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Clinical, Radiological, and Therapeutic Profiles of Patients With DYT-TOR1A: A Single-Center Study in India and Literature Review of the Asian MDSGene Cohort
Madathum Kuzhiyil Farsana, Vikram V. Holla, Debjyoti Dhar, Nishanth Gowda, Hansashree Padmanabha, Babylakshmi Muthusamy, Nitish Kamble, Dwarakanath Srinivas, Ravi Yadav, Pramod Kumar Pal
J Mov Disord. 2026;19(2):192-198.   Published online December 17, 2025
DOI: https://doi.org/10.14802/jmd.25256
  • 1,970 View
  • 44 Download
AbstractAbstract PDFSupplementary Material
Objective
This study aimed to characterize the phenotypic spectrum and therapeutic outcomes of patients of Indian and Asian origin with DYT-TOR1A.
Methods
A retrospective chart review of patients with genetically confirmed DYT-TOR1A (c.907_909delGAG; p.Glu303del variant) from a tertiary care center in India.
Results
Twelve patients (11 males, 91.7%) with a median age at disease onset of 10.5 years (range, 8–17 years) and a disease duration of 5 years (range, 2 months–31 years) were included. All patients had an isolated and progressive dystonia phenotype. Eight patients (66.7%) had a disease onset in childhood, and limb involvement at disease onset was noted in 10 (83.3%) patients. Five patients (41.7%) underwent bilateral globus pallidus internus deep brain stimulation within a median duration of 4 years (range, 2.5–6.5 years) from onset, with significant improvement.
Conclusion
This Indian patient cohort showed a strong male predominance and consistent early involvement of the upper limbs. A shorter disease course accompanied by greater severity highlights the need for early recognition and potential surgical intervention.
Original Articles
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Optimal Stimulation Sites and Long-Term Efficacy of Pallidal Deep-Brain Stimulation for Patients With Tardive Dystonia
Taku Nonaka, Shiro Horisawa, Kilsoo Kim, Masato Murakami, Masahiko Nishitani, Takakazu Kawamata, Takaomi Taira
J Mov Disord. 2026;19(1):49-57.   Published online October 28, 2025
DOI: https://doi.org/10.14802/jmd.25164
  • 2,143 View
  • 85 Download
AbstractAbstract PDF
Objective
Globus pallidus internus deep brain stimulation (GPi-DBS) is an established treatment for dystonia, but its specific efficacy for tardive dystonia (TD) remains insufficiently documented. To evaluate the long-term clinical outcomes of GPi-DBS and to identify optimal stimulation sites in patients with medically refractory TD.
Methods
We retrospectively analyzed data from 26 patients with TD who underwent bilateral GPi-DBS. Clinical outcomes were assessed using the Burke–Fahn–Marsden Dystonia Rating Scale (BFMDRS). Optimal stimulation sites were identified using voxelwise sweet spot analysis.
Results
At an average follow-up time of 42 months (range 12–4 months), the mean BFMDRS score improvement was 81.5%. The optimal stimulation sites were located in the posteroventral region of the GPi. Two patients experienced sustained symptom remission after DBS cessation. Complications included device-related infection (n=2), dysarthria (n=4), and gait imbalance (n= 1); no severe permanent complications occurred.
Conclusion
GPi-DBS is effective and safe for patients with medically refractory TD, providing significant long-term symptom relief. The optimal stimulation sites were located in the posteroventral GPi, which is consistent with those reported for patients with other dystonia types.
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Connectivity-Based Analysis of the Stimulation Effects of Globus Pallidus Interna Deep Brain Stimulation in Parkinson’s Disease: A Focus on Freezing of Gait
Sungyang Jo, Moongwan Choi, Jihyun Lee, Sangjin Lee, Hwon Heo, Chong Hyun Suh, Woo Hyun Shim, Junhyung Kim, Sang Ryong Jeon, Hyunna Lee, Sun Ju Chung
J Mov Disord. 2025;18(4):327-336.   Published online July 30, 2025
DOI: https://doi.org/10.14802/jmd.25005
  • 4,291 View
  • 124 Download
  • 1 Web of Science
  • 1 Crossref
AbstractAbstract PDFSupplementary Material
Objective
Freezing of gait (FOG) significantly affects quality of life and increases the risk of falls in patients with Parkinson’s disease (PD). Although deep brain stimulation (DBS) of the globus pallidus interna (GPi) is effective in managing motor complications, its efficacy in treating FOG remains inconsistent. This study aimed to determine whether preoperative structural brain connectivity can predict both the presence of FOG and its postoperative improvement following GPi DBS.
Methods
We retrospectively analyzed 58 patients with PD who underwent GPi DBS. Preoperative diffusion tensor imaging was used to assess structural connectivity between the volume of activated tissue (VAT) and 82 cortical regions. Machine learning models were developed to predict baseline FOG and postoperative FOG improvement (defined as a ≥1- or ≥2-point reduction) using demographic and connectivity features.
Results
Machine learning models incorporating structural connectivity features between the VAT and cortical regions—including the prefrontal, cingulate, and premotor cortices—outperformed models based solely on demographic variables in predicting both the presence of preoperative FOG and postoperative improvement. For example, the support vector machine model to predict FOG improvement (≥1-point improvement) achieved an accuracy of 0.65 with demographic data alone, which increased to 0.77 with the addition of structural connectivity features. Similar performance enhancements were observed in sensitivity analyses using stricter FOG thresholds (≥2-point improvement).
Conclusion
Preoperative structural connectivity between the GPi and key cortical regions involved in cognitive control and motor planning predicts FOG responsiveness to DBS. These results highlight the utility of connectomic biomarkers for personalizing DBS strategies and optimizing therapeutic outcomes in patients with advanced PD.

