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Review Articles
Historical and More Common Nongenetic Movement Disorders From Asia
Norlinah Mohamed Ibrahim, Priya Jagota, Pramod Kumar Pal, Roongroj Bhidayasiri, Shen-Yang Lim, Yoshikazu Ugawa, Zakiyah Aldaajani, Beomseok Jeon, Shinsuke Fujioka, Jee-Young Lee, Prashanth Lingappa Kukkle, Huifang Shang, Onanong Phokaewvarangkul, Cid Diesta, Cholpon Shambetova, Chin-Hsien Lin
J Mov Disord. 2023;16(3):248-260.   Published online June 9, 2023
  • 2,323 View
  • 129 Download
  • 1 Web of Science
  • 1 Crossref
AbstractAbstract PDFSupplementary Material
Nongenetic movement disorders are common throughout the world. The movement disorders encountered may vary depending on the prevalence of certain disorders across various geographical regions. In this paper, we review historical and more common nongenetic movement disorders in Asia. The underlying causes of these movement disorders are diverse and include, among others, nutritional deficiencies, toxic and metabolic causes, and cultural Latah syndrome, contributed by geographical, economic, and cultural differences across Asia. The industrial revolution in Japan and Korea has led to diseases related to environmental toxin poisoning, such as Minamata disease and β-fluoroethyl acetate-associated cerebellar degeneration, respectively, while religious dietary restriction in the Indian subcontinent has led to infantile tremor syndrome related to vitamin B12 deficiency. In this review, we identify the salient features and key contributing factors in the development of these disorders.


Citations to this article as recorded by  
  • Diabetic striatopathy and other acute onset de novo movement disorders in hyperglycemia
    Subhankar Chatterjee, Ritwik Ghosh, Payel Biswas, Shambaditya Das, Samya Sengupta, Souvik Dubey, Biman Kanti Ray, Alak Pandit, Julián Benito-León, Rana Bhattacharjee
    Diabetes & Metabolic Syndrome: Clinical Research & Reviews.2024; 18(3): 102997.     CrossRef
Adult-Onset Genetic Leukoencephalopathies With Movement Disorders
Mu-Hui Fu, Yung-Yee Chang
J Mov Disord. 2023;16(2):115-132.   Published online March 7, 2023
  • 4,667 View
  • 379 Download
AbstractAbstract PDF
Genetic leukoencephalopathies (GLEs) are a group of white matter abnormalities with heterogeneous radiological and phenotypic features. Although these conditions have mostly been described in children, adult-onset cases are increasingly recognized owing to the widespread use of neuroimaging and advances in molecular genetic testing. The disease course is often progressive with a varied spectrum of presentations, trapping neurologists in the dilemma of differential diagnosis. Movement disorders are among the most common symptoms, and their diversity makes diagnosis challenging. In this review, we focus on adult-onset GLEs with movement disorders and offer a step-by-step diagnostic approach by clarifying the phenomenology of movement, advising investigations for acquired causes, describing the clinical and radiological clues to each disease, emphasizing the limitations of advanced molecular testing, and discussing the future application of artificial intelligence. We provide a list summarizing the leukoencephalopathies associated with different categories of movement disorders. In addition to guiding clinicians on how to narrow the list of differential diagnoses with the tools currently available, another aim of this review is to emphasize the inevitable trend toward applying advanced technology in diagnosing these difficult diseases.
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
  • 3,784 View
  • 310 Download
  • 2 Web of Science
  • 1 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 to this article as recorded by  
  • 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
Brief communication
Movement Disorders Resulting From Bilateral Basal Ganglia Lesions in End-Stage Kidney Disease: A Systematic Review
Kah Hui Yap, Nurul Husna Baharudin, Abdul Halim Abdul Gafor, Rabani Remli, Shen-Yang Lim, Wan Asyraf Wan Zaidi, Shahrul Azmin, Shahizon Azura Mohamed Mukari, Raihanah Abdul Khalid, Norlinah Mohamed Ibrahim
J Mov Disord. 2022;15(3):258-263.   Published online May 26, 2022
  • 2,895 View
  • 102 Download
AbstractAbstract PDFSupplementary Material
The basal ganglia (BG) are susceptible to fluctuations in blood urea levels, sometimes resulting in movement disorders. We described patients with end-stage kidney disease (ESKD) presenting with movement disorders associated with bilateral BG lesions on imaging.
We report four patients and systematically reviewed all published cases of ESKD presenting with movement disorders and bilateral BG lesions (EBSCOhost and Ovid).
Of the 72 patients identified, 55 (76.4%) were on regular dialysis. Parkinsonism was the most common movement disorder (n = 39; 54.2%), followed by chorea (n = 24; 33.3%). Diabetes mellitus (n = 51; 70.8%) and hypertension (n = 16; 22.2%) were the most common risk factors. Forty-three (59.7%) were of Asian ethnicity. Complete clinical resolution was reported in 17 (30.9%) patients, while 38 (69.1%) had incomplete clinical resolution with relapse. Complete radiological resolution occurred in 14 (34.1%) patients.
Movement disorders associated with BG lesions should be recognized as a rare and potentially reversible metabolic movement disorder in patients with ESKD.
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
  • 7,188 View
  • 111 Download
  • 4 Web of Science
  • 3 Crossref
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.


