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Volume 12(3); September 2019
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Review Articles
Modeling α-Synuclein Propagation with Preformed Fibril Injections
Hyun Kyung Chung, Hoang-Anh Ho, Dayana Pérez-Acuña, Seung-Jae Lee
J Mov Disord. 2019;12(3):139-151.   Published online September 30, 2019
DOI: https://doi.org/10.14802/jmd.19046
Correction in: J Mov Disord 2020;13(1):77
  • 11,890 View
  • 738 Download
  • 35 Citations
AbstractAbstract PDF
The aggregation of α-synuclein (α-syn) has been implicated in the pathogenesis of many neurodegenerative disorders, including Parkinson’s disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA). Postmortem analyses of α-syn pathology, especially that of PD, have suggested that aggregates progressively spread from a few discrete locations to wider brain regions. The neuron-to-neuron propagation of α-syn has been suggested to be the underlying mechanism by which aggregates spread throughout the brain. Many cellular and animal models has been created to study cell-to-cell propagation. Recently, it has been shown that a single injection of preformed fibrils (PFFs) made of recombinant α-syn proteins into various tissues and organs of many different animal species results in widespread α-syn pathology in the central nervous system (CNS). These PFF models have been extensively used to study the mechanism by which aggregates spread throughout the brain. Here, we review what we have learned from PFF models, describe the nature of PFFs and the neuropathological features, neurophysiological characteristics, and behavioral outcomes of the models.

