Journal of Movement Disorders

Sun Ju Chung

23 Articles

Evaluating the Validity and Reliability of the Korean Version of the Scales for Outcomes in Parkinson’s Disease–Cognition: Jinse Park, Eungseok Oh, Seong-Beom Koh, In-Uk Song, Tae-Beom Ahn, Sang Jin Kim, Sang-Myung Cheon, Yoon-Joong Kim, Jin Whan Cho, Hyeo-Il Ma, Mee Young Park, Jong Sam Baik, Phil Hyu Lee, Sun Ju Chung, Jong-Min Kim, Han-Joon Kim, Young-Hee Sung, Do Young Kwon, Jae-Hyeok Lee, Jee-Young Lee, Ji Seon Kim, Ji Young Yun, Hee Jin Kim, Jin Yong Hong, Mi-Jung Kim, Jinyoung Youn, Hui-Jun Yang, Won Tae Yoon, Sooyeoun You, Kyum-Yil Kwon, Su-Yun Lee, Younsoo Kim, Hee-Tae Kim, Joong-Seok Kim, Ji-Young Kim; J Mov Disord. 2024;17(3):328-332. Published online April 3, 2024; DOI: https://doi.org/10.14802/jmd.24061

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Abstract PDF Supplementary Material: Objective
The Scales for Outcomes in Parkinson’s Disease–Cognition (SCOPA-Cog) was developed to assess cognition in patients with Parkinson’s disease (PD). In this study, we aimed to evaluate the validity and reliability of the Korean version of the SCOPACog (K-SCOPA-Cog).
Methods
We enrolled 129 PD patients with movement disorders from 31 clinics in South Korea. The original version of the SCOPA-Cog was translated into Korean using the translation-retranslation method. The test–retest method with an intraclass correlation coefficient (ICC) and Cronbach’s alpha coefficient were used to assess reliability. Spearman’s rank correlation analysis with the Montreal Cognitive Assessment-Korean version (MOCA-K) and the Korean Mini-Mental State Examination (K-MMSE) were used to assess concurrent validity.
Results
The Cronbach’s alpha coefficient was 0.797, and the ICC was 0.887. Spearman’s rank correlation analysis revealed a significant correlation with the K-MMSE and MOCA-K scores (r = 0.546 and r = 0.683, respectively).
Conclusion
Our results demonstrate that the K-SCOPA-Cog has good reliability and validity.

Caregiver Burden of Patients With Huntington’s Disease in South Korea: Chan Young Lee, Chaewon Shin, Yun Su Hwang, Eungseok Oh, Manho Kim, Hyun Sook Kim, Sun Ju Chung, Young Hee Sung, Won Tae Yoon, Jin Whan Cho, Jae-Hyeok Lee, Han-Joon Kim, Hee Jin Chang, Beomseok Jeon, Kyung Ah Woo, Seong-Beom Koh, Kyum-Yil Kwon, Jangsup Moon, Young Eun Kim, Jee-Young Lee; J Mov Disord. 2024;17(1):30-37. Published online September 11, 2023; DOI: https://doi.org/10.14802/jmd.23134

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Objective
This is the first prospective cohort study of Huntington’s disease (HD) in Korea. This study aimed to investigate the caregiver burden in relation to the characteristics of patients and caregivers.
Methods
From August 2020 to February 2022, we enrolled patients with HD from 13 university hospitals in Korea. We used the 12-item Zarit Burden Interview (ZBI-12) to evaluate the caregiver burden. We evaluated the clinical associations of the ZBI-12 scores by linear regression analysis and investigated the differences between the low- and high-burden groups.
Results
Sixty-five patients with HD and 45 caregivers were enrolled in this cohort study. The average age at onset of motor symptoms was 49.3 ± 12.3 years, with an average cytosine-adenine-guanine (CAG)n of 42.9 ± 4.0 (38–65). The median ZBI-12 score among our caregivers was 17.6 ± 14.2. A higher caregiver burden was associated with a more severe Shoulson–Fahn stage (p = 0.038) of the patients. A higher ZBI-12 score was also associated with lower independence scale (B = -0.154, p = 0.006) and functional capacity (B = -1.082, p = 0.002) scores of patients. The caregiving duration was longer in the high- than in the low-burden group. Caregivers’ demographics, blood relation, and marital and social status did not affect the burden significantly.
Conclusion
HD patients’ neurological status exerts an enormous impact on the caregiver burden regardless of the demographic or social status of the caregiver. This study emphasizes the need to establish an optimal support system for families dealing with HD in Korea. A future longitudinal analysis could help us understand how disease progression aggravates the caregiver burden throughout the entire disease course.

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A Practical Guide for Clinical Approach to Patients With Huntington’s Disease in Korea
Chaewon Shin, Ryul Kim, Dallah Yoo, Eungseok Oh, Jangsup Moon, Minkyeong Kim, Jee-Young Lee, Jong-Min Kim, Seong-Beom Koh, Manho Kim, Beomseok Jeon
Journal of Movement Disorders.2024; 17(2): 138. CrossRef

GBA1 Variants and Parkinson’s Disease: Paving the Way for Targeted Therapy: Young Eun Huh, Tatiana Usnich, Clemens R. Scherzer, Christine Klein, Sun Ju Chung; J Mov Disord. 2023;16(3):261-278. Published online June 12, 2023; DOI: https://doi.org/10.14802/jmd.23023

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Glucosylceramidase beta 1 (GBA1) variants have attracted enormous attention as the most promising and important genetic candidates for precision medicine in Parkinson’s disease (PD). A substantial correlation between GBA1 genotypes and PD phenotypes could inform the prediction of disease progression and promote the development of a preventive intervention for individuals at a higher risk of a worse disease prognosis. Moreover, the GBA1-regulated pathway provides new perspectives on the pathogenesis of PD, such as dysregulated sphingolipid metabolism, impaired protein quality control, and disrupted endoplasmic reticulum-Golgi trafficking. These perspectives have led to the development of novel disease-modifying therapies for PD targeting the GBA1-regulated pathway by repositioning treatment strategies for Gaucher’s disease. This review summarizes the current hypotheses on a mechanistic link between GBA1 variants and PD and possible therapeutic options for modulating GBA1-regulated pathways in PD patients.

