发现表明，运动神经元病中的动态皮质变化可能取决于相对 UMN 和/或 LMN 受累，以及 LMN 优势疾病中增加的皮质厚度可能代表补偿性、适应性过程。