Citations

Citations to this article as recorded by  
  • Predictors of motor outcome with pallidal stimulation for Parkinson’s disease from the CSP468 cohort
    Shawn D’Souza, Aashish Batheja, Jeffrey Chen, Harsh P. Shah, Vikram Seshadri, Nina Opem, Omar Al-Dulaimi, Jamie Toms, Pierre D’Haese, Benoit M. Dawant, Rui Li, Paul Koch, Paul Larson, Kathryn L. Holloway
    npj Parkinson's Disease.2026;[Epub]     CrossRef
Brief communications
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Clinical and Genetic Characterization of Woodhouse-Sakati Syndrome in Iranian Patients: A Case Series
Sepehr Khosravi, Toktam Moosavian, Shadab Salehpour, Seyed Amir Hassan Habibi, Afagh Alavi, Mohammad Rohani
J Mov Disord. 2025;18(3):257-261.   Published online April 16, 2025
DOI: https://doi.org/10.14802/jmd.25043
  • 2,569 View
  • 61 Download
AbstractAbstract PDFSupplementary Material
Objective
Woodhouse-Sakati syndrome (WSS) is a rare autosomal recessive neuroendocrine disorder characterized by a variety of endocrine and neurological manifestations, including extrapyramidal symptoms and intellectual disability.
Methods
This report presents the genetic characterization of five Iranian patients with WSS, including the first Iranian patient to undergo deep brain stimulation (DBS).
Results
We highlight five Iranian patients with mutations in the DCAF17 gene presenting with variable features of WSS, with symptom onset in early adolescence. Whole exome sequencing identified four homozygous variants (c.436delC, c.982-2A>G, c.580C>T, and c.838+1G>A) within the DCAF17 gene in the probands. Patients had variable responses to common therapies, and one patient achieved significant improvement following DBS.
Conclusion
We expand the clinical and genetic heterogeneity among Iranian patients and suggest the c.436delC variant as a founder mutation in the region. We highlight the importance of considering WSS in patients with both neurological and endocrine symptoms and suggest DBS as a potential treatment option.
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Modified Ratio of Tremor/Postural Instability Gait Difficulty Score as an Indicator of Short-Term Outcomes of Subthalamic Nucleus Deep Brain Stimulation in Parkinson’s Disease
Chakradhar Reddy, Kanchana Pillai, Shejoy Joshua, Anup Nair, Harshad Chavotiya, Manas Chacko, Asha Kishore
J Mov Disord. 2025;18(2):165-169.   Published online January 2, 2025
DOI: https://doi.org/10.14802/jmd.24175
  • 3,224 View
  • 76 Download
AbstractAbstract PDFSupplementary Material
Objective
The outcomes of motor and nonmotor features of Parkinson’s disease (PD) following deep brain stimulation (DBS) vary among its subtypes. We tested whether preoperative motor subtyping using the modified tremor/postural instability and gait difficulty ratio (T/P ratio) could indicate the short-term motor, nonmotor and quality of life (QOL) outcomes of subthalamic nucleus (STN) DBS.
Methods
In this prospective study, 39 consecutive STN DBS patients were assessed in the drug-OFF state before surgery and subtyped according to the T/P ratio. Patients were reassessed 6 months after surgery in the stimulation ON-drug-OFF state, and the percentage changes in motor, nonmotor and QOL scores (Parkinson’s Disease Quality of Life Questionnaire [PDQ-39]) were calculated.
Results
The modified T/P ratio was moderately and positively correlated with the percentage change in the Unified Parkinson’s Disease Rating Scale III score in the OFF state, the sum of cardinal motor signs, the Non-Motor Symptom Scale score, and QOL (PDQ-39).
Conclusion
Preoperative PD motor subtyping can be used as an indicator of the short-term outcomes of STN DBS in PD patients.
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Monitoring Cognitive Functions During Deep Brain Stimulation Interventions by Real Time Neuropsychological Testing
Ilaria Guarracino, Christian Lettieri, Massimo Mondani, Stanislao D’Auria, Giovanni Sciacca, Flavia Lavezzi, Miran Skrap, Serena D’Agostini, Gian Luigi Gigli, Mariarosaria Valente, Barbara Tomasino
J Mov Disord. 2024;17(4):442-446.   Published online September 23, 2024
DOI: https://doi.org/10.14802/jmd.24102
  • 4,799 View
  • 89 Download
  • 1 Crossref
AbstractAbstract PDFSupplementary Material
Objective
We monitored cognition in 14 Parkinson’s disease (PD) patients during deep brain stimulation (DBS) surgery when the electrode was positioned at the target subthalamic nucleus (STN) (i.e., the STN motor area).
Methods
We present the DBS-real-time neuropsychological testing (DBS-RTNT) protocol and our preliminary experience with it; we also compared the intraoperative patient performance with the baseline data.
Results
Compared with the baseline data, patients undergoing DBS-RTNT in the target area demonstrated a significantly decreased performance on some tasks belonging to the memory and executive function domains. Patients undergoing right hemisphere DBS-RTNT had significantly lower short-term memory and sequencing scores than did patients undergoing left hemisphere DBS-RTNT.
Conclusion
PD patient cognitive performance should be monitored during DBS surgery, as STN-DBS may induce changes. These preliminary data contribute to improving our understanding of the anatomo-functional topography of the STN during DBS surgery, which will enable the identification of the best site for producing positive motor effects without causing negative cognitive and/or emotional changes in individual patients in the future. In principle, medications (i.e., patients who underwent surgery in a levodopa-off state) could have influenced our results; therefore, future studies are needed to address the possible confounding effects of levodopa use.

Citations

Citations to this article as recorded by  
  • Use of a Novel Intraoperative Neurocognitive Monitoring Task During Awake Craniotomy for Tumor Resection in the Language-Dominant Parietal Lobe
    Mercy H. Mazurek, Amy J. Maguire, James D. Sixkiller, Timothy R. West, Nicole A. Perez, Ethan A. Wetzel, Alexander F. Wang, Zsombor T. Gal, Reiner B. See, Eva K. Ritzl, Otto Rapalino, Bryan D. Choi, Brian V. Nahed
    Operative Neurosurgery.2026;[Epub]     CrossRef
Review Article
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Pallidus Stimulation for Chorea-Acanthocytosis: A Systematic Review and Meta-Analysis of Individual Data
Weibin He, Chenhui Li, Hongjuan Dong, Lingmin Shao, Bo Yin, Dianyou Li, Liguo Ye, Ping Hu, Chencheng Zhang, Wei Yi
J Mov Disord. 2022;15(3):197-205.   Published online July 26, 2022
DOI: https://doi.org/10.14802/jmd.22003
  • 8,723 View
  • 356 Download
  • 6 Web of Science
  • 6 Crossref
AbstractAbstract PDFSupplementary Material
A significant proportion of patients with chorea-acanthocytosis (ChAc) fail to respond to standard therapies. Recent evidence suggests that globus pallidus internus (GPi) deep brain stimulation (DBS) is a promising treatment option; however, reports are few and limited by sample sizes. We conducted a systematic literature review to evaluate the clinical outcome of GPi-DBS for ChAc. PubMed, Embase, and Cochrane Library databases were searched for relevant articles published before August 2021. The improvement of multiple motor and nonmotor symptoms was qualitatively presented. Improvements in the Unified Huntington’s Disease Rating Scale motor score (UHDRS-MS) were also analyzed during different follow-up periods. A multivariate linear regression analysis was conducted to identify potential predictors of clinical outcomes. Twenty articles, including 27 patients, were eligible. Ninety-six percent of patients with oromandibular dystonia reported significant improvement. GPi-DBS significantly improved the UHDRS-motor score at < 6 months (p < 0.001) and ≥ 6 months (p < 0.001). The UHDRS-motor score improvement rate was over 25% in 75% (15/20 cases) of patients at long-term follow-up (≥ 6 months). The multiple linear regression analysis showed that sex, age at onset, course of disease, and preoperative movement score had no linear relationship with motor improvement at long-term follow-up (p > 0.05). GPi-DBS is an effective and safe treatment in most patients with ChAc, but no reliable predictor of efficacy has been found. Oromandibular dystonia-dominant patients might be the best candidates for GPi-DBS.

