Louis Elan D
GH Sergievsky Center, College of Physicians and Surgeons, Columbia University, New York, N.Y., USA.
Neuroepidemiology. 2014;42(2):81-9. doi: 10.1159/000356351. Epub 2013 Dec 3.
There is accumulating evidence that the entity referred to as 'essential tremor' (ET) is not a single disease. It may be a family of diseases better referred to as 'the ETs'. This review will summarize the following evidence: (1) the presence of etiological heterogeneity; (2) the heterogeneity of findings in postmortem studies, thus suggesting several diseases; (3) the recent discussion that age of onset may be an important marker of disease heterogeneity; (4) the clinical expansion of the concept of ET in recent years to include a broader range of tremor phenomenology, other motor features (gait ataxia), other involuntary movements (dystonia), and nonmotor features (cognitive problems, psychiatric problems), some of which could be primary; (5) the heterogeneity of pharmacological response profiles and clinical progression, and (6) the association of ET with Parkinson's disease, Alzheimer's disease, and possibly progressive supranuclear palsy, with the possibility that some ET patients are more predisposed to develop one of these. © 2013 S. Karger AG, Basel.
越来越多的证据表明,被称为“特发性震颤”(ET)的病症并非单一疾病。它可能是一类疾病,更宜称作“多种特发性震颤”。本综述将总结以下证据:(1)病因异质性的存在;(2)尸检研究结果的异质性,从而提示存在多种疾病;(3)近期关于发病年龄可能是疾病异质性重要标志物的讨论;(4)近年来ET概念在临床上的扩展,涵盖更广泛的震颤现象学、其他运动特征(步态共济失调)、其他不自主运动(肌张力障碍)以及非运动特征(认知问题、精神问题),其中一些可能是原发性的;(5)药物反应谱和临床进展的异质性,以及(6)ET与帕金森病、阿尔茨海默病以及可能的进行性核上性麻痹的关联,部分ET患者可能更易患其中之一。© 2013 S. Karger AG,巴塞尔。