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A Comparative Biochemical and Pathological Evaluation of Brain Samples from Knock-In Murine Models of Gaucher Disease
Makaila L. Furderer, Bahafta Berhe, Tiffany C. Chen, Stephen Wincovitch, Xuntian Jiang, Nahid Tayebi, Ellen Sidransky, Tae-Un Han
International Journal of Molecular Sciences.2024; 25(3): 1827. CrossRef
Towards a Global View of Parkinson's Disease Genetics
Marzieh Khani, Catalina Cerquera‐Cleves, Mariam Kekenadze, Peter Wild Crea, Andrew B. Singleton, Sara Bandres‐Ciga
Annals of Neurology.2024; 95(5): 831. CrossRef
Exploring the Association between Cathepsin B and Parkinson’s Disease
Changhao Lu, Xinyi Cai, Shilin Zhi, Xiaofen Wen, Jiaxin Shen, Tommaso Ercoli, Elena Rita Simula, Carla Masala, Leonardo A. Sechi, Paolo Solla
Brain Sciences.2024; 14(5): 482. CrossRef
Clinical, mechanistic, biomarker, and therapeutic advances in GBA1-associated Parkinson’s disease
Xuxiang Zhang, Heng Wu, Beisha Tang, Jifeng Guo
Translational Neurodegeneration.2024;[Epub] CrossRef

Association of Depression With Early Occurrence of Postural Instability in Parkinson’s Disease: Yun Su Hwang, Sungyang Jo, Kye Won Park, Seung Hyun Lee, Sangjin Lee, Sun Ju Chung; J Mov Disord. 2023;16(1):68-78. Published online December 20, 2022; DOI: https://doi.org/10.14802/jmd.22091

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Abstract PDF Supplementary Material: Objective
Depression in Parkinson’s disease (PD) affects the quality of life of patients. Postural instability and gait disturbance are associated with the severity and prognosis of PD. We investigated the association of depression with axial involvement in early-stage PD patients.
Methods
This study involved 95 PD patients unexposed to antiparkinsonian drugs. After a baseline assessment for depression, the subjects were divided into a depressed PD group and a nondepressed PD group. Analyses were conducted to identify an association of depression at baseline with the following outcome variables: the progression to Hoehn and Yahr scale (H-Y) stage 3, the occurrence of freezing of gait (FOG), levodopa-induced dyskinesia, and wearing-off. The follow-up period was 53.40 ± 16.79 months from baseline.
Results
Kaplan–Meier survival curves for H-Y stage 3 and FOG showed more prominent progression to H-Y stage 3 and occurrences of FOG in the depressed PD group than in the nondepressed PD group (log-rank p = 0.025 and 0.003, respectively). Depression in drug-naïve, early-stage PD patients showed a significant association with the progression to H-Y stage 3 (hazard ratio = 2.55; 95% confidence interval = 1.32–4.93; p = 0.005), as analyzed by Cox regression analyses. In contrast, the occurrence of levodopa-induced dyskinesia and wearing-off did not differ between the two groups (log-rank p = 0.903 and 0.351, respectively).
Conclusion
Depression in drug-naïve, early-stage PD patients is associated with an earlier occurrence of postural instability. This suggests shared nondopaminergic pathogenic mechanisms and potentially enables the prediction of early development of postural instability.

The Effect of Blood Lipids, Type 2 Diabetes, and Body Mass Index on Parkinson’s Disease: A Korean Mendelian Randomization Study: Kye Won Park, Yun Su Hwang, Seung Hyun Lee, Sungyang Jo, Sun Ju Chung; J Mov Disord. 2023;16(1):79-85. Published online January 12, 2023; DOI: https://doi.org/10.14802/jmd.22175

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Objective
Associations between various metabolic conditions and Parkinson’s disease (PD) have been previously identified in epidemiological studies. We aimed to investigate the causal effect of lipid levels, type 2 diabetes mellitus (T2DM), and body mass index (BMI) on PD in a Korean population via Mendelian randomization (MR).
Methods
Two-sample MR analyses were performed with inverse-variance weighted (IVW), weighted median, and MR-Egger regression approaches. We identified genetic variants associated with lipid concentrations, T2DM, and BMI in publicly available summary statistics, which were either collected from genome-wide association studies (GWASs) or from meta-analyses of GWAS that targeted only Korean individuals or East Asian individuals, including Korean individuals. The outcome dataset was a GWAS on PD performed in a Korean population.
Results
From previous GWASs and meta-analyses, we selected single nucleotide polymorphisms as the instrumental variables. Variants associated with serum levels of low-density lipoprotein cholesterol, high-density lipoprotein cholesterol, and triglycerides, as well as with T2DM and BMI, were selected (n = 11, 19, 17, 89, and 9, respectively). There were no statistically significant causal associations observed between the five exposures and PD using either the IVW, weighted median, or MR-Egger methods (p-values of the IVW method: 0.332, 0.610, 0.634, 0.275, and 0.860, respectively).
Conclusion
This study does not support a clinically relevant causal effect of lipid levels, T2DM, and BMI on PD risk in a Korean population.

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Causal effect of systemic lupus erythematosus on psychiatric disorders: A two-sample Mendelian randomization study
Hua Xue, Shuangjuan Liu, Li Zeng, Wenhui Fan
Journal of Affective Disorders.2024; 347: 422. CrossRef
Causal relationship between diabetes mellitus, glycemic traits and Parkinson’s disease: a multivariable mendelian randomization analysis
Qitong Wang, Benchi Cai, Lifan Zhong, Jitrawadee Intirach, Tao Chen
Diabetology & Metabolic Syndrome.2024;[Epub] CrossRef
Association of Body Mass Index and Parkinson Disease
Cloé Domenighetti, Pierre-Emmanuel Sugier, Ashwin Ashok Kumar Sreelatha, Claudia Schulte, Sandeep Grover, Berta Portugal, Pei-Chen Lee, Patrick May, Dheeraj Bobbili, Milena Radivojkov Blagojevic, Peter Lichtner, Andrew B. Singleton, Dena Hernandez, Connor
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Causal association between common rheumatic diseases and arrhythmia: a Mendelian randomization study
Yuchen Zhang, Ling Tang, Ke Zhang, Xinai Meng, Tian Liu, Yanjia Chen, Xingfu Huang
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Unraveling the link: exploring the causal relationship between diabetes, multiple sclerosis, migraine, and Alzheimer’s disease through Mendelian randomization
Hua Xue, Li Zeng, Shuangjuan Liu
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Glycated hemoglobin A1c, cerebral small vessel disease burden, and disease severity in Parkinson's disease
Xinxin Ma, Shuhua Li, Fengzhi Liu, Yu Du, Haibo Chen, Wen Su
Annals of Clinical and Translational Neurology.2023; 10(12): 2276. CrossRef

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

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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.

