Journal of Movement Disorders

Original Articles

Efficacy and Safety of Taltirelin Hydrate in Patients With Ataxia Due to Spinocerebellar Degeneration: Jin Whan Cho, Jee-Young Lee, Han-Joon Kim, Joong-Seok Kim, Kun-Woo Park, Seong-Min Choi, Chul Hyoung Lyoo, Seong-Beom Koh; J Mov Disord. 2025;18(1):35-44. Published online October 21, 2024; DOI: https://doi.org/10.14802/jmd.24127; Funded: HLB Pharmaceutical Co., LTD.

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Abstract PDF Supplementary Material: Objective
We conducted this study to assess the efficacy and safety of taltirelin hydrate (TH) in patients with ataxia due to spinocerebellar degeneration (SCD).
Methods
Patients were randomly assigned to either the taltirelin group (5 mg orally, twice daily) or the control group. The primary endpoint was the change in the Korean version of the Scale for the Assessment and Rating of Ataxia (K-SARA) score at 24 weeks. The secondary endpoints included changes in the K-SARA score at 4 and 12 weeks as well as the Clinical Global Impression Scale, the five-level version of the EuroQol five-dimensional questionnaire, the Tinetti balance test, and gait analysis at 4, 12, and 24 weeks.
Results
A total of 149 patients (hereditary:nonhereditary=86:63) were enrolled. There were significant differences in the change in the K-SARA score at 24 weeks from baseline between the taltirelin group and the control group (-0.51±2.79 versus 0.36±2.62, respectively; p=0.0321). For the K-SARA items, the taltirelin group had significantly lower “Stance” and “Speech disturbance” subscores than the control group (-0.04±0.89 versus 0.23±0.79 and -0.07±0.74 versus 0.18±0.67; p=0.0270 and 0.0130, respectively). However, there were no significant differences in changes in other secondary efficacy outcome measures at 24 weeks from baseline between the two treatment arms (p>0.05).
Conclusion
Clinicians might consider the use of TH in the treatment of patients with ataxia due to SCD.

Gait Parameters in Healthy Older Adults in Korea: Han-Kyeol Kim, Sung-Woo Kim, Jin Yong Hong, Min Seok Baek; J Mov Disord. 2025;18(1):55-64. Published online November 25, 2024; DOI: https://doi.org/10.14802/jmd.24181; Funded: National Research Foundation of Korea, Ministry of Science and ICT, Ministry of Education, Ministry of Trade, Industry and Energy

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Abstract PDF Supplementary Material: Objective
Gaits constitute the most fundamental and common form of human locomotion and are essential in daily activities. We aimed to investigate gait parameters in medically and cognitively healthy older adults to determine the independent effects of age, physical attributes, and cognition on these parameters.
Methods
This retrospective study enrolled healthy older adult participants aged 50 years or older with normal cognition and no neurological symptoms or medical/surgical history that could affect gait. Quantitative gait analysis was conducted via the GAITRite Electronic Walkway, which categorizes gait parameters into spatiotemporal, spatial, temporal, phase, and variability. Gait parameters were compared between sexes across different age groups. The independent effects of age, Mini-Mental State Examination score, and physical characteristics were analyzed via a multiple regression model.
Results
This study included 184 participants with an average age of 72.2 years. After adjusting for age, height, and footwear, only the base width and its variability differed between the sexes. Gait parameters varied significantly among different age groups, revealing multiple interparameter associations. Age was independently correlated with decreased velocity, step and stride lengths, single support time percentage and increased double support time, double support time percentage, and variability parameters, excluding the coefficient of variance of base width. Height was positively correlated with velocity, step and stride lengths, and base width, whereas leg length was negatively associated with cadence and positively associated with temporal parameters of gait.
Conclusion
Gait parameters in healthy older adults were not only associated with age and physical characteristics but also had interparameter correlations.

