Amyotrophic lateral sclerosis : the progression mechanism across neuronal junctions

肌萎缩侧索硬化症：神经元连接的进展机制

• 批准号: 05680653
• 负责人: OYANAGI Kiyomitsu
• 金额: $ 1.28万
• 依托单位: Niigata University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for General Scientific Research (C)
• 财政年份: 1993
• 资助国家: 日本
• 起止时间: 1993 至 1994
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/en/grant/KAKENHI-PROJECT-05680653/
• 关键词: Amyotrophic lateral sclerosis; Degeneration; Spinal cord; Anterior horn; Intermediate zone; Intermediolateral nucleus; Transneuronal degeneration; Guam ALS; 運動ニューロン; 肢切断

Was the degeneration in the extra-motor neuron system of the amyotrophic lateral sclerosis (ALS), induced secondarily to the loss of motor neurons? In relation to this, loss of neurons was found not only in the anterior horn but also in the intermediate zone of the cervical spinal cord in a 56-year-old man who had suffered accidental amputation of the right upper arm 38 years prior to death. This finding indicates that the loss of neurons in the intermediate zone of the spinal cord in ALS patients is sequentially induced at least partly by loss of the anterior horn cells.The neurons in the intermediolateral nucleus at the thoracic segment of the ALS patients, were involved primarily in the disease process without the formation of Bunina bodies, and were more severely decreased in the respirator-supported patients than non-respirator-supported patients.We elucidated that the mechanism of neuronal degeneration of Guam ALS is basically different from that of the parkinsonism-dementia complex of Guam, and that Guam ALS occurs initially as classic ALS.

肌萎缩侧索硬化症 (ALS) 的运动外神经元系统变性是否继发于运动神经元的丧失？与此相关的是，一名 56 岁男性在去世前 38 年右上臂意外截肢，不仅前角神经元缺失，颈脊髓中间区也出现神经元缺失。 。这一发现表明，ALS 患者脊髓中间区神经元的丢失至少部分是由前角细胞的丢失引起的。ALS 患者胸段的中间外侧核的神经元参与其中主要在疾病过程中不形成布尼纳小体，并且在呼吸机支持的患者中比非呼吸机支持的患者下降更严重。我们阐明了关岛ALS的神经元变性机制与关岛的帕金森病-痴呆症综合征基本不同，关岛的 ALS 最初是作为典型的 ALS 出现的。

项目成果

Takeda S: "Motor neuron disease with multi-system involvement presenting as tetraparesis, ophthalmoplegia and sensori-autonomic dysfunction" Acta Neuropathologica. 88. 193-200 (1994)

Takeda S：“多系统受累的运动神经元疾病，表现为四肢轻瘫、眼肌麻痹和感觉自主神经功能障碍”《神经病理学报》。

Wakabayashi K: "Corticobasal degeneration:etiopathological significance of the cytoskeletal alterations." Acta Neuropathologica. 87. 545-553 (1994)

Wakabayashi K：“皮质基底节变性：细胞骨架改变的病因病理学意义。”

Suzuki H: "A quantitative pathological investigation of the cervical cord,roots and ganglia after long-term amputation of the unilateral upper arm." Acta Neuropathologica. 85. 666-673 (1993)

Suzuki H：“单侧上臂长期截肢后颈髓、颈根和神经节的定量病理学研究。”

小〓清光: "筋萎縮性側索硬化症の脊髄側索前方部に関する定量的検討" Neuropathology. suppl.14. 153 (1994)

Kiyomitsu Ko：“肌萎缩侧索硬化症脊髓前部的定量研究”神经病理学 153 (1994)。

Oyanagi K: "Topographic investigation of brain atrophy in parkinsonismdementia complex of Guam:a comparison with Alzheimer's disease and progressive supranuclear palsy" Neurodegeneration. 3. 301-304 (1994)

Oyanagi K：“关岛帕金森痴呆症脑萎缩的地形学研究：与阿尔茨海默病和进行性核上性麻痹的比较”神经变性。