Citations to this article as recorded by  
  • Deep brain stimulation for movement disorders after stroke: a systematic review of the literature
    Mitch R. Paro, Michal Dyrda, Srinath Ramanan, Grant Wadman, Stacey-Ann Burke, Isabella Cipollone, Cory Bosworth, Sarah Zurek, Patrick B. Senatus
    Journal of Neurosurgery.2022; : 1.     CrossRef
  • Deep brain stimulation for post-thalamic stroke complex movement disorders
    A. Macerollo, B. Hammersley, M. Bonello, J. Somerset, D. Bhargava, K. Das, J. Osman-Farah, P. R. Eldridge, S. H. Alusi
    Neurological Sciences.2021; 42(1): 337.     CrossRef
  • Neurologic Manifestations of Systemic Disease: Movement Disorders
    Giulietta M. Riboldi, Steven J. Frucht
    Current Treatment Options in Neurology.2021;[Epub]     CrossRef
Review Articles
Untangling the Thorns: Advances in the Neuroacanthocytosis Syndromes
Ruth H. Walker
J Mov Disord. 2015;8(2):41-54.   Published online May 31, 2015
  • 28,880 View
  • 365 Download
  • 39 Web of Science
  • 32 Crossref
AbstractAbstract PDF
There have been significant advances in neuroacanthocytosis (NA) syndromes in the past 20 years, however, confusion still exists regarding the precise nature of these disorders and the correct nomenclature. This article seeks to clarify these issues and to summarise the recent literature in the field. The four key NA syndromes are described here–chorea-acanthocytosis, McLeod syndrome, Huntington’s disease-like 2, and pantothenate kinase- associated neurodegeneration. In the first two, acanthocytosis is a frequent, although not invariable, finding; in the second two, it occurs in approximately 10% of patients. Degeneration affecting the basal ganglia is the key neuropathologic finding, thus the clinical presentations can be remarkably similar. The characteristic phenotype comprises a variety of movement disorders, including chorea, dystonia, and parkinsonism, and also psychiatric and cognitive symptoms attributable to basal ganglia dysfunction. The age of onset, inheritance patterns, and ethnic background differ in each condition, providing diagnostic clues. Other investigations, including routine blood testing and neuroimaging can be informative. Genetic diagnosis, if available, provides a definitive diagnosis, and is important for genetic counseling, and hopefully molecular therapies in the future. In this article I provide a historical perspective on each NA syndrome. The first 3 disorders, chorea-acanthocytosis, McLeod syndrome, Huntington’s disease-like 2, are discussed in detail, with a comprehensive review of the literature to date for each, while pantothenate kinase-associated neurodegeneration is presented in summary, as this disorder has recently been reviewed in this journal. Therapy for all of these diseases is, at present, purely symptomatic.