Citations

Citations to this article as recorded by  
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  • Neurons with Cat’s Eyes: A Synthetic Strain of α-Synuclein Fibrils Seeding Neuronal Intranuclear Inclusions
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    Molecular Neurobiology.2021; 58(7): 3031.     CrossRef
  • Trans-synaptic spreading of alpha-synuclein pathology through sensory afferents leads to sensory nerve degeneration and neuropathic pain
    Nelson Ferreira, Nádia Pereira Gonçalves, Asad Jan, Nanna Møller Jensen, Amelia van der Laan, Simin Mohseni, Christian Bjerggaard Vægter, Poul Henning Jensen
    Acta Neuropathologica Communications.2021;[Epub]     CrossRef
  • Pharmacological Inhibition of Brain EGFR Activation By a BBB-penetrating Inhibitor, AZD3759, Attenuates α-synuclein Pathology in a Mouse Model of α-Synuclein Propagation
    Omid Tavassoly, Esther del Cid Pellitero, Frederique Larroquette, Eddie Cai, Rhalena A. Thomas, Vincent Soubannier, Wen Luo, Thomas M. Durcan, Edward A. Fon
    Neurotherapeutics.2021; 18(2): 979.     CrossRef
  • The Future of Incretin-Based Approaches for Neurodegenerative Diseases in Older Adults: Which to Choose? A Review of their Potential Efficacy and Suitability
    Christine Girges, Nirosen Vijiaratnam, Dilan Athauda, Grace Auld, Sonia Gandhi, Thomas Foltynie
    Drugs & Aging.2021; 38(5): 355.     CrossRef
  • Prodromal neuroinvasion of pathological α-synuclein in brainstem reticular nuclei and white matter lesions in a model of α-synucleinopathy
    Nelson Ferreira, Mette Richner, Amelia van der Laan, Ida Bergholdt Jul Christiansen, Christian B Vægter, Jens R Nyengaard, Glenda M Halliday, Joachim Weis, Benoit I Giasson, Ian R Mackenzie, Poul H Jensen, Asad Jan
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  • Impact of the apelin/APJ axis in the pathogenesis of Parkinson’s disease with therapeutic potential
    Efthalia Angelopoulou, Yam Nath Paudel, Anastasia Bougea, Christina Piperi
    Journal of Neuroscience Research.2021; 99(9): 2117.     CrossRef
  • Adenovirus-induced Reactive Astrogliosis Exacerbates the Pathology of Parkinson’s Disease
    Heeyoung An, Hyowon Lee, Seulkee Yang, Woojin Won, C. Justin Lee, Min-Ho Nam
    Experimental Neurobiology.2021; 30(3): 222.     CrossRef
  • Brain iron enrichment attenuates α‐synuclein spreading after injection of preformed fibrils
    Karina Dauer née Joppe, Lars Tatenhorst, Lucas Caldi Gomes, Shuyu Zhang, Mojan Parvaz, Eleonora Carboni, Anna‐Elisa Roser, Hazem El DeBakey, Mathias Bähr, Katarina Vogel‐Mikuš, Chi Wang Ip, Stefan Becker, Markus Zweckstetter, Paul Lingor
    Journal of Neurochemistry.2021; 159(3): 554.     CrossRef
  • Increased oscillatory power in a computational model of the olfactory bulb due to synaptic degeneration
    J. Kendall Berry, Daniel Cox
    Physical Review E.2021;[Epub]     CrossRef
  • Combining Automated Organoid Workflows with Artificial Intelligence‐Based Analyses: Opportunities to Build a New Generation of Interdisciplinary High‐Throughput Screens for Parkinson's Disease and Beyond
    Henrik Renner, Hans R. Schöler, Jan M. Bruder
    Movement Disorders.2021; 36(12): 2745.     CrossRef
  • Is Multiple System Atrophy a Prion-like Disorder?
    Kurt A. Jellinger, Gregor K. Wenning, Nadia Stefanova
    International Journal of Molecular Sciences.2021; 22(18): 10093.     CrossRef
  • Cortical alpha-synuclein preformed fibrils do not affect interval timing in mice
    Qiang Zhang, Hisham Abdelmotilib, Travis Larson, Cameron Keomanivong, Mackenzie Conlon, Georgina M. Aldridge, Nandakumar S. Narayanan
    Neuroscience Letters.2021; 765: 136273.     CrossRef
  • Temporal Evolution of Inflammation and Neurodegeneration With Alpha-Synuclein Propagation in Parkinson's Disease Mouse Model
    Thuy Thi Lai, Yun Joong Kim, Phuong Thi Nguyen, Young Ho Koh, Tinh Thi Nguyen, Hyeo-il Ma, Young Eun Kim
    Frontiers in Integrative Neuroscience.2021;[Epub]     CrossRef
  • The Concept of α-Synuclein Strains and How Different Conformations May Explain Distinct Neurodegenerative Disorders
    Katja Malfertheiner, Nadia Stefanova, Antonio Heras-Garvin
    Frontiers in Neurology.2021;[Epub]     CrossRef
  • A historical review of multiple system atrophy with a critical appraisal of cellular and animal models
    David J. Marmion, Wouter Peelaerts, Jeffrey H. Kordower
    Journal of Neural Transmission.2021; 128(10): 1507.     CrossRef
  • Exploring the Etiological Links behind Neurodegenerative Diseases: Inflammatory Cytokines and Bioactive Kynurenines
    Masaru Tanaka, József Toldi, László Vécsei
    International Journal of Molecular Sciences.2020; 21(7): 2431.     CrossRef
  • Modeling Parkinson’s Disease With the Alpha-Synuclein Protein
    Mónica Gómez-Benito, Noelia Granado, Patricia García-Sanz, Anne Michel, Mireille Dumoulin, Rosario Moratalla
    Frontiers in Pharmacology.2020;[Epub]     CrossRef
  • Enhanced Susceptibility of PINK1 Knockout Rats to α-Synuclein Fibrils
    Rose B. Creed, Matthew S. Goldberg
    Neuroscience.2020; 437: 64.     CrossRef
  • Pathogenic Mutations Differentially Regulate Cell-to-Cell Transmission of α-Synuclein
    Yuan Guan, Xiaofang Zhao, Fengwei Liu, Shuxin Yan, Yalong Wang, Cuilian Du, Xiuyu Cui, Rena Li, Claire Xi Zhang
    Frontiers in Cellular Neuroscience.2020;[Epub]     CrossRef
  • Dopamine, Alpha-Synuclein, and Mitochondrial Dysfunctions in Parkinsonian Eyes
    Alessia Indrieri, Rocco Pizzarelli, Brunella Franco, Elvira De Leonibus
    Frontiers in Neuroscience.2020;[Epub]     CrossRef
  • Classic and evolving animal models in Parkinson's disease
    Tanvi Pingale, Girdhari Lal Gupta
    Pharmacology Biochemistry and Behavior.2020; 199: 173060.     CrossRef
Oro-Pharyngeal Dysphagia in Parkinson’s Disease and Related Movement Disorders
Miseon Kwon, Jae-Hong Lee
J Mov Disord. 2019;12(3):152-160.   Published online September 30, 2019
DOI: https://doi.org/10.14802/jmd.19048
  • 36,193 View
  • 946 Download
  • 25 Citations
AbstractAbstract PDFSupplementary Material
Oro-pharyngeal dysphagia is a common symptom in patients with Parkinson’s disease (PD) and related disorders, even in their early stage of diseases. Dysphagia in these patients has been underdiagnosed, probably due to poor the self-awareness of the conditions and the underuse of validated tools and objective instruments for assessment. The early detection and intervention of dysphagia are closely related to improving the quality of life and decreasing the mortality rate in these patients. The purpose of this paper is to give an overview of the characteristics of dysphagia, including the epidemiology, pathophysiology, and clinical symptomatology, in patients with PD compared with other parkinsonian disorders and movement disorders. The management of dysphagia and future research directions related to these disorders are also discussed.

