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Long-Term Outcomes of Deep Brain Stimulation in Pantothenate Kinase-Associated Neurodegeneration-Related Dystonia
OBJECTIVE: To investigate the long-term clinical outcomes of pallidal deep brain stimulation (GPi-DBS) in patients with pantothenate kinase-associated neurodegeneration (PKAN). METHODS: We reviewed the records of patients with genetically confirmed PKAN who received bilateral GPi-DBS for refractory...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Korean Movement Disorder Society
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9536911/ https://www.ncbi.nlm.nih.gov/pubmed/35880383 http://dx.doi.org/10.14802/jmd.22002 |
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author | 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 |
author_facet | 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 |
author_sort | Woo, Kyung Ah |
collection | PubMed |
description | OBJECTIVE: To investigate the long-term clinical outcomes of pallidal deep brain stimulation (GPi-DBS) in patients with pantothenate 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 qualitative 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 patients’ subjective disability may have a shorter duration despite improvements in dystonia owing to the complex manifestations of PKAN. |
format | Online Article Text |
id | pubmed-9536911 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | The Korean Movement Disorder Society |
record_format | MEDLINE/PubMed |
spelling | pubmed-95369112022-10-24 Long-Term Outcomes of Deep Brain Stimulation in Pantothenate Kinase-Associated Neurodegeneration-Related Dystonia 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 J Mov Disord Original Article OBJECTIVE: To investigate the long-term clinical outcomes of pallidal deep brain stimulation (GPi-DBS) in patients with pantothenate 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 qualitative 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 patients’ subjective disability may have a shorter duration despite improvements in dystonia owing to the complex manifestations of PKAN. The Korean Movement Disorder Society 2022-09 2022-07-26 /pmc/articles/PMC9536911/ /pubmed/35880383 http://dx.doi.org/10.14802/jmd.22002 Text en Copyright © 2022 The Korean Movement Disorder Society https://creativecommons.org/licenses/by-nc/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) ) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Original Article 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 Long-Term Outcomes of Deep Brain Stimulation in Pantothenate Kinase-Associated Neurodegeneration-Related Dystonia |
title | Long-Term Outcomes of Deep Brain Stimulation in Pantothenate Kinase-Associated Neurodegeneration-Related Dystonia |
title_full | Long-Term Outcomes of Deep Brain Stimulation in Pantothenate Kinase-Associated Neurodegeneration-Related Dystonia |
title_fullStr | Long-Term Outcomes of Deep Brain Stimulation in Pantothenate Kinase-Associated Neurodegeneration-Related Dystonia |
title_full_unstemmed | Long-Term Outcomes of Deep Brain Stimulation in Pantothenate Kinase-Associated Neurodegeneration-Related Dystonia |
title_short | Long-Term Outcomes of Deep Brain Stimulation in Pantothenate Kinase-Associated Neurodegeneration-Related Dystonia |
title_sort | long-term outcomes of deep brain stimulation in pantothenate kinase-associated neurodegeneration-related dystonia |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9536911/ https://www.ncbi.nlm.nih.gov/pubmed/35880383 http://dx.doi.org/10.14802/jmd.22002 |
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