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Most-download articles are from the articles published in 2020 during the last three month.

Review Articles
Treatable Ataxias: How to Find the Needle in the Haystack?
Albert Stezin, Pramod Kumar Pal
J Mov Disord. 2022;15(3):206-226.   Published online September 7, 2022
DOI: https://doi.org/10.14802/jmd.22069
  • 1,626 View
  • 249 Download
AbstractAbstract PDF
Treatable ataxias are a group of ataxic disorders with specific treatments. These disorders include genetic and metabolic disorders, immune-mediated ataxic disorders, and ataxic disorders associated with infectious and parainfectious etiology, vascular causes, toxins and chemicals, and endocrinopathies. This review provides a comprehensive overview of different treatable ataxias. The major metabolic and genetic treatable ataxic disorders include ataxia with vitamin E deficiency, abetalipoproteinemia, cerebrotendinous xanthomatosis, Niemann-Pick disease type C, autosomal recessive cerebellar ataxia due to coenzyme Q10 deficiency, glucose transporter type 1 deficiency, and episodic ataxia type 2. The treatment of these disorders includes the replacement of deficient cofactors and vitamins, dietary modifications, and other specific treatments. Treatable ataxias with immune-mediated etiologies include gluten ataxia, anti-glutamic acid decarboxylase antibody-associated ataxia, steroid-responsive encephalopathy associated with autoimmune thyroiditis, Miller-Fisher syndrome, multiple sclerosis, and paraneoplastic cerebellar degeneration. Although dietary modification with a gluten-free diet is adequate in gluten ataxia, other autoimmune ataxias are managed by short-course steroids, plasma exchange, or immunomodulation. For autoimmune ataxias secondary to malignancy, treatment of tumor can reduce ataxic symptoms. Chronic alcohol consumption, antiepileptics, anticancer drugs, exposure to insecticides, heavy metals, and recreational drugs are potentially avoidable and treatable causes of ataxia. Infective and parainfectious causes of cerebellar ataxias include acute cerebellitis, postinfectious ataxia, Whipple’s disease, meningoencephalitis, and progressive multifocal leukoencephalopathy. These disorders are treated with steroids and antibiotics. Recognizing treatable disorders is of paramount importance when dealing with ataxias given that early treatment can prevent permanent neurological sequelae.
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
  • 1,356 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.
Diagnosis and Clinical Features in Autoimmune-Mediated Movement Disorders
Pei-Chen Hsieh, Yih-Ru Wu
J Mov Disord. 2022;15(2):95-105.   Published online May 26, 2022
DOI: https://doi.org/10.14802/jmd.21077
  • 2,099 View
  • 370 Download
AbstractAbstract PDFSupplementary Material
Movement disorders are common manifestations in autoimmune-mediated encephalitis. This group of diseases is suspected to be triggered by infection or neoplasm. Certain phenotypes correlate with specific autoantibody-related neurological disorders, such as orofacial-lingual dyskinesia with N-methyl-D-aspartate receptor encephalitis and faciobrachial dystonic seizures with leucine-rich glioma-inactivated protein 1 encephalitis. Early diagnosis and treatment, especially for autoantibodies targeting neuronal surface antigens, can improve prognosis. In contrast, the presence of autoantibodies against intracellular neuronal agents warrants screening for underlying malignancy. However, early clinical diagnosis is challenging because these diseases can be misdiagnosed. In this article, we review the distinctive clinical phenotypes, magnetic resonance imaging findings, and current treatment options for autoimmune-mediated encephalitis.
Letter to the editor
Mosapride-Induced Movement Disorders
Sang-Wook Hong, Hae-Won Shin
J Mov Disord. 2022;15(3):273-276.   Published online May 10, 2022
DOI: https://doi.org/10.14802/jmd.21149
  • 1,239 View
  • 170 Download
PDF
Original Article
Potential Link Between Cognition and Motor Reserve in Patients With Parkinson’s Disease