Citations

Citations to this article as recorded by  
  • Subthalamic Deep Brain Stimulation for Chorea-Acanthocytosis: A Single-Center Case Series
    Tianyu Ma, Meitong Zhou, Suzhen Lin, Tao Wang, Peng Huang, Dianyou Li, Hongxia Li, Yiwen Wu
    Neuromodulation: Technology at the Neural Interface.2026;[Epub]     CrossRef
  • Chorea-acanthocytosis presenting with epilepsy at onset harboring novel compound heterozygous VPS13A mutations
    Mitsuyoshi Tamura, Shogo Furukawa, Masahiro Namiki, Yukiko Ozawa, Yuka Urata, Masayuki Nakamura, Mitsuru Watanabe, Hideki Oshima, Toshiki Obuchi, Tadashi Ichikawa, Yuriko Kikkawa
    Clinical Parkinsonism & Related Disorders.2026; 14: 100433.     CrossRef
  • 38-jähriger Patient mit Epilepsie, hyperkinetischer Bewegungsstörung und Hyper-CKämie
    E. Fischer, A. Großmann, H. Ludwig, M. Lanz, A. Mühlbäck, A. Hermann, K. Peikert
    DGNeurologie.2026; 9(3): 221.     CrossRef
  • Immunology of familial chorea-acanthocytosis with presenting generalized tonic-clonic seizure: Blood cell study for early diagnosis and management
    Amineh Salem, Narges Omidvar
    Cellular Immunology.2025; 411-412: 104946.     CrossRef
  • Clinical neurophysiology in the treatment of movement disorders: IFCN handbook chapter
    Jean-Pascal Lefaucheur, Elena Moro, Yuichiro Shirota, Yoshikazu Ugawa, Talyta Grippe, Robert Chen, David H Benninger, Bahman Jabbari, Sanaz Attaripour, Mark Hallett, Walter Paulus
    Clinical Neurophysiology.2024; 164: 57.     CrossRef
  • Bilateral deep brain stimulation (DBS) of globus pallidus internus (GPi) for the treatment of benign hereditary chorea and other childhood onset choreas: a single-center experience
    Arthur R. Kurzbuch, Ben Cooper, Gina Lumsdon, Nicola Idowu, Helen Gedrim, Philipa Mulholland, Volker Tronnier, Ram Kumar, Jonathan R. Ellenbogen
    Neurosurgical Review.2024;[Epub]     CrossRef
Original Articles
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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
  • 7,616 View
  • 236 Download
  • 8 Web of Science
  • 8 Crossref
AbstractAbstract PDFSupplementary 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
  • Pantothenate kinase-associated neurodegeneration
    L. B. Novikova, A. P. Akopyan, K. M. Ziultsle
    Russian neurological journal.2025; 30(4): 44.     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
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Development of Clinical Milestones in Parkinson’s Disease After Bilateral Subthalamic Deep Brain Stimulation
Jed Noel A. Ong, Jung Hwan Shin, Seungho Jeon, Chan Young Lee, Han-Joon Kim, Sun Ha Paek, Beomseok Jeon
J Mov Disord. 2022;15(2):124-131.   Published online May 26, 2022
DOI: https://doi.org/10.14802/jmd.21106
  • 6,262 View
  • 178 Download
  • 2 Web of Science
  • 2 Crossref
AbstractAbstract PDFSupplementary Material
Objective
Deep brain stimulation of the subthalamic nucleus (STN-DBS) in Parkinson’s disease (PD) patients does not halt disease progression, as these patients will progress and develop disabling non-levodopa responsive symptoms. These features may act as milestones that represent the overall functionality of patients after DBS. The objective of this study was to investigate the development of clinical milestones in advanced PD patients who underwent bilateral STN-DBS.
Methods
The study evaluated PD patients who underwent STN-DBS at baseline up to their last follow-up using the Unified Parkinson’s Disease Rating Scale and Hoehn and Yahr scale. The symptoms of hallucinations, dysarthria, dysphagia, frequent falls, difficulty walking, cognitive impairment and the loss of autonomy were chosen as the clinical milestones.
Results
A total of 106 patients with a mean age of 47.21 ± 10.52 years at disease onset, a mean age of 58.72 ± 8.74 years at surgery and a mean disease duration of 11.51 ± 4.4 years before surgery were included. Initial improvement of motor symptoms was seen after the surgery with the appearance of clinical milestones over time. Using the moderately disabling criteria, 81 patients (76.41%) developed at least one clinical milestone, while 48 patients (45.28%) developed a milestone when using the severely disabling criteria.
Conclusion
STN-DBS has a limited effect on axial and nonmotor symptoms of the PD patients, in contrast to the effect on motor symptoms. These symptoms may serve as clinical milestones that can convey the status of PD patients and its impact on the patients and their caregivers. Therefore, advanced PD patients, even those treated with bilateral STN-DBS, will still require assistance and cannot live independently in the long run.

Citations

Citations to this article as recorded by  
  • Survival after Subthalamic Deep Brain Stimulation for Parkinson's Disease: 25‐Year Experience from a Single Center
    Sayooja Sachithanandan, Athul Sreelatha Sanjeev, Jissa Vinoda Thulaseedharan, Krishnakumar Kesavapisharady, Divya Kalikavil Puthanveedu, Asish Vijayaraghavan, Gangadhara Sarma, Syam Krishnan, Asha Kishore
    Movement Disorders Clinical Practice.2026; 13(3): 723.     CrossRef
  • Unveiling the Impact of Outpatient Physiotherapy on Specific Motor Symptoms in Parkinson’s Disease: A Prospective Cohort Study
    Yuta Terasawa, Koki Ikuno, Shintaro Fujii, Yuki Nishi, Emi Tanizawa, Sachio Nabeshima, Yohei Okada
    Brain & Neurorehabilitation.2023;[Epub]     CrossRef
Case Report
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Deep Brain Stimulation for Cockayne Syndrome-Associated Movement Disorder
Joseph S. Domino, Rose Gelineau-Morel, Christian Kaufman
J Mov Disord. 2022;15(1):62-65.   Published online November 3, 2021
DOI: https://doi.org/10.14802/jmd.21005
  • 6,770 View
  • 259 Download
  • 2 Web of Science
  • 3 Crossref
AbstractAbstract PDFSupplementary Material
Cockayne syndrome (CS) is a rare progeroid disorder characterized by multisystem degeneration, including neurological dysfunction, for which deep brain stimulation (DBS) is a proposed treatment. This study represents only the third case of DBS for CS-associated movement disorder and the first in which both proposed targets had devices implanted, allowing for direct comparison. A case of DBS for CS-associated movement disorder is presented. Previous literature documents two cases with one targeting the ventral intermediate nucleus of the thalamus (VIM) and the other targeting the globus pallidus interna (GPi). Our patient underwent stimulation of GPi nuclei followed by repositioning to VIM nuclei with improved symptom control using VIM stimulation. In all cases, there was a significant clinical benefit without off-target effects. CS-associated movement disorder exhibits phenotypic variability for which DBS is a viable treatment. Target selection should be driven by clinical phenotype.

