Cerebellar Involvement in Progressive Supranuclear Palsy: A Clinicopathological Study
Cerebellar Involvement in Progressive Supranuclear Palsy: A Clinicopathological Study
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DOI：
10.1002/mds.22583
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发表时间：
2009-07-15
影响因子：
8.6
通讯作者：
Nishizawa, Masatoyo
中科院分区：
文献类型：
--
作者：
Kanazawa, Masato;Shimohata, Takayoshi;Nishizawa, Masatoyo
进行性核上性麻痹（PSP）分为经典的理查森综合征（RS）和PSP-帕金森病（PSP- p），其临床异质性已被讨论。我们回顾性分析了22例经病理证实的日本PSP患者，以探讨其临床病理异质性。我们调查了早期和疾病过程中的任何时间的临床特征。并对神经胶质瘤和tau病理的神经元丧失的病理程度进行了评价。根据临床特征，10例为RS， 8例为PSP-P。4例出现小脑性共济失调或大脑皮层体征的患者被归类为无法分类的PSP。其中3例以小脑性共济失调为首发和主要症状。值得注意的是，在小脑性共济失调患者中，浦肯野细胞中tau阳性包涵体的出现频率明显高于非小脑性共济失调患者。所有小脑性共济失调患者均表现出更多的神经元丢失和胶质瘤，且小脑齿状核内的盘绕体密度高于非小脑性共济失调患者。本研究证实了与PSP相关的广泛临床病理表现，而不受不同种族的影响，并表明PSP患者表现为小脑性共济失调。(C) 2009运动障碍学会
The clinical heterogeneity of progressive supranuclear palsy (PSP), which is classified as classic Richardson's syndrome (RS) and PSP-Parkinsonism (PSP-P), has been previously discussed. We retrospectively analyzed 22 consecutive Japanese patients with pathologically proven PSP to investigate the clinicopathological heterogeneity. We investigated the clinical features both early in and at any time during the disease course. The pathological severities of neuronal loss with gliosis and tau pathology were also evaluated. On the basis of the clinical features, 10 patients were categorized as having RS, and 8 were categorized as having PSP-P. Four patients presenting with cerebellar ataxia or cerebral cortical signs were categorized as having unclassifiable PSP. Among them, 3 developed cerebellar ataxia as the initial and principal symptom. Notably, tau-positive inclusion bodies in Purkinje cells were significantly more frequently observed in the patients with cerebellar ataxia than in those without cerebellar ataxia. All the patients with cerebellar ataxia exhibited more neuronal loss with gliosis and higher densities of coiled bodies in the cerebellar dentate nucleus than those without cerebellar ataxia. This study confirms the wide spectrum of clinicopathological manifestations associated with PSP regardless of different ethnic origin, and demonstrates that PSP patients manifest cerebellar ataxia. (C) 2009 Movement Disorder Society