Citations

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  • Dysphagia Presentation, Airway Invasion, and Gender Differences in a Clinically Based Sample of People with Parkinson’s Disease
    Matthew Dumican, Christopher Watts, Teresa Drulia, Yan Zhang
    Dysphagia.2023; 38(1): 353.     CrossRef
  • Progression of Self-Perceived Speech and Swallowing Impairment in Early Stage Parkinson's Disease: Longitudinal Analysis of the Unified Parkinson's Disease Rating Scale
    Christopher R. Watts, Yan Zhang
    Journal of Speech, Language, and Hearing Research.2022; 65(1): 146.     CrossRef
  • Terminal Choking in Parkinson's Disease
    Andrea Ling, Fiona Herbert, Bethany Wright, Edward Richfield
    Journal of Pain and Symptom Management.2022; 63(6): e757.     CrossRef
  • Effects of a tongue training program in Parkinson's disease: Analysis of electrical activity and strength of suprahyoid muscles
    Exequiel Plaza, Angela Ruviaro Busanello-Stella
    Journal of Electromyography and Kinesiology.2022; 63: 102642.     CrossRef
  • Antidepressants Usage and Risk of Pneumonia Among Elderly Patients With the Parkinson's Disease: A Population-Based Case-Control Study
    Wei-Yin Kuo, Kuang-Hua Huang, Yu-Hsiang Kuan, Yu-Chia Chang, Tung-Han Tsai, Chien-Ying Lee
    Frontiers in Medicine.2022;[Epub]     CrossRef
  • Neuropathological Aspects of SARS-CoV-2 Infection: Significance for Both Alzheimer’s and Parkinson’s Disease
    Jaime Silva, Felipe Patricio, Aleidy Patricio-Martínez, Gerardo Santos-López, Lilia Cedillo, Yousef Tizabi, Ilhuicamina Daniel Limón
    Frontiers in Neuroscience.2022;[Epub]     CrossRef
  • Identifying rates and risk factors for medication errors during hospitalization in the Australian Parkinson’s disease population: A 3-year, multi-center study
    Michael Bakker, Michaela E. Johnson, Lauren Corre, Deanna N. Mill, Xingzhuo Li, Richard J. Woodman, Jacinta L. Johnson, Ismaeel Yunusa
    PLOS ONE.2022; 17(5): e0267969.     CrossRef
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    International Journal of Environmental Research and Public Health.2021; 18(17): 9410.     CrossRef
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  • Parkinson Hastalığında Yutma Bozuklukları
    Merve SAPMAZ ATALAR, Gençer GENÇ
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    Matthew Dumican, Christopher Watts
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Viewpoint
Recent Advances in the Development of Experimental Therapeutics for Levodopa-Induced Dyskinesia
Michael L. Martini, Sean N. Neifert, J Mocco, Fedor Panov, Winona Tse, Ruth H. Walker, Jian Jin, Fiona Gupta
J Mov Disord. 2019;12(3):161-165.   Published online September 30, 2019
DOI: https://doi.org/10.14802/jmd.19029
  • 5,166 View
  • 181 Download
  • 1 Citations
PDF

