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[一例以显著早发性失用症为特征的皮质基底节变性尸检病例:病例报告及以失用症为重点的文献综述]

[An autopsy case of corticobasal degeneration with notable early onset apraxia: a case report and literature review focused on apraxia].

作者信息

Homma Taku, Bandoh Mitsuaki, Mochizuki Yoko, Miura Naoaki, Okiyama Ryoichi, Matsubara Shiro, Mizutani Toshio

机构信息

Department of Pathology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan.

出版信息

Brain Nerve. 2013 Jul;65(7):887-93.

Abstract

We report the autopsy case of a 74-year-old woman. Onset of gait disturbance and left-side dominant bilateral motor disturbance in the patient led to bilateral progressive apraxia. This was associated with a decline in motor imagery, right-side dominant atrophy of the central sulcus region, and a decrease in cerebral blood flow during illness. She died of respiratory failure that had progressively worsened over a 9-year period. Pathologically, she exhibited right-side dominant cerebral atrophy; neuronal loss, gliosis, and astrocytic plaques were mainly present in the frontal lobe. She was subsequently diagnosed with corticobasal degeneration (CBD). The premotor and primary motor areas revealed marked degeneration; in addition, severe myelin pallor was observed in these regions, and it was suggested that such pathological features were responsible for the apraxia. We believe the present case is valuable since very few reports have provided a detailed description of clinicopathological apraxia in association with CBD.

摘要

我们报告一例74岁女性的尸检病例。患者出现步态障碍和左侧优势的双侧运动障碍,导致双侧进行性失用症。这与运动想象能力下降、中央沟区域右侧优势萎缩以及患病期间脑血流量减少有关。她死于呼吸衰竭,该症状在9年期间逐渐恶化。病理检查显示,她存在右侧优势的脑萎缩;神经元丢失、胶质增生和星形细胞斑块主要出现在额叶。她随后被诊断为皮质基底节变性(CBD)。运动前区和初级运动区显示出明显的变性;此外,在这些区域观察到严重的髓鞘苍白,提示这些病理特征是失用症的原因。我们认为本病例很有价值,因为很少有报告详细描述与CBD相关的临床病理失用症。

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