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Review Article
Pallidus Stimulation for Chorea-Acanthocytosis: A Systematic Review and Meta-Analysis of Individual Data
Weibin He, Chenhui Li, Hongjuan Dong, Lingmin Shao, Bo Yin, Dianyou Li, Liguo Ye, Ping Hu, Chencheng Zhang, Wei Yi
J Mov Disord. 2022;15(3):197-205.   Published online July 26, 2022
DOI: https://doi.org/10.14802/jmd.22003
  • 1,355 View
  • 161 Download
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.
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
DOI: https://doi.org/10.14802/jmd.21185
  • 1,088 View
  • 56 Download
AbstractAbstract PDFSupplementary Material
Objective
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.
Methods
We report four patients and systematically reviewed all published cases of ESKD presenting with movement disorders and bilateral BG lesions (EBSCOhost and Ovid).
Results
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.
Conclusion
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
DOI: https://doi.org/10.14802/jmd.18032
  • 5,240 View
  • 105 Download
  • 3 Citations
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

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
DOI: https://doi.org/10.14802/jmd.15009
  • 25,384 View
  • 316 Download
  • 31 Citations
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

Citations to this article as recorded by  
  • 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; 44(10): 2200106.     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
  • 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.2021;[Epub]     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 heterozygousVPS13Alarge 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; 4(3): e242.     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 forXKdeletions 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
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  • 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
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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
DOI: https://doi.org/10.14802/jmd.14001
  • 12,791 View
  • 86 Download
  • 5 Citations
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

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.
DOI: https://doi.org/10.14802/jmd.13004
  • 14,919 View
  • 89 Download
  • 10 Citations
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

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
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    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
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    Yong Chuan Chee, Beng Hooi Ong
    Neurology and Clinical Neuroscience.2020; 8(1): 44.     CrossRef
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    Kazuki Muguruma, Atsuko Motoda, Takamichi Sugimoto, Takeshi Kitamura
    Rinsho Shinkeigaku.2019; 59(8): 509.     CrossRef
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    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.
DOI: https://doi.org/10.14802/jmd.13003
  • 10,172 View
  • 93 Download
  • 3 Citations
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

Citations to this article as recorded by  
  • 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
  • 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
Hemichorea-Hemiballism with a Diabetic Patient
Yoo Hwan Kim, Ju Yeon Kim, Hung Youl Seok, Seong-Beom Koh
J Mov Disord. 2010;3(1):20-21.
DOI: https://doi.org/10.14802/jmd.10006
  • 9,369 View
  • 55 Download
  • 2 Citations
AbstractAbstract PDF

Chorea and ballism are movement disorders that result from a variety of conditions. They are an uncommon manifestation of diabetes mellitus. We report a 52-year-old diabetic man who presented with acute onset chorea-ballism with a putaminal high-signal-intensity lesion on T1-weighted magnetic resonance imaging (MRI).

Citations

Citations to this article as recorded by  
  • “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
  • NEUROLOGINĖ PATOLOGIJA SERGANT 2 TIPO CUKRINIU DIABETU
    Liudmila Kimševaitė
    Medicinos teorija ir praktika.2017; 22(4): 328.     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.
DOI: https://doi.org/10.14802/jmd.09027
  • 17,404 View
  • 78 Download
  • 4 Citations
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.

Citations

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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.
DOI: https://doi.org/10.14802/jmd.09021
  • 15,712 View
  • 43 Download
  • 3 Citations
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.

Citations

Citations to this article as recorded by  
  • 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.
DOI: https://doi.org/10.14802/jmd.09020
  • 18,956 View
  • 74 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.
DOI: https://doi.org/10.14802/jmd.09016
  • 16,701 View
  • 378 Download
  • 9 Citations
AbstractAbstract PDF
Background:

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.

Purpose:

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.

Methods:

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.

Results:

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.

Conclusions:

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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  • 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
  • A CASE SUPPORTING THE ROLE OF THE CEREBELLUM IN DYSTONIA
    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.
DOI: https://doi.org/10.14802/jmd.09009
  • 7,814 View
  • 81 Download
  • 2 Citations
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.

Citations

Citations to this article as recorded by  
  • 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.
DOI: https://doi.org/10.14802/jmd.08016
  • 8,242 View
  • 46 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.

Chorea as an Initial Manifestation of Polycythemia Vera
Ji Eun Lee, Hae-Won Shin, Young H. Sohn
J Mov Disord. 2008;1(2):82-85.
DOI: https://doi.org/10.14802/jmd.08015
  • 9,918 View
  • 122 Download
AbstractAbstract PDF

Chorea is a rare complication of polycythemia vera (PV). We report a 58-year-old woman with acute onset chorea without structural lesion in the basal ganglia. The physical and laboratory findings were compatible with the diagnosis of PV. After repeated phlebotomies her chorea was improved. PV should be considered as one of the possible etiologies of chorea, as early diagnosis is important to lead to the effective treatment and prevention of complications.


JMD : Journal of Movement Disorders