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)运动外神经元系统的变性是否继发于运动神经元的丧失？与此相关的是，在一名56岁的男性患者中，不仅在前角发现了神经元的丢失，而且在颈髓的中间区也发现了神经元的丢失，他在死亡前38年意外地遭受了右上臂截肢。这一发现表明ALS患者脊髓中间带神经元的丢失至少部分是由前角细胞的丢失所致。ALS患者胸段中间外侧核的神经元主要参与疾病过程，但没有形成布尼娜小体，且在佩戴呼吸机的患者中神经元减少得更严重。我们阐明了关岛ALS的神经元变性机制与GUAM的帕金森-痴呆复合体根本不同，GUAM ALS最初发生时为典型的ALS。

项目成果

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。”

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。

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。”

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)。

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》。