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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
Surgical treatment of movement disorders in neurometabolic conditions
Alonso Zea Vera, Andrea L. Gropman
Frontiers in Neurology.2023;[Epub] CrossRef

Syringomyelia Manifesting With Pseudodystonia: A Case Report: Yun Su Hwang, Seung Hyun Lee, Sungyang Jo, Sun Ju Chung; J Mov Disord. 2022;15(2):175-177. Published online November 3, 2021; DOI: https://doi.org/10.14802/jmd.21121

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Pseudodystonia and Neuropathic Tremor in a Patient With Monomelic Amyotrophy: Seung Hyun Lee, Yun Su Hwang, Sungyang Jo, Sun Ju Chung; J Mov Disord. 2022;15(2):181-183. Published online March 22, 2022; DOI: https://doi.org/10.14802/jmd.21138

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Factors Associated with Medication Beliefs in Patients with Parkinson’s Disease: A Cross-Sectional Study: Sung Reul Kim, Ji Young Kim, Hye Young Kim, Hui Young So, Sun Ju Chung; J Mov Disord. 2021;14(2):133-143. Published online May 3, 2021; DOI: https://doi.org/10.14802/jmd.20147

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Objective
Medication beliefs are a significant determinant of medication adherence in chronic illness. This study aimed to identify demographic, clinical, and medication-related factors associated with medication beliefs in patients with Parkinson’s disease (PD).
Methods
We used a descriptive cross-sectional design with a convenience sample of 173 PD patients who had been taking antiparkinson drugs for more than one year.
Results
The subjects who believed PD medication was more necessary had more severe illness, younger age of onset, longer illness duration, and longer duration of levodopa therapy. They had higher levels of non-motor symptoms and depression, number of medication uses, number of drugs, and levodopa equivalent dose, and they reported fluctuation of motor symptoms and dyskinesia. The subjects who used catechol-O-methyltransferase (COMT) inhibitors, dopamine agonists, amantadine, and monoamine oxidase-B (MAO-B) inhibitors had significantly higher necessity scores than those who did not use them. The subjects who had higher concerns about PD medications had higher levels of non-motor symptoms and depression. The subjects using amantadine and anticholinergics had significantly higher concern scores than those who did not use them. Positive necessity-concerns differentials were associated with severe illness, the presence of motor fluctuation and dyskinesia, and the use of COMT inhibitors. Based on stepwise multiple regression, the most significant factors influencing necessity beliefs were severe illness, followed by depression and motor fluctuation.
Conclusion
Severe illness, higher levels of depression, and motor fluctuation are independent factors influencing patients’ beliefs regarding medication necessity. Therefore, these characteristics should be considered in medication belief assessment and interventions for PD patients.

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Adherence to Parkinson's disease medication: A case study to illustrate reasons for non-adherence, implications for practice and engaging under-represented participants in research
Delyth James, Joshua Smith, Emma Lane, Rhian Thomas, Sarah Brown, Heidi Seage
Exploratory Research in Clinical and Social Pharmacy.2024; 14: 100450. CrossRef
Relationship Between Medication Literacy and Beliefs Among Persons with Type 2 Diabetes Mellitus in Guangdong, China
Sifen Jiang, Zhuoqi Zhu, Guisheng Liao, Yanling Huang, Lichang Li, Kun Zeng
Patient Preference and Adherence.2023; Volume 17: 2039. CrossRef
Effect of Pillbox Organizers with Alarms on Adherence to Pharmacotherapy in Parkinson Disease Patients Taking Three and More Daily Doses of Dopaminergic Medications
Igor Straka, Michal Minar, Milan Grofik, Matej Skorvanek, Veronika Bolekova, Andrea Gazova, Jan Kyselovic, Peter Valkovic
Journal of Personalized Medicine.2022; 12(2): 179. CrossRef
Factors Related to Beliefs about Medication in Ischemic Stroke Patients
Gye-Gyoung Kim, Sung-Hee Yoo, Man-Seok Park, Hyun-Young Park, Jae-Kwan Cha
Journal of Clinical Medicine.2022; 11(13): 3825. CrossRef
Lycium barbarum polysaccharide improves dopamine metabolism and symptoms in an MPTP-induced model of Parkinson’s disease
Jiangbo Song, Lian Liu, Zhiquan Li, Ting Mao, Jianfei Zhang, Lei Zhou, Xin Chen, Yunzhu Shang, Tao Sun, Yuxin Luo, Yu Jiang, Duan Tan, Xiaoling Tong, Fangyin Dai
BMC Medicine.2022;[Epub] CrossRef

Therapeutic Effect of Levodopa/Carbidopa/Entacapone on Sleep Disturbance in Patients with Parkinson’s Disease: Kye Won Park, Sungyang Jo, Seung Hyun Lee, Yun Su Hwang, Dagyo Lee, Ho-Sung Ryu, Sun Ju Chung; J Mov Disord. 2020;13(3):205-212. Published online September 9, 2020; DOI: https://doi.org/10.14802/jmd.20055

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Objective
To investigate the efficacy of levodopa/carbidopa/entacapone (LCE) at bedtime for treating sleep disturbance in patients with Parkinson’s disease (PD) with motor fluctuations.
Methods
Participants included 128 PD patients with motor fluctuations. All patients were assessed for motor, nonmotor, and sleep-specific symptoms using the United Parkinson’s Disease Rating Scale (UPDRS), the Korean version of the Nonmotor Symptom Scale, the Parkinson’s Disease Sleep Scale (PDSS), the Epworth Sleepiness Scale, and the Rapid Eye Movement Sleep Behavior Disorder Screening Questionnaire (RBDSQ). We compared the baseline characteristics of patients with sleep disturbance (PDSS score < 120) and those without sleep disturbance (PDSS score ≥ 120). Thirty-nine patients with sleep disturbance who agreed to take LCE at bedtime completed 3-month follow-ups. We analyzed changes in the scores of motor, nonmotor, and sleep symptom scales over the 3 months.
Results
PD patients with sleep disturbance were at more advanced disease stages and had more severe motor, nonmotor, and sleep symptoms than those without sleep disturbance. Patients who took LCE at night showed improvements in motor (UPDRS part III, p = 0.007) and sleep symptoms (total PDSS, p < 0.001). Sleep features that benefitted from LCE included not only nocturnal motor components but also insomnia (PDSS items 2 and 3, p = 0.005 and p < 0.001) and rapid eye movement behavior disorder (PDSS item 6, p = 0.002; and RBDSQ, p < 0.001).
Conclusion
The use of LCE at bedtime may be a useful treatment for sleep disturbance in advanced PD patients with motor fluctuations.

