Benito-León Julián
Department of Neurology, University Hospital "12 de Octubre", Madrid, Spain ; Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Madrid, Spain ; Department of Medicine, Complutense University, Madrid, Spain.
Tremor Other Hyperkinet Mov (N Y). 2014 Jul 22;4:252. doi: 10.7916/D8765CG0. eCollection 2014.
Essential tremor (ET) is one of the most common neurological disorders among adults, and is the most common of the many tremor disorders. It has classically been viewed as a benign monosymptomatic condition, yet over the past decade, a growing body of evidence indicates that ET is a progressive condition that is clinically heterogeneous, as it may be associated with a spectrum of clinical features, with both motor and non-motor elements. In this review, I will describe the most significant emerging milestones in research which, when taken together, suggest that ET is a neurodegenerative condition.
A PubMed search conducted in June 2014 crossing the terms "essential tremor" (ET) and "neurodegenerative" yielded 122 entries, 20 of which included the term "neurodegenerative" in the article title. This was supplemented by articles in the author's files that pertained to this topic.
RESULTS/DISCUSSION: There is an open and active dialogue in the medical community as to whether ET is a neurodegenerative disease, with considerable evidence in favor of this. Specifically, ET is a progressive disorder of aging associated with neuronal loss (reduction in Purkinje cells) as well as other post-mortem changes that occur in traditional neurodegenerative disorders. Along with this, advanced neuroimaging techniques are now demonstrating distinct structural changes, several of which are consistent with neuronal loss, in patients with ET. However, further longitudinal clinical and neuroimaging longitudinal studies to assess progression are required.
特发性震颤(ET)是成年人中最常见的神经系统疾病之一,也是众多震颤疾病中最常见的一种。传统上,它被视为一种良性单症状疾病,但在过去十年中,越来越多的证据表明,ET是一种进行性疾病,在临床上具有异质性,因为它可能与一系列临床特征相关,包括运动和非运动因素。在这篇综述中,我将描述研究中最重要的新进展,这些进展综合起来表明ET是一种神经退行性疾病。
2014年6月在PubMed上进行的一次搜索,将“特发性震颤”(ET)和“神经退行性”这两个术语交叉检索,得到122条记录,其中20条在文章标题中包含“神经退行性”一词。作者文件中与该主题相关的文章对其进行了补充。
结果/讨论:医学界就是否将ET视为神经退行性疾病展开了开放且活跃的讨论,有大量证据支持这一观点。具体而言,ET是一种与衰老相关的进行性疾病,伴有神经元丢失(浦肯野细胞减少)以及传统神经退行性疾病中出现的其他尸检变化。与此同时,先进的神经影像学技术现在显示,ET患者存在明显的结构变化,其中一些与神经元丢失一致。然而,需要进一步进行纵向临床和神经影像学纵向研究来评估疾病进展。