原发性进行性失语症中的双语现象：临床与语言特征的回顾性研究。
Bilingualism in Primary Progressive Aphasia: A Retrospective Study on Clinical and Language Characteristics.
机构信息
Neurocognition Unit, Department of Neurology, Hospital de Braga, Sete Fontes-São Victor, Braga, Portugal.
Department of Neurology, RWTH Aachen University, Pauwelsstr, Aachen.
出版信息
Alzheimer Dis Assoc Disord. 2019 Jan-Mar;33(1):47-53. doi: 10.1097/WAD.0000000000000288.
BACKGROUND
Primary progressive aphasia (PPA) is a neurodegenerative disorder characterized by progressive deterioration of language. Being rare, reports of PPA in multilingual individuals are scarce, despite more than half of the world population being multilingual.
METHODS
We describe clinical characteristics of 33 bilingual patients with PPA, including symptom presentation and language deficits pattern in their first (L1) and second language (L2), through a systematic literature review and new cases retrospectively identified in 5 countries.
RESULTS
In total, 14 patients presented with nonfluent/agrammatic variant, 6 with semantic variant, and 13 with logopenic variant, with a median symptom onset of 2 years. Word-finding difficulties was the first symptom in 65% of all cases, initially noticed in L2, and not always the dominant language. Our group had 22 different languages as L1, and 9 as L2. At the whole-group level there was a tendency for parallel impairment in both languages, in line with the shared bilingual neural substrate hypothesis, but each PPA variant showed some heterogeneity.
DISCUSSION
Each PPA variant showed heterogeneity, showing the need for comprehensive language and cognitive assessment across languages, as well as further clarification on the role of language mediators.
背景
原发性进行性失语症（PPA）是一种以语言逐渐恶化为特征的神经退行性疾病。尽管世界上超过一半的人口是双语或多语者，但由于 PPA 较为罕见，因此在多语言个体中报告的 PPA 病例很少。
方法
我们通过系统的文献回顾和在 5 个国家回顾性确定的新病例，描述了 33 名双语 PPA 患者的临床特征，包括他们的第一语言（L1）和第二语言（L2）中的症状表现和语言缺陷模式。
结果
共有 14 名患者表现为非流利/语法障碍型，6 名表现为语义障碍型，13 名表现为失语法型，中位症状起始时间为 2 年。在所有病例中，65%的患者最初出现找词困难的症状，首先在 L2 中发现，且并不总是主导语言。我们的患者组有 22 种不同的语言作为 L1，9 种作为 L2。从整个组的水平来看，两种语言都存在平行的损伤倾向，这与共享的双语神经基质假说一致，但每种 PPA 变体都表现出一些异质性。
讨论
每个 PPA 变体都表现出异质性，这表明需要在跨语言中进行全面的语言和认知评估，并进一步阐明语言中介的作用。
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