KMDS
E-submission
Search
- Page Path
- HOME > Search
Original Article
- Long-Term Outcomes of Deep Brain Stimulation in Pantothenate Kinase-Associated Neurodegeneration-Related Dystonia
- 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
- J Mov Disord. 2022;15(3):241-248. Published online July 26, 2022
- DOI: https://doi.org/10.14802/jmd.22002
- 4,647 View
- 207 Download
- 7 Web of Science
- 7 Crossref
-
Abstract
PDF
Supplementary Material - 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, BFMDRSD 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. -
Citations
Citations to this article as recorded by
- Imaging Findings of Intracerebral Infection after Deep Brain Stimulation: Pediatric Case Series and Literature Review
Andrew Z. Yang, Alexandre Boutet, Vivek Pai, Michael J. Colditz, Artur Vetkas, Brendan Santyr, Nardin Samuel, Jurgen Germann, Sara Breitbart, Lior Elkam, Birgit Ertl‐Wagner, Alfonso Fasano, Andres M. Lozano, George M Ibrahim, Carolina Gorodetsky
Movement Disorders Clinical Practice.2025; 12(2): 242. CrossRef - Hallerworden – Spatz DISEASE. Clinical case
L. B. Novikova, K. M. Ziultsle, A. P. Akopian
Medical alphabet.2025; (33): 34. CrossRef - Deep Brain Stimulation for Refractory Status Dystonicus in Children: Multicenter Case Series and Systematic Review
Lindsey M. Vogt, Han Yan, Brendan Santyr, Sara Breitbart, Melanie Anderson, Jürgen Germann, Karlo J. Lizarraga, Angela L. Hewitt, Alfonso Fasano, George M. Ibrahim, Carolina Gorodetsky
Annals of Neurology.2024; 95(1): 156. CrossRef - Illustration of the long-term efficacy of pallidal deep brain stimulation in a patient with PKAN dystonia
Luigi M. Romito, Fabiana Colucci, Giovanna Zorzi, Barbara Garavaglia, Ahmet Kaymak, Alberto Mazzoni, Celeste Panteghini, Nico Golfrè Andreasi, Sara Rinaldo, Vincenzo Levi, Miryam Carecchio, Roberto Eleopra
Parkinsonism & Related Disorders.2024; 123: 106977. CrossRef - Case of Hallervorden–Spatz Syndrome: A Tale of Twin Sisters
Naveen Reddy, Jitender Sharma, Anmol Sharma
Neurology India.2024; 72(2): 411. CrossRef - Patient Selection for Deep Brain Stimulation for Pantothenate Kinase-Associated Neurodegeneration
Jason L. Chan, Ashley E. Rawls, Joshua K. Wong, Penelope Hogarth, Justin D. Hilliard, Michael S. Okun
Tremor and Other Hyperkinetic Movements.2024;[Epub] CrossRef - Surgical treatment of movement disorders in neurometabolic conditions
Alonso Zea Vera, Andrea L. Gropman
Frontiers in Neurology.2023;[Epub] CrossRef
- Imaging Findings of Intracerebral Infection after Deep Brain Stimulation: Pediatric Case Series and Literature Review
Invited Review
- Apomorphine and Levodopa Infusion Therapies for Advanced Parkinson’s Disease
- Angelo Antonini
- J Mov Disord. 2009;2(1):4-9.
- DOI: https://doi.org/10.14802/jmd.09002
- 42,984 View
- 174 Download
- 15 Crossref
-
Abstract
PDF
Continuous infusion of levodopa or apomorphine provide constant dopaminergic stimulations are good alternatives to deep brain stimulation to control motor fluctuations in patients with advanced Parkinson’s disease (PD). Apomorphine provides motor benefit similar to dopamine, but its long-term use is limited by compliance, mostly injection site skin reactions. Administration of levodopa/carbidopa by continuous duodenal infusion allows replacement of all oral medications and permits achievement of a satisfactory therapeutic response paralleled by a reduction in motor complication severity. However, this procedure is more invasive than apomorphine as it requires a percutaneous endoscopic gastrostomy Clinical experience with infusions shows that continuous dopaminergic stimulation of dopaminergic medications reduces dyskinesia and widens the therapeutic window in advanced PD.
