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下运动神经元中的颗粒丝状结构:一例散发的肌萎缩侧索硬化症病例,伴有许多路易小体样包涵体。

Granulofilamentous profiles in lower motor neurons: a sporadic case of amyotrophic lateral sclerosis with many Lewy body-like inclusions.

作者信息

Kusaka H, Matsumoto S, Imai T

机构信息

Department of Neurology, Kitano Hospital, Osaka, Japan.

出版信息

Clin Neuropathol. 1992 Jan-Feb;11(1):20-4.

PMID:1312402
Abstract

In a 62-year-old man with an 8-month course of sporadic classical amyotrophic lateral sclerosis, many Lewy body-like hyaline inclusions (LI) were observed in spinal anterior horn cells, hypoglossal nuclei, nucleus ambiguus, and motor nuclei of the trigeminal nerve. These motor neurons showed a mild degree of neuronal loss, several Bunina bodies, spheroids and chromatolytic neurons. Tract degeneration was limited to pyramidal tracts. In addition to intensely stained LI, immunoreactive skeins or granules were recognized by a polyclonal anti-ubiquitin antibody. Thick filaments of 15 to 20 nm in diameters with granules formed conglomerated masses with varying amounts of neurofilaments in the anterior horns, corresponding to light microscopically observed LI. More commonly, these thick granulofilamentous profiles were dispersed in small bundles or individually in the cytoplasm. Electron microscopically, there was no close association of filaments with Bunina bodies.

摘要

在一名患有8个月散发性经典肌萎缩侧索硬化症病程的62岁男性中,在脊髓前角细胞、舌下神经核、疑核和三叉神经运动核中观察到许多路易小体样透明包涵体(LI)。这些运动神经元显示出轻度的神经元丢失、多个布尼亚小体、球形小体和染色质溶解神经元。束状变性仅限于锥体束。除了强染色的LI外,多克隆抗泛素抗体还识别出免疫反应性细丝或颗粒。直径为15至20纳米的粗丝与颗粒在前角形成聚集块,其中含有不同数量的神经丝,对应于光镜下观察到的LI。更常见的是,这些粗的颗粒状细丝轮廓分散成小束或单独存在于细胞质中。在电子显微镜下,细丝与布尼亚小体没有紧密关联。

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Clin Neuropathol. 1992 Jan-Feb;11(1):20-4.
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