Hans-Berger Department of Neurology, University Hospital Jena , Jena , Germany.
Amyotroph Lateral Scler Frontotemporal Degener. 2013 Dec;14(7-8):507-15. doi: 10.3109/21678421.2013.812661. Epub 2013 Jul 29.
Amyotrophic lateral sclerosis is a multisystemic neurodegenerative disease in which degenerative processes are not exclusively restricted to the upper and lower motor neurons. Herein, imaging and neuropathological evidence for involvement of the cerebellum, which to date is not thought to be involved in ALS, is reviewed. Evidence for involvement of the cerebellum in ALS comes from several neuropathological studies. Especially ubiquitinated forms of TDP-43 and ubiquitinated p62-positive inclusions were frequently observed. The widely used transgenic SOD1-G93A ALS mice model showed prominent cerebellar immunostaining of pERK and alterations of tau expression. Studies using advanced MRI techniques demonstrated that several cerebral areas, including the cerebellum, were recruited in order to compensate for functional motor decline. Functional MRI, voxel based morphometry, and diffusion-tensor imaging showed these cerebellar alterations as being of functional and structural nature.
肌萎缩侧索硬化症是一种多系统神经退行性疾病，其退行性过程不仅限于上下运动神经元。在此，回顾了小脑受累的影像学和神经病理学证据，迄今为止，小脑被认为与 ALS 无关。小脑参与 ALS 的证据来自几项神经病理学研究。特别是 TDP-43 的泛素化形式和泛素化 p62 阳性包涵体经常被观察到。广泛使用的 SOD1-G93A ALS 转基因小鼠模型显示出明显的小脑 pERK 免疫染色和 tau 表达的改变。使用先进的 MRI 技术的研究表明，包括小脑在内的几个大脑区域被招募以补偿运动功能下降。功能 MRI、体素基于形态计量学和弥散张量成像显示这些小脑改变具有功能和结构性质。