Citations to this article as recorded by  
  • Identification of four novel mutations in VSP13A in Iranian patients with Chorea-acanthocytosis (ChAc)
    Vadieh Ghodsinezhad, Abdoreza Ghoreishi, Mohammad Rohani, Mahdi Dadfar, Akbar Mohammadzadeh, Ali Rostami, Hamzeh Rahimi
    Molecular Genetics and Genomics.2024;[Epub]     CrossRef
  • Acupuncture for treating symptoms associated with chorea-acanthocytosis: A CARE-compliant case report
    Qiqi Wu, Zengtu Li, Yingying Cheng, Hantong Hu, Hong Gao, Jiawei Wang, Dexiong Han
    EXPLORE.2023; 19(1): 127.     CrossRef
  • Two case reports of chorea-acanthocytosis and review of literature
    Shuangfeng Huang, Junliang Zhang, Manli Tao, Yaodong Lv, Luyao Xu, Zhigang Liang
    European Journal of Medical Research.2022;[Epub]     CrossRef
  • Skin Conditions and Movement Disorders: Hiding in Plain Sight
    Kristina Kulcsarova, Janette Baloghova, Jan Necpal, Matej Skorvanek
    Movement Disorders Clinical Practice.2022; 9(5): 566.     CrossRef
  • The XK plasma membrane scramblase and the VPS13A cytosolic lipid transporter for ATP‐induced cell death
    Yuta Ryoden, Shigekazu Nagata
    BioEssays.2022;[Epub]     CrossRef
  • 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
    Journal of Movement Disorders.2022; 15(3): 197.     CrossRef
  • Chorea-acanthocytosis: Time-dependent changes of symptoms and imaging findings
    Fumio Suzuki, Noriko Sato, Atsuhiko Sugiyama, Keiya Iijima, Yoko Shigemoto, Emiko Morimoto, Yukio Kimura, Hiroyuki Fujii, Yuji Takahashi, Yasuhiro Nakata, Hiroshi Matsuda, Osamu Abe
    Journal of Neuroradiology.2021; 48(6): 419.     CrossRef
  • Prionopathies and Prionlike Protein Aberrations in Neurodegenerative Diseases
    K.N. Anderson, W.B. Overcast, J.R. Brosch, B.D. Graner, M.C. Veronesi
    Neurographics.2021; 11(2): 127.     CrossRef
  • Brain F-18 FDG and F-18 FP-CIT PET/CT Findings of c.856_860delCTCTA Mutation McLeod Syndrome
    Ho-Sung Ryu, Chae Moon Hong
    Cognitive and Behavioral Neurology.2021; 34(3): 207.     CrossRef
  • Vacuolar Protein-Sorting Receptor MoVps13 Regulates Conidiation and Pathogenicity in Rice Blast Fungus Magnaporthe oryzae
    Xueming Zhu, Lin Li, Jiaoyu Wang, Lili Zhao, Huanbin Shi, Jiandong Bao, Zhenzhu Su, Xiaohong Liu, Fucheng Lin
    Journal of Fungi.2021; 7(12): 1084.     CrossRef
  • Involuntary movements, vocalizations and cognitive decline
    Olafur Sveinsson, Bjarne Udd, Per Svenningsson, Christoph Gassner, Charlotte Engström, José Laffita-Mesa, Göran Solders, Stellan Hertegård, Irina Savitcheva, Hans H. Jung, Markus Tolnay, Beat M. Frey, Martin Paucar
    Parkinsonism & Related Disorders.2020; 79: 135.     CrossRef
  • Discriminating chorea-acanthocytosis from Huntington's disease with single-case voxel-based morphometry analysis
    Fumio Suzuki, Noriko Sato, Miho Ota, Atsuhiko Sugiyama, Yoko Shigemoto, Emiko Morimoto, Yukio Kimura, Noritaka Wakasugi, Yuji Takahashi, Akinori Futamura, Mitsuru Kawamura, Kenjiro Ono, Masayuki Nakamura, Akira Sano, Masako Watanabe, Hiroshi Matsuda, Osam
    Journal of the Neurological Sciences.2020; 408: 116545.     CrossRef
  • Identification of two compound heterozygous VPS13A large deletions in chorea‐acanthocytosis only by protein and quantitative DNA analysis
    Derek Spieler, Antonio Velayos‐Baeza, Alžbeta Mühlbäck, Florian Castrop, Christian Maegerlein, Julia Slotta‐Huspenina, Benedikt Bader, Bernhard Haslinger, Adrian Danek
    Molecular Genetics & Genomic Medicine.2020;[Epub]     CrossRef
  • Neuroacanthocytosis with unusual clinical features
    Hui Zhu, Xue-min Feng, Teng Zhao, Jing-yao Liu
    Medicine.2019; 98(2): e14050.     CrossRef
  • VPS13A, a closely associated mitochondrial protein, is required for efficient lysosomal degradation
    Sandra Muñoz-Braceras, Alba R. Tornero-Écija, Olivier Vincent, Ricardo Escalante
    Disease Models & Mechanisms.2019;[Epub]     CrossRef
  • Juvenile parkinsonism: Differential diagnosis, genetics, and treatment
    Nicki Niemann, Joseph Jankovic
    Parkinsonism & Related Disorders.2019; 67: 74.     CrossRef
  • Chorea
    Pichet Termsarasab
    CONTINUUM: Lifelong Learning in Neurology.2019; 25(4): 1001.     CrossRef
  • Chorea-acanthocytosis
    Susan Walker, Rubina Dad, Bhooma Thiruvahindrapuram, Muhammed Ikram Ullah, Arsalan Ahmad, Muhammad Jawad Hassan, Stephen W. Scherer, Berge A. Minassian
    Neurology Genetics.2018;[Epub]     CrossRef
  • Huntington's disease-like disorders in Latin America and the Caribbean
    Ruth H. Walker, Emilia M. Gatto, M. Leonor Bustamante, Oscar Bernal-Pacheco, Francisco Cardoso, Raphael M. Castilhos, Pedro Chana-Cuevas, Mario Cornejo-Olivas, Ingrid Estrada-Bellmann, Laura B. Jardim, Ricardo López-Castellanos, Ricardo López-Contreras, D
    Parkinsonism & Related Disorders.2018; 53: 10.     CrossRef
  • Feeding Dystonia
    Ritu Shree, Sahil Mehta, Venugopalan Y. Vishnu
    Movement Disorders Clinical Practice.2018; 5(4): 442.     CrossRef
  • Comment on: Consequences of Delayed Dental Extraction in Lesch‐Nyhan Disease
    Harriet Van Den Tooren, Benjamin M. Davies, Monty Silverdale, David McKee
    Movement Disorders Clinical Practice.2017; 4(3): 463.     CrossRef
  • Novel VPS13A Gene Mutations Identified in Patients Diagnosed with Chorea-acanthocytosis (ChAc): Case Presentation and Literature Review
    Yan Shen, Xiaoming Liu, Xi Long, Chao Han, Fang Wan, Wenliang Fan, Xingfang Guo, Kai Ma, Shiyi Guo, Luxi Wang, Yun Xia, Ling Liu, Jinsha Huang, Zhicheng Lin, Nian Xiong, Tao Wang
    Frontiers in Aging Neuroscience.2017;[Epub]     CrossRef
  • Stepwise partitioning of Xp21: a profiling method for XK deletions causative of the McLeod syndrome
    Christoph Gassner, Chantal Brönnimann, Yvonne Merki, Maja P. Mattle‐Greminger, Sonja Sigurdardottir, Eduardo Meyer, Charlotte Engström, John D. O'Sullivan, Hans H. Jung, Beat M. Frey
    Transfusion.2017; 57(9): 2125.     CrossRef
  • Disruption of GABA(A)-mediated intracortical inhibition in patients with chorea-acanthocytosis
    Raffaele Dubbioso, Marcello Esposito, Silvio Peluso, Rosa Iodice, Giuseppe De Michele, Lucio Santoro, Fiore Manganelli
    Neuroscience Letters.2017; 654: 107.     CrossRef
  • The First Genetically Confirmed McLeod Syndrome in Korea
    Kye Won Park, Soo Jeong, Eul-Ju Seo, Chong S. Lee
    Journal of the Korean Neurological Association.2017; 35(2): 85.     CrossRef
  • Hippocampal sclerosis and mesial temporal lobe epilepsy in chorea‐acanthocytosis: a case with clinical, pathologic and genetic evaluation
    K. Mente, S. A. Kim, C. Grunseich, M. M. Hefti, J. F. Crary, A. Danek, B. I. Karp, R. H. Walker
    Neuropathology and Applied Neurobiology.2017; 43(6): 542.     CrossRef
  • Yeast and other lower eukaryotic organisms for studies of Vps13 proteins in health and disease
    Weronika Rzepnikowska, Krzysztof Flis, Sandra Muñoz‐Braceras, Regina Menezes, Ricardo Escalante, Teresa Zoladek
    Traffic.2017; 18(11): 711.     CrossRef
  • Peripheral neuropathy in complex inherited diseases: an approach to diagnosis
    Alexander M Rossor, Aisling S Carr, Helen Devine, Hoskote Chandrashekar, Ana Lara Pelayo-Negro, Davide Pareyson, Michael E Shy, Steven S Scherer, Mary M Reilly
    Journal of Neurology, Neurosurgery & Psychiatry.2017; 88(10): 846.     CrossRef
  • Huntington's Disease, Huntington's Disease Look‐Alikes‎, and Benign Hereditary Chorea: What's New?
    Susanne A. Schneider, Thomas Bird
    Movement Disorders Clinical Practice.2016; 3(4): 342.     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,
    Journal of Movement Disorders.2016; 9(1): 20.     CrossRef
  • Neurological Disorders Associated with Striatal Lesions: Classification and Diagnostic Approach
    Davide Tonduti, Luisa Chiapparini, Isabella Moroni, Anna Ardissone, Giovanna Zorzi, Federica Zibordi, Sergio Raspante, Celeste Panteghini, Barbara Garavaglia, Nardo Nardocci
    Current Neurology and Neuroscience Reports.2016;[Epub]     CrossRef
  • Clinical variability of neuroacanthocytosis syndromes—a series of six patients with long follow-up
    J. Dulski, W. Sołtan, M. Schinwelski, M. Rudzińska, M. Wójcik-Pędziwiatr, L. Wictor, F. Schön, A. Puschmann, J. Klempíř, L. Tilley, J. Roth, P. Tacik, S. Fujioka, W. Drozdowski, E.J. Sitek, Z. Wszolek, J. Sławek
    Clinical Neurology and Neurosurgery.2016; 147: 78.     CrossRef
Cell Therapy Strategies vs. Paracrine Effect in Huntington’s Disease
Wooseok Im, Manho Kim
J Mov Disord. 2014;7(1):1-6.   Published online April 30, 2014
  • 14,233 View
  • 88 Download
  • 6 Web of Science
  • 5 Crossref
AbstractAbstract PDF
Huntington’s disease (HD) is a genetic neurodegenerative disorder. The most common symptom of HD is abnormal involuntary writhing movements, called chorea. Antipsychotics and tetrabenazine are used to alleviate the signs and symptoms of HD. Stem cells have been investigated for use in neurodegenerative disorders to develop cell therapy strategies. Recent evidence indicates that the beneficial effects of stem cell therapies are actually mediated by secretory molecules, as well as cell replacement. Although stem cell studies show that cell transplantation provides cellular improvement around lesions in in vivo models, further work is required to elucidate some issues before the clinical application of stem cells. These issues include the precise mechanism of action, delivery method, toxicity and safety. With a focus on HD, this review summarizes cell therapy strategies and the paracrine effect of stem cells.


