Deep brain stimulation for pediatric dystonia: a meta‐analysis with individual participant data

Abstract

Aim We performed a meta‐analysis with individual participant data of deep brain stimulation (DBS) for dystonia in children and young people. Method Three databases (PubMed, Embase, and Web of Science) were queried from January 1999 to August 2017 with no language restrictions to identify case studies and cohort studies reporting on pediatric patients (age ≤21y) with dystonia. The primary outcomes were changes in Burke‐Fahn‐Marsden (BFM) or Barry‐Albright Dystonia Scale scores. A mixed‐effects regression was used to identify associations between clinical covariates and outcomes. Results Of 2509 citations reviewed, 72 articles (321 children) were eligible. At last follow‐up (median 12mo, 25th centile=9.0; 75th centile=32.2), 277 (86.3%) patients showed improvement in dystonia, while 66.1 percent showed clinically significant (>20%) BFM Dystonia Rating Scale‐motor improvement. On multivariable hierarchical regression, older age at dystonia onset, inherited dystonia without nervous system pathology and idiopathic dystonia (vs inherited with nervous system pathology or acquired dystonia), and truncal involvement indicated a better outcome (p<0.05). Interpretation The data suggest that DBS is effective and should be considered in selected children with inherited or idiopathic dystonia. What this paper adds Deep brain stimulation is effective in selected children with inherited or idiopathic dystonia.

Subthalamic Nucleus Stimulation in Pediatric Isolated Dystonia: A 10-Year Follow-up

“…To be eligible, they had to meet the following surgical inclusion criteria: (1) clinical diagnosis of dystonia either with or without a positive family history based on clinical diagnostic assessments and a family history method; (2) dystonia refractory to oral medications and to chemodenervation with botulinum toxin; (3) significant functional impairment; (4) age at illness onset younger than 18 years; and (5) younger than 21 years at the time of STN-DBS surgery. 17 Surgical exclusion criteria were (1) dystonia due to an acquired cause; (2) contradiction to neurosurgery or anesthesia; and (3) a follow-up duration less than 12 months. All patients underwent genetic testing of the DYT-1 and DYT-6 loci pre-or post-operatively.…”

Deep brain stimulation for Gilles de la Tourette syndrome in children and youth: a meta-analysis with individual participant data

“…16,23,47 These comorbidities may further contribute to decreased quality of life. 17 The pathophysiology of GTS is incompletely understood. Involvement of the cortico-striato-thalamo-cortical network is generally described.…”

Current therapies and therapeutic decision making for childhood‐onset movement disorders

“…Overall, the role of DBS can be summarized from literatures as below: DBS is beneficial in children and adults with primary dystonia such as DYT1 . Genetic etiology is likely to influence expected benefit from DBS The benefit from DBS is likely to be more the less the duration of life lived with dystonia …”

“…202 Genetic etiology is likely to influence expected benefit from DBS. 203,204 • The benefit from DBS is likely to be more the less the duration of life lived with dystonia. 205,206 • DBS can benefit children with dystonia attributed to progressive disorders like PKAN [207][208][209] or with dystonia associated with brain injury (hypoxia-or kernicterusrelated CP), 200,210 though the magnitude of motor effects measured by conventional scales is less compared to primary dystonia, and benefit reported is variable.…”