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皮克病和阿尔茨海默病:一种表现为皮质基底节综合征的罕见共病。

Pick and Alzheimer diseases: a rare comorbidity presenting as corticobasal syndrome.

作者信息

Rusina Robert, Pazdera Ladislav, Kulišťák Petr, Vyšata Oldřich, Matěj Radoslav

机构信息

*Department of Neurology, Thomayer Hospital and Institute for Postgraduate Education in Medicine, Prague, Czech Republic †Department of Neurology and Centre of Clinical Neuroscience, First Faculty of Medicine, Charles University in Prague, and General University Hospital in Prague, Czech Republic ‡Neurocenter Caregroup Rychnov nad Kněžnou, Czech Republic §Department of Neurology, Faculty of Medicine in Hradec Králové, Charles University in Prague, Czech Republic ∥Department of Pathology and Molecular Medicine, Thomayer Hospital, Prague, Czech Republic.

出版信息

Cogn Behav Neurol. 2013 Dec;26(4):189-94. doi: 10.1097/WNN.0000000000000011.

Abstract

We describe a patient with corticobasal syndrome in whom neuropathological examination on autopsy revealed Pick and Alzheimer diseases in comorbidity. Corticobasal degeneration is a tauopathy usually associated with asymmetric parkinsonism, parietal lobe involvement, and cognitive impairment. Corticobasal syndrome is the clinical presentation of corticobasal degeneration without neuropathological confirmation. A 66-year-old right-handed man slowly developed speech difficulties, right-hand clumsiness, and forgetfulness. His speech apraxia progressed to mutism with preserved comprehension, and his clumsiness progressed to severe apraxia involving both hands. He developed behavioral changes and severe amnesia. All of these features were consistent with corticobasal syndrome. His loss of episodic, verbal, and visuospatial memory suggested Alzheimer disease; however, beyond his frontotemporal neuropsychological profile, he had few symptoms characteristic of frontal lobe dementia. Magnetic resonance imaging scans showed worsening temporal, frontal, and parietal atrophy, predominant in the left hemisphere. Neuropathological examination at autopsy revealed abundant neuritic plaques and neurofibrillary tangles consistent with fully developed Alzheimer disease, as well as numerous intraneuronal Pick bodies in the frontotemporal lobes. Our findings confirm the importance of clinical and neuropathological correlations in patients with atypical neurodegenerative dementias.

摘要

我们描述了一名患有皮质基底节综合征的患者,其尸检时的神经病理学检查显示合并有Pick病和阿尔茨海默病。皮质基底节变性是一种tau蛋白病,通常与不对称帕金森综合征、顶叶受累及认知障碍相关。皮质基底节综合征是未经神经病理学证实的皮质基底节变性的临床表现。一名66岁右利手男性逐渐出现言语困难、右手笨拙及健忘。他的言语失用进展为缄默但理解力保留,笨拙进展为累及双手的严重失用。他出现了行为改变和严重失忆。所有这些特征均符合皮质基底节综合征。他情景记忆、言语记忆和视觉空间记忆丧失提示阿尔茨海默病;然而,除了额颞叶神经心理学表现外,他几乎没有额叶痴呆的特征性症状。磁共振成像扫描显示颞叶、额叶和顶叶萎缩加重,以左侧半球为主。尸检时的神经病理学检查显示有大量与完全发展的阿尔茨海默病一致的神经炎性斑块和神经原纤维缠结,以及额颞叶内大量神经元内Pick小体。我们的发现证实了非典型神经退行性痴呆患者临床与神经病理学相关性的重要性。

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