Journal of Movement Disorders

Volume 12(1); January 2019

Review Articles

Abnormal Eye Movements in Parkinsonism and Movement Disorders: Ileok Jung, Ji-Soo Kim; J Mov Disord. 2019;12(1):1-13. Published online January 30, 2019; DOI: https://doi.org/10.14802/jmd.18034

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Abnormal eye movements are commonly observed in movement disorders. Ocular motility examination should include bedside evaluation and laboratory recording of ocular misalignment, involuntary eye movements, including nystagmus and saccadic intrusions/oscillations, triggered nystagmus, saccades, smooth pursuit (SP), and the vestibulo-ocular reflex. Patients with Parkinson’s disease (PD) mostly show hypometric saccades, especially for the selfpaced saccades, and impaired SP. Early vertical saccadic palsy is characteristic of progressive supranuclear palsy-Richardson’s syndrome. Patients with cortico-basal syndrome typically show a delayed onset of saccades. Downbeat and gaze-evoked nystagmus and hypermetric saccades are characteristic ocular motor findings in ataxic disorders due to cerebellar dysfunction. In this review, we discuss various ocular motor findings in movement disorders, including PD and related disorders, ataxic syndromes, and hyperkinetic movement disorders. Systemic evaluation of the ocular motor functions may provide valuable information for early detection and monitoring of movement disorders, despite an overlap in the abnormal eye movements among different movement disorders.

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Oculomotor Abnormalities in a Sheep (Ovis aries) Model of Huntington’s Disease: Towards a Biomarker for Assessing Therapeutic Efficacy
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A Case of AOA2 With Compound Heterozygous SETX Mutations
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Reading Warm-Up, Reading Skill, and Reading Prosody When Reading the My Grandfather Passage: An Exploratory Study Born Out of the Motor Planning Theory of Prosody and Reading Prosody Research
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The effects of age and sex on the incidence of multiple step saccades and corrective saccades
Wenbo Ma, Mingsha Zhang
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Oculomotor impairments in de novo Parkinson’s disease
Meng-Xi Zhou, Qin Wang, Yin Lin, Qian Xu, Li Wu, Ya-Jing Chen, Yu-Han Jiang, Qing He, Lei Zhao, You-Rong Dong, Jian-Ren Liu, Wei Chen
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Eye movement especially vertical oculomotor impairment as an aid to assess Parkinson’s disease
JianYuan Zhang, Bin Zhang, QingGuo Ren, Qing Zhong, Ye Li, GuoTao Liu, XiaoTian Ma, CuiPing Zhao
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Abnormalities of smooth pursuit in Parkinson’s disease: A systematic review
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Central nystagmus in progressive supranuclear palsy: A neglected clinical feature?
Maja Klarendic, Manja Hribar, Nina Bozanic Urbancic, Nina Zupancic, Milica G. Kramberger, Maja Trost, Saba Battelino, Diego Kaski, Maja Kojovic
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Hypomania and saccadic changes in Parkinson’s disease: influence of D2 and D3 dopaminergic signalling
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Management of Visual Dysfunction in Patients with Parkinson’s Disease
Joseph Savitt, Rachid Aouchiche, Bastiaan R. Bloem, Patrik Brundin
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Ocular motor manifestations of movement disorders
Grace F. Crotty, Bart K. Chwalisz
Current Opinion in Ophthalmology.2019; 30(6): 443. CrossRef

Pseudobulbar Affect in Parkinsonian Disorders: A Review: Mathew Hakimi, Carine W. Maurer; J Mov Disord. 2019;12(1):14-21. Published online January 30, 2019; DOI: https://doi.org/10.14802/jmd.18051