Seok Jong Chung, Yae Ji Kim, Yun Joong Kim, Hye Sun Lee, Mijin Yun, Phil Hyu Lee, Yong Jeong, Young H. Sohn
J Mov Disord. 2022;15(3):249-257.   Published online September 7, 2022
DOI: https://doi.org/10.14802/jmd.22063
  • 515 View
  • 59 Download
AbstractAbstract PDFSupplementary Material
Objective
To investigate whether there is a link between cognitive function and motor reserve (i.e., individual capacity to cope with nigrostriatal dopamine depletion) in patients with newly diagnosed Parkinson’s disease (PD).
Methods
A total of 163 patients with drug-naïve PD who underwent 18F-FP-CIT PET, brain MRI, and a detailed neuropsychological test were enrolled. We estimated individual motor reserve based on initial motor deficits and striatal dopamine depletion using a residual model. We performed correlation analyses between motor reserve estimates and cognitive composite scores. Diffusion connectometry analysis was performed to map the white matter fiber tracts, of which fractional anisotropy (FA) values were well correlated with motor reserve estimates. Additionally, Cox regression analysis was used to assess the effect of initial motor reserve on the risk of dementia conversion.
Results
The motor reserve estimate was positively correlated with the composite score of the verbal memory function domain (γ = 0.246) and with the years of education (γ = 0.251). Connectometry analysis showed that FA values in the left fornix were positively correlated with the motor reserve estimate, while no fiber tracts were negatively correlated with the motor reserve estimate. Cox regression analysis demonstrated that higher motor reserve estimates tended to be associated with a lower risk of dementia conversion (hazard ratio, 0.781; 95% confidence interval, 0.576–1.058).
Conclusion
The present study demonstrated that the motor reserve estimate was well correlated with verbal memory function and with white matter integrity in the left fornix, suggesting a possible link between cognition and motor reserve in patients with PD.
Review Article
Diagnostic Criteria for Dementia with Lewy Bodies: Updates and Future Directions
Masahito Yamada, Junji Komatsu, Keiko Nakamura, Kenji Sakai, Miharu Samuraki-Yokohama, Kenichi Nakajima, Mitsuhiro Yoshita
J Mov Disord. 2020;13(1):1-10.   Published online November 8, 2019
DOI: https://doi.org/10.14802/jmd.19052
  • 20,997 View
  • 1,495 Download
  • 15 Citations
AbstractAbstract PDF
The aim of this article is to describe the 2017 revised consensus criteria for the clinical diagnosis of dementia with Lewy bodies (DLB) with future directions for the diagnostic criteria. The criteria for the clinical diagnosis of probable and possible DLB were first published as the first consensus report in 1996 and were revised in the third consensus report in 2005. After discussion at the International DLB Conference in Fort Lauderdale, Florida, USA, in 2015, the International DLB Consortium published the fourth consensus report including the revised consensus criteria in 2017. The 2017 revised criteria clearly distinguish between clinical features and diagnostic biomarkers. Significant new information about previously reported aspects of DLB has been incorporated, with increased diagnostic weighting given to rapid eye movement (REM) sleep behavior disorder (RBD) and iodine-123-metaiodobenzylguanidine (MIBG) myocardial scintigraphy. Future directions include the development of the criteria for early diagnosis (prodromal DLB) and the establishment of new biomarkers that directly indicate Lewy-related pathology, including α-synuclein imaging, biopsies of peripheral tissues (skin, etc.) for the demonstration of α-synuclein deposition, and biochemical markers (cerebrospinal fluid/blood), as well as the pathological evaluation of the sensitivity and specificity of the 2017 revised diagnostic criteria. In conclusion, the revised consensus criteria for the clinical diagnosis of DLB were reported with the incorporation of new information about DLB in 2017. Future directions include the development of the criteria for early diagnosis and the establishment of biomarkers directly indicative of Lewy-related pathology.

