Clinical subtypes of essential tremor
Clinical subtypes of essential tremor
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DOI：
10.1001/archneur.57.8.1194
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发表时间：
2000-08-01
影响因子：
--
通讯作者：
Barnes, LF
中科院分区：
文献类型：
--
作者：
Louis, ED;Ford, B;Barnes, LF
背景：特发性震颤 (ET) 存在临床变异，但尚不清楚这种变异是否是由于存在不同的 ET 临床亚型（即，病因、进展速度或治疗反应可能不同的 ET 形式）。目的：检查一组 ET 病例的震颤发病年龄、解剖分布和进展速度，并寻找这些因素之间的关联。方法：确定 ET 病例来自纽约州曼哈顿北部的一个社区 (n=60) 和一个三级转诊诊所 (n=55)。所有受试者均接受采访并进行录像震颤检查。根据评估时震颤的严重程度和报告的疾病持续时间来估计进展率。结果：临床病例中的发病年龄呈双峰分布。震颤的解剖分布存在差异（仅手臂震颤与头部震颤以及手臂震颤与孤立的头部震颤）。进展率呈指数分布；有一大群受试者的进展速度较慢，而少数受试者的进展速度较快。发病年龄与进展速度之间存在相关性（r = 0.46-0.50，P60 岁），进展较快（P60 岁和无头部震颤的患者）进展较快，表明这些 ET 病例可能定义不同的临床亚型。应进一步评估这些亚型的病因和遗传异质性以及对治疗药物反应性的差异。
Background: There is clinical variability in essential tremor (ET), but it is not clear whether this variability is because of the existence of distinct clinical subtypes of ET (ie, forms of ET that may differ in their etiology, rate of progression, or response to treatment).Objectives: To examine in a group of ET cases the age of onset, anatomic distribution, and rate of progression of tremor, and to look for associations between these factors.Methods: Cases of ET were ascertained from a community (n=60) and a tertiary referral clinic (n=55) in northern Manhattan, New York, NY. All subjects underwent an interview and videotaped tremor examination. Rate of progression was estimated based on the tremor severity and reported disease duration at the time of evaluation.Results: Age of onset was bimodally distributed in clinic cases. There were differences in the anatomic distribution of the tremor (arm tremor only vs head and ann tremor vs isolated head tremor). Rate of progression was distributed exponentially; there was a large cluster of subjects with slower rates of progression, and a smaller number who had faster rates. There was an association between age of onset and rate of progression (r = 0.46-0.50, P60 years) progressed more rapidly (P60 years and those without head tremor) progressed moro rapidly, suggesting that these ET cases may define distinct clinical subtypes. These subtypes should be further assessed for etiologic and genetic heterogeneity as well as differences in responsiveness to therapeutic agents.