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Abstract PDF

Pseudobulbar affect (PBA) is a neurological symptom of inappropriate and uncontrollable laughter or crying that occurs secondary to a variety of neurological conditions, including parkinsonian disorders. PBA is a socially and emotionally debilitating symptom that has been estimated to affect 3.6% to 42.5% of the population with Parkinson’s disease. While indexing measures and treatment options for PBA have been extensively studied in neurological conditions such as amyotrophic lateral sclerosis and multiple sclerosis, there has been considerably less attention given in the literature to PBA in parkinsonian disorders. The purpose of this review is to discuss the pathophysiology of PBA, its prevalence and impact on quality of life in parkinsonian disorders, and the treatment options currently available. Areas requiring further study, including the development of standardized, cross-culturally validated methods of symptom assessment, and evidence-based studies exploring the efficacy of current treatment options in parkinsonian disorders, are also highlighted.

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Original Articles

Sensory Trick Frames: A New Device for Blepharospasm Patients: Daniele Lorenzano, Steven Tansley, Daniel G. Ezra; J Mov Disord. 2019;12(1):22-26. Published online January 16, 2019; DOI: https://doi.org/10.14802/jmd.18010

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Abstract PDF

Objective
To determine whether the use of unique customized spectacles provided with modified side arms may be helpful in reducing benign essential blepharospasm (BEB) in patients describing periocular sensory tricks (ST).
Methods
A prospective descriptive study of patients with BEB with positive periocular or temporal region ST phenomenon response under the care of the Botox Clinic at Moorfields Eye Hospital, London, UK. Nine consecutive patients with BEB describing ST were recruited, and the disease frequency and severity were assessed with the Jankovic Rating Scale (JRS) and the Blepharospasm Disability Index (BSDI) before and after the use of the sensory trick frames (STF).
Results
A reduction in the score was noted in both severity (p = 0.0115) and frequency patterns (p = 0.0117) in the JRS in patients using the STF. A significant reduction of the BSDI score was also observed (p = 0.0314).
Conclusion
All the patients selected and fitted with the STF had a reduction in spasms and related symptoms. This new device may be helpful in some selected BEB patients who previously responded positively to periocular pressure alleviating maneuvers.

Citations

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Electrically Induced Sensory Trick in a Patient with Musician’s Dystonia: A Case Report
Daisuke Nishida, Katsuhiro Mizuno, Osamu Takahashi, Meigen Liu, Tetsuya Tsuji
Brain Sciences.2023; 13(2): 223. CrossRef
Evaluation of Blepharoptosis in Patients With Refractory Blepharospasm by VISA—Video Recordings, Idiosyncratic Expressions, Sensory Tricks, and Ancillary Procedures
Chung-Sheng Lai, Savitha Ramachandran, Chia-Chen Lee, Ya-Wei Lai, Yang-Pei Chang, Shu-Hung Huang
Annals of Plastic Surgery.2023; 90(5S): S172. CrossRef
Variability of Movement Disorders: The Influence of Sensation, Action, Cognition, and Emotions
Rok Berlot, John C. Rothwell, Kailash P. Bhatia, Maja Kojović
Movement Disorders.2021; 36(3): 581. CrossRef
Impact of Upper Eyelid Surgery on Symptom Severity and Frequency in Benign Essential Blepharospasm
Hannah Mary Timlin, Kailun Jiang, Daniel George Ezra
Journal of Movement Disorders.2021; 14(1): 53. CrossRef
Benign essential blepharospasm: epidemiology, clinical manifestations, pathophysiology, botulinum toxin therapy
Z. A. Zalyalova
Neurology, Neuropsychiatry, Psychosomatics.2021; 13(1): 119. CrossRef
Best Practices in the Clinical Management of Progressive Supranuclear Palsy and Corticobasal Syndrome: A Consensus Statement of the CurePSP Centers of Care
Brent Bluett, Alexander Y. Pantelyat, Irene Litvan, Farwa Ali, Diana Apetauerova, Danny Bega, Lisa Bloom, James Bower, Adam L. Boxer, Marian L. Dale, Rohit Dhall, Antoine Duquette, Hubert H. Fernandez, Jori E. Fleisher, Murray Grossman, Michael Howell, Di
Frontiers in Neurology.2021;[Epub] CrossRef
Ocular motor manifestations of movement disorders
Grace F. Crotty, Bart K. Chwalisz
Current Opinion in Ophthalmology.2019; 30(6): 443. CrossRef

