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目的：认识常见神经变性疾病的脊髓组织病理及其蛋白质表达特征。方法对解放军总医院1955年至2013年间经临床与病理解剖确诊的3例多系统萎缩，4例肌萎缩侧索硬化症，5例阿尔茨海默病（其中2例合并帕金森病），2例进行性核上性麻痹，1例路易体痴呆和1例皮质基底节变性病例的脊髓组织进行常规组织学及Gallyas-Braak 银染色检查，相应切片进行tau 蛋白、α-synuclein、TDP-43及泛素免疫组织化学染色；以4例同龄、无神经疾病症状及脑病理改变的脊髓组织标本作为对照。结果3例多系统萎缩的胸髓侧角及骶髓Onuf核神经细胞重度脱失，颈髓前角细胞中度脱失，轻度脊髓前束及外侧束变性，病变区存在大量泛素和α-synuclein 阳性少突胶质细胞包涵体；4例肌萎缩侧索硬化症的脊髓前角细胞重度脱失，残存细胞萎缩变性，2例见Bunina小体，脊髓前束、外侧束存在中、重度髓鞘变性，其中3例脊髓组织存在泛素、TDP-43阳性神经细胞包涵体及TDP-43阳性胶质细胞包涵体；5例阿尔茨海默病中4例脊髓前角细胞存在tau阳性神经原纤维缠结；2例阿尔茨海默病合并帕金森病及1例路易体痴呆的胸脊髓侧角也存在重度神经细胞脱失，见α-synuclein阳性路易小体及轴索变性；2例进行性核上性麻痹及1例皮质基底节变性存在脊髓前角细胞轻度脱失，并见tau阳性球形神经原纤维缠结及大量线丝结构。结论多系统萎缩、肌萎缩侧索硬化症以及阿尔茨海默病、帕金森病和进行性核上性麻痹等神经变性疾病的脊髓组织存在各自特征性组织学改变及相应的蛋白质病理表达，值得重视对这些神经变性疾病的脊髓病理研究。

Objective To investigate histopathology and proteinopathy in the spinal cord of patients with common neurodegenerative diseases. Methods Spinal cord tissues from clinically and neuropathologically confirmed neruodegnerative diseases were enrolled in this study , including 3 cases of multiple system strophy , 4 cases of amyotrophic lateral sclerosis , 5 cases of Alzheimer′s disease ( AD, included 2 cases of AD combined with Parkinson′s disease ) , 2 cases of progressive supranuclear palsy , 1 case of dementia with lewy body and 1 case of corticobasal degeneration from 1955 to 2013 at Chinese People′s Liberation Army General Hospital.Four normal control cases were also included.Routine HE and Gallyas-Braak staining , and immunohistochemical stainings for anti-PHF tau ( AT8 ) , anti-α-synuclein , anti-TDP-43 and anti-ubiquitin were performed.Results Examination of the spinal cord in 3 cases with multiple system strophy revealed severe neuron loss in the intermediolateral nucleus of thoracic segment and Onuf′s nucleus of the sacral segment , along with moderate neuron loss in the anterior horn of the cervical segment and mild myelin pallor in the anterior funiculus and anterolateral funiculus in the cervical and thoracic segments.Large amount of argentophilic , ubiquitin and synuclein positive oligodendroglial cytoplasmic inclusions were found widely distributed in the anterior horn and the anterior funiculus and anterolateral funiculus of the full spinal cord.Severe neuron loss and several morphological changes with gliosis in the anterior horn and severe loss of myelin in the anterior funiculus and anterolateral funiculus of the full spinal cord were observed in 4 cases of amyotrophic lateral sclerosis , 2 of which were found with Bunina bodies in neurons of the anterior horn.Three amyotrophic lateral sclerosis cases had ubiquitin-positive neuronal inclusions and TDP-43 positive neuronal and glial inclusions in the anterior horn at cervical and lumbar segments.A few argentophilic , tau positive neurofibrillary tangles ( NFTs) and neuropil threads in the anterior horn at cervical and lumbar segments were found in 4 AD cases.Examination of spinal cord in 2 cases with Parkinson′s disease combined with AD and 1 case with dementia with lewy body revealed severe neuron loss in the intermediolateral nucleus of thoracic segment , and a few synuclein positive lewy bodies and neuritis were also observed.There was mild neuron loss in the anterior horn at cervical and lumbar segments, along with some argentophilic , tau positive globous NFTs and many argentophilic , tau positive neutrophil threads were observed in 2 progressive supranuclear palsy cases and 1 corticobasal degeneration case.Conclusion Each common neurodegenerative diseases of the spinal cord including multiple system strophy,amyotrophic lateral sclerosis and Parkinson′s disease has its own specific histopathology and proteinopathy characteristics .