表现为非流畅性/语法缺失型原发性进行性失语的皮质基底节变性：一例报告
Corticobasal Degeneration Presenting as Non-Fluent/Agrammatic Primary Progressive Aphasia: A Case Report.
作者信息
Kim Ji Sun, Lee Geum Bong, Hong Yun Jeong, Park Kyung Won
机构信息
Department of Neurology, Cognitive Disorders and Dementia Center, College of Medicine, Dong-A University, Busan, Korea.
出版信息
Dement Neurocogn Disord. 2016 Jun;15(2):55-58. doi: 10.12779/dnd.2016.15.2.55. Epub 2016 Jun 30.
BACKGROUND
Non-fluent agrammatic primary progressive aphasia (naPPA) is characterized by progressive non-fluent speech disorder and might be associated with taupathy such as corticobasal degeneration (CBD) and progressive supranuclear palsy. We report a case of overlap syndrome presented with language impairment, and diagnosed as naPPA with possible CBD.
CASE REPORT
A 58-year-old woman visited a memory and dementia clinic, with a 10-month history of progressive language disturbance. She was diagnosed as naPPA and overlapping CBD, based on the clinical features and neuroimaging findings including florbetaben PET.
CONCLUSIONS
naPPA is pathologically caused by taupathy, and might progress to asymmetrical parkinsonism and apraxia, suggestive of CBD. Overlapping clinical features in our case represent various phenotypes of taupathy.
背景
非流畅性语法缺失型原发性进行性失语（naPPA）的特征为进行性非流畅性言语障碍，可能与tau蛋白病相关，如皮质基底节变性（CBD）和进行性核上性麻痹。我们报告1例以语言障碍为表现的重叠综合征病例，诊断为可能合并CBD的naPPA。
病例报告
一名58岁女性因进行性语言障碍10个月就诊于记忆与痴呆门诊。根据临床特征及包括氟比他班PET在内的神经影像学检查结果，她被诊断为naPPA并合并CBD。
结论
naPPA在病理上由tau蛋白病引起，可能进展为不对称性帕金森综合征和失用症，提示CBD。我们病例中的重叠临床特征代表了tau蛋白病的各种表型。
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