Citations

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  • Simultaneous, dual-target, bilateral deep brain stimulation for treatment of galactosemia-induced dystonia and tremor in a pediatric patient
    Adip G. Bhargav, Paige A. Lundy, Keith A. Coffman, Christian B. Kaufman
    Child's Nervous System.2025;[Epub]     CrossRef
  • Cockayne syndrome type 3 with dystonia‐ataxia and clicking blinks
    Özge Berna Gültekin‐Zaim, Gül Yalçın‐Çakmaklı, Ayşe İlksen Çolpak, Pelin Özlem Şimşek‐Kiper, Gülen Eda Utine, Bülent Elibol
    Movement Disorders Clinical Practice.2023;[Epub]     CrossRef
  • Hypomyelinating leukodystrophy and movement disorders
    Jacky Ganguly, Jigyasha Sinha, Purba Basu, Anushree Pal, Banashree Mondal, Mona Tiwari, Hrishikesh Kumar
    Annals of Movement Disorders.2023; 6(2): 58.     CrossRef
Original Article
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The Queensland Parkinson’s Project: An Overview of 20 Years of Mortality from Parkinson’s Disease
Peter Cornelis Poortvliet, Alexander Gluch, Peter A. Silburn, George D. Mellick
J Mov Disord. 2021;14(1):34-41.   Published online December 7, 2020
DOI: https://doi.org/10.14802/jmd.20034
  • 22,557 View
  • 270 Download
  • 27 Web of Science
  • 27 Crossref
AbstractAbstract PDF
Objective
The consensus is that life expectancy for individuals with Parkinson’s disease (PD) is reduced, but estimations vary. We aimed to provide an overview of 20 years of mortality and risk factor data from the Queensland Parkinson’s Project.
Methods
The analysis included 1,334 PD and 1,127 control participants. Preliminary analysis of baseline characteristics (sex, age at onset, family history, smoking status, pesticide exposure, depression and neurosurgery) was conducted, and Kaplan–Meier curves were generated for each potential risk factor. Standardized mortality ratios (SMRs) were calculated comparing this cohort to the general Australian population. Cox proportional hazards regression modeling was used to analyze potential predictors of mortality.
Results
In total, 625 (46.8%) PD and 237 (21.0%) control participants were deceased. Mean disease duration until death was 15.3 ± 7.84 years. Average ages at death were 78.0 ± 7.4 years and 80.4 ± 8.4 years for the deceased PD and control participants, respectively. Mortality was significantly increased for PD in general {SMR = 2.75 [95% confidence interval (CI): 2.53–2.96]; p = 0.001}. SMRs were slightly higher for women and those with an age of onset before 60 years. Multivariate analysis showed that deep brain stimulation (DBS) treatment was associated with lower mortality [hazard ratio (HR) = 0.76; 95% CI: 0.59–0.98], while occasional pesticide exposure increased mortality risk (HR = 1.48; 95% CI: 1.17–1.88). Family history of PD, smoking and depression were not independent predictors of mortality.
Conclusion
Mortality in PD is increased. Sex, age at onset and occasional pesticide exposure were independent determinants of increased mortality, while DBS treatment was associated with reduced mortality.

Citations

Citations to this article as recorded by  
  • Survival after Subthalamic Deep Brain Stimulation for Parkinson's Disease: 25‐Year Experience from a Single Center
    Sayooja Sachithanandan, Athul Sreelatha Sanjeev, Jissa Vinoda Thulaseedharan, Krishnakumar Kesavapisharady, Divya Kalikavil Puthanveedu, Asish Vijayaraghavan, Gangadhara Sarma, Syam Krishnan, Asha Kishore
    Movement Disorders Clinical Practice.2026; 13(3): 723.     CrossRef
  • Physical Exercise and Survival in Parkinson’s Disease: A Cohort Study
    Priscila Rodrigues Gil, Juliana da Silveira, Adriano Ferreti Borgatto, Jéssica Amaro Moratelli, Anelise Sonza, Adriana Coutinho de Azevedo Guimarães
    Research Quarterly for Exercise and Sport.2026; 97(2): 341.     CrossRef
  • Biological aging predicts mortality in Parkinson’s patients: evidence from UK Biobank
    Qing-Qing Duan, Wei-Ming Su, Kang-Fu Yin, Sheng-Yi He, Ru-Yin Liu, Xiang-Jin Wen, Xiao-Jing Gu, Ting Chen, Bei Cao, Yong-Ping Chen
    npj Parkinson's Disease.2026;[Epub]     CrossRef
  • Long-Term Mortality and 10-Year Outcomes after Subthalamic Nucleus Deep Brain Stimulation in Parkinson’s Disease
    Hikaru Kamo, Genko Oyama, Mai Shimizu, Masanobu Ito, Hirokazu Iwamuro, Atsushi Umemura, Taku Hatano, Nobutaka Hattori
    Stereotactic and Functional Neurosurgery.2026; : 1.     CrossRef
  • Dementia Incidence in Individuals With Parkinson's Disease in the Framingham Heart Study
    Joshi Dookhy, Michael Cummings, Eden Price, Yulin Liu, Santiago Gutierrez‐Gomez, Sarah O'Shea, Samuel Frank, Rhoda Au, Ashita S. Gurnani, Ting Fang Alvin Ang, Jesse Mez, Ludy C. Shih
    Annals of Clinical and Translational Neurology.2026;[Epub]     CrossRef
  • Case Definitions and Data Sources for NNCSS Parkinson Disease Surveillance
    Christine D. Esper, Kelly N. Hogan, Purni Abeysekara, Kasey Nichole Smith, Tenbroeck G. Smith, Shawna L. Mercer
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Brief communications
Article image
Knowledge, Attitude, and Perceptions about Deep Brain Stimulation for Parkinson’s Disease: Observations from a Single Indian Center
Shweta Prasad, Amitabh Bhattacharya, Lulup Kumar Sahoo, Dhruv Batra, Nitish Kamble, Ravi Yadav, Dwarakanath Srinivas, Pramod Kumar Pal
J Mov Disord. 2021;14(1):60-64.   Published online September 21, 2020
DOI: https://doi.org/10.14802/jmd.20066
  • 9,153 View
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AbstractAbstract PDFSupplementary Material
Objective
Willingness to undergo deep brain stimulation (DBS) among patients with Parkinson’s disease (PD) and their overall satisfaction with the procedure is highly dependent upon expectations, which are based on the core concepts of knowledge, attitude and perceptions. The present study aims to evaluate these factors in patients and caregivers with PD from a single tertiary care hospital in India.
Methods
A structured questionnaire designed to assess the knowledge, attitude and perceptions about DBS in PD was administered to 400 patients with PD and their caregivers.
Results
A very small proportion of patients and caregivers were aware of DBS. Even those who claimed to be aware of DBS were inadequately informed and had incorrect knowledge, which led to wrong attitudes and perceptions.
Conclusion
There are very significant knowledge gaps and misconceptions regarding DBS among patients with PD and caregivers. Adequate and appropriate education is necessary to clarify these misconceptions to avoid the development of unrealistic expectations and poor satisfaction.

Citations

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Article image
Deep Brain Stimulation Battery Exhaustion during the COVID-19 Pandemic: Crisis within a Crisis
Vikram Venkappayya Holla, Koti Neeraja, Bharath Kumar Surisetti, Shweta Prasad, Nitish Kamble, Dwarakanath Srinivas, Ravi Yadav, Pramod Kumar Pal
J Mov Disord. 2020;13(3):218-222.   Published online August 31, 2020
DOI: https://doi.org/10.14802/jmd.20073
  • 15,291 View
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AbstractAbstract PDF
Objective
The novel coronavirus disease (COVID-19) pandemic and public health measures to control it have resulted in unique challenges in the management of patients with deep brain stimulation (DBS). We report our experience with the management of acute worsening of symptoms due to battery exhaustion in 3 patients with DBS.
Methods
Patients with DBS for movement disorders who visited the emergency room due to battery exhaustion during the nationwide lockdown from April to May 2020 were included.
Results
Two patients with subthalamic nucleus-DBS for Parkinson’s disease (PD) and one with globus pallidus interna-DBS for generalized dystonia presented with acute worsening of symptoms due to battery exhaustion. Urgent battery replacement was performed in both patients with PD. The patient with generalized dystonia was managed with medication adjustment as he chose to defer battery replacement.
Conclusion
DBS battery replacement can be an emergency. Decisions regarding DBS battery replacement should be individualized during this COVID-19 pandemic.

