Matsumoto S, Kusaka H, Ito H, Shibata N, Asayama T, Imai T
Department of Neurology, Neurological Center, Tazuke-Kofukai Medical Research Institute and Kitano Hospital, Osaka, Japan.
Clin Neuropathol. 1996 Jan-Feb;15(1):41-6.
This report concerns a case of sporadic amyotrophic lateral sclerosis (ALS) with dementia and Lewy body-like hyaline inclusions (LBHIs). The patient was a 70-year-old woman who initially showed memory disturbance and later developed bulbar palsy, muscle atrophy and weakness. The total clinical course was 51 months. The postmortem examination revealed superficial sponginess and subcortical gliosis in the frontotemporal cortices. Ubiquitin-positive intraneuronal inclusions were found in small cortical neurons of the frontotemporal lobe. Neuronal loss was marked in the spinal anterior horn with degeneration of the pyramidal tracts. The anterior horn cells had ubiquitin-immunoreactive skein-like inclusions and Bunina bodies. LBHIs were present in the lumbar horn; ultrastructurally they were composed of randomly arranged thick filamentous structures studded with granules. The LBHIs were intensely stained with anti-ubiquitin antibody. As in familial ALS and in certain cases of sporadic ALS, some of these inclusions reacted with an antibody against Cu/Zn superoxide dismutase, the enzyme whose gene was recently found to be mutated in some forms of familial ALS.
本报告涉及一例伴有痴呆和路易小体样透明包涵体（LBHIs）的散发性肌萎缩侧索硬化症（ALS）。患者为一名70岁女性，最初表现为记忆障碍，随后出现延髓麻痹、肌肉萎缩和无力。整个临床病程为51个月。尸检显示额颞叶皮质有表面海绵状变性和皮质下胶质增生。在额颞叶的小皮质神经元中发现泛素阳性的神经元内包涵体。脊髓前角有明显的神经元丢失，锥体束变性。前角细胞有泛素免疫反应性的丝状包涵体和布尼亚小体。LBHIs存在于腰角；超微结构上，它们由随机排列的粗丝状结构组成，上面布满颗粒。LBHIs被抗泛素抗体强烈染色。与家族性ALS以及某些散发性ALS病例一样，其中一些包涵体与抗铜/锌超氧化物歧化酶抗体发生反应，最近发现该酶的基因在某些形式的家族性ALS中发生了突变。