Citations

Citations to this article as recorded by  
  • Autonomic symptoms are predictive of dementia with Lewy bodies
    Wenzheng Hu, Shuai Liu, Fei Wang, Han Zhu, Xiaoshan Du, Lingyun Ma, Jinghuan Gan, Hao Wu, Xiaodan Wang, Yong Ji
    Parkinsonism & Related Disorders.2022; 95: 1.     CrossRef
  • Delusion and Delirium in Neurodegenerative Disorders: An Overlooked Relationship?
    Daniele Urso, Valentina Gnoni, Marco Filardi, Giancarlo Logroscino
    Frontiers in Psychiatry.2022;[Epub]     CrossRef
  • Clinical Trajectories at the End of Life in Autopsy-Confirmed Dementia Patients With Alzheimer Disease and Lewy Bodies Pathologies
    Yian Gu, Anton Kociolek, Kayri K. Fernandez, Stephanie A. Cosentino, Carolyn Wei Zhu, Zhezhen Jin, James B. Leverenz, Yaakov B. Stern
    Neurology.2022; 98(21): e2140.     CrossRef
  • Diagnostic Performance for Differential Diagnosis of Atypical Parkinsonian Syndromes from Parkinson’s Disease Using Quantitative Indices of 18F-FP-CIT PET/CT
    Miju Cheon, Seung Min Kim, Sang-Won Ha, Min Ju Kang, Hea-Eun Yang, Jang Yoo
    Diagnostics.2022; 12(6): 1402.     CrossRef
  • The promise of amplification assays for accurate early detection of α-synucleinopathies: A review
    Regina Kurapova, Leonidas Chouliaras, John T. O'Brien
    Experimental Gerontology.2022; 165: 111842.     CrossRef
  • Progressive Olfactory Impairment and Cardiac Sympathetic Denervation in REM Sleep Behavior Disorder
    Annette Janzen, David Vadasz, Jan Booij, Markus Luster, Damiano Librizzi, Martin T. Henrich, Lars Timmermann, Mahboubeh Habibi, Elisabeth Sittig, Geert Mayer, Fanni Geibl, Wolfgang Oertel
    Journal of Parkinson's Disease.2022; 12(6): 1921.     CrossRef
  • A Systematic Review and Comparison of Neurocognitive Features of Late-Life Attention-Deficit/Hyperactivity Disorder and Dementia With Lewy Bodies
    Jennifer L. Prentice, Morgan J. Schaeffer, Alexandra K. Wall, Brandy L. Callahan
    Journal of Geriatric Psychiatry and Neurology.2021; 34(5): 466.     CrossRef
  • Dementia with Lewy bodies in first-generation immigrants in a European memory clinic
    Kurt Segers, Florence Benoit, Jean-Marie Meyts, Gérald Glibert, Sophie Levy, Murielle Surquin
    Acta Neurologica Belgica.2021; 121(1): 219.     CrossRef
  • The development of new method to differentiate between Dementia with Lewy bodies and Alzheimer’s disease by cerebral perfusion SPECT-comparison to CIScore
    Gaku Honda, Shigeki Nagamachi, Masanari Nonokuma, Koichi Takano, Yasuo Kuwabara, Kengo Yoshimitsu, Hitoshi Iida, Koji Ogomori, Hiroaki Kawasaki, Yoshio Tsuboi
    Japanese Journal of Radiology.2021; 39(2): 198.     CrossRef
  • Impaired meningeal lymphatic drainage in patients with idiopathic Parkinson’s disease
    Xue-Bing Ding, Xin-Xin Wang, Dan-Hao Xia, Han Liu, Hai-Yan Tian, Yu Fu, Yong-Kang Chen, Chi Qin, Jiu-Qi Wang, Zhi Xiang, Zhong-Xian Zhang, Qin-Chen Cao, Wei Wang, Jia-Yi Li, Erxi Wu, Bei-Sha Tang, Ming-Ming Ma, Jun-Fang Teng, Xue-Jing Wang
    Nature Medicine.2021; 27(3): 411.     CrossRef
  • Mechanisms of Neurodegeneration in Various Forms of Parkinsonism—Similarities and Differences
    Dariusz Koziorowski, Monika Figura, Łukasz M. Milanowski, Stanisław Szlufik, Piotr Alster, Natalia Madetko, Andrzej Friedman
    Cells.2021; 10(3): 656.     CrossRef
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    Raymond Wong, Yishan Luo, Vincent Chung-tong Mok, Lin Shi
    Brain Science Advances.2021; 7(1): 26.     CrossRef
  • Convolutional neural network-based automatic heart segmentation and quantitation in 123I-metaiodobenzylguanidine SPECT imaging
    Shintaro Saito, Kenichi Nakajima, Lars Edenbrandt, Olof Enqvist, Johannes Ulén, Seigo Kinuya
    EJNMMI Research.2021;[Epub]     CrossRef
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    Kurt Segers, Florence Benoit, Jean‐Marie Meyts, Gerald Glibert, Murielle Surquin
    Psychogeriatrics.2020; 20(5): 785.     CrossRef
  • Calibrated scintigraphic imaging procedures improve quantitative assessment of the cardiac sympathetic nerve activity
    Koichi Okuda, Kenichi Nakajima, Chiemi Kitamura, Yumiko Kirihara, Mitsumasa Hashimoto, Seigo Kinuya
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Letter to the editor
Multiple Sclerosis-Related Paroxysmal Kinesigenic Dyskinesia: Long Term, Favorable Response to Lacosamide
Vasiliki Poulidou, Martha Spilioti, Maria Moschou, Nickolas Papanikolaou, Antonios Drevelegas, Sotirios Papagiannopoulos, Dimitrios Kazis, Vasilios K. Kimiskidis
