PDF(Pubmed)
Abstract：
Multiple system atrophy (MSA) is an adult-onset neurodegenerative disorder that presents with variable combinations of autonomic dysfunction, cerebellar ataxia, parkinsonism, and pyramidal signs. The inferior olivary nucleus is targeted in MSA, with a phenotype of olivopontocerebellar atrophy in particular, and involvement of the olivocerebellar tract is well known. However, degeneration of the olivospinal tract has not been studied in MSA. We examined 97 spinal cords from consecutively autopsied patients with MSA. Myelin staining revealed that 22 cords (22.7%) had small, bilateral, triangular-shaped tract degeneration in the boundary of the anterior and lateral funiculi, which appeared continuously from C1 to C5. The anatomical pathway of the degenerated tract was consistent with the description of the olivospinal tract provided by Helweg in 1888. The MSA patients showing degeneration of this tract were younger at disease onset (average: 56.4 ± 8.7 years, range: 42-74), and had longer disease duration (average: 10.1 ± 4.8 years, range: 2-25) and more severe olivopontocerebellar changes compared to other MSA patients. Quantitative analyses revealed that patients with olivospinal tract degeneration had a lower neuronal density in the inferior olivary nucleus compared to other patients. Microglial density in this tract was negatively correlated with the neuronal density in the inferior olivary nucleus. The densities of glial cytoplasmic inclusions in the inferior olivary nucleus and in the olivospinal tract were strongly correlated with each other. Neurologically healthy controls (n = 22) and disease controls with Lewy body disease (n = 30), amyotrophic lateral sclerosis (n = 30), and progressive supranuclear palsy (n = 30) did not present the olivospinal tract degeneration. Our results indicate an impairment of the neural connection between the inferior olivary nucleus and the spinal cord in MSA patients, which may develop in a descending manner.
摘要：
多系统萎缩（MSA）是一种成人发作的神经退行性疾病，表现为自主神经功能障碍的各种组合，小脑共济失调,帕金森病,和锥体的迹象。下橄榄核以MSA为目标，特别是橄榄桥脑小脑萎缩的表型，和小脑管的参与是众所周知的。然而,在MSA中尚未研究橄榄脊髓束的变性。我们检查了连续尸检MSA患者的97条脊髓。髓磷脂染色显示22根(22.7%)有小,双边,前侧壁边界的三角形束变性，从C1到C5连续出现。退化道的解剖途径与Helweg在1888年提供的橄榄脊髓道的描述一致。显示该道变性的MSA患者在疾病发作时年龄较小（平均：56.4±8.7岁，范围：42-74），病程较长（平均：10.1±4.8年，范围：2-25）和与其他MSA患者相比更严重的橄榄桥脑变化。定量分析显示，与其他患者相比，橄榄脊髓束变性患者的下橄榄核中的神经元密度较低。该束中的小胶质细胞密度与下橄榄核中的神经元密度呈负相关。下橄榄核和橄榄脊髓束中神经胶质细胞质内含物的密度彼此密切相关。神经学健康对照（n=22）和路易体病疾病对照（n=30），肌萎缩侧索硬化症（n=30），进行性核上性麻痹（n=30）没有表现出小肠脊髓束变性。我们的结果表明，MSA患者下橄榄核与脊髓之间的神经连接受损，可能以下降的方式发展。
