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病例报告:对一名原发性进行性失语症语言迟缓型变体的中国随访病例进行的神经语言学和神经影像学研究。

Case Report: A neurolinguistic and neuroimaging study on a Chinese follow-up case with logopenic-variant of primary progressive aphasia.

作者信息

Huang Binyao, Wang Xiaolu, Jiang Biao, Kong Linlin, Hou Haifeng, Zhou Jiong

机构信息

School of Foreign Languages, Zhejiang University of Finance and Economics, Hangzhou, China.

School of Foreign Languages, Zhejiang University City College, Hangzhou, China.

出版信息

Front Neurol. 2022 Sep 20;13:963970. doi: 10.3389/fneur.2022.963970. eCollection 2022.

DOI:10.3389/fneur.2022.963970
PMID:36203977
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9530806/
Abstract

Primary progressive aphasia (PPA), typically resulting from a neurodegenerative disease, is characterized by a progressive loss of specific language functions while other cognitive domains are relatively unaffected. The logopenic variant, characterized by impairments of word retrieval and sentence repetition along with preserved semantic, syntactic, and motor speech abilities, is the most recently described and remains less understood than other variants due to a comparatively small number of case studies and a lack of investigations with a thorough specification. In this article, we report a 2-year follow-up case study of a 74-year-old Chinese female patient with a logopenic variant of primary progressive aphasia, including its neurolinguistic study, magnetic resonance imaging (MRI), and 11C-Pittsburgh compound B-Positron emission tomography imaging analyses, as well as gene sequencing. This case confirms that, in addition to word-finding and sentence repetition difficulties, the logopenic variant may also present with mild auditory comprehension and naming deficits attributed to impaired access to lexical representations. The observation of clinical treatment suggests the efficacy of memantine hydrochloride tablet and rivastigmine transdermal patch in slowing down the cognitive deterioration of this patient. The description and exploration of this case may shed new insights into a better understanding of the Chinese logopenic variant of primary progressive aphasia.

摘要

原发性进行性失语(PPA)通常由神经退行性疾病引起,其特征是特定语言功能逐渐丧失,而其他认知领域相对未受影响。语音性变异型原发性进行性失语的特征是词汇检索和句子复述受损,同时语义、句法和言语运动能力保留,这是最近描述的一种类型,由于病例研究数量相对较少且缺乏全面详细的调查,与其他变异型相比,人们对它的了解仍然较少。在本文中,我们报告了一名74岁中国女性语音性变异型原发性进行性失语患者的2年随访病例研究,包括神经语言学研究、磁共振成像(MRI)、11C-匹兹堡化合物B-正电子发射断层扫描成像分析以及基因测序。该病例证实,除了找词和句子复述困难外,语音性变异型原发性进行性失语还可能表现为轻度听觉理解和命名缺陷,这归因于词汇表征获取受损。临床治疗观察表明,盐酸美金刚片和卡巴拉汀透皮贴剂在减缓该患者认知衰退方面具有疗效。该病例的描述和探索可能为更好地理解中国语音性变异型原发性进行性失语提供新的见解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/babe/9530806/84a5eaf0b4f7/fneur-13-963970-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/babe/9530806/ec2f3c8eec47/fneur-13-963970-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/babe/9530806/84a5eaf0b4f7/fneur-13-963970-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/babe/9530806/ec2f3c8eec47/fneur-13-963970-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/babe/9530806/84a5eaf0b4f7/fneur-13-963970-g0002.jpg

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本文引用的文献

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A Longitudinal Study of a Chinese Man Presenting with Non-Fluent/Agrammatic Variant of Primary Progressive Aphasia.一名表现为非流畅性/语法缺失型原发性进行性失语的中国男性的纵向研究。
Front Neurol. 2018 Feb 16;9:75. doi: 10.3389/fneur.2018.00075. eCollection 2018.
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Cholinergic depletion and basal forebrain volume in primary progressive aphasia.原发性进行性失语中的胆碱能耗竭与基底前脑体积
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Preclinical Alzheimer's disease: Definition, natural history, and diagnostic criteria.
临床前阿尔茨海默病:定义、自然史及诊断标准。
Alzheimers Dement. 2016 Mar;12(3):292-323. doi: 10.1016/j.jalz.2016.02.002.
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Is the logopenic-variant of primary progressive aphasia a unitary disorder?原发性进行性失语的言语流畅性变异型是一种单一的疾病吗?
Cortex. 2015 Jun;67:122-33. doi: 10.1016/j.cortex.2015.03.011. Epub 2015 Apr 1.
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Deciphering logopenic primary progressive aphasia: a clinical, imaging and biomarker investigation.破解失读性原发性进行性失语症:一项临床、影像和生物标志物研究。
Brain. 2013 Nov;136(Pt 11):3474-88. doi: 10.1093/brain/awt266. Epub 2013 Oct 8.
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Clinic, neuropathology and molecular genetics of frontotemporal dementia: a mini-review.额颞叶痴呆的临床、神经病理学和分子遗传学:综述。
Transl Neurodegener. 2013 Apr 19;2(1):8. doi: 10.1186/2047-9158-2-8.
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