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特发性震颤的演变定义:我们在应对什么?

The evolving definition of essential tremor: What are we dealing with?

机构信息

Department of Neurology, Yale School of Medicine, Yale University, New Haven, CT, USA; Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, CT, USA; Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, Yale University, New Haven, CT, USA.

出版信息

Parkinsonism Relat Disord. 2018 Jan;46 Suppl 1(Suppl 1):S87-S91. doi: 10.1016/j.parkreldis.2017.07.004. Epub 2017 Jul 8.

Abstract

BACKGROUND

Although essential tremor (ET) is commonly encountered in clinical practice, historically, there has been considerable disagreement as how to best define it, and now with a growing sense of its clinical complexity, how to best encapsulate it. Here, I draw attention to five issues of current uncertainty.

METHODS

A PubMed search conducted on June 19, 2017 crossed "essential tremor" with 9 second search terms (e.g., definition, diagnosis).

RESULTS

There are several major issues of clinical and diagnostic uncertainty. Underlying each issue is a larger question about the nature of the underlying pathophysiology of ET. Does age of onset of ET matter? How much dystonia is acceptable in ET? How much in the way of "cerebellar signs" are acceptable? Are non-motor features due to the underlying disease or merely secondary to the clinical features? Is ET a single disease entity or something else?

CONCLUSIONS

We are learning more about ET and, as a by-product of these efforts, are struggling with its definition. Further understanding the nature of the underlying disease pathogenesis as well as the role the cerebellum and cerebellar relays play in this process will likely provide important clues to enable us to bring order to areas of uncertainty.

摘要

背景

尽管特发性震颤(ET)在临床实践中很常见,但历史上,如何最好地定义它存在相当大的分歧,而现在随着对其临床复杂性的认识不断提高,如何最好地描述它也存在不确定性。在这里,我提请注意当前存在的五个不确定性问题。

方法

2017 年 6 月 19 日,通过在 PubMed 上进行搜索,将“essential tremor”与 9 个搜索词(例如,定义、诊断)进行交叉搜索。

结果

存在几个主要的临床和诊断不确定性问题。每个问题的背后都存在一个更大的问题,即特发性震颤潜在病理生理学的本质。特发性震颤的发病年龄是否重要?特发性震颤中可以接受多少肌张力障碍?可以接受多少“小脑体征”?非运动症状是由潜在疾病引起的,还是仅仅是由临床症状引起的?特发性震颤是一种单一的疾病实体还是其他什么?

结论

我们对特发性震颤有了更多的了解,并且作为这些努力的副产品,我们正在努力定义它。进一步了解潜在疾病发病机制的本质,以及小脑及其中继在这一过程中所起的作用,可能会为我们提供重要的线索,使我们能够解决不确定性领域的问题。

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