Citations to this article as recorded by  
  • Current Understanding of Stem Cell and Secretome Therapies in Liver Diseases
    Dongkyu Kim, Gun-Sik Cho, Choongseong Han, Dong-Hyuk Park, Hee-Kyung Park, Dong-Hun Woo, Jong-Hoon Kim
    Tissue Engineering and Regenerative Medicine.2017; 14(6): 653.     CrossRef
  • Induced Pluripotent Stem Cells in Huntington’s Disease: Disease Modeling and the Potential for Cell-Based Therapy
    Ling Liu, Jin-Sha Huang, Chao Han, Guo-Xin Zhang, Xiao-Yun Xu, Yan Shen, Jie Li, Hai-Yang Jiang, Zhi-Cheng Lin, Nian Xiong, Tao Wang
    Molecular Neurobiology.2016; 53(10): 6698.     CrossRef
  • Stem Cells in Neurological Disorders: Emerging Therapy with Stunning Hopes
    Ghanshyam Upadhyay, Sharmila Shankar, Rakesh K. Srivastava
    Molecular Neurobiology.2015; 52(1): 610.     CrossRef
  • Genome Modification Leads to Phenotype Reversal in Human Myotonic Dystrophy Type 1 Induced Pluripotent Stem Cell-Derived Neural Stem Cells
    Guangbin Xia, Yuanzheng Gao, Shouguang Jin, S.H. Subramony, Naohiro Terada, Laura P.W. Ranum, Maurice S. Swanson, Tetsuo Ashizawa
    Stem Cells.2015; 33(6): 1829.     CrossRef
  • Glycogen synthase kinase 3β inhibition enhanced proliferation, migration and functional re-endothelialization of endothelial progenitor cells in hypercholesterolemia microenvironment
    Bin Cui, Jun Jin, Xiaohan Ding, Mengyang Deng, Shiyong Yu, MingBao Song, Yang Yu, Xiaohui Zhao, Jianfei Chen, Lan Huang
    Experimental Biology and Medicine.2015; 240(12): 1752.     CrossRef
Case Reports
Acute Hemichorea as an Unusual Presentation of Internal Carotid Artery Stenosis
Dong Wook Kim, Youngchai Ko, Sang Hyun Jang, Soo Jin Yoon, Gun-Sei Oh, Soo Joo Lee, Dong Joo Yun
J Mov Disord. 2013;6(1):17-20.
  • 16,539 View
  • 100 Download
  • 10 Crossref
AbstractAbstract PDF

