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首页> 外文期刊>Amyotrophic lateral sclerosis eofficial publication of the World Federation of Neurology Research Group on Motor Neuron Diseases >The syndromes of frontotemporal dysfunction in amyotrophiclateral sclerosis
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The syndromes of frontotemporal dysfunction in amyotrophiclateral sclerosis

机译：肌萎缩性侧索硬化症的额颞功能异常的症状

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摘要

Amyotrophic lateral sclerosis is increasingly recognized to be a complex multisystems disorder both at the level of itspathobiology and in the breadth of non-motor manifestations that can accompany it. Paramount among these are disordersof frontotemporal function which can be associated with syndromes of behavioural, cognitive or executive dysfunction ormanifest as a frontotemporal dementia (FTD). While these may occur in isolation and precede the development of motordeficits, more commonly they insidiously onset following the initial neuromuscular dysfunction. The earliest clinicalmanifestation is a loss of verbal fluency, disproportionate to impairments in oromotor control. There is good correlationbetween the presence of a syndrome of frontotemporal dysfunction and alterations in brain structure or function asidentified with a wide variety of neuroimaging techniques and which reflect a frontotemporal lobar degeneration (FTLD).Although the cause(s) of this process remain to be defined, as with the clinical heterogeneity, there is likely to be significantbiochemical heterogeneity. This includes alterations in tau protein metabolism which are present in a proportion of familialand sporadic ALS cases, as well as the western Pacific variant, and recently described alterations in the metabolism of theTAR DNA binding protein 43 (TDP-43).
机译：肌萎缩性侧索硬化症在其病理生物学水平和伴随其的非运动表现的广度上都日益被认为是一种复杂的多系统疾病。其中最重要的是额颞功能障碍，它可能与行为，认知或执行功能障碍综合征或表现为额颞痴呆（FTD）有关。尽管这些可能孤立发生并在运动障碍发展之前发生，但更常见的是，它们会在最初的神经肌肉功能障碍后隐匿发作。最早的临床表现是口语流利性的丧失，与口语运动控制的损害不成比例。额颞功能不全综合征的存在与脑结构或功能的变化之间存在良好的相关性，这些变化已被多种神经影像学技术鉴定并反映了额颞叶变性（FTLD）。定义为与临床异质性一样，可能存在显着的生化异质性。这包括在一部分家族性ALS病例以及Western Pacific变体中存在的tau蛋白代谢改变，并且最近描述了TAR DNA结合蛋白43（TDP-43）的代谢改变。

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