Basophilic cytoplasmic inclusions in a case of sporadic juvenile amyotrophic lateral sclerosis
Basophilic cytoplasmic inclusions in a case of sporadic juvenile amyotrophic lateral sclerosis
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DOI：
10.1016/s0022-510x(00)00321-x
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发表时间：
2000-06-15
影响因子：
4.4
通讯作者：
Kikuchi, K
中科院分区：
文献类型：
--
作者：
Aizawa, H;Kimura, T;Kikuchi, K
一位34岁女性，右上肢肌肉萎缩，无力。随后，她的虚弱迅速扩展到左上肢、颈部和下肢。神经系统检查发现下运动神经元系统受累。她在发病后7个月死亡。在脊髓前缘有神经元丢失和反应性胶质增生，而在运动皮质中则少得多。在丘脑和脑干以及上下运动神经元中观察到嗜碱性胞质包涵体。超微结构上，内含物缺乏一个限制膜，并由一个网状的丝状结构与颗粒。包涵体不能与抗磷酸化神经丝或胱抑素C的抗体反应。大多数包涵体与抗泛素抗体不反应，但少数包涵体与该抗体呈颗粒状反应产物沉积。包涵体未用抗TGN 46和MG-160的抗体进行免疫染色，TGN 46和MG-160分别是高尔基体的trans-Golgi网络和内侧池的标记物，这表明它们不是来自破碎的高尔基体。(C)2000 Elsevier Science B. V.保留所有权利。
A 34-year-old woman presented with progressive muscle atrophy and weakness of the right upper extremity. Subsequently her weakness rapidly extended to the left upper extremity, neck and lower extremities. Neurological examination disclosed involvement of the lower motor neuron system. She died 7 months after the onset. There was neuronal loss and reactive gliosis in the anterior hems of the spinal cord and much less frequently in the motor cortex. Basophilic cytoplasmic inclusions were observed in the thalamus and brain stem as well as the upper and lower motor neurons. Ultrastructurally, the inclusions lacked a limiting membrane and consisted of a meshwork of filamentous structures associated with granules. The inclusions failed to react with antibodies against phosphorylated neurofilament or cystatin C. Most of the inclusions show no reaction with anti-ubiquitin antibody, however, a few inclusions show granular reaction product deposits with this antibody. The inclusions were not immunostained with antibodies against TGN46 and MG-160, markers of the trans-Golgi network and the medial cisternae of the Golgi apparatus, respectively, suggesting that they were not derived from the Golgi apparatus which was fragmented. (C) 2000 Elsevier Science B.V. All rights reserved.