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Review Article
Article image
Update on Current Technologies for Deep Brain Stimulation in Parkinson’s Disease
Michelle Paff, Aaron Loh, Can Sarica, Andres M. Lozano, Alfonso Fasano
J Mov Disord. 2020;13(3):185-198.   Published online August 31, 2020
DOI: https://doi.org/10.14802/jmd.20052
  • 30,092 View
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  • 83 Web of Science
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AbstractAbstract PDF
Deep brain stimulation (DBS) is becoming increasingly central in the treatment of patients with Parkinson’s disease and other movement disorders. Recent developments in DBS lead and implantable pulse generator design provide increased flexibility for programming, potentially improving the therapeutic benefit of stimulation. Directional DBS leads may increase the therapeutic window of stimulation by providing a means of avoiding current spread to structures that might give rise to stimulation-related side effects. Similarly, control of current to individual contacts on a DBS lead allows for shaping of the electric field produced between multiple active contacts. The following review aims to describe the recent developments in DBS system technology and the features of each commercially available DBS system. The advantages of each system are reviewed, and general considerations for choosing the most appropriate system are discussed.

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Original Article
Long-term Effects of Bilateral Subthalamic Deep Brain Stimulation on Postural Instability and Gait Difficulty in Patients with Parkinson’s Disease
Hae-Won Shin, Mi Sun Kim, Sung Reul Kim, Sang Ryong Jeon, Sun Ju Chung
J Mov Disord. 2020;13(2):127-132.   Published online May 29, 2020
DOI: https://doi.org/10.14802/jmd.19081
  • 9,917 View
  • 233 Download
  • 16 Web of Science
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AbstractAbstract PDF
Objective
The long-term effects of bilateral subthalamic nucleus deep brain stimulation (STN-DBS) on postural instability and gait difficulty (PIGD) in patients with Parkinson’s disease (PD) remain unclear. In this study, we aimed to evaluate the longterm effects of STN-DBS surgery on PIGD symptoms in patients with advanced-stage PD. Methods This study included 49 consecutively included patients with PD who underwent bilateral STN-DBS. The Unified Parkinson’s Disease Rating Scale (UPDRS) scores and subscores for PIGD were assessed at baseline and at 1, 3, and 5 years postoperatively. The PIGD subscore was divided into PIGD-motor and PIGD-activities of daily living (ADL) scores according to parts III and II of the UPDRS, respectively. Results The PIGD-motor and PIGD-ADL scores at the “medication-off” state improved at 3 and 5 years, respectively. Overall, the UPDRS III and II scores at “medication-off” improved at 5 years. The UPDRS IV score also significantly improved and the levodopa equivalent daily dosage decreased at all follow-ups. Finally, the PIGD-motor score at baseline was able to predict long-term improvement in the PIGD-motor score at the 5-year follow-up. Conclusion The STN-DBS has both short- and long-term effects on PIGD, as well as overall motor function, in patients with advanced PD. The degree of PIGD at the preoperative evaluation can be used to predict long-term outcomes after STN-DBS surgery.

Citations

Citations to this article as recorded by  
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Case Report
Successful Pallidal Stimulation in a Patient with KMT2B-Related Dystonia
Jun Kyu Mun, Ah Reum Kim, Jong Hyeon Ahn, Minkyeong Kim, Jin Whan Cho, Jung-Il Lee, Kyung Rae Cho, Jinyoung Youn
J Mov Disord. 2020;13(2):154-158.   Published online April 6, 2020
DOI: https://doi.org/10.14802/jmd.19087
  • 13,217 View
  • 189 Download
  • 14 Web of Science
  • 14 Crossref
AbstractAbstract PDFSupplementary Material
Although the KMT2B gene was identified as a causative gene for early-onset generalized dystonia, the efficacy of deep brain stimulation (DBS) in KMT2B-related dystonia has not been clearly elucidated. Here, we describe a 28-year-old woman who developed generalized dystonia with developmental delay, microcephaly, short stature, and cognitive decline. She was diagnosed with KMT2B- related dystonia using whole-exome sequencing with a heterozygous frameshift insertion of c.515dupC (p.T172fs) in the KMT2B gene. Oral medications and botulinum toxin injection were not effective. The dystonia markedly improved with bilateral pallidal DBS (the Burke-Fahn-Marsden Dystonia Rating Scale score was reduced from 30 to 5 on the dystonia movement scale and from 11 to 1 on the disability scale), and she could walk independently. From this case, we suggest that bilateral globus pallidus internus DBS can be an effective treatment option for patients with KMT2B-related generalized dystonia.

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Brief communications
Article image
Rescue Levodopa/Carbidopa Intestinal Gel for Secondary Deep Brain Stimulation Failure
Juan Miguel Pilar Bautista, Genko Oyama, Maierdanjiang Nuermaimaiti, Satoko Sekimoto, Fuyuko Sasaki, Taku Hatano, Kenya Nishioka, Masanobu Ito, Atsushi Umemura, Yuji Ishibashi, Yasushi Shimo, Nobutaka Hattori
J Mov Disord. 2020;13(1):57-61.   Published online January 31, 2020
DOI: https://doi.org/10.14802/jmd.19051
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AbstractAbstract PDF
Objective
The long-term efficacy of deep brain stimulation (DBS) for motor fluctuations in advanced Parkinson’s disease (PD) has been well established; however, motor fluctuations may recur over time despite multiple adjustments of DBS settings and medications.
Methods
We conducted a retrospective chart review of three patients for whom levodopa-carbidopa intestinal gel (LCIG) was additionally administered as a rescue therapy for secondary DBS failure due to the recurrence of motor fluctuations.
Results
The three patients had advanced PD with a disease duration of 14–19 years, and had undergone DBS for motor fluctuations refractory to standard medical management. LCIG was administered to the patients because of symptom recurrence years after DBS and provided complementary effects in all patients.
Conclusion
The cases presented here show that rescue LCIG therapy may be a complementary treatment option for patients with post-DBS advanced PD who have a recurrence of troublesome motor complications.

