Deep brain stimulation for childhood dystonia: current evidence and emerging practice

Abstract

Dystonia, one of the most common childhood movement disorders, is often medically refractory and can lead to profound impacts on the child and their caretakers' quality of life. Limited efficacy of pharmacological treatments has fueled enthusiasm for innovative neurosurgical approaches, notably deep brain stimulation (DBS) as a treatment for refractory dystonia. Areas covered: DBS is increasingly applied to successfully treat childhood dystonia. While generally safe and effective, results vary widely depending on underlying dystonia etiology. The current work synthesizes and highlights advances in research pertaining to the use of DBS for childhood dystonia. The efficacy of DBS for children and youth with dystonia is discussed, with analysis divided among etiological subtypes. The role of DBS as a lifesaving treatment for status dystonicus is also reviewed. Expert commentary: When carefully selected, certain children and youth with dystonia experience significant symptomatic improvement after DBS. Beyond dystonic symptoms, DBS can improve quality of life and reduce caretaker burden.

Deep brain stimulation versus pallidotomy for status dystonicus: a single-center case series

“…Recently, Elkaim et al showed that the grade of motor improvement is not very predictable after surgery for SD. 13 Patients who had undergone surgical intervention, in fact, showed improvement compared to pre-SD baseline in 65.5% of cases, with the other cases (34.5%) remaining unchanged or even worsening. These data are not negligible, especially if they are compared to the out-standing long-term surgical motor outcomes reported for the general dystonic population.…”

A Proposed Diagnostic Algorithm for Inborn Errors of Metabolism Presenting With Movements Disorders

“…The mean age of surgery was 12.2 ± 4.6 years (range: 1-18 y) and the most frequent targets were internal globus pallidus (19 studies) and subthalamic nucleus (5 studies) . In summary, DBS can be recommended for treatment of dystonia in pantothenate kinase associated neurodegeneration or Lesch-Nyhan syndrome (182). Patients with pantothenate kinase associated neurodegeneration repeatedly respond well to DBS even if there may not be sustained at long-term effect because of ongoing neurodegeneration in the pallidum.…”

Pediatric Deep Brain Stimulation for Dystonia: Current State and Ethical Considerations

“…Inherited dystonia, commonly known as primary dystonia, is caused by mutations in single genes (e.g., TOR1A), which may or may not accompany degeneration or structural lesions. 7,8 Acquired dystonia, commonly known as secondary dystonia, generally develops out of neurological disease or injury (e.g., cerebral palsy). Dystonia may also be idiopathic and have no known cause.…”