Eye Movement and Gait Variability Analysis in Chinese Patients With Huntington’s Disease: Shu-Xia Qian, Yu-Feng Bao, Xiao-Yan Li, Yi Dong, Zhi-Ying Wu; J Mov Disord. 2025;18(1):65-76. Published online December 9, 2024; DOI: https://doi.org/10.14802/jmd.24151; Funded: Medical and Health Science and Technology Plan of Zhejiang Provincial Health Commission, Key Research and Development project of Zhejiang Province

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Abstract PDF Supplementary Material: Objective
Huntington’s disease (HD) is characterized by motor, cognitive, and neuropsychiatric symptoms. Oculomotor impairments and gait variability have been independently considered as potential markers in HD. However, an integrated analysis of eye movement and gait is lacking. We performed multiple examinations of eye movement and gait variability in HTT mutation carriers, analyzed the consistency between these parameters and clinical severity, and then examined the associations between oculomotor impairments and gait deficits.
Methods
We included 7 patients with pre-HD, 30 patients with HD and 30 age-matched controls. We collected demographic data and assessed the Unified Huntington’s Disease Rating Scale (UHDRS) score. Examinations, including saccades, smooth pursuit tests, and optokinetic (OPK) tests, were performed to evaluate eye movement function. The parameters of gait include stride length, walking velocity, step deviation, step length, and gait phase.
Results
HD patients have significant impairments in the latency and velocity of saccades, the gain of smooth pursuit, and the gain and slow phase velocities of OPK tests. Only the speed of saccades significantly differed between pre-HD patients and controls. There are significant impairments in stride length, walking velocity, step length, and gait phase in HD patients. The parameters of eye movement and gait variability in HD patients were consistent with the UHDRS scores. There were significant correlations between eye movement and gait parameters.
Conclusion
Our results show that eye movement and gait are impaired in HD patients and that the speed of saccades is impaired early in pre-HD. Eye movement and gait abnormalities in HD patients are significantly correlated with clinical disease severity.

Video-Oculography for Enhancing the Diagnostic Accuracy of Early Oculomotor Dysfunction in Progressive Supranuclear Palsy: Harshad Chovatiya, Kanchana Pillai, Chakradhar Reddy, Amiya Thalakkattu, Ayana Avarachan, Manas Chacko, Asha Kishore; J Mov Disord. 2025;18(1):77-86. Published online December 9, 2024; DOI: https://doi.org/10.14802/jmd.24171; Funded: Aster Medcity, Kerala, India

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Abstract PDF Supplementary Material: Objective
Oculomotor impairment is an important diagnostic feature of progressive supranuclear palsy (PSP) and PSP subtypes. We assessed the role of video-oculography (VOG) in confirming clinically suspected slow saccades in PSP and differentiating PSP from Parkinson’s disease (PD). We also measured the correlation of both saccadic velocity and latency in PSP patients with scores on the PSP Rating Scale, Montreal Cognitive Assessment, and frontal assessment battery. We assessed the frequency of apraxia of eyelid opening (ALO) and reflex blepharospasm in PSP and PD patients.
Methods
A total of 112 PSP patients with slow saccades but not gaze palsy, 50 PD patients, and 50 healthy controls (HCs) were recruited. The Movement Disorders Society task force-PSP and PD criteria were used for the diagnoses. All the subjects underwent VOG.
Results
Horizontal and vertical saccadic velocities and latencies differentiated PSP patients from PD patients and HCs (p<0.001). Vertical saccadic velocity and latency accurately differentiated PSP with predominant parkinsonism (PSP-P) patients from PD patients (p<0.001 and 0.012, respectively). A couple of vertical and horizontal saccadic velocities differentiated PSP-Richardson’s syndrome (PSP-RS) patients from PSP-P patients (vertical velocity of left eye: p=0.024; horizontal velocity of right eye: p=0.030). In vertical gaze, the mean velocity cutoff showed good sensitivity and specificity in differentiating PSP patients from HCs and PD patients. Prolonged horizontal gaze latency was associated with more severe PSP and worse global cognitive and frontal dysfunction. ALO and reflex blepharospasm were observed only in PSP patients.
Conclusion
VOG is useful for confirming slow saccades in PSP-RS and PSP-P patients and for differentiating PSP-P patients from PD patients. Prolonged horizontal gaze latency was associated with more severe PSP and worse cognitive dysfunction. ALO and reflex blepharospasm were observed only in PSP patients.

