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MRI-guided Focused Ultrasound Thalamotomy for Patients with Medically-refractory Essential Tremor
Published on: December 13, 2017
Essential tremor among children
Joseph Jankovic1, Jaswanth Madisetty, Kevin Dat Vuong
1Department 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
Insights
Essential tremor (ET) in children is often misdiagnosed and presents with comorbidities like dystonia. Standard adult treatments, such as propranolol, show potential effectiveness in pediatric essential tremor cases.
Area of Science:
- Neurology
- Pediatric Neurology
- Movement Disorders
Background:
- Essential tremor (ET) is a prevalent autosomal dominant disorder, primarily studied in adults.
- Limited research exists on childhood-onset ET, leading to potential misdiagnosis and underestimation of its impact in pediatric populations.
- Previous studies on pediatric ET have been constrained by small sample sizes.
Purpose of the Study:
- To comprehensively characterize the clinical presentation and therapeutic responses of essential tremor in children.
- To address the knowledge gap regarding the occurrence, clinical features, treatment, and prognosis of ET in pediatric patients.
Main Methods:
- A retrospective analysis of clinical data from consecutive patients diagnosed with childhood-onset ET.
- Data collected included demographics, age at onset, family history, associated neurological disorders, and treatment outcomes.
- Patients were evaluated at the Movement Disorders Clinic at Baylor College of Medicine.
Main Results:
- The study included 39 pediatric patients with ET; 74.4% were male, with a mean age at onset of 8.8 years.
- A significant family history of tremor was reported in 79.5% of cases.
- Neurologic comorbidities, notably dystonia (28.2%), were present in 46.2% of patients. Of those treated, 5 out of 12 improved with propranolol.
Conclusions:
- Childhood-onset ET frequently co-occurs with other movement disorders like dystonia, underscoring its heterogeneity.
- Therapeutic approaches utilized for adult ET appear applicable to children, though further research is warranted.
- Controlled trials are needed to establish definitive treatment guidelines for pediatric ET.
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.
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