The ‘Hot Cross Bun’ Sign Is Not Always Multiple System Atrophy: Etiologies of 11 Cases: Christopher Way, David Pettersson, Amie Hiller; J Mov Disord. 2019;12(1):27-30. Published online December 19, 2018; DOI: https://doi.org/10.14802/jmd.18031

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Abstract PDF

Objective
To clarify the specificity of the ‘hot cross bun’ sign (HCBS) for multiple system atrophy (MSA) in adult cerebellar ataxia or parkinsonism.
Methods
The radiologic information systems at an academic center and affiliated veterans’ hospital were queried using the keywords ‘hot cross bun,’ ‘pontocerebellar,’ ‘cruciate,’ ‘cruciform,’ ‘MSA,’ ‘multiple system atrophy,’ and ‘multisystem atrophy.’ Scans were reviewed by a neurologist and neuroradiologist to identify the HCBS. Subjects with the HCBS were reviewed by 2 neurologists to identify the most likely etiology of the patient’s neurologic symptoms.
Results
Eleven cases were identified. Etiologies included MSA (4 probable, 2 possible), hereditary cerebellar ataxia (3/11), probable dementia with Lewy bodies (1/11), and uncertain despite autopsy (1/11).
Conclusion
MSA was the most common etiology. However, 5 of the 11 patients did not have MSA. The most common alternate etiology was an undefined hereditary cerebellar ataxia (3/11).

