Objective: Dystonia is a movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both. It can be classified as primary or secondary. Pediatric population, however, can also be afflicted by functionally uncapacitating neurological conditions that remain refractory despite the clinicians’ best efforts. In such cases, deep brain stimulation (DBS) offers an alternative treatment.

Background: There is no cure for dystonia and the goal of treatment is to provide a better quality of life for the patient. After DBS surgery, improvement was satisfactory in secondary dystonia group, however, clinical results were better in primary dystonia group.

Methods: From 2012 to the present, we performed DBS for 5 children ( 3 boys and 2 girls) with dystonia. The mean age at the time of treatment was 10,6 ( range 5 – 15 years). Clinical indications for DBS treatment was primary dystonia in 3 patients, secondary dystonia with pantothenate kinase-associated neurodegeneration (PKAN) in 1 patient and secondary dystonia with mitochondrial membrane protein-associated neurodegeneration (MPAN) in 1 patient. All patients had undergone unsuccesful medical treatment for their movement disorder prior to DBS procedure. Bilateral Gpi DBS was performed for all patients. 

Results: No complication occured during or after procedures. After six months, improvement of motor symptoms was seen in all patients. All patients showed significant improvement in their motor symptoms. No DBS related side effects were documented. 

Conclusions: Gpi DBS is a safe and effective treatment for patients with both primary and secondary dystonia and the improvement appears durable.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/gpi-dbs-for-dystonia-in-pediatric-patients/