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  • Selection of patients with Parkinson’s disease for interventional therapies: A review of available tools — scales and questionnaires
    E.V. Bril, E.N. Antyukhova, A.A. Tomskiy, A.A. Gamaleya, N.V. Fedorova
    S.S. Korsakov Journal of Neurology and Psychiatry.2025; 125(11): 11.     CrossRef
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    Iro Boura, Nikolaos Haliasos, Ιrene‐Areti Giannopoulou, Dimitrios Karabetsos, Cleanthe Spanaki
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    Daniël van Poppelen, Annelie N.M. Tromp, Rob M.A. de Bie, Joke M. Dijk
    Journal of Personalized Medicine.2021; 11(6): 547.     CrossRef
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    Valentina Leta, Haidar S. Dafsari, Anna Sauerbier, Vinod Metta, Nataliya Titova, Lars Timmermann, Keyoumars Ashkan, Michael Samuel, Eero Pekkonen, Per Odin, Angelo Antonini, Pablo Martinez-Martin, Miriam Parry, Daniel J. van Wamelen, K. Ray Chaudhuri
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  • Parkinson’s Kinetigraph in the Selection of Levodopa-Carbidopa Intestinal Gel for Motor Fluctuations Refractory to Deep Brain Stimulation
    Yassine Noui, Monty Adam Silverdale, Julian Evans, Lucy Partington-Smith, Christopher Kobylecki
    Journal of Movement Disorders.2021; 14(3): 239.     CrossRef
  • The Choice Between Advanced Therapies for Parkinson’s Disease Patients: Why, What, and When?
    Joke M. Dijk, Alberto J. Espay, Regina Katzenschlager, Rob M.A. de Bie, Bastiaan R. Bloem, Patrik Brundin
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The Effect of Globus Pallidus Interna Deep Brain Stimulation on a Dystonia Patient with the GNAL Mutation Compared to Patients with DYT1 and DYT6
Jong Hyeon Ahn, Ah Reum Kim, Nayoung K. D. Kim, Woong-Yang Park, Ji Sun Kim, Minkyeong Kim, Jongkyu Park, Jung-Il Lee, Jin Whan Cho, Kyung Rae Cho, Jinyoung Youn
J Mov Disord. 2019;12(2):120-124.   Published online May 30, 2019
DOI: https://doi.org/10.14802/jmd.19006
  • 9,537 View
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  • 19 Web of Science
  • 19 Crossref
AbstractAbstract PDFSupplementary Material
Objective
The aim of this study was to investigate the efficacy of globus pallidus interna deep brain stimulation (GPi-DBS) for treating dystonia due to the GNAL mutation.
Methods
We provide the first report of a dystonia patient with a genetically confirmed GNAL mutation in the Korean population and reviewed the literature on patients with the GNAL mutation who underwent GPi-DBS. We compared the effectiveness of DBS in patients with the GNAL mutation compared to that in patients with DYT1 and DYT6 in a previous study.
Results
Patients with the GNAL mutation and those with DYT1 had higher early responder rates (GNAL, 5/5, 100%; DYT1, 7/7, 100%) than did patients with DYT6 (p = 0.047). The responder rates at late follow-up did not differ statistically among the three groups (p = 0.278). The decrease in the dystonia motor scale score in the GNAL group was 46.9% at early follow-up and 63.4% at late follow-up.
Conclusion
GPi-DBS would be an effective treatment option for dystonia patients with the GNAL mutation who are resistant to medication or botulinum toxin treatment.

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Original Article
Article image
Pilot Study for Considering Subthalamic Nucleus Anatomy during Stimulation Using Directional Leads
Takashi Asahi, Kiyonobu Ikeda, Jiro Yamamoto, Hiroyuki Tsubono, Shuji Sato
J Mov Disord. 2019;12(2):97-102.   Published online April 5, 2019
DOI: https://doi.org/10.14802/jmd.18054
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AbstractAbstract PDF
Objective
Directional leads are used for deep brain stimulation (DBS). Two of the four contacts of the leads are divided into three parts, enabling controlled stimulation in a circumferential direction. The direction of adverse effects evoked by DBS in the subthalamic nucleus (STN) and stimulation strategies using directional leads were evaluated. Methods Directional leads were implanted into the bilateral STN of six parkinsonian patients (1 man, 5 women; mean age 66.2 years). The contact centers were located within the upper border of the STN, and the locations were identified electrically using microrecordings. Adverse effects were evaluated with electrical stimulation (30 μs, 130 Hz, limit 11 mA) using the directional part of each lead after surgery, and the final stimulation direction was investigated. Unified Parkinson’s disease rating scale (UPDRS) scores were evaluated before and after DBS. Results Fifty-six motor and four sensory symptoms were evoked by stimulation; no adverse effect was evoked in 14 contacts. Motor and sensory symptoms were evoked by stimulation in the anterolateral direction and medial to posterolateral direction, respectively. Stimulation in the posteromedial direction produced adverse effects less frequently. The most frequently used contacts were located above the STN (63%), followed by the upper part of the STN (32%). The mean UPDRS part III and dyskinesia scores decreased after DBS from 30.2 ± 11.7 to 7.2 ± 2.9 and 3.3 ± 2.4 to 0.5 ± 0.8, respectively. Conclusion The incidence of adverse effects was low for the posteromedial stimulation of the STN. Placing the directional part of the lead above the STN may facilitate the control of dyskinesia.

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Case Report
Treatment of Hemichoreoathetosis with Arrhythmic Proximal Tremor after Stroke: The Role of Zona Incerta as a Target for Deep Brain Stimulation
Andrei Koerbel, Augusto Radünz do Amaral, Helena Bedatti Zeh, Eduardo Wollmann, Renata Fabiola Heil Koerbel, Carla Moro, Alexandre Luiz Longo
J Mov Disord. 2019;12(1):47-51.   Published online January 30, 2019
DOI: https://doi.org/10.14802/jmd.18032
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AbstractAbstract PDFSupplementary Material
Deep brain stimulation (DBS) of the zona incerta has shown promising results in the reduction of medically refractory movement disorders. However, evidence supporting its efficacy in movement disorders secondary to hemorrhagic stroke or hemichoreoathetosis is limited. We describe a 48-year-old man who developed progressive hemichoreoathetosis with an arrhythmic, proximal tremor in his right arm following a thalamic hemorrhagic stroke. Pharmacological treatment was carried out with no change in the Abnormal Involuntary Movement Scale (AIMS) score after 4 weeks (14). After six sessions of botulinum toxin treatment, a subtle improvement in the AIMS score (13) was registered, but no clinical improvement was noted. The arrhythmic proximal movements were significantly improved after DBS of the zona incerta with a major decrease in the patient’s AIMS score (8). The response to DBS occurring after the failure of pharmacological and botulinum toxin treatments suggests that zona incerta DBS may be an alternative for postthalamic hemorrhage movement disorders.

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Review Article
Article image
The Relationship between Saccades and Locomotion
Anshul Srivastava, Omar F. Ahmad, Christopher Pham Pacia, Mark Hallett, Codrin Lungu
J Mov Disord. 2018;11(3):93-106.   Published online August 9, 2018
DOI: https://doi.org/10.14802/jmd.18018
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AbstractAbstract PDFSupplementary Material
Human locomotion involves a complex interplay among multiple brain regions and depends on constant feedback from the visual system. We summarize here the current understanding of the relationship among fixations, saccades, and gait as observed in studies sampling eye movements during locomotion, through a review of the literature and a synthesis of the relevant knowledge on the topic. A significant overlap in locomotor and saccadic neural circuitry exists that may support this relationship. Several animal studies have identified potential integration nodes between these overlapping circuitries. Behavioral studies that explored the relationship of saccadic and gait-related impairments in normal conditions and in various disease states are also discussed. Eye movements and locomotion share many underlying neural circuits, and further studies can leverage this interplay for diagnostic and therapeutic purposes.