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Comparison of sleep characteristics between Parkinson's disease with and without freezing of gait: A systematic review
Tracy Milane, Clint Hansen, Mathias Baptiste Correno, Matthias Chardon, Fabio A. Barbieri, Edoardo Bianchini, Nicolas Vuillerme
Sleep Medicine.2024; 114: 24. CrossRef
Sleep and sleep disorders in people with Parkinson's disease
Alex Iranzo, Valerie Cochen De Cock, María Livia Fantini, Laura Pérez-Carbonell, Lynn Marie Trotti
The Lancet Neurology.2024; 23(9): 925. CrossRef
Pharmacokinetics and Dose Proportionality Study of a Novel Antiparkinsonian Agent, a 1H-1,2,4-Triazol-3-ylthio-conjugate of Prottremine
Daria S. Gorina, Anastasiya V. Lastovka, Artem D. Rogachev, Alexandra V. Podturkina, Alla V. Pavlova, Oleg V. Ardashov, Nikolai S. Li-Zhulanov, Tatyana G. Tolstikova, Konstantin P. Volcho, Nariman F. Salakhutdinov
Molecules.2024; 29(18): 4498. CrossRef
Opicapone versus entacapone: Head‐to‐head retrospective data‐based comparison of healthcare resource utilization in people with Parkinson's disease new to catechol‐O‐methyltransferase (COMT) inhibitor treatment
Glynn Harrison‐Jones, Xiaocong Li Marston, Francesca Morgante, K. Ray Chaudhuri, Guillermo Castilla‐Fernández, Valentina Di Foggia
European Journal of Neurology.2023; 30(10): 3132. CrossRef
Management of REM sleep behavior disorder: an American Academy of Sleep Medicine systematic review, meta-analysis, and GRADE assessment
Michael Howell, Alon Y. Avidan, Nancy Foldvary-Schaefer, Roneil G. Malkani, Emmanuel H. During, Joshua P. Roland, Stuart J. McCarter, Rochelle S. Zak, Gerard Carandang, Uzma Kazmi, Kannan Ramar
Journal of Clinical Sleep Medicine.2023; 19(4): 769. CrossRef
The real-life effect of catechol-O-methyltransferase inhibition on non-motor symptoms in levodopa-treated Parkinson’s disease: opicapone versus entacapone
Valentina Leta, Daniel J. van Wamelen, Federico Aureli, Vinod Metta, Dhaval Trivedi, Pietro Cortelli, Carmen Rodriguez-Blazquez, Alexandra Rizos, K. Ray Chaudhuri
Journal of Neural Transmission.2023; 130(7): 925. CrossRef
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P. Tall, M. A. Qamar, L. Batzu, V. Leta, C. Falup-Pecurariu, K. Ray Chaudhuri
Journal of Neural Transmission.2023; 130(11): 1443. CrossRef
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Di Zhang, Wenjing Zhang, Shumin Deng, Lu Liu, Hua Wei, Fenqin Xue, Hui Yang, Xiaomin Wang, Zheng Fan
Biomedicine & Pharmacotherapy.2023; 165: 115259. CrossRef
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Subramanian Thangaleela, Bhagavathi Sundaram Sivamaruthi, Periyanaina Kesika, Subramanian Mariappan, Subramanian Rashmi, Thiwanya Choeisoongnern, Phakkharawat Sittiprapaporn, Chaiyavat Chaiyasut
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Heinz Reichmann
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Karina A. Atanasova-Ivanova, Sonya Ivanova Hristova-Chakmakova, Ivan G. Milanov
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The Home-Based Sleep Laboratory
Yael Hanein, Anat Mirelman, Anat Mirelman, E. Ray Dorsey, Patrik Brundin, Bastiaan R. Bloem
Journal of Parkinson's Disease.2021; 11(s1): S71. CrossRef
Shudi Pingchan Decoction combined with repetitive transcranial magnetic stimulation in the treatment of Parkinson’s disease with sleep disorders
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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

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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.

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Effects of subthalamic nucleus deep brain stimulation using different frequency programming paradigms on axial symptoms in advanced Parkinson’s disease
Yifeng Cheng, Guangrui Zhao, Lei Chen, Deqiu Cui, Chunjuan Wang, Keke Feng, Shaoya Yin
Acta Neurochirurgica.2024;[Epub] CrossRef
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Yun Su Hwang, Sungyang Jo, Seung Hyun Lee, Nayoung Kim, Mi-Sun Kim, Sang Ryong Jeon, Sun Ju Chung
Journal of the Neurological Sciences.2023; 444: 120484. CrossRef
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WITHDRAWN: Laterality and frequency settings of subthalamic nucleus DBS for Parkinson's disease: A systematic review and network meta-analysis
Rajiv Dharnipragada, Lalitha S. Denduluri, Anant Naik, Mario Bertogliat, Matthew Awad, Salman Ikramuddin, Michael C. Park
Parkinsonism & Related Disorders.2023; : 105455. CrossRef
Frequency settings of subthalamic nucleus DBS for Parkinson's disease: A systematic review and network meta-analysis
Rajiv Dharnipragada, Lalitha S. Denduluri, Anant Naik, Mario Bertogliat, Matthew Awad, Salman Ikramuddin, Michael C. Park
Parkinsonism & Related Disorders.2023; 116: 105809. CrossRef
Unlocking potential: low frequency subthalamic nucleus stimulation enhances executive function in Parkinson’s disease patients with postural instability/gait disturbance
Guofan Qin, Hutao Xie, Lin Shi, Baotian Zhao, Yifei Gan, Zixiao Yin, Yichen Xu, Xin Zhang, Yaojing Chen, Yin Jiang, Quan Zhang, Jianguo Zhang
Frontiers in Neuroscience.2023;[Epub] CrossRef
The Role of Microelectrode Recording in Deep Brain Stimulation Surgery for Parkinson’s Disease: A Systematic Review and Meta-Analysis
R. Saman Vinke, Martin Geerlings, Ashok K. Selvaraj, Dejan Georgiev, Bastiaan R. Bloem, Rianne A.J. Esselink, Ronald H.M.A. Bartels
Journal of Parkinson's Disease.2022; 12(7): 2059. CrossRef
Axial impairment and falls in Parkinson’s disease: 15 years of subthalamic deep brain stimulation
Alessandro Zampogna, Francesco Cavallieri, Francesco Bove, Antonio Suppa, Anna Castrioto, Sara Meoni, Pierre Pélissier, Emmanuelle Schmitt, Amélie Bichon, Eugénie Lhommée, Andrea Kistner, Stephan Chabardès, Eric Seigneuret, Valerie Fraix, Elena Moro
npj Parkinson's Disease.2022;[Epub] CrossRef

Fragile X-Associated Tremor/Ataxia Syndrome: An Illustrative Case: Chaewon Lee, Kye Won Park, Nari Choi, Ho-Sung Ryu, Sun Ju Chung; J Mov Disord. 2019;12(3):184-186. Published online July 17, 2019; DOI: https://doi.org/10.14802/jmd.18060

