Joseph Jankovic1, Jaswanth Madisetty, Kevin Dat Vuong. 1. Department of Neurology, Parkinson's Disease Center and Movement Disorders Clinic, Baylor College of Medicine, 6550 Fannin St, Suite 1801, Houston, TX 77030, USA. josephj@bcm.tmc.edu
Abstract
OBJECTIVE: To characterize the clinical and therapeutic aspects of essential tremor (ET) among children. BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
OBJECTIVE: To characterize the clinical and therapeutic aspects of essential tremor (ET) among children. BACKGROUND: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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.