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Original Article
Comparison of Pallidal and Subthalamic Deep Brain Stimulation in Parkinson’s Disease: Therapeutic and Adverse Effects
Ho-Sung Ryu, Mi-Sun Kim, Sooyeoun You, Mi-Jung Kim, Young Jin Kim, Juyeon Kim, Kiju Kim, Sun Ju Chung
J Mov Disord. 2017;10(2):80-86.   Published online May 8, 2017
DOI: https://doi.org/10.14802/jmd.17001
  • 12,736 View
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AbstractAbstract PDFSupplementary Material
Objective
To compare the therapeutic and adverse effects of globus pallidus interna (GPi) and subthalamic nucleus (STN) deep brain stimulation (DBS) for the treatment of advanced Parkinson’s disease (PD).
Methods
We retrospectively analyzed the clinical data of patients with PD who underwent GPi (n = 14) or STN (n = 28) DBS surgery between April 2002 and May 2014. The subjects were matched for age at surgery and disease duration. The Unified Parkinson’s Disease Rating Scale (UPDRS) scores and levodopa equivalent dose (LED) at baseline and 12 months after surgery were used to assess the therapeutic effects of DBS. Adverse effects were also compared between the two groups.
Results
At 12 months, the mean changes in the UPDRS total and part I–IV scores did not differ significantly between the two groups. However, the subscores for gait disturbance/postural instability and dyskinesia were significantly more improved after GPi DBS than those after STN DBS (p = 0.024 and 0.016, respectively). The LED was significantly more reduced in patients after STN DBS than that after GPi DBS (p = 0.004). Serious adverse effects did not differ between the two groups (p = 0.697).
Conclusion
The patients with PD showed greater improvement in gait disturbance/postural instability and dyskinesia after GPi DBS compared with those after STN DBS, although the patients had a greater reduction in LED after STN DBS. These results may provide useful information for optimal target selection for DBS in PD.

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Case Report
Holmes’ Tremor with Shoulder Pain Treated by Deep Brain Stimulation of Unilateral Ventral Intermediate Thalamic Nucleus and Globus Pallidus Internus
Sabri Aydın, Huseyin Canaz, Ezgi Tuna Erdogan, Nazlı Durmaz, Barıs Topcular
J Mov Disord. 2017;10(2):92-95.   Published online April 18, 2017
DOI: https://doi.org/10.14802/jmd.16051
  • 11,076 View
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AbstractAbstract PDF
A 21-year-old male was admitted with severe right arm and hand tremors after a thalamic hemorrhage caused by a traffic accident. He was also suffering from agonizing pain in his right shoulder that manifested after the tremor. Neurologic examination revealed a disabling, severe, and irregular kinetic and postural tremor in the right arm during target-directed movements. There was also an irregular ipsilateral rest tremor and dystonic movements in the distal part of the right arm. The amplitude was moderate at rest and extremely high during kinetic and intentional movements. The patient underwent left globus pallidum internus and ventral intermediate thalamic nucleus deep brain stimulation. The patient improved by more than 80% as rated by the Fahn-Tolosa-Marin Tremor Rating Scale and Visual Analog Scale six months after surgery.

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Review Articles
The Current Status of Deep Brain Stimulation for the Treatment of Parkinson Disease in the Republic of Korea
Jung-Il Lee
J Mov Disord. 2015;8(3):115-121.   Published online September 10, 2015
DOI: https://doi.org/10.14802/jmd.15043
  • 26,424 View
  • 129 Download
  • 15 Web of Science
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AbstractAbstract PDF
Parkinson disease (PD) is a common neurodegenerative disease with an increasing prevalence in Korea. Deep brain stimulation (DBS) is a safe and effective surgical treatment option for this disease. The aim of this review was to provide an update regarding current DBS practices with respect to the treatment of PD in the Republic of Korea. The first DBS in Korea was performed in 2000; approximately 2,000 patients have undergone DBS for a variety of neurological disorders, the majority of whom were patients with PD. Approximately 150 new patients with PD receive DBS annually, and more than 20 centers perform DBS. However, DBS remains underutilized for many reasons, and the clinical case burden at many institutions is below the level presumed adequate for qualified practice. With a rapidly aging population and an evolving socioeconomic environment, the need for surgical intervention for PD is likely to increase significantly in the future. Many issues such as finances, education, and quality assurance must be resolved to cope with this need.

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Nonmotor Symptoms and Subthalamic Deep Brain Stimulation in Parkinson’s Disease
Han-Joon Kim, Beom S. Jeon, Sun Ha Paek
J Mov Disord. 2015;8(2):83-91.   Published online May 31, 2015
DOI: https://doi.org/10.14802/jmd.15010
  • 30,919 View
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AbstractAbstract PDF
Subthalamic deep brain stimulation (STN DBS) is an established treatment for the motor symptoms in patients with advanced Parkinson’s disease (PD). In addition to improvements in motor symptoms, many studies have reported changes in various nonmotor symptoms (NMSs) after STN DBS in patients with PD. Psychiatric symptoms, including depression, apathy, anxiety, and impulsivity, can worsen or improve depending on the electrical stimulation parameters, the locations of the stimulating contacts within the STN, and changes in medications after surgery. Global cognitive function is not affected by STN DBS, and there is no increase in the incidence of dementia after STN DBS compared to that after medical treatment, although clinically insignificant declines in verbal fluency have been consistently reported. Pain, especially PD-related pain, improves with STN DBS. Evidence regarding the effects of STN DBS on autonomic symptoms and sleep-related problems is limited and remains conflicting. Many symptoms of nonmotor fluctuations, which are occasionally more troublesome than motor fluctuations, improve with STN DBS. Although it is clear that NMSs are not target symptoms for STN DBS, NMSs have a strong influence on the quality of life of patients with PD, and clinicians should thus be aware of these NMSs when deciding whether to perform surgery and should pay attention to changes in these symptoms after STN DBS to ensure the optimal care for patients.

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    Han‐Joon Kim, Beomseok Jeon, Jee‐Young Lee, Sun Ha Paek
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    Margherita Fabbri, Miguel Coelho, Leonor Correia Guedes, Mario M. Rosa, Daisy Abreu, Nilza Gonçalves, Angelo Antonini, Joaquim J. Ferreira
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    Kathrin Badstuebner, Ulrike Gimsa, Immo Weber, Armin Tuchscherer, Jan Gimsa
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    Maja Kojovic, Andrea Higgins, Marjan Jahanshahi
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Case Reports
Globus Pallidus Interna Deep Brain Stimulation in a Patient with Medically Intractable Meige Syndrome
Dae-Woong Bae, Byung-chul Son, Joong-Seok Kim
J Mov Disord. 2014;7(2):92-94.   Published online October 30, 2014
DOI: https://doi.org/10.14802/jmd.14013
  • 17,090 View
  • 107 Download
  • 7 Web of Science
  • 7 Crossref
AbstractAbstract PDF
Medical therapies in patients with Meige syndrome, including botulinum toxin injection, have been limited because of incomplete response or adverse side effects. We evaluated a patient with Meige syndrome who was successfully treated with deep brain stimulation (DBS) in the globus pallidus interna (GPi). This case report and other previous reports suggest that bilateral GPi DBS may be an effective treatment for medically refractory Meige syndrome, without significant adverse effects.