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Alteration in the Local and Global Functional Connectivity of Resting State Networks in Parkinson’s Disease: Maryam Ghahremani, Jaejun Yoo, Sun Ju Chung, Kwangsun Yoo, Jong C. Ye, Yong Jeong; J Mov Disord. 2018;11(1):13-23. Published online January 23, 2018; DOI: https://doi.org/10.14802/jmd.17061

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Abstract PDF

Objective
Parkinson’s disease (PD) is a neurodegenerative disorder that mainly leads to the impairment of patients’ motor function, as well as of cognition, as it progresses. This study tried to investigate the impact of PD on the resting state functional connectivity of the default mode network (DMN), as well as of the entire brain.
Methods
Sixty patients with PD were included and compared to 60 matched normal control (NC) subjects. For the local connectivity analysis, the resting state fMRI data were analyzed by seed-based correlation analyses, and then a novel persistent homology analysis was implemented to examine the connectivity from a global perspective.
Results
The functional connectivity of the DMN was decreased in the PD group compared to the NC, with a stronger difference in the medial prefrontal cortex. Moreover, the results of the persistent homology analysis indicated that the PD group had a more locally connected and less globally connected network compared to the NC.
Conclusion
Our findings suggest that the DMN is altered in PD, and persistent homology analysis, as a useful measure of the topological characteristics of the networks from a broader perspective, was able to identify changes in the large-scale functional organization of the patients’ brain.

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Topological disruption of high‐order functional networks in cognitively preserved Parkinson's disease
Song'an Shang, Siying Zhu, Jingtao Wu, Yao Xu, Lanlan Chen, Weiqiang Dou, Xindao Yin, Yu‐Chen Chen, Dejuan Shen, Jing Ye
CNS Neuroscience & Therapeutics.2023; 29(2): 566. CrossRef
IABC: A Toolbox for Intelligent Analysis of Brain Connectivity
Yuhui Du, Yanshu Kong, Xingyu He
Neuroinformatics.2023; 21(2): 303. CrossRef
Topological data analysis in biomedicine: A review
Yara Skaf, Reinhard Laubenbacher
Journal of Biomedical Informatics.2022; 130: 104082. CrossRef
Altered Long- and Short-Range Functional Connectivity Density in Patients With Thyroid-Associated Ophthalmopathy: A Resting-State fMRI Study
Wen-Hao Jiang, Huan-Huan Chen, Wen Chen, Qian Wu, Lu Chen, Jiang Zhou, Xiao-Quan Xu, Hao Hu, Fei-Yun Wu
Frontiers in Neurology.2022;[Epub] CrossRef
Modulations of static and dynamic functional connectivity among brain networks by electroacupuncture in post-stroke aphasia
Minjie Xu, Ying Gao, Hua Zhang, Binlong Zhang, Tianli Lyu, Zhongjian Tan, Changming Li, Xiaolin Li, Xing Huang, Qiao Kong, Juan Xiao, Georg S. Kranz, Shuren Li, Jingling Chang
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Multi-dimensional persistent feature analysis identifies connectivity patterns of resting-state brain networks in Alzheimer’s disease
Jin Li, Chenyuan Bian, Haoran Luo, Dandan Chen, Luolong Cao, Hong Liang
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Characterizing resting‐state networks in Parkinson’s disease: A multi‐aspect functional connectivity study
Mahdieh Ghasemi, Ali Foroutannia, Abbas Babajani‐Feremi
Brain and Behavior.2021;[Epub] CrossRef
The role of the medial prefrontal cortex in cognition, ageing and dementia
Dan D Jobson, Yoshiki Hase, Andrew N Clarkson, Rajesh N Kalaria
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Image Target Recognition Model of Multi- Channel Structure Convolutional Neural Network Training Automatic Encoder
Sen Zhang, Qiuyun Cheng, Dengxi Chen, Haijun Zhang
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Sleepiness and Depression in Parkinson’s Disease Patients Treated with Ropinirole and Levodopa: Suk Yun Kang, Ho-Sung Ryu, Mun-Kyung Sunwoo, Sang-Jin Kim, Jong-Sam Baik, Mee-Young Park, Hyung-Eun Park, Joong-Seok Kim, Kyum-Yil Kwon, Seong-Beom Koh, Young-Eun Kim, Mi-Kyong Lee, Jong-Min Kim, Sun Ju Chung, Young-Ho Sohn; J Mov Disord. 2017;10(3):123-129. Published online September 22, 2017; DOI: https://doi.org/10.14802/jmd.17048

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Abstract PDF

Objective
We aimed to investigate the effect of ropinirole on excessive daytime sleepiness (EDS) and depression in Parkinson’s disease (PD) with a large population.
Methods
We conducted a cross-sectional observational study at nine hospitals in Korea between April 24, 2013, and April 22, 2015. We analyzed the demographic and clinical features, other medical history, history of antiparkinsonian medication within 6 months, Hoehn and Yahr stage (HY stage), Unified Parkinson’s Disease Rating Scale (UPDRS) part II and III, Epworth Sleepiness Scale (ESS), and 30-item Geriatric Depression Scale (GDS-30).
Results
Four-hundred-thirteen patients with PD (mean age: 65.2 ± 9.0 years; men: 227 patients) were analyzed. Multivariate logistic regression analysis showed that age at examination, UPDRS II, and GDS-30 were independent risk factors for EDS and that sex, UPDRS II, and ESS were independent risk factors for depression.
Conclusion
Our large group study did not find any significant associations of ropinirole with EDS and depression in Korean PD patients.