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Progressive supranuclear palsy: A case report and brief review of the literature
Vivek Batheja, Morgan Fish, Aneri B. Balar, Jeffery P. Hogg, Dhairya A. Lakhani, Musharaf Khan
Radiology Case Reports.2024; 19(1): 250. CrossRef
A case of bilateral middle cerebellar peduncle infarction with hot cross bun sign
Daisuke Kuzume, Yuko Morimoto, Satoshi Tsutsumi, Masahiro Yamasaki, Naohisa Hosomi
Rinsho Shinkeigaku.2024; 64(3): 190. CrossRef
Immune‐mediated spastic ataxia masquerading as clinically probable multisystem atrophy in an elderly woman
Rithvik Ramesh, Anuhya Chadalawada, Pedapati Radhakrishna, Lakshmi Narasimhan Ranganathan, Philo Hazeena, Sundar Shanmugam, Deepa Avadhani
Clinical and Experimental Neuroimmunology.2024;[Epub] CrossRef
Food for Thought: A Review of Neuroradiographic Signs Inspired by Food
C.T. Zoppo, T. Taros, J. Singh, A.S. Puri, A.L. Kuhn
Neurographics.2024; 14(1): 29. CrossRef
Structural and Molecular Imaging for Clinically Uncertain Parkinsonism
Sanskriti Sasikumar, Antonio P. Strafella
Seminars in Neurology.2023; 43(01): 095. CrossRef
Anti‐kelchlike protein 11 antibody‐associated encephalitis: Two case reports and review of the literature
Alejandro León Betancourt, Anina Schwarzwald, Alban Millonig, Michael Oberholzer, Lidia Sabater, Helly Hammer, Nicole Kamber, Lara Diem, Andrew Chan, Robert Hoepner, Anke Salmen, Christoph Friedli
European Journal of Neurology.2023; 30(6): 1801. CrossRef
Hjerne med kors
Linh Tran, Tuba Ahmad, Anniken Haslund, Phuoc Ngoc Thi Nguyen
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Clinical Neurology and Neurosurgery.2023; 233: 107975. CrossRef
Spinocerebellar ataxia-type 34: A case report and brief review of the literature
Vivek Batheja, Morgan Fish, Aneri B. Balar, Siddhi Hedge, Jeffery P. Hogg, Dhairya A. Lakhani, Musharaf Khan
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The “Black Straight-Line Sign” in the Putamen in Diffusion-Weighted Imaging: A Potential Diagnostic MRI Marker for Multiple System Atrophy
Yiming Zheng, Xiwen Wang, Huajian Zhao, Yanyan Jiang, Ying Zhu, Jing Chen, Wei Sun, Zhaoxia Wang, Yunchuang Sun
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Hung-Chieh Chen, Li-Hua Lee, Jiing-Feng Lirng, Bing-wen Soong
Scientific Reports.2022;[Epub] CrossRef
The “Hot Cross Bun Sign” in Spinocerebellar Ataxia Types 2 and 7–Case Reports and Review of Literature
Ansuya Kasavelu Naidoo, Cait‐Lynn Deanne Wells, Yashvir Rugbeer, Neil Naidoo
Movement Disorders Clinical Practice.2022; 9(8): 1105. CrossRef
Magnetic resonance imaging abnormalities as a marker of multiple system atrophy in isolated rapid eye movement sleep behavior disorder
Amaia Muñoz-Lopetegi, Joan Berenguer, Alex Iranzo, Monica Serradell, Teresa Pujol, Carles Gaig, Esteban Muñoz, Eduard Tolosa, Joan Santamaría
Sleep.2021;[Epub] CrossRef
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Margiben Tusharbhai Bhatt, R. Sunil
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Inna Page, Frank Gaillard
Practical Neurology.2020; 20(6): 463. CrossRef
Various Diseases and Clinical Heterogeneity Are Associated With “Hot Cross Bun”
Shuzhen Zhu, Hualing Li, Bin Deng, Jialing Zheng, Zifeng Huang, Zihan Chang, Yanjun Huang, Zhibo Wen, Yanran Liang, Mengjue Yu, Ling-Ling Chan, Eng-King Tan, Qing Wang
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Hot cross bun sign
M. Portet, M. Filyridou, D. C. Howlett
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Progressive Supranuclear Palsy, Corticobasal Degeneration, and Multiple System Atrophy
Paul Greene
CONTINUUM: Lifelong Learning in Neurology.2019; 25(4): 919. CrossRef
Differential value of brain magnetic resonance imaging in multiple system atrophy cerebellar phenotype and spinocerebellar ataxias
Minkyeong Kim, Jong Hyeon Ahn, Yoonsu Cho, Ji Sun Kim, Jinyoung Youn, Jin Whan Cho
Scientific Reports.2019;[Epub] CrossRef

Clinical Characteristics of Involuntary Movement in Hospitalized Patients: Kyum-Yil Kwon, Hye Mi Lee, Seon-Min Lee, Seong-Beom Koh; J Mov Disord. 2019;12(1):31-36. Published online December 20, 2018; DOI: https://doi.org/10.14802/jmd.18040

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Abstract PDF

Objective
Neurological symptoms in hospitalized patients are not rare, and neurological consultation for movement disorders is especially important in evaluating or managing those with various movement disorders. Therefore, we investigated a clinical pattern of in-hospital consultations for various movement disorders in a tertiary care university hospital.
Methods
Over two years, a total of 202 patients (70.7 ± 11.8 years of age) presenting with movement disorders referred to movement disorder specialists were investigated.
Results
The main symptoms referred by nonneurologists were tremor (56.9%), parkinsonism (16.8%), and gait disturbance (8.9%). The most frequent diagnostic category was toxic/metabolic-caused movement disorder (T/MCMD) (35%) with regard to medications, followed by Parkinson’s disease (PD) (16%). Regarding the mode of onset, T/MCMD was the leading cause for acute (68%) and subacute onset (46%), while PD was the leading disorder (31%) for chronic onset.
Conclusion
The current study showed a characteristic pattern of inpatients presenting with movement disorders. Furthermore, our findings highlighted the clinical significance of drug use or metabolic problems for treating this patient population.

