Jankovic Joseph, Madisetty Jaswanth, Vuong Kevin Dat
Department of Neurology, Parkinson's Disease Center and Movement Disorders Clinic, Baylor College of Medicine, 6550 Fannin St, Suite 1801, Houston, TX 77030, USA.
Pediatrics. 2004 Nov;114(5):1203-5. doi: 10.1542/peds.2004-0031.
To characterize the clinical and therapeutic aspects of essential tremor (ET) among children.
ET, an autosomal dominant disorder, has been studied extensively among adults, but little is known regarding its occurrence, clinical characteristics, treatment, and prognosis in pediatric populations. Often stigmatized as a disorder of the elderly, ET may be misdiagnosed among children. Previous studies of childhood-onset ET were limited by small sample sizes.
Clinical data, including gender, age at onset, family history, associated disorders, and response to treatment, were collected for consecutive patients diagnosed with childhood-onset ET at the Movement Disorders Clinic at Baylor College of Medicine.
Of the 39 patients with ET, 29 (74.4%) were male. The mean age at onset was 8.8 +/- 5.0 years, and the mean age at evaluation was 20.3 +/- 14.4 years. A family history of tremor was noted for 79.5% of the patients. Eighteen (46.2%) had some neurologic comorbidity, such as dystonia, which was noted for 11 patients (28.2%). Only 24 of the patients (61.5%) were treated with a specific antitremor medication; 5 of the 12 patients treated with propranolol experienced improvement.
Concomitant movement disorders, such as dystonia, are common among patients with childhood-onset ET, which supports the concept that ET is a heterogeneous disorder. Treatment strategies used for adult patients with ET seem to be effective also for children with ET, although controlled therapeutic trials in this population of patients with ET are lacking.
描述儿童特发性震颤(ET)的临床和治疗方面的特征。
ET是一种常染色体显性疾病,在成年人中已得到广泛研究,但关于其在儿科人群中的发生、临床特征、治疗和预后知之甚少。ET常被污名化为老年人的疾病,在儿童中可能会被误诊。先前关于儿童期发病ET的研究受样本量小的限制。
收集了在贝勒医学院运动障碍诊所连续诊断为儿童期发病ET的患者的临床数据,包括性别、发病年龄、家族史、相关疾病和治疗反应。
在39例ET患者中,29例(74.4%)为男性。平均发病年龄为8.8±5.0岁,平均评估年龄为20.3±14.4岁。79.5%的患者有震颤家族史。18例(46.2%)有一些神经合并症,如肌张力障碍,11例(28.2%)有此症状。只有24例患者(61.5%)接受了特定的抗震颤药物治疗;12例接受普萘洛尔治疗的患者中有5例病情改善。
儿童期发病ET患者常伴有运动障碍,如肌张力障碍,这支持了ET是一种异质性疾病的概念。用于成年ET患者的治疗策略对儿童ET患者似乎也有效,尽管缺乏针对该ET患者群体的对照治疗试验。