Brief communication

Spatiotemporal Gait Parameters During Turning and Imbalance in Parkinson’s Disease: Video-Based Analysis From a Single Camera: HoYoung Jeon, Jung Hwan Shin, Ri Yu, Min Kyung Kang, Seungmin Lee, Seoyeon Kim, Bora Jin, Kyung Ah Woo, Han-Joon Kim, Beomseok Jeon; J Mov Disord. 2025;18(1):87-92. Published online December 23, 2024; DOI: https://doi.org/10.14802/jmd.24210; Funded: National Research Foundation of Korea, Ministry of Science and ICT, Institute of Information & communications Technology Planning & Evaluation

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Abstract PDF Supplementary Material: Objective
This study aims to objectively evaluate turning gait parameters in Parkinson’s disease (PD) patients using 2D-RGB video-based analysis and explore their relationships with imbalance.
Methods
We prospectively enrolled PD patients for clinical assessment, balance analysis and gait with 180º turning. Spatiotemporal gait parameters during turning were derived using video-based analysis and correlated with modified Hoehn and Yahr (mHY) stages and center of pressure (COP) oscillations.
Results
A total of 64 PD patients were enrolled. The PD patients with higher mHY stages (≥2.5) had significantly longer turning times, greater numbers of steps, wider step bases and less variability in step length during turns. COP oscillations were positively correlated with the mean turning time on both the anterior-posterior and right-left axes.
Conclusion
Spatiotemporal gait parameter during turning, derived from video-based gait analysis, may represent apromising biomarker for monitoring postural instability in PD patients.

Review Article

A Practical Guide for Diagnostic Investigations and Special Considerations in Patients With Huntington’s Disease in Korea: Jangsup Moon, Eungseok Oh, Minkyeong Kim, Ryul Kim, Dallah Yoo, Chaewon Shin, Jee-Young Lee, Jong-Min Kim, Seong-Beom Koh, Manho Kim, Beomseok Jeon; J Mov Disord. 2025;18(1):17-30. Published online December 26, 2024; DOI: https://doi.org/10.14802/jmd.24232; Funded: Seoul Metropolitan Government-Seoul National University Boramae Medical Center , Chungnam National University Sejong Hospital

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Abstract PDF: This review provides a comprehensive framework for the diagnostic approach and management of Huntington’s disease (HD) tailored to the Korean population. Key topics include genetic counseling, predictive testing, and reproductive options like preimplantation genetic testing. Strategies for assessing disease progression in premanifest HD through laboratory investigations, biofluid, and imaging biomarkers are highlighted. Special considerations for juvenile and late-onset HD, along with associated comorbidities like diabetes mellitus, hypertension, and cardiovascular abnormalities, are discussed. The guide emphasizes personalized symptom management, including pharmacotherapy, physical therapy, and nutritional support, while exploring emerging disease-modifying treatments. A multidisciplinary care model is advocated to improve outcomes for HD patients and caregivers in Korea.

Letters to the editor

Thalamic Deep Brain Stimulation for SPG56-Related Focal Hand Dystonia: Momo Uchida, Shiro Horisawa, Kenkou Azuma, Hiroyuki Akagawa, Shinichi Tokushige, Takakazu Kawamata, Takaomi Taira; J Mov Disord. 2024;17(4):447-449. Published online June 27, 2024; DOI: https://doi.org/10.14802/jmd.24022; Funded: Japan Brain Foundation, Japan Society for the Promotion of Science

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PDF Supplementary Material

Olfactory and Gustatory Function in Early-Stage Parkinson’s Disease: Implications for Cognitive Association: Jeongjae Lee, Young Eun Kim, Joong Seob Lee, Suk Yun Kang, Min Seung Kim, In Hee Kwak, Jaeseol Park, Jung Yeon Nam, Hyeo-il Ma; J Mov Disord. 2024;17(4):450-452. Published online June 27, 2024; DOI: https://doi.org/10.14802/jmd.23243; Funded: Korea Health Industry Development Institute, Ministry of Health and Welfare, National Research Foundation of Korea, Ministry of Education, Ministry of Science and ICT

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Brief communication

Efficacy and Safety of Zolpidem for Musician’s Dystonia: Shiro Horisawa, Kilsoo Kim, Masato Murakami, Masahiko Nishitani, Takakazu Kawamata, Takaomi Taira; J Mov Disord. 2024;17(4):425-429. Published online July 1, 2024; DOI: https://doi.org/10.14802/jmd.24121; Funded: Japan Society for the Promotion of Science, Japan Brain Foundation