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  • Antioxidant Effect of Alpha-Lipoic Acid in 6-Hydroxydopamine Unilateral Intrastriatal Injected Rats
    Pavlina Andreeva-Gateva, Lubomir Traikov, Zafer Sabit, Dimitar Bakalov, Radka Tafradjiiska-Hadjiolova
    Antioxidants.2020; 9(2): 122.     CrossRef
Original Articles
Increased Signal in the Superior Cerebellar Peduncle of Patients with Progressive Supranuclear Palsy
Hiroshi Kataoka, Yukako Nishimori, Takao Kiriyama, Hitoki Nanaura, Tesseki Izumi, Nobuyuki Eura, Naoki Iwasa, Kazuma Sugie
J Mov Disord. 2019;12(3):166-171.   Published online August 9, 2019
DOI: https://doi.org/10.14802/jmd.19002
  • 7,039 View
  • 195 Download
  • 2 Citations
AbstractAbstract PDF
Objective
The provisional diagnosis of progressive supranuclear palsy (PSP) depends on a combination of typical clinical features and specific MRI findings, such as atrophy of the tegmentum in the midbrain. Atrophy of the superior cerebellar peduncle (SCP) distinguishes PSP from other types of parkinsonism. Histological factors affect the conventional fluid-attenuated inversion recovery (FLAIR) signals, such as the extent of neuronal loss and gliosis.
Methods
We investigated patients with PSP to verify the percentage of patients with various PSP phenotypes presenting a high signal intensity in the SCP. Three interviewers, who were not informed about the clinical data, visually inspected the presence or absence of a high signal intensity in the SCP on the FLAIR images. We measured the pixel value in the SCP of each patient. Clinical characteristics were evaluated using the Mann-Whitney test, followed by the χ2 test.
Results
Ten of the 51 patients with PSP showed a high signal intensity in the SCP on FLAIR MRI. Higher pixel values were observed within the SCP of patients with a high signal intensity in the SCP than in patients without a high signal intensity (p < 0.001). The sensitivity and specificity of the high signal intensity in the SCP of patients with PSP was 19.6% and 100%, respectively. This finding was more frequently observed in patients with PSP with Richardson’s syndrome (PSP-RS) (25.7%) than other phenotypes (6.2%).
Conclusion
The high signal intensity in the SCP on FLAIR MRI might be an effective diagnostic tool for PSP-RS.