Involuntary movement associated with deep watershed ischemic lesions has been rarely reported. A 67-year-old woman presented with acute hemichorea on the left side. Magnetic resonance imaging showed acute infarcts in the anterior border zone. On perfusion studies, impaired cerebral blood flow was observed on the subcortical region sparing the basal ganglia. Cerebral angiogram confirmed severe stenosis in the right internal carotid artery. Her hemichorea gradually improved along with normalization of perfusion after carotid artery stenting with angioplasty. We suggest that impaired cerebral blood flow in critical watershed territories may be an important contributing factor in hemichorea associated with carotid occlusive disease.


Citations to this article as recorded by  
  • Hemichorea associated with subcortical border-zone infarction and middle cerebral artery stenosis
    So-Ei Ann, Suho Ro, Yun Hyeong Jeong, Sumin Kim, Pil-Wook Chung
    Journal of Geriatric Neurology.2022; 1(1): 41.     CrossRef
  • Movement Disorders Associated With Cerebral Artery Stenosis: A Nationwide Study
    Kye Won Park, Nari Choi, Eungseok Oh, Chul Hyoung Lyoo, Min Seok Baek, Han-Joon Kim, Dalla Yoo, Jee-Young Lee, Ji-Hyun Choi, Jae Hyeok Lee, Seong-Beom Koh, Young Hee Sung, Jin Whan Cho, Hui-Jun Yang, Jinse Park, Hae-Won Shin, Tae-Beom Ahn, Ho-Sung Ryu, So
    Frontiers in Neurology.2022;[Epub]     CrossRef
  • Hemiballism-hemichorea revealing carotidal stenosis
    I. Ion, T. Parvu, G. Farouil, D. Sablot
    Revue Neurologique.2022; 178(8): 859.     CrossRef
  • The Impact of Revascularization in a Patient with Atypical Manifestations of Hypoperfusion
    Sintija Strautmane, Zanda Priede, Andrejs Millers
    Medicina.2022; 58(10): 1328.     CrossRef
  • A case of hemichorea in RNF213-related vasculopathy
    Satoshi Hosoki, Takeshi Yoshimoto, Masafumi Ihara
    BMC Neurology.2021;[Epub]     CrossRef
  • Acute hemichorea with T1 ischemic hyperintensity associated with steno‐occlusive middle cerebral artery dissection
    Yong Chuan Chee, Beng Hooi Ong
    Neurology and Clinical Neuroscience.2020; 8(1): 44.     CrossRef
  • A case of hemichorea caused by right internal carotid artery stenosis
    Kazuki Muguruma, Atsuko Motoda, Takamichi Sugimoto, Takeshi Kitamura
    Rinsho Shinkeigaku.2019; 59(8): 509.     CrossRef
  • Movement Disorders Following Cerebrovascular Lesions: Etiology, Treatment Options and Prognosis
    Do-Young Kwon
    Journal of Movement Disorders.2016; 9(2): 63.     CrossRef
  • Hemichorea improvement following endarterectomy for internal carotid artery stenosis
    Kazuyuki Noda, Reo Ishimoto, Nobutaka Hattori, Yasuyuki Okuma, Takuji Yamamoto
    Journal of the Neurological Sciences.2016; 371: 45.     CrossRef
  • Hemichorea Resulting from Ischemic Stroke in the Ipsilateral Caudate Nucleus
    Seung-Keun Lee, Ji Sun Kim, Kyung-Bok Lee, Hakjae Roh, Moo-young Ahn
    Journal of Neurocritical Care.2015; 8(2): 109.     CrossRef
A Case of Isolated Middle Cerebral Artery Stenosis with Hemichorea and Moyamoya Pattern Collateralization
Seok Jong Chung, Hyung Seok Lee, Han Soo Yoo, Kyung Min Kim, Ki Jeong Lee, Jong-Soo Kim, Jae-Wook Lee, Jong Hun Kim, Jeong Hee Cho, Gyu Sik Kim, Jun Hong Lee, Sun-Ah Choi
J Mov Disord. 2013;6(1):13-16.
  • 11,634 View
  • 98 Download
  • 5 Crossref
AbstractAbstract PDF

