Pallidal deep brain stimulation (DBS) is an established treatment for patients 
with severe isolated dystonia. However, clinical evidence for the long-term use 
of DBS in children is limited and controlled trials have not yet been conducted. 
Here, we provide the long-term results of up to 13 years of pallidal DBS in 
eight pediatric patients with generalized idiopathic or hereditary isolated 
dystonia (five males, mean age at surgery 12.5 ± 3.5 years), as assessed by 
retrospective video rating. Video rating was performed at three time points: 
pre-operative, 1-year short-term follow-up (1y-FU) and long-term last FU (LT-FU, 
up to 13 years). Symptom severity and disability were assessed using the 
Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). Disability scores were 
obtained from clinical charts and during the last FU. The mean improvement in 
BFMDRS motor score was 54.4 ± 8.9 % at 1y-FU and 42.9 ± 11.6 % at LT-FU; the 
disability scores improved by 59.8 ± 10.3 and 63.3 ± 7.8 %, respectively. 
Electrode dislocation was noted in one patient and implantable pulse generator 
dislocation in another, both requiring surgical intervention; no further serious 
adverse events occurred. Our study presents the first blinded video rating 
assessment of the short- and long-term effects of pallidal DBS in children with 
idiopathic or hereditary isolated dystonia. Results confirm that pallidal DBS is 
a safe and efficacious long-term treatment in children, with overall motor 
improvement similar to that described in controlled trials in adults.