Citations

Citations to this article as recorded by  
  • Diffusion tractography of superior cerebellar peduncle and dentatorubrothalamic tracts in two autopsy confirmed progressive supranuclear palsy variants: Richardson syndrome and the speech-language variant
    Rodolfo G. Gatto, Peter R. Martin, Farwa Ali, Heather M. Clark, Joseph R. Duffy, Rene L. Utianski, Hugo Botha, Mary M. Machulda, Dennis W. Dickson, Keith A. Josephs, Jennifer L. Whitwell
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    Lydia Chougar, Nadya Pyatigorskaya, Bertrand Degos, David Grabli, Stéphane Lehéricy
    Frontiers in Neurology.2020;[Epub]     CrossRef
The Impact of Impulsivity on Quality of Life in Early Drug-Naïve Parkinson’s Disease Patients
Dong-Woo Ryu, Joong-Seok Kim, Sang-Won Yoo, Yoon-Sang Oh, Kwang-Soo Lee
J Mov Disord. 2019;12(3):172-176.   Published online August 9, 2019
DOI: https://doi.org/10.14802/jmd.19004
Correction in: J Mov Disord 2021;14(2):176
  • 4,182 View
  • 103 Download
  • 4 Citations
AbstractAbstract PDF
Objective
Impulse control disorders (ICDs) in Parkinson’s disease (PD) are mostly related to dopamine replacement therapy (DRT); however, drug-naïve PD patients have also frequently experienced impulsivity. This phenomenon makes clinicians hesitate treating patients with DRT. In this study, we assessed the effect of impulsivity on quality of life (QOL) in drug-naïve PD patients.
Methods
Two hundred three newly diagnosed, nonmedicated PD patients were enrolled, and they received structured clinical interviews, physical examinations and validated questionnaires to evaluate motor and nonmotor symptoms and QOL. Impulsivity was evaluated using the Questionnaire for Impulsive-Compulsive Disorders in Parkinson’s Disease-Rating Scale (QUIP-RS).
Results
Thirty-eight patients (18.7%) had impulsivity with QUIP-RS scores ≥ 1 and 4 patients (2.0%) were diagnosed with combined ICDs. Motor and nonmotor symptoms were significantly correlated with the Parkinson’s Disease Questionnaire-39 summary index. Female sex and QUIP-RS scores were also correlated with QOL in drug-naïve PD patients.
Conclusion
The results of the present study showed that impulsivity negatively influences QOL in early drug-naïve PD patients. In addition, more severe motor and nonmotor symptoms were also associated with lower QOL. Such findings complicate treatment but provide valuable information for managing early PD.

Citations

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  • Neuropsychiatric Symptoms in Parkinson's Disease After Subthalamic Nucleus Deep Brain Stimulation
    Weibing Liu, Tatsuya Yamamoto, Yoshitaka Yamanaka, Masato Asahina, Tomoyuki Uchiyama, Shigeki Hirano, Keisuke Shimizu, Yoshinori Higuchi, Satoshi Kuwabara
    Frontiers in Neurology.2021;[Epub]     CrossRef
  • Dopamine genetic risk score predicts impulse control behaviors in Parkinson’s disease
    Alison Hall, Samuel R. Weaver, Lindsey J. Compton, Winston D. Byblow, Ned Jenkinson, Hayley J. MacDonald
    Clinical Parkinsonism & Related Disorders.2021; 5: 100113.     CrossRef
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    Mohammed Munther Al-Hammouri, Jehad A. Rababah, Celeste Shawler
    Advances in Nursing Science.2021; 44(4): 357.     CrossRef
  • Assessment of therapeutic strategies for management of impulse control disorder in Parkinson’s disease
    Mayela Rodríguez-Violante, Yazmín Ríos-Solís, Oscar Esquivel-Zapata, Fanny Herrera, Susana López-Alamillo, Cynthia Sarabia-Tapia, Amin Cervantes-Arriaga
    Arquivos de Neuro-Psiquiatria.2021; 79(11): 989.     CrossRef
Clinical Milestones Preceding the Diagnosis of Multiple System Atrophy and Progressive Supranuclear Palsy: A Retrospective Cohort Study
Louise Wiblin, Rory Durcan, Brook Galna, Mark Lee, David Burn
J Mov Disord. 2019;12(3):177-183.   Published online August 9, 2019
DOI: https://doi.org/10.14802/jmd.19015
  • 5,664 View
  • 193 Download
  • 4 Citations
AbstractAbstract PDF
Objective
Multiple System Atrophy (MSA) and progressive supranuclear palsy (PSP) are rapidly progressive forms of degenerative Parkinsonism. The difficulties of diagnosing MSA and PSP in their early stages may lead to delayed referral to appropriate specialists and distress to patients, as well as delaying symptomatic treatment and participation in clinical trials. This work aimed to describe the symptoms that patients with MSA and PSP developed and plot their emergence relative to final diagnosis using a median onset in months.
Methods
Forty-seven patients from the United Kingdom with MSA or PSP diagnosed by a movement disorder specialist were interviewed with carers or relatives to establish milestone onset. This was corroborated using clinical notes and letters.
Results
In the MSA cohort (n = 23), autonomic symptoms (median 5.5 months before diagnosis) and falls (median 1 month before diagnosis) were the two clinical milestones which occurred before diagnosis. In the PSP cohort (n = 24), falling was the only milestone which occurred before diagnosis (median of 18.5 months).
Conclusion
This Study Shows That Psp Patients Experience Falling More Than A Year And A Half An Average Before Receiving A Diagnosis And Although Msa Patients Also Tended To Fall, This Was Much Closer To The Time Of Diagnosis. Further Work With Larger Cohorts May Illustrate Whether These Preliminary Findings Can Be Generalised To Guide Diagnosis And Management.