Isolated middle cerebral artery (MCA) stenosis in young patients with no other medical condition may be a unique pathologic entity with a benign long-term course. Generally, moyamoya disease shows a progression of stenosis from internal cerebral artery (ICA) to other intracranial vessel. A 26-year-old woman was admitted for choreic movements of the right arm and leg. Brain magnetic resonance imaging showed no stroke. Conventional angiography revealed 48% stenosis of the left M1 without ICA stenosis. Single photon emission computed tomography revealed perfusion asymmetry after acetazolamide injection, suggesting decreased uptake in the left basal ganglia and the cerebral cortex. Her hemichorea was mildly decreased with risperidone. One year later, follow-up angiography showed complete occlusion of the left M1 with neovascularization suggestive of moyamoya disease. The patient underwent bypass surgery and her hemichorea disappeared. This may be an atypical presentation of moyamoya disease. The bypass surgery was an effective measure for restoring the vascular insufficiency and, resultantly, controlling her hemichorea.


Citations to this article as recorded by  
  • Hemichorea in patients with temporal lobe infarcts: Two case reports
    Xu-Dong Wang, Xing Li, Chun-Lian Pan
    World Journal of Clinical Cases.2024; 12(4): 806.     CrossRef
  • Truncal dystonia with isolated middle cerebral artery ischemia: A case report of revascularization therapy for dystonia
    Shinichi Matsumoto, Yuki Yamamoto, Koji Fujita, Ryosuke Miyamoto, Hidetaka Koizumi, Akihiro Tateishi, Naoaki Yamada, Yuishin Izumi
    Surgical Neurology International.2022; 13: 155.     CrossRef
  • Geriatric Case of Moyamoya Disease Presenting with Hemiballismus as an Initial Manifestation
    Koichiro SATO, Masaru IDEI, Kenichirou NOGAMI, Makoto KURESHIMA, Kaname HOKAO, Rina TORII, Takenori UOZUMI, Junkoh YAMAMOTO
    Surgery for Cerebral Stroke.2022; 50(6): 508.     CrossRef
  • Persistent Hemichorea as a Preceding Symptom of Cerebral Infarction Due to Middle Cerebral Artery Stenosis
    Yuki Ueta, Haruhisa Kato, Makiko Naito, Takeshi Taguchi, Hiroo Terashi, Hitoshi Aizawa
    Internal Medicine.2021; 60(23): 3805.     CrossRef
  • Clinical findings of a patient with hemiballism after superficial temporal artery-middle cerebral artery anastomosis for idiopathic middle cerebral artery stenosis
    Hideaki Shibata, Yuichi Hayashi, Nobuaki Yoshikura, Megumi Yamada, Akio Kimura, Takayoshi Shimohata
    Rinsho Shinkeigaku.2019; 59(12): 829.     CrossRef
Chorea in the Both Lower Limbs Associated with Nonketotic Hyperglycemia
Young-Hee Sung, Ki-Hyung Park, Yeung-Bae Lee, Hyeon-Mi Park, Dong-Jin Shin
J Mov Disord. 2009;2(2):98-100.
  • 19,013 View
  • 87 Download
  • 4 Crossref
AbstractAbstract PDF

Hemichorea-hemiballism (HC-HB) is a complication of non-ketotic hyperglycemia (NKH); in NKH patients, the frequency of occurrence of HC-HB is greater than that of bilateral chorea. We report the case of a hyperglycemic patient who showed chorea in both the lower limbs. Magnetic resonance imaging (MRI) of the brain revealed high signal intensity on T1-weighted images of the bilateral dorsolateral putamen. The abnormal involuntary movements disappeared after oral administration of haloperidol. Our case report that chorea associated with NKH is correlated with the topography of the basal ganglia.


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  • Mechanistic insights into the role of serum-glucocorticoid kinase 1 in diabetic nephropathy: A systematic review
    Saba Noor, Taj Mohammad, Gulam M. Ashraf, Joviana Farhat, Anwar L. Bilgrami, Mathew Suji Eapen, Sukhwinder Singh Sohal, Dharmendra Kumar Yadav, Md Imtaiyaz Hassan
    International Journal of Biological Macromolecules.2021; 193: 562.     CrossRef
  • “Diabetic striatopathy”: clinical presentations, controversy, pathogenesis, treatments, and outcomes
    Choon-Bing Chua, Cheuk-Kwan Sun, Chih-Wei Hsu, Yi-Cheng Tai, Chih-Yu Liang, I-Ting Tsai
    Scientific Reports.2020;[Epub]     CrossRef
  • T2*-based MR imaging of hyperglycemia-induced hemichorea-hemiballism
    Fang Yu, Andrew Steven, Lee Birnbaum, Wilson Altmeyer
    Journal of Neuroradiology.2017; 44(1): 24.     CrossRef
  • Acute chorea in the diabetic nonketotic hyperosmolar state
    Aaron de Souza, C. Shyam Babu, Paresh K. Desai
    Basal Ganglia.2013; 3(2): 85.     CrossRef
A Case of Vascular Hemichorea Responding to Topiramate
Jee-Ae Kim, San Jung, Min-Ju Kim, Seok-Beom Kwon, Sung-Hee Hwang, Ki-Han Kwon
J Mov Disord. 2009;2(2):80-81.
  • 16,911 View
  • 48 Download
  • 3 Crossref
AbstractAbstract PDF