-
Citations
Citations to this article as recorded by- Integrating technology into a successful apomorphine delivery program in Thailand: a 10-year journey of achievements with a five-motto concept
Onanong Phokaewvarangkul, Nithinan Kantachadvanich, Vijittra Buranasrikul, Kanyawat Sanyawut, Saisamorn Phumphid, Chanawat Anan, Roongroj Bhidayasiri
Frontiers in Neurology.2024;[Epub] CrossRef - What was first and what is next in selecting device-aided therapy in Parkinson’s disease? Balancing evidence and experience
Onanong Phokaewvarangkul, Manon Auffret, Sergiu Groppa, Vladana Markovic, Igor Petrovic, Roongroj Bhidayasiri
Journal of Neural Transmission.2024; 131(11): 1307. CrossRef - Small Particles, Big Potential: Polymeric Nanoparticles for Drug Delivery in Parkinson's Disease
Sasivimol Virameteekul, Andrew J. Lees, Roongroj Bhidayasiri
Movement Disorders.2024; 39(11): 1922. CrossRef - Safety and tolerability of long-term apomorphine infusion in advanced Parkinson's disease: an Indian multi-center (APO-IND) experience
Vinod Metta, Rajinder K. Dhamija, Lucia Batzu, Rukmini Mrudula, Natuva Sai Sampath Kumar, Arunan S., Cristian Falup-Pecurariu, Carmen Rodriguez-Blazquez, Vinay Goyal, Prashanth L.K., Kalyan Bhattacharya, Suresh Kumar, Kallol Ray Chaudhuri, Rupam Borgohain
Scientific Reports.2023;[Epub] CrossRef - A Randomized Trial Assessing the Safety, Pharmacokinetics, and Efficacy During Morning Off of AZ‐009
Eva Thijssen, Jonas den Heijer, David Puibert, Laurence Moss, Mingzu Lei, David Hasegawa, Kyo Keum, Ken Mochel, Mohammed Ezzeldin Sharaf, Tom Alfredson, Wenxiang Zeng, Emilie van Brummelen, Tatjana Naranda, Geert Jan Groeneveld
Movement Disorders.2022; 37(4): 790. CrossRef - Why do ‘OFF’ periods still occur during continuous drug delivery in Parkinson’s disease?
Silvia Rota, Daniele Urso, Daniel J. van Wamelen, Valentina Leta, Iro Boura, Per Odin, Alberto J. Espay, Peter Jenner, K. Ray Chaudhuri
Translational Neurodegeneration.2022;[Epub] CrossRef - Parkinson's disease and skin
Nicki Niemann, Andrew Billnitzer, Joseph Jankovic
Parkinsonism & Related Disorders.2021; 82: 61. CrossRef - Subcutaneous apomorphine in advanced Parkinson’s disease and its use in Indian population
Vinod Metta, Rupam Borgohain, Prashanth L Kukkle, Rukmini Mridula, Pankaj Agarwal, Asha Kishore, Vinay Goyal, Ray Chaudhuri
Annals of Movement Disorders.2020; 3(3): 145. CrossRef - Apomorphine for Parkinson’s Disease: Efficacy and Safety of Current and New Formulations
Federico Carbone, Atbin Djamshidian, Klaus Seppi, Werner Poewe
CNS Drugs.2019; 33(9): 905. CrossRef - Platinum/Graphene Oxide Coated Microfabricated Arrays for Multinucleus Neural Activities Detection in the Rat Models of Parkinson’s Disease Treated by Apomorphine
Guihua Xiao, Yilin Song, Yu Zhang, Shengwei Xu, Yu Xing, Mixia Wang, Xinxia Cai
ACS Applied Bio Materials.2019; 2(9): 4010. CrossRef - Pharmacological Insights into the Use of Apomorphine in Parkinson’s Disease: Clinical Relevance
Manon Auffret, Sophie Drapier, Marc Vérin
Clinical Drug Investigation.2018; 38(4): 287. CrossRef - The Many Faces of Apomorphine: Lessons from the Past and Challenges for the Future
Manon Auffret, Sophie Drapier, Marc Vérin
Drugs in R&D.2018; 18(2): 91. CrossRef - Apomorphine en perfusion sous-cutanée continue dans la maladie de Parkinson : le point de vue pharmacologique
M. Auffret, S. Drapier, M. Vérin, P. Sauleau
Pratique Neurologique - FMC.2016; 7(1): 25. CrossRef - Apomorphine en perfusion sous-cutanée continue dans la maladie de Parkinson : le point de vue clinique
S. Drapier, M. Auffret, M. Vérin, P. Sauleau
Pratique Neurologique - FMC.2016; 7(1): 33. CrossRef - Infusion treatments and deep brain stimulation in Parkinson's Disease: The role of nursing
Anna De Rosa, Alessandro Tessitore, Leonilda Bilo, Silvio Peluso, Giuseppe De Michele
Geriatric Nursing.2016; 37(6): 434. CrossRef
- Integrating technology into a successful apomorphine delivery program in Thailand: a 10-year journey of achievements with a five-motto concept
First
Prev
