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进行性核上性麻痹:神经病理学与临床异质性

Progressive supranuclear palsy: neuropathologic and clinical heterogeneity.

作者信息

Gearing M, Olson D A, Watts R L, Mirra S S

机构信息

Veterans Affairs Medical Center, Emory University School of Medicine, Atlanta, GA.

出版信息

Neurology. 1994 Jun;44(6):1015-24. doi: 10.1212/wnl.44.6.1015.

DOI:10.1212/wnl.44.6.1015
PMID:8208392
Abstract

To investigate potential heterogeneity in progressive supranuclear palsy (PSP), we examined 13 patients with neuropathologically diagnosed PSP. The clinical diagnosis of PSP was made in eight of these individuals, whereas probable AD was the primary diagnosis in the remaining five. In addition to PSP neuropathology, seven of the 13 patients (54%) showed concomitant pathologic changes of Alzheimer's disease (AD), Parkinson's disease (PD), or both disorders. These observations indicate that AD and PD changes coexist with PSP neuropathology in a substantive proportion of patients and provide further evidence of clinical and neuropathologic heterogeneity in neurodegenerative disorders. Moreover, our results suggest that PSP may be underdiagnosed and deserves more prominence in the differential diagnosis of dementing illness.

摘要

为研究进行性核上性麻痹(PSP)潜在的异质性,我们检查了13例经神经病理学诊断为PSP的患者。其中8例患者临床诊断为PSP,而其余5例主要诊断为可能的阿尔茨海默病(AD)。除PSP神经病理学表现外,13例患者中有7例(54%)同时出现阿尔茨海默病(AD)、帕金森病(PD)或两种疾病的病理改变。这些观察结果表明,在相当一部分患者中,AD和PD改变与PSP神经病理学共存,为神经退行性疾病的临床和神经病理学异质性提供了进一步证据。此外,我们的结果表明,PSP可能诊断不足,在痴呆症的鉴别诊断中应得到更多重视。

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Progressive supranuclear palsy: neuropathologic and clinical heterogeneity.进行性核上性麻痹:神经病理学与临床异质性
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Midbrain catecholaminergic neurons co-express α-synuclein and tau in progressive supranuclear palsy.中脑儿茶酚胺能神经元在进行性核上性麻痹中共同表达α-突触核蛋白和tau。
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