An autopsy case of early ("minimal change") olivopontocerebellar atrophy (multiple system atrophy-cerebellar)
An autopsy case of early ("minimal change") olivopontocerebellar atrophy (multiple system atrophy-cerebellar)
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DOI：
10.1007/s00401-005-1029-1
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发表时间：
2005-08-01
影响因子：
12.7
通讯作者：
Kuroda, N
中科院分区：
文献类型：
--
作者：
Wakabayashi, K;Mori, F;Kuroda, N
我们报告一位57岁的女性多系统萎缩（MSA）的15个月的持续时间。患者出现构音障碍，随后步态平衡受损，轻度肢体共济失调和扫视性眼球运动。在她被发现死在浴缸里后进行的尸检显示，神经元损失仅限于橄榄脑桥小脑系统，在脑桥核中更为严重。在前蚓部和下橄榄核也发现轻度神经元丢失。α-突触核蛋白免疫组化显示在中枢神经系统中广泛存在胶质细胞胞质内含物，其在脑桥基底和小脑白色物质中更多。相反，神经元α-突触核蛋白的积累仅限于脑桥和下橄榄核。神经元核内包涵体的数量远高于神经元胞质包涵体。此外，α-突触核蛋白的积累是更严重的神经突比在细胞质或细胞核。本例显示了脑病理学的早期模式在MSA-小脑（橄榄脑桥小脑萎缩）。
We report a 57-year-old woman with multiple system atrophy (MSA) of 15-month duration. The patient developed dysarthria, followed by impaired balance of gait, mild limb ataxia, and saccadic eye movement. A postmortem examination performed after she was found dead in a bathtub revealed neuronal loss restricted to the olivopontocerebellar system, being more severe in the pontine nucleus. Mild neuronal loss was also found in the anterior vermis and inferior olivary nucleus. alpha-Synuclein immunohistochemistry demonstrated widespread occurrence of glial cytoplasmic inclusions in the central nervous system, which were more numerous in the pontine base and cerebellar white matter. In contrast, neuronal alpha-synuclein accumulation was confined to the pontine and inferior olivary nuclei. The number of neuronal intranuclear inclusions was much higher than that of neuronal cytoplasmic inclusions. Moreover, alpha-synuclein accumulation was more severe in the neurites than in the cytoplasm or nucleus. This case demonstrates the early pattern of brain pathology in MSA-cerebellar (olivopontocerebellar atrophy).