Related Experiment Video
Updated: Sep 28, 2025
Targeting Neuronal Fiber Tracts for Deep Brain Stimulation Therapy Using Interactive, Patient-Specific Models
Published on: August 12, 2018
Deep Brain Stimulation (DBS) with Subthalamic Nucleus (STN) as Target for Pediatric Patients with PKAN
Hui Li1, Dapeng Li2, Wuyang Yang3
1Department of Pediatrics, Peking University First Hospital, Peking University, Beijing, People's Republic of China.
Objective:
Dystonia in pantothenate kinase-associated neurodegeneration (PKAN) is progressive despite medication. Deep brain stimulation (DBS) was reported to effectively provide symptom relief. No consensus exists in candidate and target selection for DBS. We aimed to demonstrate effectiveness of subthalamic DBS (STN-DBS) placement in pediatric patients with PKAN.
Methods:
We reviewed consecutive series of pediatric patients diagnosed with PKAN and treated with STN-DBS from 2016 to 2019 in our institution. Each case was described in detail. Preoperative and postoperative Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) were assessed to evaluate functional improvement at follow-up.
Results:
Seven pediatric patients were included. Mean age of initial onset was 0.6 ± 0.5 years and presentation to clinics was 6.6 ± 1.3 years. Mean preoperative BFMDRS was 73.3 ± 3.5. Following STN-DBS, for mean follow-up duration of 13.0 ± 10.7 months, mean BFMDRS was 37.3 ± 12.6, translating to score improvement of 36.0 ± 12.9 (P < 0.001), and percentage improvement of 49.0 ± 18.0%.
Conclusions:
This case series demonstrated that STN-DBS is an effective symptom-based treatment for pediatric patients with PKAN.
