INTRODUCTION: Despite promising results of DBS in adults with refractory dystonia, various factors such as, effect on developing brain, ethical issues, cost constraints, lack of optimal stimulation parameters on paediatric DBS has resulted in less application of this procedure. We describe our experience with DBS for primary and secondary dystonia.

METHOD: A retrospective review of 7 patients with refractory dystonia with varied aetiology, were evaluated by a neurologist, neuropsychologist and a neurosurgeon and advised DBS. Preoperative and postoperative dystonia severity using the Barry-Albright-Dystonia(BAD) and Burke-Fahn-Marsden Dystonia Rating scale-Motor(BFMDRS-M) were obtained. Patients were followed up regularly for an average duration of 3-10 years.

RESULTS: 2/7 patients were primary generalized dystonia, one being genetics positive(DYT1). 5/7 patients were secondary dystonia(3/5 were genetically confirmed Pantothenate Kinasae-Associated Neurodegeneration(PKAN), 2/5 were dystonic cerebral palsy(CP)). The median age of presentation for patients with primary dystonia was 7.5 yrs, with PKAN 2yrs and dystonic Cerebral palsy  8 months. The average age of DBS procedure was 10 years(8-14yrs).

On follow up, both patients with primary dystonia showed significant immediate & sustained improvement in functionality and quality of life. Among patients with secondary dystonia, PKAN group showed improvement in quality of life, but not much functionally. However, given PKAN being a neurodegenerative condition, this is a substantial benefit. Patients with dystonic-CP did not show much early or late improvement and eventually failed to follow-up.

CONCLUSION: DBS is a promising alternative treatment option for children with severe dystonia. Those with primary dystonia showed best results, hence should be considered earlier.