Early- and late-onset essential tremor patients represent clinically distinct subgroups
Early- and late-onset essential tremor patients represent clinically distinct subgroups
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DOI：
10.1002/mds.26708
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发表时间：
2016-10-01
影响因子：
8.6
通讯作者：
Deuschl, Guenther
中科院分区：
文献类型：
--
作者：
Hopfner, Franziska;Ahlf, Anjuli;Deuschl, Guenther
原发性震颤是一种非常常见的疾病，由稀疏的临床标准定义。原发性震颤不太可能是一种病因相同的疾病。使用临床特征对广泛定义的疾病进行分层通常有助于对病因病理学的理解。大多数特发性震颤的研究显示2个不同的发病高峰年龄：早期和晚期。本研究探讨了早发性和迟发性原发性震颤patients.MethodsWe研究了1137震颤患者的样本之间的表型差异。在这些患者中，978例患有明确或可能的原发性震颤。所有的患者进行了相同的标准化检查，包括，除其他项目外，绘制阿基米德螺旋和评估的Fahn-Tolosa-Marin scale.ResultsTwo亚组的早发性（24岁，n = 317）和迟发性（46岁，n = 356）患者的发病年龄分布的视觉和数学分析的基础上选择。两组的震颤严重程度相当。与早发患者相比，晚发患者的震颤进展（以阿基米德螺旋评分和Fahn-Tolosa-Marin分量表除以10年的病程）明显更快。早发性患者更频繁地报告了积极的家族史和酒精敏感性的tremer.ConclusionsThe发病年龄分布表明早发性和晚发性震颤之间的区别。早发性和晚发性特发性震颤的进展速度和阳性家族史的频率以及酒精对震颤的积极影响的历史不同。(c)2016国际帕金森和运动障碍协会
ObjectiveEssential tremor is a very common disease defined by sparse clinical criteria. It is unlikely that essential tremor is an etiologically homogeneous disease. Stratifying broadly defined diseases using clinical characteristics has often aided the etiopathological understanding. Most studies of essential tremor show 2 distinct age at onset peaks: early and late. This study investigates phenotypical differences between early- and late-onset essential tremor patients.MethodsWe studied a sample of 1137 tremor patients. Of these patients, 978 suffered from definite or probable essential tremor. All of the patients underwent the same standardized examination encompassing, among other items, drawing of the Archimedes spiral and assessment of the Fahn-Tolosa-Marin scale.ResultsTwo subgroups of early-onset ( 24 years of age, n = 317) and late-onset ( 46 years of age, n = 356) patients were selected based on the visual and mathematical analysis of the age-at-onset distribution. Tremor severity in both groups was comparable. Tremor progression measured as Archimedes spiral score and the Fahn-Tolosa-Marin subscales divided by the disease duration in 10-year bins was significantly faster in late-onset patients when compared with early-onset patients. Early-onset patients more frequently reported a positive family history and alcohol sensitivity of the tremor.ConclusionsThe age-at-onset distribution suggests a distinction between early- and late-onset tremor. Early-onset and late-onset essential tremor differ in the progression rates and the frequencies of a positive family history and history of a positive effect of alcohol on tremor. (c) 2016 International Parkinson and Movement Disorder Society