Long-Term Outcomes of Deep Brain Stimulation in Pantothenate Kinase-Associated Neurodegeneration-Related Dystonia bropen access
- Authors
- Woo, Kyung Ah; Kim, Han-Joon; Jeon, Seung-Ho; Park, Hye Ran; Park, Kye Won; Lee, Seung Hyun; Chung, Sun Ju; Chae, Jong-Hee; Paek, Sun Ha; Jeon, Beomseok
- Issue Date
- Sep-2022
- Publisher
- 대한파킨슨병및이상운동질환학회
- Keywords
- Deep brain stimulation (DBS); Dystonia; Globus pallidus; Neurodegeneration with brain iron accumulation (NBIA); Pantothenate kinase-associated neurodegeneration (PKAN)
- Citation
- Journal Of Movement Disorders, v.15, no.3, pp 241 - +
- Journal Title
- Journal Of Movement Disorders
- Volume
- 15
- Number
- 3
- Start Page
- 241
- End Page
- +
- URI
- https://scholarworks.bwise.kr/sch/handle/2021.sw.sch/22107
- DOI
- 10.14802/jmd.22002
- ISSN
- 2005-940X
2093-4939
- Abstract
- Objective To investigate the long-term clinical outcomes of pallidal deep brain stimulation (GPi-DBS) in patients with panto-thenate kinase-associated neurodegeneration (PKAN). Methods We reviewed the records of patients with genetically confirmed PKAN who received bilateral GPi-DBS for refractory dystonia and were clinically followed up for at least 2 years postoperatively at two centers in Korea. Pre- and postoperative Burke - Fahn-Marsden Dystonia Rating Scale motor subscale (BFMDRS-M) scores, disability subscale (BFMDRS-D) scores, and quali-tative clinical information were prospectively collected. Descriptive analysis was performed for BFMDRS-M scores, BFMDRS-D scores, and the orofacial, axial, and limb subscores of the BFMDRS-M at 6-12, 24-36, and 60-72 months postoperatively. Results Five classic-type, four atypical-type, and one unknown-type PKAN cases were identified. The mean preoperative BFMDRS-M score was 92.1 for the classic type and 38.5 for the atypical or unknown type, with a mean BFMDRS follow-up of 50.7 months and a clinical follow-up of 69.0 months. The mean improvements in BFMDRS-M score were 11.3%, 41.3%, and 30.5% at 6-12, 24-36, and 60-72 months, respectively. In four patients with full regular evaluations until 60-72 months, improvements in the orofacial, axial, and limb subscores persisted, but the disability scores worsened from 24-36 months post-operation compared to the baseline, mainly owing to the aggravation of eating and feeding disabilities. Conclusion The benefits of GPi-DBS on dystonia may persist for more than 5 years in PKAN. The effects on patient's subjec-tive disability may have a shorter duration despite improvements in dystonia owing to the complex manifestations of PKAN
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Collections - College of Medicine > Department of Neurosurgery > 1. Journal Articles
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