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Associations between non-motor symptoms and patient characteristics in Parkinson’s disease: a multicenter cross-sectional study
Remi Morimoto, Mutsumi Iijima, Yasuyuki Okuma, Keisuke Suzuki, Fumihito Yoshii, Shigeru Nogawa, Takashi Osada, Kazuo Kitagawa
Frontiers in Aging Neuroscience.2023;[Epub] CrossRef
Excessive Daytime Sleepiness in Parkinson’s Disease
Hanshu Liu, Jingwen Li, Xinyi Wang, Jinsha Huang, Tao Wang, Zhicheng Lin, Nian Xiong
Nature and Science of Sleep.2022; Volume 14: 1589. CrossRef
Excessive daytime sleepiness in Parkinson's disease: A systematic review and meta-analysis
Fei Feng, YingYing Cai, YanBing Hou, Ruwei Ou, Zheng Jiang, HuiFang Shang
Parkinsonism & Related Disorders.2021; 85: 133. CrossRef
Sleep Disorders and Cognitive Dysfunctions in Parkinson’s Disease: A Meta-Analytic Study
Gianpaolo Maggi, Luigi Trojano, Paolo Barone, Gabriella Santangelo
Neuropsychology Review.2021; 31(4): 643. CrossRef
Longitudinal risk factors for developing depressive symptoms in Parkinson's disease
Tarek Antar, Huw R. Morris, Faraz Faghri, Hampton L. Leonard, Mike A. Nalls, Andrew B. Singleton, Hirotaka Iwaki
Journal of the Neurological Sciences.2021; 429: 117615. CrossRef
The effect and safety of ropinirole in the treatment of Parkinson disease
Jiali Zhu, Min Chen
Medicine.2021; 100(46): e27653. CrossRef
Pramipexole regulates depression-like behavior via dopamine D3 receptor in a mouse model of Parkinson’s disease
Shi-Zhuang Wei, Xiao-Yu Yao, Chen-Tao Wang, An-Qi Dong, Dan Li, Yu-Ting Zhang, Chao Ren, Jin-Bao Zhang, Cheng-Jie Mao, Fen Wang, Chun-Feng Liu
Brain Research Bulletin.2021; 177: 363. CrossRef
A selective D2 dopamine receptor agonist alleviates depression through up-regulation of tyrosine hydroxylase and increased neurogenesis in hippocampus of the prenatally stressed rats
Mahino Fatima, Mir Hilal Ahmad, Saurabh Srivastav, Moshahid Alam Rizvi, A.C. Mondal
Neurochemistry International.2020; 136: 104730. CrossRef
‘Dopamine agonist Phobia’ in Parkinson’s disease: when does it matter? Implications for non-motor symptoms and personalized medicine
Silvia Rota, Iro Boura, Lucia Batzu, Nataliya Titova, Peter Jenner, Cristian Falup-Pecurariu, K Ray Chaudhuri
Expert Review of Neurotherapeutics.2020; 20(9): 953. CrossRef
An Investigation on the Clinical Features and Neurochemical Changes in Parkinson's Disease With Depression
Teng-Hong Lian, Peng Guo, Li-Jun Zuo, Yang Hu, Shu-Yang Yu, Li Liu, Zhao Jin, Qiu-Jin Yu, Rui-Dan Wang, Li-Xia Li, Ying-Shan Piao, Wei Zhang
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Oculodentodigital Dysplasia Presenting as Spastic Paraparesis: The First Genetically Confirmed Korean Case and a Literature Review: Kye Won Park, Ho-Sung Ryu, Juyeon Kim, Sun Ju Chung; J Mov Disord. 2017;10(3):149-153. Published online September 22, 2017; DOI: https://doi.org/10.14802/jmd.17050

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Oculodentodigital dysplasia (ODDD) is a rare autosomal dominant inherited disease caused by mutations of the human gap junction alpha 1 gene, which encodes the protein Connexin-43. Patients with ODDD may present with neurological deficits with a typical pleiotropic combination of characteristic craniofacial, ophthalmological, phalangeal, and dental anomalies. In this report, we describe the first genetically confirmed Korean ODDD patient, who presented with spastic paraparesis. We will also review the neurological aspects of ODDD as reported in the literature.

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Glial Connexins and Pannexins in the Healthy and Diseased Brain
Christian Giaume, Christian C. Naus, Juan C. Sáez, Luc Leybaert
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Oculodentodigital Dysplasia: A Case Report and Major Review of the Eye and Ocular Adnexa Features of 295 Reported Cases
Virang Kumar, Natario L. Couser, Arti Pandya
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Zhirong Wang, Limei Sun, Panfeng Wang, Chonglin Chen, Aiyuan Zhang, Weiqing Wang, Xiaoyan Ding
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I. Harting, S. Karch, U. Moog, A. Seitz, P.J.W. Pouwels, N.I. Wolf
American Journal of Neuroradiology.2019; 40(5): 903. CrossRef

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

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Abstract PDF Supplementary 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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Long-term motor outcomes of deep brain stimulation of the globus pallidus interna in Parkinson's disease patients: Five-year follow-up
Yun Su Hwang, Sungyang Jo, Seung Hyun Lee, Nayoung Kim, Mi-Sun Kim, Sang Ryong Jeon, Sun Ju Chung
Journal of the Neurological Sciences.2023; 444: 120484. CrossRef
The Role of Microelectrode Recording in Deep Brain Stimulation Surgery for Parkinson’s Disease: A Systematic Review and Meta-Analysis
R. Saman Vinke, Martin Geerlings, Ashok K. Selvaraj, Dejan Georgiev, Bastiaan R. Bloem, Rianne A.J. Esselink, Ronald H.M.A. Bartels
Journal of Parkinson's Disease.2022; 12(7): 2059. CrossRef
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Heidi R. Tucker, Emily Mahoney, Kainat Akhtar, Tzu-Jen Kao, Gianna Mamone, Saisree Mikkilineni, Maya Ravi, Hanel Watkins, Danielle-Lee Terrelonge, Caryn Martin, Kristen Unger, Gabrielle Kim, Kyra Fiber, Megan Gupta, Jonathan Indajang, Eliyahu M. Kochman,
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Michał Hutny, Jagoda Hofman, Aleksandra Klimkowicz-Mrowiec, Agnieszka Gorzkowska
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Estimating Risk for Future Intracranial, Fully Implanted, Modular Neuroprosthetic Systems: A Systematic Review of Hardware Complications in Clinical Deep Brain Stimulation and Experimental Human Intracortical Arrays
Autumn J. Bullard, Brianna C. Hutchison, Jiseon Lee, Cynthia A. Chestek, Parag G. Patil
Neuromodulation: Technology at the Neural Interface.2020; 23(4): 411. CrossRef
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
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Validation of the Korean Version of the Scale for Outcomes in Parkinson’s Disease-Autonomic: Ji-Young Kim, In-Uk Song, Seong-Beom Koh, Tae-Beom Ahn, Sang Jin Kim, Sang-Myung Cheon, Jin Whan Cho, Yun Joong Kim, Hyeo-Il Ma, Mee-Young Park, Jong Sam Baik, Phil Hyu Lee, Sun Ju Chung, Jong-Min Kim, Han-Joon Kim, Young-Hee Sung, Do Young Kwon, Jae-Hyeok Lee, Jee-Young Lee, Ji Sun Kim, Ji Young Yun, Hee Jin Kim, Jin Young Hong, Mi-Jung Kim, Jinyoung Youn, Ji Seon Kim, Eung Seok Oh, Hui-Jun Yang, Won Tae Yoon, Sooyeoun You, Kyum-Yil Kwon, Hyung-Eun Park, Su-Yun Lee, Younsoo Kim, Hee-Tae Kim, Joong-Seok Kim; J Mov Disord. 2017;10(1):29-34. Published online January 18, 2017; DOI: https://doi.org/10.14802/jmd.16057