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  • Pallidal versus subthalamic deep brain stimulation for Meige syndrome: A systematic review and meta-analysis
    Xin Wu, Tao Xue, Shiqing Pan, Weikang Xing, Chuanjun Huang, Jianguo Zhang, Guozheng Zhao
    Heliyon.2024; 10(6): e27945.     CrossRef
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    Joshua Lucas, Dorian Kusyk, Donald Whiting
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    Hongying Ma, Jian Qu, Liangjun Ye, Yi Shu, Qiang Qu
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    Xin Wang, Zhiqi Mao, Zhiqiang Cui, Xin Xu, Longsheng Pan, Shuli Liang, Zhipei Ling, Xinguang Yu
    Journal of Neurosurgery.2020; 132(5): 1367.     CrossRef
  • Long-Term Efficacy of Deep Brain Stimulation of Bilateral Globus Pallidus Internus in Primary Meige Syndrome
    Hong Tian, Yanbing Yu, Xueke Zhen, Li Zhang, Yue Yuan, Bo Zhang, Liang Wang
    Stereotactic and Functional Neurosurgery.2019; 97(5-6): 356.     CrossRef
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    Sanjay Pandey, Soumya Sharma
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Hypomania Induced by Subthalamic Nucleus Stimulation in a Parkinson’s Disease Patient: Does It Suggest a Dysfunction of the Limbic Circuit?
Ji Seon Kim, Hee Jin Kim, Ji-Young Lee, Jong Min Kim, Ji Young Yun, Beom S. Jeon
J Mov Disord. 2012;5(1):14-17.
DOI: https://doi.org/10.14802/jmd.12004
  • 17,600 View
  • 87 Download
  • 9 Crossref
AbstractAbstract PDF

The aim of this report was to describe a case of hypomania after deep brain stimulation of the subthalamic nucleus (STN DBS) in a Parkinson’s disease (PD) patient. 59-year-old man with a 15-year history of PD underwent bilateral implantation of electrodes to the STN. Immediately after surgery, his motor function was markedly improved and his mood was elevated to hypomania. Fusion images of the preoperative MRI and postoperative CT scan showed that the electrodes were located in the medial portion of the STN. In this case, behavioral mood change was related to the deep brain stimulation. Moreover, the anatomical location and the functional alteration of the STN after the DBS surgery might be related to the regulatory system of the associative and limbic cortico-subcortical circuits.

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  • Is the Medial Subthalamic Region in Humans Homologous to That in Rodents? Relevance to Neuropsychiatric Disorders and Their Treatment With Deep Brain Stimulation
    Marie Barbier, Yvan Peterschmitt, Matthieu Béreau, Pierre-Yves Risold
    Biological Psychiatry.2026; 99(3): 200.     CrossRef
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    Barbara Buccilli, Patricia E. Buccilli
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    Asheeta A. Prasad, Åsa Wallén-Mackenzie
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    Mircea Polosan, Fabien Droux, Astrid Kibleur, Stephan Chabardes, Thierry Bougerol, Olivier David, Paul Krack, Valerie Voon
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Original Article
Lateralized Effects of Unilateral Thalamotomy and Thalamic Stimulation in Patients with Essential Tremor
Mi J. Kim, Sang R. Jeon, Sung R. Kim, Myoung C. Lee, Sun J. Chung
J Mov Disord. 2011;4(2):64-67.
DOI: https://doi.org/10.14802/jmd.11013
  • 20,607 View
  • 82 Download
  • 9 Crossref
AbstractAbstract PDF
Background and Purpose

Stereotactic thalamotomy has been an effective surgical procedure in the treatment of medically refractory essential tremor (ET), however, little is known about the bilateral effects of unilateral ventralis intermedius (Vim) thalamotomy and Vim deep brain stimulation (DBS). We studied the lateralized effects of unilateral Vim thalamotomy and Vim DBS in ET patients.

Methods

Vim thalamotomy was performed in 6 patients and Vim DBS in 6. Patients were evaluated preoperatively and at 3 and 6 months postoperatively using the Clinical Rating Scale for Tremor (CRST).

Results

The contralateral Part A (tremor localization/severity rating) and Part B (specific motor tasks/function rating) subscores, and axial subscores of CRST significantly improved after unilateral Vim thalamotomy or Vim DBS. On the side ipsilateral to surgery, ET patients demonstrated no significant improvements in the Part A and Part B subscores of CRST. The Part C (functional disabilities resulting from tremor) subscores and total scores of CRST were significantly improved after surgery.

Conclusions

Vim thalamotomy and DBS may be equally effective for the management of contralateral and axial tremor in ET patients, but both interventions may not improve tremor on the side ipsilateral to surgery.

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  • Wearable peripheral nerve stimulator reduces essential tremor symptoms through targeted brain modulation
    Cuong P. Luu, Jordan Ranum, Youngwon Youn, Jennifer L. Perrault, Bryan M. Krause, Matthew I. Banks, Laura Buyan-Dent, Kip A. Ludwig, Wendell B. Lake, Aaron J. Suminski
    Brain Stimulation.2025; 18(4): 1162.     CrossRef
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    Sagun Ghimire, Bibechan Thapa, Durga Neupane, Pashupati Pokharel
    Journal of Clinical Neuroscience.2024; 126: 38.     CrossRef
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    Atsuya Sato, Takaomi Taira, Kazuya Kitada, Toshiki Ando, Toyohiro Hamaguchi, Michiko Konno, Yoshinori Kitabatake, Toshiyuki Ishioka
    Frontiers in Neurology.2023;[Epub]     CrossRef
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    Prashin Unadkat, David Eidelberg
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  • Deep Brain Stimulation for Tremor: Update on Long-Term Outcomes, Target Considerations and Future Directions
    Naomi I. Kremer, Rik W. J. Pauwels, Nicolò G. Pozzi, Florian Lange, Jonas Roothans, Jens Volkmann, Martin M. Reich
    Journal of Clinical Medicine.2021; 10(16): 3468.     CrossRef
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    J. Levi Chazen, Harini Sarva, Philip E. Stieg, Robert J. Min, Douglas J. Ballon, Kane O. Pryor, Paul M. Riegelhaupt, Michael G. Kaplitt
    Journal of Neurosurgery.2018; 129(2): 315.     CrossRef
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    Lauren K. Dunn, Marcel E. Durieux, W. Jeffrey Elias, Edward C. Nemergut, Bhiken I. Naik
    Journal of Neurosurgical Anesthesiology.2018; 30(1): 18.     CrossRef
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    Rodger J. Elble, Ludy Shih, Jeffrey W. Cozzens
    Expert Review of Neurotherapeutics.2018; 18(4): 303.     CrossRef
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    Jung-Il Lee
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Review Article
One View of the Current State of Understanding in Basal Ganglia Pathophysiology and What is Needed for the Future
Erwin B. Montgomery
J Mov Disord. 2011;4(1):13-20.
DOI: https://doi.org/10.14802/jmd.11003
  • 19,941 View
  • 57 Download
  • 4 Crossref
AbstractAbstract PDF

Deep Brain Stimulation (DBS), arguably, is the most dramatic development in movement disorders since the levodopa for Parkinson’s disease. Yet, its mechanisms of action of DBS are unknown. However, DBS related research already has demonstrated that current concepts of basal ganglia pathophysiology are wrong. Specifically, the notion that over-activity of the globus pallidus interna causes parkinsonism, the basis for the most current theories, is no longer tenable. The development of any new theory will be aided by an understanding of how current theories are wrong and why have these flawed theories persist. Many of the problems of current theories are more matters of inference, assumptions, presumptions, and the accepted level of ambiguity than they are of fact. Consequently, it is imperative that these issues be addressed. Just as the inappropriate use of a tool or method is grounds for criticism, methods of reasoning are tools that can be used inappropriately and should be subject to discussion just as misuse of any other tool. Thorough criticism can provide very important lesions though the process could be mistaken as harsh or personal; neither is the case here. At the least, such analyzes can point to potential pitfalls that could be avoided in the development of new theories. As will be discussed, theories are important for the development of therapies but perhaps most important, for the acceptance of new therapies, as was the case for the recent resurgence of interest in surgical therapies.

Citations

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    Alexander Reinshagen
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