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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
  • 10,834 View
  • 722 Download
  • 31 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.
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
  • 33,993 View
  • 865 Download
  • 20 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.
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
  • 4,566 View
  • 174 Download
  • 1 Citations
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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
  • 6,488 View
  • 183 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.
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
  • 3,696 View
  • 98 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.
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
  • 4,879 View
  • 177 Download
  • 3 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.
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
  • 4,015 View
  • 97 Download
  • 1 Citations
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Hyperglycemia-Associated Hemichorea-Hemiballismus with Predominant Ipsilateral Putaminal Abnormality on Neuroimaging
Si Lei Fong, Ai Huey Tan, Kar Foo Lau, Norlisah Ramli, Shen-Yang Lim
J Mov Disord. 2019;12(3):187-189.   Published online August 9, 2019
DOI: https://doi.org/10.14802/jmd.19014
  • 5,106 View
  • 143 Download
  • 3 Citations
PDFSupplementary Material
Successful Pallidal Deep Brain Stimulation in a Patient with Childhood-Onset Generalized Dystonia with ANO3 Mutation
Dallah Yoo, Han-Joon Kim, Jong-Hee Chae, Sun Ha Paek, Beomseok Jeon
J Mov Disord. 2019;12(3):190-191.   Published online July 17, 2019
DOI: https://doi.org/10.14802/jmd.19016
  • 3,979 View
  • 105 Download
  • 5 Citations
PDFSupplementary Material
Benefits of Levodopa-Carbidopa Intestinal Gel Infusion in Patients with Parkinson’s Disease Experiencing Gait Dysfunction Following Subthalamic Deep Brain Stimulation
Thomas Edmund Kimber, YiZhong Zhuang, Philip Douglas Thompson
J Mov Disord. 2019;12(3):192-194.   Published online September 30, 2019
DOI: https://doi.org/10.14802/jmd.19022
  • 3,238 View
  • 64 Download
  • 1 Citations
PDFSupplementary Material
Hemichorea-Hemiballism after External Ventricular Drainage
Mirza Masoom Abbas, Ravi Gopal Varma, Nirmala Sankar, Raghavendra Pai
J Mov Disord. 2019;12(3):195-197.   Published online September 30, 2019
DOI: https://doi.org/10.14802/jmd.19033
  • 2,951 View
  • 55 Download
  • 1 Citations
PDFSupplementary Material

JMD : Journal of Movement Disorders