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Objective
Autonomic symptoms are commonly observed in patients with Parkinson’s disease (PD) and often limit the activities of daily living. The Scale for Outcomes in Parkinson’s disease-Autonomic (SCOPA-AUT) was developed to evaluate and quantify autonomic symptoms in PD. The goal of this study was to translate the original SCOPA-AUT, which was written in English, into Korean and to evaluate its reliability and validity for Korean PD patients.
Methods
For the translation, the following processes were performed: forward translation, backward translation, expert review, pretest of the pre-final version and development of the final Korean version of SCOPA-AUT (K-SCOPA-AUT). In total, 127 patients with PD from 31 movement disorder clinics of university-affiliated hospitals in Korea were enrolled in this study. All patients were assessed using the K-SCOPA-AUT and other motor, non-motor, and quality of life scores. Test-retest reliability for the K-SCOPA-AUT was assessed over a time interval of 10−14 days.
Results
The internal consistency and reliability of the K-SCOPA-AUT was 0.727 as measured by the mean Cronbach’s α-coefficient. The test-retest correlation reliability was 0.859 by the Guttman split-half coefficient. The total K-SCOPA-AUT score showed a positive correlation with other non-motor symptoms [the Korean version of non-motor symptom scale (K-NMSS)], activities of daily living (Unified Parkinson’s Disease Rating Scale part II) and quality of life [the Korean version of Parkinson’s Disease Quality of Life 39 (K-PDQ39)].
Conclusion
The K-SCOPA-AUT had good reliability and validity for the assessment of autonomic dysfunction in Korean PD patients. Autonomic symptom severities were associated with many other motor and non-motor impairments and influenced quality of life.

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Clinical Characteristics of Parkinson’s Disease Developed from Essential Tremor
Dong-Woo Ryu, Si-Hoon Lee, Yoon-Sang Oh, Jae-Young An, Jeong-Wook Park, In-Uk Song, Kwang-Soo Lee, Joong-Seok Kim
Journal of Parkinson's Disease.2017; 7(2): 369. CrossRef

Clinical Heterogeneity of Atypical Pantothenate Kinase-Associated Neurodegeneration in Koreans: Jae-Hyeok Lee, Jongkyu Park, Ho-Sung Ryu, Hyeyoung Park, Young Eun Kim, Jin Yong Hong, Sang Ook Nam, Young-Hee Sung, Seung-Hwan Lee, Jee-Young Lee, Myung Jun Lee, Tae-Hyoung Kim, Chul Hyoung Lyoo, Sun Ju Chung, Seong Beom Koh, Phil Hyu Lee, Jin Whan Cho, Mee Young Park, Yun Joong Kim, Young H. Sohn, Beom Seok Jeon, Myung Sik Lee; J Mov Disord. 2016;9(1):20-27. Published online January 25, 2016; DOI: https://doi.org/10.14802/jmd.15058

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Abstract PDF Supplementary Material

Objective
Neurodegeneration with brain iron accumulation (NBIA) represents a group of inherited movement disorders characterized by iron accumulation in the basal ganglia. Recent advances have included the identification of new causative genes and highlighted the wide phenotypic variation between and within the specific NBIA subtypes. This study aimed to investigate the current status of NBIA in Korea.
Methods
We collected genetically confirmed NBIA patients from twelve nationwide referral hospitals and from a review of the literature. We conducted a study to describe the phenotypic and genotypic characteristics of Korean adults with atypical pantothenate kinase-associated neurodegeneration (PKAN).
Results
Four subtypes of NBIA including PKAN (n = 30), PLA2G6-related neurodegeneration (n = 2), beta-propeller protein-associated neurodegeneration (n = 1), and aceruloplasminemia (n = 1) have been identified in the Korean population. The clinical features of fifteen adults with atypical PKAN included early focal limb dystonia, parkinsonism-predominant feature, oromandibular dystonia, and isolated freezing of gait (FOG). Patients with a higher age of onset tended to present with parkinsonism and FOG. The p.R440P and p.D378G mutations are two major mutations that represent approximately 50% of the mutated alleles. Although there were no specific genotype-phenotype correlations, most patients carrying the p.D378G mutation had a late-onset, atypical form of PKAN.
Conclusions
We found considerable phenotypic heterogeneity in Korean adults with atypical PKAN. The age of onset may influence the presentation of extrapyramidal symptoms.

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Yilun Tao, Chen Zhao, Dong Han, Yiju Wei, Lihong Wang, Wenxia Song, Xiaoze Li
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Van Khanh Tran, Chi Dung Vu, Hai Anh Tran, Nguyen Thi Kim Lien, Nguyen Van Tung, Nguyen Ngoc Lan, Huy Thinh Tran, Nguyen Huy Hoang
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Nationwide Survey of Patient Knowledge and Attitudes towards Human Experimentation Using Stem Cells or Bee Venom Acupuncture for Parkinson’s Disease: Sun Ju Chung, Seong Beom Koh, Young-Su Ju, Jae Woo Kim; J Mov Disord. 2014;7(2):84-91. Published online October 30, 2014; DOI: https://doi.org/10.14802/jmd.14012

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Objective Stem cell treatment is a well-recognized experimental treatment among patients with Parkinson’s disease (PD), for which there are high expectations of a positive impact. Acupuncture with bee venom is one of the most popular complementary and alternative treatments for PD. Patient knowledge and attitudes towards these experimental treatments are unknown.
Methods Using a 12-item questionnaire, a nationwide survey was conducted of 963 PD patients and 267 caregivers in 44 Korean Movement Disorders Society member hospitals from April 2013 to June 2013. The survey was performed by trained interviewers using conventional methods.
Results Regarding questions on experimental treatments using stem cells or bee venom acupuncture, 5.1–17.7% of PD patients answered questions on safety, efficacy, and evidence-based practice incorrectly; however, more than half responded that they did not know the correct answer. Although safety and efficacy have not been established, 55.5% of PD patients responded that they were willing to receive stem cell treatment. With regard to participating in experimental treatments, there was a strong correlation between stem cell treatment and bee venom acupuncture (p < 0.0001, odds ratio = 5.226, 95% confidence interval 3.919–6.969). Younger age, higher education, and a longer duration of PD were all associated with a correct understanding of experimental treatments.
Conclusions Our data suggest that relatively few PD patients correctly understand the safety and efficacy of experimental treatments and that PD patients are greatly interested in new treatments. We hope that our data will be used to educate or to plan educational programs for PD patients and caregivers.

