Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, BC, Canada.
Amyotroph Lateral Scler Frontotemporal Degener. 2020 Nov;21(sup1):47-51. doi: 10.1080/21678421.2020.1837173.
Published descriptions of the neuropathology of clinically defined primary lateral sclerosis (PLS) are reviewed in order to clarify the pathogenesis and the relationship between PLS and classical amyotrophic lateral sclerosis (ALS). Degeneration of the primary motor cortex and corticospinal tracts with preservation of lower motor neurons (LMN) has been reported in most cases. Studies that employed immunohistochemistry found ubiquitin and/or TDP-43-positive neuronal inclusions in the motor cortex and often in the extramotor neocortex. Ubiquitin/TDP-43-immunoreactive inclusions in LMN have been reported in just over half of cases; however, these have never been numerous. The finding of TDP-43 pathology in most cases indicates that PLS and ALS are closely related conditions; however, the fact that cases of PLS consistently show minimal involvement of LMN suggests that PLS represents a distinct entity, rather than an early stage of ALS.
为了阐明原发性侧索硬化症（PLS）的发病机制及其与经典肌萎缩性侧索硬化症（ALS）的关系，本文对临床上定义的 PLS 的神经病理学描述进行了回顾。在大多数情况下，报道了原发性运动皮层和皮质脊髓束的退化，而下运动神经元（LMN）得以保留。使用免疫组织化学研究的发现，在运动皮层中，通常在外运动新皮层中，存在泛素和/或 TDP-43 阳性神经元包涵体。超过一半的病例报告了 LMN 中存在泛素/TDP-43 免疫反应性包涵体；然而，这些包涵体从未大量存在过。在大多数情况下发现 TDP-43 病理学表明 PLS 和 ALS 密切相关；然而，PLS 病例始终表现出 LMN 最小程度受累的事实表明，PLS 代表一种独特的实体，而不是 ALS 的早期阶段。