Citations

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Pattern and frequency of involuntary movements: hospital-based study
H. N. El Tallawy, A. S. Shalash, M. A. Abdelhamed, R. E. Elsabrout
The Egyptian Journal of Neurology, Psychiatry and Neurosurgery.2023;[Epub] CrossRef
Clinical Spectrum of Movement Disorders in Neurology Inpatients in a Tertiary Care Centre
Shabeer Ahmad Paul, Gouranga Prasad Mondal, Ramesh Bhattacharyya, Kartik Chandra Ghosh, Sarbajit Das, Suman Das, Hema Krishna, Chandrakanta Patra
Journal of Neurosciences in Rural Practice.2021; 12: 581. CrossRef

Less Pulsatile Levodopa Therapy (6 Doses Daily) Is Associated with a Reduced Incidence of Dyskinesia: Mark M. Lin, Robert Laureno; J Mov Disord. 2019;12(1):37-42. Published online January 30, 2019; DOI: https://doi.org/10.14802/jmd.18046

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Abstract PDF

Objective
To evaluate whether less pulsatile levodopa therapy (LPT) can reduce the development of levodopa-induced dyskinesia (LID).
Methods
This is a retrospective cohort study of patients with Parkinson’s disease at the movement disorders clinic of Medstar Washington Hospital Center. The study was not blinded or randomized. Patients were seen between August 2002 and August 2018. During these years, we treated patients with less pulsatile (6 doses daily) levodopa treatment to reduce LID. Occurrence of LID was recorded.
Results
Ninety-five patients with Parkinson’s disease taking levodopa were divided into two groups: 1) patients who were initially managed on LPT or who switched from traditional therapy (TT) (n = 61) (mean disease duration: 7.7 ± 4.8 years, mean levodopa duration: 5.6 ± 4.5 years and mean observation time: 4.3 ± 3.4 years), and 2) patients on TT throughout the observation period or until they developed dyskinesia (n = 34) (mean disease duration: 8.3 ± 3.8 years, mean levodopa duration: 6.2 ± 4.2 years and mean observation time: 4.1 ± 3.4 years). Three of the 61 LPT patients developed dyskinesia during the observation period. One of the patients developed dyskinesia after being switched to pulsatile doses by another doctor. In the other two, dyskinesia was minimal. In contrast to this 4.9% cumulative incidence, dyskinesia occurred in 50% (17/34) of TT patients, an incidence similar to that in published data (p < 0.001).
Conclusion
Less pulsatile levodopa with 6 daily doses was associated with a low incidence of LID. Further study of this method of treatment is warranted.

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The Gut Microbiome: A Therapeutically Targetable Site of Peripheral Levodopa Metabolism
Eoin Mulroy, Kailash P. Bhatia
Movement Disorders Clinical Practice.2019; 6(7): 547. CrossRef

Brief communication

Assessment of Bone Mineral Density of Patients with Spinocerebellar Ataxia Type 3: Aline MS Farias, Simone Appenzeller, Marcondes C França, Alberto RM Martinez, Elba E Etchebehere, Thiago F Souza, Allan O Santos; J Mov Disord. 2019;12(1):43-46. Published online January 30, 2019; DOI: https://doi.org/10.14802/jmd.18041

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Objective
Machado-Joseph disease (MJD) is a spinocerebellar ataxia, and osteoporosis is a multifactor disease that may affect patients with neurologic conditions. The frequency of osteoporosis among MJD patients, however, has not been studied. The purpose of this study is to evaluate bone mineral density (BMD) and identify correlations between clinical factors and frequency of vertebral fractures in patients with MJD.
Methods
Clinical data, lumbar X-rays and BMD data were obtained in 30 patients with MJD.
Results
Ten patients (33.3%) showed low BMD in at least one of the sites studied based on Z-scores. The Z-score correlated directly with body mass index, and the femoral neck Z-score was inversely correlated with cytosine-adenine-guanine (CAG) expansion. There was no correlation between BMD and other clinical factors. Forty-three percent of the patients reported previous pathologic fractures. Five patients (16.7%) had at least one fracture detected by lumbar X-ray.
Conclusion
Low BMD and fractures are frequent among MJD patients, and careful management of BMD may be beneficial for these patients.

