Sone Mie, Yoshida Mari, Hashizume Yoshio, Hishikawa Nozomi, Sobue Gen
Department of Neurology, Nagoya University Graduate School of Medicine, Showa-ku, Japan.
Acta Neuropathol. 2005 Jul;110(1):19-26. doi: 10.1007/s00401-005-1013-9. Epub 2005 May 14.
We immunohistochemically examined the sympathetic ganglia (SG) and brains of 26 patients with multiple system atrophy (MSA), 19 age-matched controls, and 25 patients with amyotrophic lateral sclerosis (ALS). alpha-Synuclein-immunoreactive structures were found in the neuronal cytoplasm and processes of the SG in 11 of the 26 MSA cases (42.3%) and 1 of the 25 ALS cases (4%), but not in the 19 controls. No alpha-synuclein-immunoreactive structures were found in Schwann cells or the neuronal nucleus. Mean disease duration of MSA cases with alpha-synuclein-immunoreactive structures was significantly longer than that of MSA cases without alpha-synuclein-immunoreactive structures. alpha-Synuclein-immunoreactive structures in 4 cases proved to be Lewy bodies (LB) based on hematoxylin-eosin staining. A few LB were also found in the brains of 3 of these 4 cases. In the other 7 MSA cases, diffuse or focal neuronal cytoplasmic aggregates and swollen neurites were detected with alpha-synuclein immunostaining, but not with hematoxylin-eosin staining. However, a few LB-like structures with ring-like staining were observed in those aggregates, which suggested those aggregates had progressed to form LB. Immunoelectron microscopically, those aggregates were composed of filaments and granular materials which closely resembled the ultrastructural features of LB. We inferred that alpha-synuclein aggregates found in the SG in our study evidenced LB-related pathologies. MSA, a type of synucleinopathy, is characterized by glial cytoplasmic inclusions in oligodendrocytes, but also frequently develops LB pathology in the late stage, especially in the SG.
我们采用免疫组织化学方法检查了26例多系统萎缩（MSA）患者、19例年龄匹配的对照者以及25例肌萎缩侧索硬化（ALS）患者的交感神经节（SG）和脑。在26例MSA病例中的11例（42.3%）以及25例ALS病例中的1例（4%）的SG神经元细胞质和突起中发现了α-突触核蛋白免疫反应性结构，而在19例对照者中未发现。在施万细胞或神经元核中未发现α-突触核蛋白免疫反应性结构。具有α-突触核蛋白免疫反应性结构的MSA病例的平均病程显著长于无α-突触核蛋白免疫反应性结构的MSA病例。基于苏木精-伊红染色，4例中的α-突触核蛋白免疫反应性结构被证实为路易小体（LB）。在这4例中的3例脑内也发现了少数路易小体。在其他7例MSA病例中，通过α-突触核蛋白免疫染色检测到弥漫性或局灶性神经元细胞质聚集物和肿胀的神经突，但苏木精-伊红染色未检测到。然而，在这些聚集物中观察到一些具有环状染色的类路易小体结构，这表明这些聚集物已发展形成路易小体。免疫电子显微镜检查显示，这些聚集物由细丝和颗粒物质组成，与路易小体的超微结构特征非常相似。我们推断，在本研究中SG中发现的α-突触核蛋白聚集物证明了与路易小体相关的病理学特征。MSA是一种突触核蛋白病，其特征为少突胶质细胞中的胶质细胞质包涵体，但在晚期也经常出现路易小体病理学改变，尤其是在SG中。