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Abstract PDF Supplementary Material: Objective
The efficacy and safety of zolpidem for treating musician’s dystonia are not well understood. We aimed to retrospectively investigate the efficacy and safety of zolpidem for treating musician’s dystonia.
Methods
We retrospectively reviewed medical records between January 2021 and December 2023 to identify patients with musician’s dystonia who had been prescribed zolpidem. Tubiana’s Musician’s Dystonia Rating Scale (range, 1–5; lower scores indicating greater severity) was used to evaluate musician’s dystonia.
Results
Fifteen patients were included in this study. The mean effective dose of zolpidem was 5.3 ± 2.0 mg. The mean effective duration of zolpidem was 4.3 ± 1.2 h. With zolpidem administration, Tubiana’s musician’s dystonia rating scale score significantly improved from 2.2 ± 1.0 to 4.3 ± 0.8 (48.9% improvement, p < 0.001). Two patients (13.3%) discontinued the drug owing to unsatisfactory results or sleepiness.
Conclusion
The results of this study suggest that zolpidem may be an alternative treatment option for musician’s dystonia.

Original Article

Ocular Vestibular-Evoked Myogenic Potential Assists in the Differentiation of Multiple System Atrophy From Parkinson’s Disease: Keun-Tae Kim, Kyoungwon Baik, Sun-Uk Lee, Euyhyun Park, Chan-Nyoung Lee, Tonghoon Woo, Yukang Kim, Seoui Kwag, Hyunsoh Park, Ji-Soo Kim; J Mov Disord. 2024;17(4):398-407. Published online July 9, 2024; DOI: https://doi.org/10.14802/jmd.24120; Funded: National Research Foundation of Korea, Ministry of Science and ICT

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Abstract PDF Supplementary Material

Objective
Vestibular-evoked myogenic potentials (VEMPs) can help in assessing otolithic neural pathway in the brainstem, which may also contribute to the cardiovascular autonomic function. Parkinson’s disease (PD) is associated with altered VEMP responses; however, the associations between VEMP abnormalities and multiple system atrophy (MSA) remain unknown. Therefore, we compared the extent of otolith dysfunction using ocular (oVEMP) and cervical VEMPs between patients with MSA and PD.
Methods
We analyzed the clinical features, VEMP, and head-up tilt table test (HUT) findings using the Finometer in 24 patients with MSA and 52 with de novo PD who had undergone neurotologic evaluation at a referral-based university hospital in South Korea from January 2021 to March 2023.
Results
MSA was associated with bilateral oVEMP abnormalities (odds ratio [95% confidence interval] = 9.19 [1.77–47.76], p = 0.008). The n1–p1 amplitude was negatively correlated with the Unified Multiple System Atrophy Rating Scale I-II score in patients with MSA (r = -0.571, p = 0.033), whereas it did not correlate with the Movement Disorder Society-Unified Parkinson’s Disease Rating Scale-III score in patients with PD (r = -0.051, p = 0.687). The n1 latency was negatively correlated with maximum changes in systolic blood pressure within 15 s during HUT in patients with PD (r = -0.335, p = 0.040) but not in those with MSA (r = 0.277, p = 0.299).
Conclusion
Bilaterally abnormal oVEMP responses may indicate the extent of brainstem dysfunction in MSA. oVEMP reflects the integrity of otolith-autonomic interplay, reliably assists in differentiating between MSA and PD, and helps infer clinical decline.

Citations

Citations to this article as recorded by

Vestibular Evoked Myogenic Potentials (VEMPs) in Parkinson’s Disease Patients with Monopolar Deep Brain Stimulation
Kim E. Hawkins, John Holden, Elodie Chiarovano, Simon J. G. Lewis, Ian S. Curthoys, Hamish G. MacDougall
Signals.2025; 6(1): 10. CrossRef
Update on Clinical Physiology and Pathomechanisms for Vestibulo-Autonomic Interplay
Sun-Uk Lee, Jeong-Yoon Choi
The Cerebellum.2025;[Epub] CrossRef
Selective otolithic dysfunction in patients presenting with acute spontaneous vertigo: consideration based on MRI
Keun-Tae Kim, Sangeun Park, Sun-Uk Lee, Euyhyun Park, Byungjun Kim, Ji-Soo Kim
Frontiers in Neurology.2024;[Epub] CrossRef

