Deep Brain Stimulation (DBS) with Subthalamic Nucleus (STN) as Target for Pediatric Patients with PKAN

Hui Li; Dapeng Li; Wuyang Yang; Huifang Yan; Zifang Zhao; Haibo Yang

doi:10.1016/j.wneu.2022.03.130

Deep Brain Stimulation (DBS) with Subthalamic Nucleus (STN) as Target for Pediatric Patients with PKAN

World Neurosurg. 2022 Jul:163:e317-e322. doi: 10.1016/j.wneu.2022.03.130. Epub 2022 Apr 1.

Authors

Hui Li¹, Dapeng Li², Wuyang Yang³, Huifang Yan⁴, Zifang Zhao¹, Haibo Yang⁵

Affiliations

¹ Department of Pediatrics, Peking University First Hospital, Peking University, Beijing, People's Republic of China.
² Department of Neurosurgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
³ Department of Neurosurgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
⁴ Department of Anesthesiology, Peking University First Hospital, Peking University, Beijing, People's Republic of China.
⁵ Department of Pediatrics, Peking University First Hospital, Peking University, Beijing, People's Republic of China; Department of Pediatric Surgery, Peking University First Hospital, Peking University, Beijing, People's Republic of China. Electronic address: haiboyang226@bjmu.edu.cn.

PMID: 35367641
DOI: 10.1016/j.wneu.2022.03.130

Abstract

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.

Keywords: Clinical outcome; Deep brain stimulation; PKAN; Subthalamic nucleus.

Publication types

Review
Research Support, Non-U.S. Gov't

MeSH terms

Child
Deep Brain Stimulation*
Dystonia* / therapy
Dystonic Disorders* / therapy
Globus Pallidus / surgery
Humans
Infant
Pantothenate Kinase-Associated Neurodegeneration* / therapy
Severity of Illness Index
Subthalamic Nucleus*
Treatment Outcome