Lowe J
Department of Pathology, University of Nottingham Medical School, Queen's Medical Centre, UK.
J Neurol Sci. 1994 Jul;124 Suppl:38-51. doi: 10.1016/0022-510x(94)90175-9.
There have been recent developments in the pathology of sporadic ALS. A new filamentous neuronal inclusion body in ALS detected by immunohistochemical localisation of the protein ubiquitin has been characterised at the light microscopic and ultrastructural level and appears specific for the disease. The molecular composition of underlying filaments remains unresolved but the quest for this is a major aim in ALS research. Despite being a progressive degenerative process which primarily affects motor systems, ALS is now recognised to involve several non-motor systems and in long survivors affects many subcortical structures. There is also accumulating evidence that the neurodegenerative process underlying ALS may present as a non-motor clinical syndrome, particularly as a frontal lobe dementia with characteristic inclusions present in the non-motor cortex. Considering ALS as a multisystem disease rather than simply a disease of motor neurones has major implications for research into pathogenesis.
散发性肌萎缩侧索硬化症（ALS）的病理学研究近来有了新进展。通过对泛素蛋白进行免疫组织化学定位，在ALS中检测到一种新的丝状神经元包涵体，已在光学显微镜和超微结构水平对其进行了表征，且似乎是该疾病所特有的。其潜在细丝的分子组成仍未明确，但对这方面的探索是ALS研究的一个主要目标。尽管ALS是一个主要影响运动系统的进行性退行性过程，但现在人们认识到它涉及多个非运动系统，在病程较长的患者中还会影响许多皮质下结构。越来越多的证据表明，ALS潜在的神经退行性过程可能表现为一种非运动性临床综合征，尤其是表现为额叶痴呆，且在非运动皮质中存在特征性包涵体。将ALS视为一种多系统疾病而非仅仅是运动神经元疾病，对发病机制的研究具有重要意义。
