原发性进行性失用症:一种不寻常的意识形态运动综合征。

Journal of Clinical Movement Disorders Pub Date : 2017-11-14 eCollection Date: 2017-01-01 DOI:10.1186/s40734-017-0064-0
Yeva M Fernandez, Steven J Frucht
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引用次数: 2

摘要

背景:原发性进行性失用症是一种罕见的无痴呆的失用症,其发展隐匿且进展缓慢。大多数失用症患者的报告还描述了共存的失语症或涉及额外的失用症和言语影响,通常在神经退行性疾病的背景下,如皮质基底变性、阿尔茨海默病或额颞叶痴呆。本报告的目的是描述和视频演示两个孤立的原发性进行性意识形态运动失用症在我们的诊所看到。病例介绍:我们描述了两例2-5年进行性使用双手困难的患者,尽管他们有完整的认知和影像学上缺乏相关病变。结论:我们报告了两例原发性进行性失用症,这两例患者可能都是tau病变的早期表现。在这两种情况下,都存在孤立的手部深度意识运动失用症,保留了认知、语言技能、肌肉力量和音调以及步态。影像学上未见相关病变。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Primary progressive apraxia: an unusual ideomotor syndrome.

Background: Primary progressive apraxia is a rare form of apraxia in the absence of dementia which develops insidiously and is slowly progressive. Most reports of patients with apraxia also describe coexisting aphasias or involve additional apraxias with affected speech, usually in the setting of neurodegenerative diseases such as corticobasal degeneration, Alzheimer's disease or frontotemporal dementia. The aim of this report is to describe and demonstrate by video two cases of isolated primary progressive ideomotor apraxia seen in our clinic.

Case presentation: We describe two patients with 2-5 years of progressive difficulty using their hands, despite having intact cognition and lack of correlating lesions on imaging.

Conclusion: We report two cases of primary progressive apraxia that may be early presentations of taupathic disease in both patients. In both cases, there is isolated profound ideomotor apraxia of the hands, with preserved cognition, language skills, muscle power and tone, and gait. There are no correlating lesions on imaging.

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