Kato S, Nakamura H
Division of Neuropathology, Tottori University School of Medicine, Yongao, Japan.
Acta Neuropathol. 1990;79(6):584-94. doi: 10.1007/BF00294235.
Patients with olivopontocerebellar atrophy (OPCA) were studied, and cytoplasmic inclusions were observed in some of the remaining neurons of the pontine nuclei, nuclei reticularis tegmenti pontis and arcuate nuclei. The cytoplasmic argyrophilic inclusions were demonstrated by silver impregnation techniques such as Bielschowsky and Bodian staining. With hematoxylin and eosin stain, the inclusions were sharply demarcated and appeared pale. The inclusions were not stained by the following routine histological methods: Klüver-Barrera, phosphotungstic acid hematoxylin, Holzer, periodic acid-Schiff, Mallory azan, alcian blue, nile blue, Masson trichrome, Congo red, thioflavine S, oil red O and Sudan black B stains. Immunohistochemistry with anti-ubiquitin antiserum showed that these inclusions were ubiquitinated. However, the inclusions did not react with any of the following antibodies (Abs) or antisera: anti-phosphorylated neurofilament (NF) Ab, anti-nonphosphorylated NF Abs (160 and 200 kDa), anti-paired helical filament antiserum, anti-tau antiserum, anti-tubulin Abs (alpha and beta), anti-microtubule-associated protein antiserum, anti-glial fibrillary acidic protein antiserum, anti-vimentin Ab, anti-desmin Ab, anti-cytokeratin Abs (low and high molecular weights), anti-actin antiserum, anti-skeletal myosin antiserum and anti-myelin basic protein Ab. Ultrastructurally, the inclusion bodies noted in OPCA were composed primarily of fibrils having a width ranging from about 24 to 40 nm, which were entirely coated with osmiophilic granular material along their whole length. They were occasionally intermingled with a few filaments about 10 nm in width. Electron microscopical examination on silver-impregnated specimens revealed that each granule-coated fibril had a great affinity for silver particles. In elucidating the pathogenesis of OPCA, it was considered to be an important neuropathological finding that some of the remaining pontine neurons affected by OPCA developed characteristic cytoplasmic argyrophilic inclusions.
对橄榄体脑桥小脑萎缩(OPCA)患者进行了研究,在脑桥核、脑桥被盖网状核和弓状核的一些残留神经元中观察到了胞质内包涵体。胞质嗜银性包涵体通过诸如 Bielschowsky 和 Bodian 染色等银浸染技术得以显示。用苏木精和伊红染色时,包涵体界限清晰,呈淡色。这些包涵体不能被以下常规组织学方法染色:Klüver-Barrera 染色、磷钨酸苏木精染色、Holzer 染色、过碘酸希夫染色、Mallory 阿赞染色、阿尔辛蓝染色、尼罗蓝染色、Masson 三色染色、刚果红染色、硫黄素 S 染色、油红 O 染色和苏丹黑 B 染色。用抗泛素抗血清进行免疫组织化学检测显示这些包涵体被泛素化。然而,这些包涵体不与以下任何抗体(Ab)或抗血清发生反应:抗磷酸化神经丝(NF)抗体、抗非磷酸化 NF 抗体(160 和 200 kDa)、抗双螺旋丝抗血清、抗 tau 抗血清、抗微管蛋白抗体(α和β)、抗微管相关蛋白抗血清、抗胶质纤维酸性蛋白抗血清、抗波形蛋白抗体、抗结蛋白抗体、抗细胞角蛋白抗体(低分子量和高分子量)、抗肌动蛋白抗血清、抗骨骼肌肌球蛋白抗血清和抗髓鞘碱性蛋白抗体。超微结构上,OPCA 中发现的包涵体主要由宽度约为 24 至 40 纳米的原纤维组成,并在其全长上完全被嗜锇颗粒物质包裹。它们偶尔与一些宽度约为 10 纳米的细丝混合。对银浸染标本的电子显微镜检查显示,每个被颗粒包裹的原纤维对银颗粒具有很强的亲和力。在阐明 OPCA 的发病机制时,认为受 OPCA 影响的一些残留脑桥神经元出现特征性胞质嗜银性包涵体是一项重要的神经病理学发现。