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小脑功能障碍患者的元语言缺陷:思维失调理论的实证支持。

Metalinguistic deficits in patients with cerebellar dysfunction: empirical support for the dysmetria of thought theory.

作者信息

Guell Xavier, Hoche Franziska, Schmahmann Jeremy D

机构信息

Ataxia Unit, Cognitive Behavioral Neurology Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Suite 340, Charles River Plaza South, 175 Cambridge Street, Boston, MA, 02114, USA.

出版信息

Cerebellum. 2015 Feb;14(1):50-8. doi: 10.1007/s12311-014-0630-z.

Abstract

The cerebellar cognitive affective syndrome (CCAS) includes disruption of linguistic processing such as verbal fluency, verbal working memory, grammar, and speech perception. We set out to examine linguistic capabilities in patients with cerebellar lesions to determine which domains are spared and which impaired and to evaluate the underlying cognitive structure of these deficits. Forty-four patients with cerebellar disease were compared to 40 healthy controls on the Oral Sentence Production Test (OSPT) which assesses production of sentences with correct syntactic structure and semantic quality. Twenty-five of these cerebellar patients and 25 controls received the Test of Language Competence-Expanded (TLC-E) that assesses metalinguistic ability. The OSPT failed to reveal differences between patients and controls. In contrast, all cerebellar patients were impaired on each of the four TLC-E subtests. Differences between isolated cerebellar and complex cerebrocerebellar patients were nonsignificant. These results confirm and extend prior observations of the TLC-E in patients with cerebellar lesions and suggest three separate but related language impairments following cerebellar dysfunction: (1) disruption in automatic adjustment of intact grammatical and semantic abilities to a linguistic context in sentence production, (2) disruption in automatic adjustment to a linguistic context in sentence interpretation, and (3) disruption of cognitive processes essential for linguistic skills, such as analysis and sequential logical reasoning. These findings are consistent with the unifying framework of the universal cerebellar transform and the dysmetria of thought theory and provide new insights into the nature of the cognitive impairments in patients with the CCAS.

摘要

小脑认知情感综合征(CCAS)包括语言加工障碍,如言语流畅性、言语工作记忆、语法和言语感知。我们着手研究小脑病变患者的语言能力,以确定哪些领域未受影响,哪些领域受损,并评估这些缺陷的潜在认知结构。在口语句子生成测试(OSPT)中,将44例小脑疾病患者与40名健康对照者进行比较,该测试评估具有正确句法结构和语义质量的句子的生成情况。其中25例小脑患者和25名对照者接受了语言能力扩展测试(TLC-E),该测试评估元语言能力。OSPT未能揭示患者与对照者之间的差异。相比之下,所有小脑患者在TLC-E的四个子测试中的每一个上都表现受损。孤立性小脑病变患者与复杂性脑-小脑病变患者之间的差异不显著。这些结果证实并扩展了先前对小脑病变患者TLC-E的观察结果,并表明小脑功能障碍后存在三种独立但相关的语言障碍:(1)在句子生成中,完整的语法和语义能力自动调整到语言语境时出现障碍;(2)在句子解释中,自动调整到语言语境时出现障碍;(3)对语言技能至关重要的认知过程,如分析和顺序逻辑推理出现障碍。这些发现与通用小脑转换和思维失调理论的统一框架一致,并为CCAS患者认知障碍的本质提供了新的见解。

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