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Overview of the Clinical Approach to Individuals With Cerebellar Ataxia and Neuropathy
Leslie J. Roberts, Michael McVeigh, Linda Seiderer, Ian H. Harding, Louise A. Corben, Martin Delatycki, David J. Szmulewicz
Neurology Genetics.2022;[Epub] CrossRef
Effects of Neurological Disorders on Bone Health
Ryan R. Kelly, Sara J. Sidles, Amanda C. LaRue
Frontiers in Psychology.2020;[Epub] CrossRef

Case Report

Treatment of Hemichoreoathetosis with Arrhythmic Proximal Tremor after Stroke: The Role of Zona Incerta as a Target for Deep Brain Stimulation: Andrei Koerbel, Augusto Radünz do Amaral, Helena Bedatti Zeh, Eduardo Wollmann, Renata Fabiola Heil Koerbel, Carla Moro, Alexandre Luiz Longo; J Mov Disord. 2019;12(1):47-51. Published online January 30, 2019; DOI: https://doi.org/10.14802/jmd.18032

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Deep brain stimulation (DBS) of the zona incerta has shown promising results in the reduction of medically refractory movement disorders. However, evidence supporting its efficacy in movement disorders secondary to hemorrhagic stroke or hemichoreoathetosis is limited. We describe a 48-year-old man who developed progressive hemichoreoathetosis with an arrhythmic, proximal tremor in his right arm following a thalamic hemorrhagic stroke. Pharmacological treatment was carried out with no change in the Abnormal Involuntary Movement Scale (AIMS) score after 4 weeks (14). After six sessions of botulinum toxin treatment, a subtle improvement in the AIMS score (13) was registered, but no clinical improvement was noted. The arrhythmic proximal movements were significantly improved after DBS of the zona incerta with a major decrease in the patient’s AIMS score (8). The response to DBS occurring after the failure of pharmacological and botulinum toxin treatments suggests that zona incerta DBS may be an alternative for postthalamic hemorrhage movement disorders.

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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

Letters to the editor

Paroxysmal Kinesigenic Dyskinesia Presented Following Concussion: Norman Cottrill, Brian McCully, Mary Payne; J Mov Disord. 2019;12(1):52-53. Published online December 19, 2018; DOI: https://doi.org/10.14802/jmd.18027

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Brenda Zhang, Joseph P Settineri, Alan Chajet, Purushothaman Muthukanagaraj
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Translational Neurodegeneration.2021;[Epub] CrossRef

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Call Center Operator’s Dystonia: Dokyung Lee, Jung A Kim; J Mov Disord. 2019;12(1):57-59. Published online December 19, 2018; DOI: https://doi.org/10.14802/jmd.18038

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Jason S. Gill, Megan X. Nguyen, Mariam Hull, Meike E. van der Heijden, Ken Nguyen, Sruthi P. Thomas, Roy V. Sillitoe
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Transient Hypersomnolence Provoked by Metoclopramide in a Patient with Degenerative Parkinsonism: Sang-Won Yoo, Ko Eun Choi, Joong-Seok Kim; J Mov Disord. 2019;12(1):60-62. Published online January 15, 2019; DOI: https://doi.org/10.14802/jmd.18042

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Novel Ferritin Light Chain Gene Mutation in a Korean Patient with Neuroferritinopathy: So Hoon Yoon, Nan Young Kim, Yun Joong Kim, Chul Hyoung Lyoo; J Mov Disord. 2019;12(1):63-65. Published online January 30, 2019; DOI: https://doi.org/10.14802/jmd.18062

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