Citations

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  • Toward More Accessible Fully Automated 3D Volumetric MRI Decision Trees for the Differential Diagnosis of Multiple System Atrophy, Related Disorders, and Age-Matched Healthy Subjects
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Letters to the editor
Fragile X-Associated Tremor/Ataxia Syndrome: An Illustrative Case
Chaewon Lee, Kye Won Park, Nari Choi, Ho-Sung Ryu, Sun Ju Chung
J Mov Disord. 2019;12(3):184-186.   Published online July 17, 2019
DOI: https://doi.org/10.14802/jmd.18060
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  • Brain 18F-FDG and 18F-Flumetamol PET Imaging of Fragile X-Associated Tremor Ataxia Syndrome
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Hyperglycemia-Associated Hemichorea-Hemiballismus with Predominant Ipsilateral Putaminal Abnormality on Neuroimaging
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DOI: https://doi.org/10.14802/jmd.19014
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  • Consideration for Hemiballismus in the Differential Diagnosis: A Rare Case of Hyperosmolar Hyperglycemic State
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Successful Pallidal Deep Brain Stimulation in a Patient with Childhood-Onset Generalized Dystonia with ANO3 Mutation
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J Mov Disord. 2019;12(3):190-191.   Published online July 17, 2019
DOI: https://doi.org/10.14802/jmd.19016
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  • Deep Brain Stimulation in Dystonia: Disentangling Heterogeneity
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    Frontiers in Neurology.2021;[Epub]     CrossRef
  • The expanding clinical and genetic spectrum of ANO3 dystonia
    Li-Ting Jiang, Li-Xi Li, Ying Liu, Xiao-Long Zhang, You-Gui Pan, Lin Wang, Xin-Hua Wan, Ling-Jing Jin
    Neuroscience Letters.2021; 746: 135590.     CrossRef
  • Huntington disease-like phenotype in a patient with ANO3 mutation
    Shahedah Koya Kutty, Eoin Mulroy, Francesca Magrinelli, Giulia Di Lazzaro, Anna Latorre, Kailash P. Bhatia
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Benefits of Levodopa-Carbidopa Intestinal Gel Infusion in Patients with Parkinson’s Disease Experiencing Gait Dysfunction Following Subthalamic Deep Brain Stimulation
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J Mov Disord. 2019;12(3):192-194.   Published online September 30, 2019
DOI: https://doi.org/10.14802/jmd.19022
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Hemichorea-Hemiballism after External Ventricular Drainage
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DOI: https://doi.org/10.14802/jmd.19033
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  • Efficacy and safety profile of neuroendoscopic hematoma evacuation combined with intraventricular lavage in severe intraventricular hemorrhage patients
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JMD : Journal of Movement Disorders