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一例导致皮质基底节综合征的言语失用症患者中发现的一种新的微管相关蛋白tau(MAPT)基因缺失。

A new MAPT deletion in a case of speech apraxia leading to corticobasal syndrome.

作者信息

Mazzon Giulia, Menichelli Alina, Fabretto Antonella, Cattaruzza Tatiana, Manganotti Paolo

机构信息

a Neurological Clinic, Department of Medical, Surgical and Health Sciences , University of Trieste , Trieste , Italy.

b Neuropsychology Unit, Department of Rehabilitation Medicine , University of Trieste , Trieste , Italy.

出版信息

Neurocase. 2018 Jun;24(3):140-144. doi: 10.1080/13554794.2018.1492729. Epub 2018 Jul 3.

Abstract

Speech apraxia is a disorder of speech motor planning/programming leading to slow rate, articulatory distortion, and distorted sound substitutions. We describe the clinical profile evolution of a patient presenting with slowly progressive isolated speech apraxia that eventually led to the diagnosis of corticobasal syndrome (CBS), supporting the evidence that this rare speech disorder can be the first presentation of CBS. Moreover, we found a novel variant in MAPT gene, which is hypothesized to be disease-causing mutation. These results underscore the importance of genetic analysis - particularly in selected atypical cases - for in vivo understanding of possible pathophysiological disease process.

摘要

言语失用症是一种言语运动计划/编程障碍,导致语速缓慢、发音扭曲和声音替代异常。我们描述了一名表现为缓慢进展性孤立性言语失用症患者的临床特征演变,该患者最终被诊断为皮质基底节综合征(CBS),这支持了这种罕见的言语障碍可能是CBS首发表现的证据。此外,我们在微管相关蛋白tau(MAPT)基因中发现了一个新的变异体,推测其为致病突变。这些结果强调了基因分析的重要性,特别是在选定的非典型病例中,有助于从体内了解可能的病理生理疾病过程。

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