J Mov Disord. 2022;15(3):286-289.   Published online July 26, 2022
DOI: https://doi.org/10.14802/jmd.22016
  • 503 View
  • 122 Download
PDFSupplementary Material
Original Article
Long-Term Outcomes of Deep Brain Stimulation in Pantothenate Kinase-Associated Neurodegeneration-Related Dystonia
Kyung Ah Woo, Han-Joon Kim, Seung-Ho Jeon, Hye Ran Park, Kye Won Park, Seung Hyun Lee, Sun Ju Chung, Jong-Hee Chae, Sun Ha Paek, Beomseok Jeon
J Mov Disord. 2022;15(3):241-248.   Published online July 26, 2022
DOI: https://doi.org/10.14802/jmd.22002
  • 733 View
  • 62 Download
AbstractAbstract PDFSupplementary Material
Objective
To investigate the long-term clinical outcomes of pallidal deep brain stimulation (GPi-DBS) in patients with pantothenate kinase-associated neurodegeneration (PKAN).
Methods
We reviewed the records of patients with genetically confirmed PKAN who received bilateral GPi-DBS for refractory dystonia and were clinically followed up for at least 2 years postoperatively at two centers in Korea. Pre- and postoperative Burke– Fahn–Marsden Dystonia Rating Scale motor subscale (BFMDRS-M) scores, disability subscale (BFMDRS-D) scores, and qualitative clinical information were prospectively collected. Descriptive analysis was performed for BFMDRS-M scores, BFMDRSD scores, and the orofacial, axial, and limb subscores of the BFMDRS-M at 6–12, 24–36, and 60–72 months postoperatively.
Results
Five classic-type, four atypical-type, and one unknown-type PKAN cases were identified. The mean preoperative BFMDRS-M score was 92.1 for the classic type and 38.5 for the atypical or unknown type, with a mean BFMDRS follow-up of 50.7 months and a clinical follow-up of 69.0 months. The mean improvements in BFMDRS-M score were 11.3%, 41.3%, and 30.5% at 6–12, 24–36, and 60–72 months, respectively. In four patients with full regular evaluations until 60–72 months, improvements in the orofacial, axial, and limb subscores persisted, but the disability scores worsened from 24–36 months post-operation compared to the baseline, mainly owing to the aggravation of eating and feeding disabilities.
Conclusion
The benefits of GPi-DBS on dystonia may persist for more than 5 years in PKAN. The effects on patients’ subjective disability may have a shorter duration despite improvements in dystonia owing to the complex manifestations of PKAN.
Letter to the editor
Continuous 24-h Levodopa-Carbidopa Intestinal Gel Infusion After a Levodopa Holiday Suppressed Refractory Dyskinesia Despite Increasing Levodopa Dose
Noriko Nishikawa, Taku Hatano, Daiki Kamiyama, Haruna Haginiwa-Hasegawa, Genko Oyama, Nobutaka Hattori
J Mov Disord. 2022;15(3):290-292.   Published online July 26, 2022
DOI: https://doi.org/10.14802/jmd.22021
  • 415 View
  • 96 Download
PDFSupplementary Material
Review Article
Principles of Electrophysiological Assessments for Movement Disorders
Kai-Hsiang Stanley Chen, Robert Chen
J Mov Disord. 2020;13(1):27-38.   Published online January 31, 2020
DOI: https://doi.org/10.14802/jmd.19064
  • 10,355 View
  • 879 Download
  • 23 Citations
AbstractAbstract PDFSupplementary Material
Electrophysiological studies can provide objective and quantifiable assessments of movement disorders. They are useful in the diagnosis of hyperkinetic movement disorders, particularly tremors and myoclonus. The most commonly used measures are surface electromyography (sEMG), electroencephalography (EEG) and accelerometry. Frequency and coherence analyses of sEMG signals may reveal the nature of tremors and the source of the tremors. The effects of voluntary tapping, ballistic movements and weighting of the limbs can help to distinguish between organic and functional tremors. The presence of Bereitschafts-potentials and beta-band desynchronization recorded by EEG before movement onset provide strong evidence for functional movement disorders. EMG burst durations, distributions and muscle recruitment orders may identify and classify myoclonus to cortical, subcortical or spinal origins and help in the diagnosis of functional myoclonus. Organic and functional cervical dystonia can potentially be distinguished by EMG power spectral analysis. Several reflex circuits, such as the long latency reflex, blink reflex and startle reflex, can be elicited with different types of external stimuli and are useful in the assessment of myoclonus, excessive startle and stiff person syndrome. However, limitations of the tests should be recognized, and the results should be interpreted together with clinical observations.