Letters to the editor

NBR and GBA Gene Methylation Levels in the Peripheral Blood of Parkinson’s Disease Patients: Yagmur Inalkac Gemici, Ahmet Koc; J Mov Disord. 2024;17(4):456-458. Published online July 22, 2024; DOI: https://doi.org/10.14802/jmd.24111; Funded: Inonu University

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Optimal Measurement Height and Validation of a 2D-Light Detection and Ranging Device-Based Analysis System for Spatiotemporal Gait Parameters: Seungki Woo, Chaewon Shin, Min Young Kim; J Mov Disord. 2024;17(4):459-461. Published online August 21, 2024; DOI: https://doi.org/10.14802/jmd.24134; Funded: Chungnam National University Sejong Hospital, National Research Foundation of Korea, Ministry of Science and ICT

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Brief communications

Journey Through Autosomal-Recessive Spastic Ataxia of Charlevoix–Saguenay: Insights From a Case Series of Seven Patients–A Single-Center Study and Review of an Indian Cohort: Mit Ankur Raval, Vikram V Holla, Nitish Kamble, Gautham Arunachal, Babylakshmi Muthusamy, Jitender Saini, Ravi Yadav, Pramod Kumar Pal; J Mov Disord. 2024;17(4):430-435. Published online August 29, 2024; DOI: https://doi.org/10.14802/jmd.24154; Funded: Department of Biotechnology, Government of India

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Abstract PDF Supplementary Material: Objective
In this study, we describe the clinical and investigative profiles of 7 cases of autosomal-recessive spastic ataxia of Charlevoix–Saguenay (ARSACS).
Methods
We performed a retrospective chart review of genetically proven cases of ARSACS from our database. Additionally, we reviewed the literature for reported cases of ARSACS from India.
Results
All 7 patients experienced disease onset within the first decade of life. According to the available data, all patients had walking difficulty (7/7), spastic ataxia (7/7), classical neuroimaging findings (7/7), sensory‒motor demyelinating polyneuropathy (6/6), abnormal evoked potentials (5/5), and a thickened retinal nerve fiber layer (3/3). Exome sequencing revealed 8 unique pathogenic/likely pathogenic variants (6 novel) in the SACS gene. An additional 21 cases (18 families) of ARSACS that could be identified from India had similar clinical and investigational findings. The most common c.8793delA variant may have a founder effect.
Conclusion
Our series adds to the previously reported cases of ARSACS from India and expands the genetic spectrum by adding 6 novel variants.

Clinico-Genetic Profiles of Seven Patients With PINK1-Related Parkinson’s Disease: A Case Series From a Tertiary Care Centre in India and a Review of the Literature: Aravind Gunasekaran, Vikram V Holla, Prashant Phulpagar, Sneha D Kamath, Nitish Kamble, Ravi Yadav, Babylakshmi Muthusamy, Pramod Kumar Pal; J Mov Disord. 2024;17(4):436-441. Published online September 19, 2024; DOI: https://doi.org/10.14802/jmd.24157; Funded: Department of Biotechnology, Government of India

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Abstract PDF Supplementary Material: Objective
Recessive variants in the PINK1 gene are known causes of early-onset Parkinson’s disease (EOPD). To describe the clinical features and genetic profiles of patients with PINK1-related Parkinson’s disease (PARK-PINK1) mutations.
Methods
We conducted a retrospective chart review of the demographic, clinical and genetic details of patients from our database carrying biallelic PINK1 variants.
Results
A total of 7 patients whose median age at onset was 33 years (range: 20–49) were recruited. All had asymmetrical onset, tremors were present in 4 patients, abnormal posturing was present in 2 patients, and slowness was present in 1 patient. The parkinsonism phenotype was noted in 6 patients (with dystonia in four) and isolated dystonia in one. Among the 6 patients with parkinsonism, five had rest tremors, all had good levodopa responses, and four had motor fluctuations with choreiform dyskinesia. Exome sequencing revealed biallelic pathogenic/likely pathogenic variants, five of which were novel.
Conclusion
PARK-PINK1 presents as an EOPD with tremor-predominant phenotype, good levodopa-responsiveness, early motor fluctuation and dyskinesia. We describe five novel variants in PINK1 gene.

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; Funded: Ricerca Corrente

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Abstract PDF Supplementary 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.

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