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运动神经元病-额颞叶痴呆:一种临床连续体。

Motor neuron disease-frontotemporal dementia: a clinical continuum.

作者信息

Devenney Emma, Vucic Steve, Hodges John R, Kiernan Matthew C

机构信息

Neuroscience Research Australia, Sydney, NSW, Australia.

出版信息

Expert Rev Neurother. 2015 May;15(5):509-22. doi: 10.1586/14737175.2015.1034108. Epub 2015 Apr 11.

Abstract

Overlap between motor neuron disease (MND) and frontotemporal dementia (FTD) occurs at clinical, genetic and pathological levels, and has been recently strengthened through the discovery of the C9orf72 genetic expansion. MND is now considered to be a multisystem disorder in which cognitive involvement may be present and, in some cases, may evolve to frank FTD. Identifying cognitive features in MND can be challenging, while, similarly, motor dysfunction in FTD may be overlooked. As such, the present review aims to decipher the variety of overlapping clinical features across the MND-FTD continuum.

摘要

运动神经元病(MND)和额颞叶痴呆(FTD)在临床、遗传和病理层面均存在重叠,并且最近通过对C9orf72基因扩增的发现,这种重叠关系得到了进一步强化。MND现在被认为是一种多系统疾病,其中可能存在认知功能受累情况,在某些病例中,还可能发展为明显的FTD。识别MND中的认知特征具有挑战性,同样,FTD中的运动功能障碍也可能被忽视。因此,本综述旨在解读MND - FTD连续体中各种重叠的临床特征。

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