Citations

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  • Functional Patients Referred for Deep Brain Stimulation: How Common Is it?
    Carolina Gorodetsky, Paula Azevedo, Alfonso Fasano
    Movement Disorders Clinical Practice.2022; 9(6): 841.     CrossRef
  • Myoclonus and other jerky movement disorders
    Sterre van der Veen, John N. Caviness, Yasmine E.M. Dreissen, Christos Ganos, Abubaker Ibrahim, Johannes H.T.M. Koelman, Ambra Stefani, Marina A.J. Tijssen
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    Sarah Smith, Ny-Ying Lam
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    Abhishek Lenka, Joseph Jankovic
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Letters to the editor
Catatonia in Hospitalized Patients With COVID-19: An Important Clinical Finding That Should Not be Missed
Tien Lee Ong, Sapiah Sapuan
J Mov Disord. 2022;15(3):277-280.   Published online May 10, 2022
DOI: https://doi.org/10.14802/jmd.21172
  • 1,304 View
  • 152 Download
PDFSupplementary Material
Blacksmith’s Dystonia Is Another Task-Specific Dystonia: From Past to Present
Min Seung Kim, Don Gueu Park, Jung Han Yoon
J Mov Disord. 2022;15(3):284-285.   Published online July 26, 2022
DOI: https://doi.org/10.14802/jmd.22037
  • 444 View
  • 93 Download
PDFSupplementary Material
Review Article
Update on Current Technologies for Deep Brain Stimulation in Parkinson’s Disease
Michelle Paff, Aaron Loh, Can Sarica, Andres M. Lozano, Alfonso Fasano
J Mov Disord. 2020;13(3):185-198.   Published online August 31, 2020
DOI: https://doi.org/10.14802/jmd.20052
  • 13,680 View
  • 592 Download
  • 34 Citations
AbstractAbstract PDF
Deep brain stimulation (DBS) is becoming increasingly central in the treatment of patients with Parkinson’s disease and other movement disorders. Recent developments in DBS lead and implantable pulse generator design provide increased flexibility for programming, potentially improving the therapeutic benefit of stimulation. Directional DBS leads may increase the therapeutic window of stimulation by providing a means of avoiding current spread to structures that might give rise to stimulation-related side effects. Similarly, control of current to individual contacts on a DBS lead allows for shaping of the electric field produced between multiple active contacts. The following review aims to describe the recent developments in DBS system technology and the features of each commercially available DBS system. The advantages of each system are reviewed, and general considerations for choosing the most appropriate system are discussed.

Citations

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Letter to the editor
Refractory Myoclonus as a Presentation of Metabolic Stroke in A Child With Cobalamin B Methylmalonic Acidemia After Liver and Kidney Transplant
Valerie Olson, Irene J Chang, J Lawrence Merritt nd, Dararat Mingbunjerdsuk
J Mov Disord. 2022;15(3):281-283.   Published online May 26, 2022
DOI: https://doi.org/10.14802/jmd.21196
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  • 134 Download
PDFSupplementary Material
Original Article
Increased Mortality in Young-Onset Parkinson’s Disease
Eldbjørg Hustad, Tor Åge Myklebust, Sasha Gulati, Jan O. Aasly
J Mov Disord. 2021;14(3):214-220.   Published online July 29, 2021
DOI: https://doi.org/10.14802/jmd.21029
  • 13,160 View
  • 208 Download
  • 4 Citations
AbstractAbstract PDF
Objective
Few studies have followed Parkinson’s disease (PD) patients from the time of diagnosis to the date of death. This study compared mortality in the Trondheim PD cohort to the general population, investigated causes of death and analyzed the associations between mortality and age at disease onset (AAO) and cognitive decline defined as Montreal Cognitive Assessment (MoCA) score below 26.
Methods
The cohort was followed longitudinally from 1997. By the end of January 2020, 587 patients had died. Comparisons to the Norwegian population were performed by calculating standardized mortality ratios (SMRs). Survival curves were estimated using the standard Kaplan-Meier estimator, and multivariable Cox proportional hazard models were estimated to investigate associations.
Results
SMR was 2.28 [95% confidence interval (CI): 2.13–2.44] for the whole cohort. For participants with AAO 20–39 years, the SMR was 5.55 (95% CI: 3.38–8.61). Median survival was 15 years (95% CI: 14.2–15.5) for the whole cohort. Early-onset PD (EOPD) patients (AAO < 50 years) had the longest median survival time. For all groups, there was a significant shortening in median survival time and an almost 3-fold higher age- and sex-adjusted hazard ratio for death when the MoCA score decreased below 26.
Conclusion
PD patients with an AAO before 40 years had a more than fivefold higher mortality rate compared to a similar general population. EOPD patients had the longest median survival; however, their life expectancy was reduced to a greater degree than that of late-onset PD patients. Cognitive impairment was strongly associated with mortality in PD.

Citations

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JMD : Journal of Movement Disorders