Long-Term Outcomes of Deep Brain Stimulation in Pantothenate Kinase-Associated Neurodegeneration-Related Dystonia br
DC Field | Value | Language |
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dc.contributor.author | Woo, Kyung Ah | - |
dc.contributor.author | Kim, Han-Joon | - |
dc.contributor.author | Jeon, Seung-Ho | - |
dc.contributor.author | Park, Hye Ran | - |
dc.contributor.author | Park, Kye Won | - |
dc.contributor.author | Lee, Seung Hyun | - |
dc.contributor.author | Chung, Sun Ju | - |
dc.contributor.author | Chae, Jong-Hee | - |
dc.contributor.author | Paek, Sun Ha | - |
dc.contributor.author | Jeon, Beomseok | - |
dc.date.accessioned | 2023-03-09T02:42:39Z | - |
dc.date.available | 2023-03-09T02:42:39Z | - |
dc.date.issued | 2022-09 | - |
dc.identifier.issn | 2005-940X | - |
dc.identifier.issn | 2093-4939 | - |
dc.identifier.uri | https://scholarworks.bwise.kr/sch/handle/2021.sw.sch/22107 | - |
dc.description.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 | - |
dc.language | 영어 | - |
dc.language.iso | ENG | - |
dc.publisher | 대한파킨슨병및이상운동질환학회 | - |
dc.title | Long-Term Outcomes of Deep Brain Stimulation in Pantothenate Kinase-Associated Neurodegeneration-Related Dystonia br | - |
dc.type | Article | - |
dc.publisher.location | 대한민국 | - |
dc.identifier.doi | 10.14802/jmd.22002 | - |
dc.identifier.scopusid | 2-s2.0-85138681696 | - |
dc.identifier.wosid | 000889374600005 | - |
dc.identifier.bibliographicCitation | Journal Of Movement Disorders, v.15, no.3, pp 241 - + | - |
dc.citation.title | Journal Of Movement Disorders | - |
dc.citation.volume | 15 | - |
dc.citation.number | 3 | - |
dc.citation.startPage | 241 | - |
dc.citation.endPage | + | - |
dc.type.docType | Article | - |
dc.identifier.kciid | ART002877558 | - |
dc.description.isOpenAccess | Y | - |
dc.description.journalRegisteredClass | scie | - |
dc.description.journalRegisteredClass | scopus | - |
dc.description.journalRegisteredClass | kci | - |
dc.relation.journalResearchArea | Neurosciences & Neurology | - |
dc.relation.journalWebOfScienceCategory | Clinical Neurology | - |
dc.subject.keywordPlus | PALLIDAL STIMULATION | - |
dc.subject.keywordPlus | MANAGEMENT | - |
dc.subject.keywordPlus | PANK2 | - |
dc.subject.keywordAuthor | Deep brain stimulation (DBS) | - |
dc.subject.keywordAuthor | Dystonia | - |
dc.subject.keywordAuthor | Globus pallidus | - |
dc.subject.keywordAuthor | Neurodegeneration with brain iron accumulation (NBIA) | - |
dc.subject.keywordAuthor | Pantothenate kinase-associated neurodegeneration (PKAN) | - |
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