Lou J S, Jankovic J
Department of Neurology, Baylor College of Medicine, Houston, TX 77030.
Neurology. 1991 Feb;41(2 ( Pt 1)):234-8. doi: 10.1212/wnl.41.2_part_1.234.
To study the demographic and clinical correlates of essential tremor (ET), we analyzed a comprehensive database of 350 patients evaluated at the Movement Disorders Clinic at Baylor College of Medicine from 1982 to 1989. The age at onset of tremor showed bimodal distribution for both male and female patients, with peaks in 2nd and 6th decades. ET appeared most frequently in hands, followed by head, voice, tongue, leg, and trunk. Half of the patients (47%) had associated dystonia, including cervical dystonia, writer's cramp, spasmodic dysphonia, and cranial dystonia, and 20% of the patients had associated parkinsonism. At least one 1st-degree relative of 62.5% of ET patients reported tremor. Alcohol relieved tremor in 2/3 of ET patients. Sixty-eight percent of patients who had adequate follow-up improved with propranolol, and 72% with primidone. There was no significant difference in various clinical variables between the 219 patients with familial ET and 131 with sporadic ET. Patients with early-onset ET were more likely to have hand involvement and associated dystonia than patients with late-onset ET. Dystonia was more frequently associated with mild ET than with severe ET. Patients with low-frequency tremor were older and had more head but less hand involvement than patients with high-frequency tremor. The lack of relevant differences between ET subgroups suggests that, despite variable expression, ET represents a single disease entity.
为研究特发性震颤(ET)的人口统计学和临床相关性,我们分析了一个综合数据库,该数据库包含1982年至1989年在贝勒医学院运动障碍诊所接受评估的350例患者。震颤的发病年龄在男性和女性患者中均呈双峰分布,高峰出现在第二个和第六个十年。ET最常出现在手部,其次是头部、声音、舌头、腿部和躯干。一半的患者(47%)伴有肌张力障碍,包括颈部肌张力障碍、书写痉挛、痉挛性发音障碍和颅肌张力障碍,20%的患者伴有帕金森综合征。62.5%的ET患者至少有一位一级亲属报告有震颤。2/3的ET患者饮酒后震颤减轻。在有充分随访的患者中,68%的患者使用普萘洛尔病情改善,72%的患者使用扑米酮病情改善。219例家族性ET患者和131例散发性ET患者在各种临床变量上无显著差异。早发型ET患者比晚发型ET患者更易出现手部受累和伴有肌张力障碍。肌张力障碍在轻度ET患者中比在重度ET患者中更常见。低频震颤患者比高频震颤患者年龄更大,头部受累更多但手部受累更少。ET亚组之间缺乏相关差异表明,尽管表现各异,但ET代表一种单一的疾病实体。