Department of Neurology, Baylor College of Medicine, Houston, TX, USA.
Department of Neurology, University of Texas Southwestern, Dallas, TX, USA.
Tremor Other Hyperkinet Mov (N Y). 2023 Nov 7;13:43. doi: 10.5334/tohm.812. eCollection 2023.
Essential tremor (ET) is a chronic, progressive neurological disease that may negatively affect patients' lives. While there has been considerable progress in ET research, some fundamental issues remain unaddressed. One such issue is . Staging schemes have inherent value and are part of the dialogue that clinicians have with other movement disorders patients. We highlight the value of and challenges with developing a staging system for ET and organize a discussion around the potential steps in developing such a system. Diseases for which there are staging schemes generally have a number of shared characteristics. ET has numerous features that would lend themselves to a staging scheme: emerging evidence supporting the existence of a premotor phase of disease, insidious onset, progressive worsening of arm tremor, spread of tremor to other body regions, the observation that patients seem to be at increased risk for other conditions within the same organ (i.e., emergence of Parkinson's disease and Alzheimer's disease in excessive numbers of ET patients), pathological changes in the cerebellum whose evolution can be ordered from (i) those that compromise the physical integrity and physiological function of Purkinje cells, (ii) subsequent changes that are reparative and regenerative, and (iii) eventual cell death. Challenges to formulating a staging scheme are the absence of both a biological marker and an "end stage" of disease. The sum of combined evidence suggests that a staging scheme would be of value. We provide initial thoughts as to how to begin to structure such a staging scheme.
特发性震颤（essential tremor，ET）是一种慢性进行性神经系统疾病，可能会对患者的生活产生负面影响。尽管 ET 研究已经取得了相当大的进展，但仍存在一些未解决的基本问题。其中一个问题是……分期方案具有内在价值，是临床医生与其他运动障碍患者对话的一部分。我们强调了为 ET 开发分期系统的价值和挑战，并围绕开发此类系统的潜在步骤组织了讨论。通常情况下，有分期方案的疾病具有一些共同特征。ET 有许多特征可以为分期方案提供依据：越来越多的证据支持疾病存在运动前期，起病隐匿，手臂震颤逐渐恶化，震颤向其他身体部位扩散，观察到患者似乎在同一器官内发生其他疾病的风险增加（即，帕金森病和阿尔茨海默病在 ET 患者中大量出现），小脑的病理变化可以从（i）损害浦肯野细胞的物理完整性和生理功能，（ii）随后的修复和再生变化，以及（iii）最终的细胞死亡，进行有序排列。制定分期方案的挑战在于缺乏生物标志物和疾病的“终末期”。综合证据表明，分期方案将具有价值。我们提供了一些初步的思路，以探讨如何开始构建这样的分期方案。