Although vascular chorea often comes into remission spontaneously, a few patients may remain with persistent movement disorder. Most movements respond well to neuroleptics as well as other antidopaminergic drugs, but some patients show poor responses to those neuroleptics. Topiramate is a widely used of broad-spectrum anticonvulsant possessing a complex mechanism of action. It has been proven to enhance gamma-aminobutyrate acid activity and to be effective in the control of other movement disorders. We describe a 63-year-old woman with intractable vascular hemichorea which was controlled with anti-convulsant, topiramate.


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  • A case of severe movement disorder with GNAO1 mutation responsive to topiramate
    Saori Sakamoto, Yukifumi Monden, Ryoko Fukai, Noriko Miyake, Hiroshi Saito, Akihiko Miyauchi, Ayumi Matsumoto, Masako Nagashima, Hitoshi Osaka, Naomichi Matsumoto, Takanori Yamagata
    Brain and Development.2017; 39(5): 439.     CrossRef
  • Writing Tremor Secondary to Ischemic Stroke
    Juan P. Paviolo, Gabriela B. Raina, Eugenia Conti, Federico Micheli
    Clinical Neuropharmacology.2015; 38(2): 57.     CrossRef
  • Acute Hemichorea as an Unusual Presentation of Internal Carotid Artery Stenosis
    Dong Wook Kim, Youngchai Ko, Sang Hyun Jang, Soo Jin Yoon, Gun-Sei Oh, Soo Joo Lee, Dong Joo Yun
    Journal of Movement Disorders.2013; 6(1): 17.     CrossRef
Paroxysmal Chorea as a Relapse of Myelopathy in a Patient with Neuromyelitis Optica
Sang-Soo Lee, Ho-Sung Han, Dong-Ick Shin
J Mov Disord. 2009;2(2):78-79.
  • 20,337 View
  • 78 Download
AbstractAbstract PDF

Movement disorders secondary to intrinsic spinal cord disease are rare. Paroxysmal chorea has not yet been reported in the neuromyelitis optica (NMO). We report a 43-year-old woman with relapsing-remitting cervical myelopathy who developed paroxysmal chorea during clinical exacerbation of NMO. MRI scan of the cervical spine revealed a long segmental enhancing lesion, but brain MRI did not show any responsible abnormalities. Acute exacerbation of recurrent myelopathy in NMO may be associated with transient movement disorder.

Review Article
Secondary Dystonia-Clinical Clues and Syndromic Associations
Susanne A Schneider, Kailash P Bhatia
J Mov Disord. 2009;2(2):58-63.
  • 18,533 View
  • 427 Download
  • 10 Crossref
AbstractAbstract PDF

Dystonia is a hyperkinetic movement disorder defined by involuntary sustained muscle spasms and unusual postures. Etiologically, dystonic syndromes can be broadly divided into primary and secondary forms, dystonia-plus syndromes and heredodegenerative forms. In particular, diagnosis of secondary dystonic syndromes can be challenging in view of the variety of causes.


The purpose of this article is to highlight some clinical clues and syndromic associations as well as investigational findings which may be helpful in the approach to a patient with suspected secondary dystonia.


We outline characteristic clinical and neuroimaging findings which may be directive in the diagnostic process of dystonia patients and facilitate making the correct diagnosis, thus allowing initiating the best treatment.


Secondary causes of dystonia include, among others, strategic brain lesions of various origins, metabolic disease, neurodegenerative conditions, and previous exposure to drugs or toxins. Presence of clinical signs including prominent oromandibular involvement, eye movement disorders, retinitis pigmentosa, deafness, peripheral neuropathy, parkinsonism or progressive dementia should alert the clinician to consider a secondary cause. Strategic lesions within the basal ganglia, but also within the brainstem, cerebellum or cortical areas may underlie dystonia and should thus be excluded.


When thorough clinical examination reveals features atypical of primary dystonia, syndromic associations may help the clinician to narrow down the list of differential diagnosis. Directive investigations like neuroimaging may confirm the clinical suspicion.