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A Case of Multiple System Atrophy-Cerebellar Type Preceded by Dementia: Eun Hye Jang, Joo Kyung Lee, Hyun Jung Jang, Mi-Jung Kim, Sun Ju Chung; J Mov Disord. 2012;5(2):48-52.; DOI: https://doi.org/10.14802/jmd.12011

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Abstract PDF

Multiple system atrophy (MSA) is a sporadic, adult-onset disease characterized by progressive degeneration of nervous systems including cerebellar, pyramidal, extrapyramidal, and autonomic system. Although a few recent studies reported that cognitive impairments could occur in patients with MSA, prominent dementia with progressive decline is not a typical clinical manifestation of MSA. In particular, dementia with MSA-cerebellar type is very rare. We have experienced a patient with 2-year history of severe cognitive impairment, who was finally diagnosed as MSA-cerebellar type.

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Miki Yoshitake, Atsuhiko Sugiyama, Takayoshi Shimohata, Nobuyuki Araki, Masahide Suzuki, Kazumoto Shibuya, Kengo Nagashima, Nobutaka Hattori, Satoshi Kuwabara
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Mitochondrial dysfunction and altered ribostasis in hippocampal neurons with cytoplasmic inclusions of multiple system atrophy
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Takayoshi Shimohata, Naotaka Aizawa, Hideaki Nakayama, Hiroshige Taniguchi, Yasuyoshi Ohshima, Hitoshi Okumura, Tetsuya Takahashi, Akio Yokoseki, Makoto Inoue, Masatoyo Nishizawa
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Human Genetic Variation and Parkinson’s Disease: Sun Ju Chung; J Mov Disord. 2010;3(1):1-5.; DOI: https://doi.org/10.14802/jmd.10001

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Abstract PDF

Parkinson’s disease (PD) is a chronic neurodegenerative disorder with multifactorial etiology. In the past decade, the genetic causes of monogenic forms of familial PD have been defined. However, the etiology and pathogenesis of the majority of sporadic PD cases that occur in outbred populations have yet to be clarified. The recent development of resources such as the International HapMap Project and technological advances in high-throughput genotyping have provided new basis for genetic association studies of common complex diseases, including PD. A new generation of genome-wide association studies will soon offer a potentially powerful approach for mapping causal genes and will likely change treatment and alter our perception of the genetic determinants of PD. However, the execution and analysis of such studies will require great care.

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Gene Signals and SNPs Associated with Parkinson’s Disease: A Nutrigenomics and Computational Prospective Insights
Swetha Subramaniyan, Beena Briget Kuriakose, Sakeena Mushfiq, Narayanaswamy Marimuthu Prabhu, Karthikeyan Muthusamy
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Comparison of Cerebral Glucose Metabolism between Possible and Probable Multiple System Atrophy: Kyum-Yil Kwon, Jae Seung Kim, Ki Chun Im, Myoung Chong Lee, Sun Ju Chung; J Mov Disord. 2009;2(1):22-28.; DOI: https://doi.org/10.14802/jmd.09006

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Abstract PDF

Background:

To investigate the relationship between presenting clinical manifestations and imaging features of multisystem neuronal dysfunction in MSA patients, using ¹⁸F-fluorodeoxyglucose positron emission tomography (¹⁸F-FDG PET).

Methods:

We studied 50 consecutive MSA patients with characteristic brain MRI findings of MSA, including 34 patients with early MSA-parkinsonian (MSA-P) and 16 with early MSA-cerebellar (MSA-C). The cerebral glucose metabolism of all MSA patients was evaluated in comparison with 25 age-matched controls. ¹⁸F-FDG PET results were assessed by the Statistic Parametric Mapping (SPM) analysis and the regions of interest (ROI) method.

Results:

The mean time from disease onset to ¹⁸F-FDG PET was 25.9±13.0 months in 34 MSA-P patients and 20.1±11.1 months in 16 MSA-C patients. Glucose metabolism of the putamen showed a greater decrease in possible MSA-P than in probable MSA-P (p=0.031). Although the Unified Multiple System Atrophy Rating Scale (UMSARS) score did not differ between possible MSA-P and probable MSA-P, the subscores of rigidity (p=0.04) and bradykinesia (p= 0.008) were significantly higher in possible MSA-P than in probable MSA-P. Possible MSA-C showed a greater decrease in glucose metabolism of the cerebellum than probable MSA-C (p=0.016).

Conclusions:

Our results may suggest that the early neuropathological pattern of possible MSA with a predilection for the striatonigral or olivopontocerebellar system differs from that of probable MSA, which has prominent involvement of the autonomic nervous system in addition to the striatonigral or olivopontocerebellar system.

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F-18 FP-CIT PET in Multiple System Atrophy of the Cerebellar Type: Additional Role in Treatment
Young Jin Jeong, Sang-Myung Cheon, Do-Young Kang, Jae Woo Kim
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The Role of Telephone Counseling in Management of Parkinson’s Disease Patients: Mi Sun Kim, Sun Ju Chung, Sung Reul Kim, Tai Yeon Lee, Myoung Chong Lee; J Mov Disord. 2008;1(1):33-37.; DOI: https://doi.org/10.14802/jmd.08006

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Abstract PDF: Background:
Various nonpharmacologic managements are important fundamental elements in the treatment of Parkinson’s disease (PD). We aimed to investigate the role of telephone counseling in managing PD patients.
Methods:
From November 2006 to January 2007, we studied 243 PD patients at outpatient clinic of Asan Medical Center. Detailed telephone counseling was provided using a list structured questionnaires.
Results:
There were 73 men and 170 women with an age range of 17 to 85 years (mean age, 64.9 years). Mean age at onset was 59.5 years (range, 14–82 years) and mean disease duration was 5.6 years (range, 0.3–25 years). The contents of telephone counseling included adverse effects of anti-Parkinsonian medications (24.4%), aggravation of motor symptoms (18.7%), problems due to comorbidities (17.8%), how to take medicine (13.6%), activities of daily living (diet, bowel, sleeping and safety) (12.6%), complementary or alternative medicines (3.9%) and knowledge about PD (3.0%). Persons who responded to use the telephone counseling included patients (37.9%), offspring (36.2%), spouses (17.7%) and other relatives (7.4%). Persons who received the telephone counseling were determined by level of education, sex, cohabitation and Hoehn-Yahr stage. Contents of telephone counseling varied significantly with Hoehn-Yahr stage and persons who used the telephone counseling.
Conclusions:
Our results suggest that support system with telephone counseling may provide beneficial therapeutic intervention in PD patients, especially for those with advanced PD. The most cost-effective method for telephone support needs to be studied.

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