Department of Neurology, Memory and Aging Center, University of California, San Francisco, California, USA.
Curr Opin Neurol. 2010 Dec;23(6):633-7. doi: 10.1097/WCO.0b013e32833fb93e.
The aim is to explore the evolution of the logopenic variant of primary progressive aphasia as a distinct clinical entity and to outline recent advances that have clarified its clinical characteristics, neural underpinnings, and potential genetic and pathological bases. This is particularly relevant as researchers attempt to identify clinico-pathological relationships in subtypes of primary progressive aphasia in hopes of utilizing language phenotype as a marker of underlying disease.
Recent work has served to refine and expand upon the clinical phenotype of the logopenic variant. Logopenic patients show a unique pattern of spared and impaired language processes that reliably distinguish this syndrome from other variants of progressive aphasia. Specifically, they exhibit deficits in naming and repetition in the context of spared semantic, syntactic, and motor speech abilities. Further, there is a growing body of evidence indicating a possible link between the logopenic phenotype and specific pathological and genetic correlates.
Findings indicate that the logopenic variant is a distinct subtype of progressive aphasia that may hold value as a predictor of underlying pathology. Additional research, however, is warranted in order to further clarify the cognitive-linguistic profile and to confirm its relation to certain pathological and genetic processes.
目的综述:目的在于探讨以语言流畅性损害为主要表现的原发性进行性失语的演变过程,明确其临床特征、神经基础以及潜在的遗传和病理基础,概述近期的研究进展。这在试图确定原发性进行性失语亚型的临床-病理关系时尤为重要,因为研究人员希望将语言表型作为潜在疾病的标志物。
最新发现:近期的研究工作进一步完善和扩展了以语言流畅性损害为主要表现的原发性进行性失语的临床表型。语言流畅性损害患者表现出独特的语言过程受损和保留模式,这一模式可将该综合征与其他进行性失语变体区分开来。具体而言,他们在语义、句法和运动言语能力保留的情况下表现出命名和复述缺陷。此外,越来越多的证据表明,语言流畅性损害表型与特定的病理和遗传相关性之间可能存在关联。
总结:研究结果表明,语言流畅性损害是进行性失语的一种独特亚型,可能作为潜在病理学的预测指标具有一定价值。然而,需要进一步的研究来进一步阐明其认知-语言特征,并确认其与某些病理和遗传过程的关系。