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    Faye Rawson, Matthias Christen, Jeremy Rose, Emilie Paran, Tosso Leeb, Angela Fadda
    Journal of Veterinary Internal Medicine.2024; 38(1): 277.     CrossRef
  • Oromandibular dystonia – A systematic review
    Udit Saraf, Mitesh Chandarana, KP Divya, Syam Krishnan
    Annals of Indian Academy of Neurology.2022; 25(1): 26.     CrossRef
  • Oromandibular Dystonia: A Clinical Examination of 2,020 Cases
    Laura M. Scorr, Stewart A. Factor, Sahyli Perez Parra, Rachel Kaye, Randal C. Paniello, Scott A. Norris, Joel S. Perlmutter, Tobias Bäumer, Tatiana Usnich, Brian D. Berman, Marie Mailly, Emmanuel Roze, Marie Vidailhet, Joseph Jankovic, Mark S. LeDoux, Ric
    Frontiers in Neurology.2021;[Epub]     CrossRef
  • Clinical characteristics and diagnostic clues to Neurometabolic causes of dystonia
    Chun Seng Phua, Kishore Raj Kumar, Stanley Levy
    Journal of the Neurological Sciences.2020; 419: 117167.     CrossRef
  • Botulinum toxin A in the treatment of dystonia
    Lenka Hvizdošová, Pavel Otruba, Martin Nevrlý, Petr Kaňovský
    Neurologie pro praxi.2020; 21(1): 21.     CrossRef
  • Sensory trick in upper limb dystonia
    Sabino Dagostino, Tommaso Ercoli, Angelo F. Gigante, Roberta Pellicciari, Laura Fadda, Giovanni Defazio
    Parkinsonism & Related Disorders.2019; 63: 221.     CrossRef
  • Dystonia in Patients With Spinocerebellar Ataxia Type 2
    Vladana Markovic, Natasa T. Dragasevic‐Miskovic, Iva Stankovic, Igor Petrovic, Marina Svetel, Vladimir S. Kostić
    Movement Disorders Clinical Practice.2016; 3(3): 292.     CrossRef
  • Drug-induced movement disorders
    Dénes Zádori, Gábor Veres, Levente Szalárdy, Péter Klivényi, László Vécsei
    Expert Opinion on Drug Safety.2015; 14(6): 877.     CrossRef
  • Novel GNAL mutation with intra-familial clinical heterogeneity: Expanding the phenotype
    Miryam Carecchio, Celeste Panteghini, Chiara Reale, Chiara Barzaghi, Valentina Monti, Luigi Romito, Francesco Sasanelli, Barbara Garavaglia
    Parkinsonism & Related Disorders.2015;[Epub]     CrossRef
    Anisha Doshi, Jonathon Rohrer, Tom Warner
    Journal of Neurology, Neurosurgery & Psychiatry.2013; 84(11): e2.61.     CrossRef
Case Reports
Generalized Chorea Induced by an Unilateral Anterior Cerebral Artery Territorial Infarction
Jae Hong Chang, Woo-Keun Seo, Moon-Ho Park, Jong-Mun Lee, Do-Young Kwon, Seong-Beom Koh
J Mov Disord. 2009;2(1):37-39.
  • 9,097 View
  • 85 Download
  • 3 Crossref
AbstractAbstract PDF

Generalized chorea caused by unilateral cerebral infarction has rarely been reported. A 58-year-old woman presented involuntary movement in her all extremities after acute cerebral infarction on her right anterior cerebral artery territory. The involuntary movements were diagnosed as generalized chorea. We didn’t find any cause of generalized chorea except the acute cerebral infarction. Here, we described the case of generalized chorea after unilateral cerebral infarction discussing the possible mechanisms.


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  • Cerebral infarction in centrum semiovale presenting with hemichorea: a case report and literature review
    Jingjing Yi, Lingru Zhang, Tao Zhang, Jianfeng Li, Yifan Zhang, Meining Zhou
    Frontiers in Neurology.2023;[Epub]     CrossRef
  • Movement Disorders Associated With Cerebral Artery Stenosis: A Nationwide Study
    Kye Won Park, Nari Choi, Eungseok Oh, Chul Hyoung Lyoo, Min Seok Baek, Han-Joon Kim, Dalla Yoo, Jee-Young Lee, Ji-Hyun Choi, Jae Hyeok Lee, Seong-Beom Koh, Young Hee Sung, Jin Whan Cho, Hui-Jun Yang, Jinse Park, Hae-Won Shin, Tae-Beom Ahn, Ho-Sung Ryu, So
    Frontiers in Neurology.2022;[Epub]     CrossRef
  • Acute Hemichorea as an Unusual Presentation of Internal Carotid Artery Stenosis
    Dong Wook Kim, Youngchai Ko, Sang Hyun Jang, Soo Jin Yoon, Gun-Sei Oh, Soo Joo Lee, Dong Joo Yun
    Journal of Movement Disorders.2013; 6(1): 17.     CrossRef
A Case of Chorea as the Initial Manifestation of SLE Triggered by Estrogen
Su-Hyun Kim, Dong-Jin Shin, Hyeon-Mi Park, Yeong-Bae Lee, Kee-Hyung Park, Young-Hee Sung
J Mov Disord. 2008;1(2):86-89.
  • 9,242 View
  • 47 Download
AbstractAbstract PDF

Neurological complications of systemic lupus erythematosus (SLE) are relatively common, but chorea as the initial manifestation of SLE unmasked by exogenous estrogen is very rare. A-46-year old right handed woman presented with generalized chorea since 2 weeks ago. Her medical records revealed that the chorea had appeared within one month after estrogen medication. The laboratory test revealed positive antinuclear antibody (ANA), positive anti-dsDNA and positive anti-histone antibody. After discontinuation of estrogen, her choreic movement was not diminished. We report a case of newly diagnosed SLE attribute to chorea which triggered by estrogen.

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