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 dy...
Main Authors: | , , , , , , , , , |
---|---|
Format: | Article |
Language: | English |
Published: |
Korean Movement Disorder Society
2022-09-01
|
Series: | Journal of Movement Disorders |
Subjects: | |
Online Access: | http://www.e-jmd.org/upload/jmd-22002.pdf |
_version_ | 1797711087339044864 |
---|---|
author | Kyung Ah Woo Han-Joon Kim Seung-Ho Jeon Hye Ran Park Kye Won Park Seung Hyun Lee Sun Ju Chung Jong-Hee Chae Sun Ha Paek Beomseok Jeon |
author_facet | Kyung Ah Woo Han-Joon Kim Seung-Ho Jeon Hye Ran Park Kye Won Park Seung Hyun Lee Sun Ju Chung Jong-Hee Chae Sun Ha Paek Beomseok Jeon |
author_sort | Kyung Ah Woo |
collection | DOAJ |
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. |
first_indexed | 2024-03-12T07:01:58Z |
format | Article |
id | doaj.art-272c6da3b15142a283cc4397845bdf64 |
institution | Directory Open Access Journal |
issn | 2005-940X 2093-4939 |
language | English |
last_indexed | 2024-03-12T07:01:58Z |
publishDate | 2022-09-01 |
publisher | Korean Movement Disorder Society |
record_format | Article |
series | Journal of Movement Disorders |
spelling | doaj.art-272c6da3b15142a283cc4397845bdf642023-09-02T23:41:51ZengKorean Movement Disorder SocietyJournal of Movement Disorders2005-940X2093-49392022-09-0115324124810.14802/jmd.22002399Long-Term Outcomes of Deep Brain Stimulation in Pantothenate Kinase-Associated Neurodegeneration-Related DystoniaKyung Ah Woo0Han-Joon Kim1Seung-Ho Jeon2Hye Ran Park3Kye Won Park4Seung Hyun Lee5Sun Ju Chung6Jong-Hee Chae7Sun Ha Paek8Beomseok Jeon9 Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea Department of Neurology, Jeonbuk National University Hospital, Jeonju, Korea Department of Neurosurgery, Soonchunhyang University Seoul Hospital, Seoul, Korea Department of Neurology, Uijeongbu Eulji Medical Center, Eulji University School of Medicine, Uijeongbu, Korea Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea Department of Pediatrics, Pediatric Clinical Neuroscience Center, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Korea Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, KoreaObjective 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.http://www.e-jmd.org/upload/jmd-22002.pdfdeep brain stimulation (dbs)dystoniaglobus pallidusneurodegeneration with brain iron accumulation (nbia)pantothenate kinase-associated neurodegeneration (pkan) |
spellingShingle | Kyung Ah Woo Han-Joon Kim Seung-Ho Jeon Hye Ran Park Kye Won Park Seung Hyun Lee Sun Ju Chung Jong-Hee Chae Sun Ha Paek Beomseok Jeon Long-Term Outcomes of Deep Brain Stimulation in Pantothenate Kinase-Associated Neurodegeneration-Related Dystonia Journal of Movement Disorders deep brain stimulation (dbs) dystonia globus pallidus neurodegeneration with brain iron accumulation (nbia) pantothenate kinase-associated neurodegeneration (pkan) |
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 | deep brain stimulation (dbs) dystonia globus pallidus neurodegeneration with brain iron accumulation (nbia) pantothenate kinase-associated neurodegeneration (pkan) |
url | http://www.e-jmd.org/upload/jmd-22002.pdf |
work_keys_str_mv | AT kyungahwoo longtermoutcomesofdeepbrainstimulationinpantothenatekinaseassociatedneurodegenerationrelateddystonia AT hanjoonkim longtermoutcomesofdeepbrainstimulationinpantothenatekinaseassociatedneurodegenerationrelateddystonia AT seunghojeon longtermoutcomesofdeepbrainstimulationinpantothenatekinaseassociatedneurodegenerationrelateddystonia AT hyeranpark longtermoutcomesofdeepbrainstimulationinpantothenatekinaseassociatedneurodegenerationrelateddystonia AT kyewonpark longtermoutcomesofdeepbrainstimulationinpantothenatekinaseassociatedneurodegenerationrelateddystonia AT seunghyunlee longtermoutcomesofdeepbrainstimulationinpantothenatekinaseassociatedneurodegenerationrelateddystonia AT sunjuchung longtermoutcomesofdeepbrainstimulationinpantothenatekinaseassociatedneurodegenerationrelateddystonia AT jongheechae longtermoutcomesofdeepbrainstimulationinpantothenatekinaseassociatedneurodegenerationrelateddystonia AT sunhapaek longtermoutcomesofdeepbrainstimulationinpantothenatekinaseassociatedneurodegenerationrelateddystonia AT beomseokjeon longtermoutcomesofdeepbrainstimulationinpantothenatekinaseassociatedneurodegenerationrelateddystonia |