7,413 results match your criteria Multiple System Atrophy


Recent Advances in the Neural Control of Movements: Lessons for Functional Recovery.

Phys Ther Res 2022 29;25(1):1-11. Epub 2021 Sep 29.

Department of Human Development, Graduate School of Human Development and Environment, Kobe University, Japan.

We review the current views on the control and coordination of movements following the traditions set by Nikolai Bernstein. In particular, we focus on the theory of neural control of effectors - from motor units to individual muscles, to joints, limbs, and to the whole body - with spatial referent coordinates organized into a hierarchy with multiple few-to-many mappings. Further, we discuss synergies ensuring stability of natural human movements within the uncontrolled manifold hypothesis. Read More

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September 2021

Symptomatic Care in Multiple System Atrophy: State of the Art.

Cerebellum 2022 May 17. Epub 2022 May 17.

Department of Neurology, Medical University of Innsbruck, Anichstrasse 35, A-6020, Innsbruck, Austria.

Without any disease-modifying treatment strategy for multiple system atrophy (MSA), the therapeutic management of MSA patients focuses on a multidisciplinary strategy of symptom control. In the present review, we will focus on state of the art treatment in MSA and additionally give a short overview about ongoing randomized controlled trials in this field. Read More

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Kidney Biopsy Features Most Predictive of Clinical Outcomes in the Spectrum of Minimal Change Disease and Focal Segmental Glomerulosclerosis.

J Am Soc Nephrol 2022 May 17. Epub 2022 May 17.

Department of Pathology, Division of AI and Computational Pathology, and Department of Medicine, Division of Nephrology, Duke University, Durham, North Carolina

Background: Heterogeneity in disease course and treatment response among patients with MCD/FSGS necessitates a granular evaluation of kidney tissue features. This study aimed to identify histologic and ultrastructural descriptors of structural changes most predictive of clinical outcomes in the Nephrotic Syndrome Study Network (NEPTUNE).

Methods: Forty-eight histologic (37 glomerular, 9 tubulointerstitial, 2 vascular) and 20 ultrastructural descriptors were quantified by applying the NEPTUNE Digital Pathology Scoring System to NEPTUNE kidney biopsies. Read More

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Can We Treat Neurodegenerative Proteinopathies by Enhancing Protein Degradation?

Mov Disord 2022 May 17. Epub 2022 May 17.

Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy.

Neurodegenerative proteinopathies are defined as a class of neurodegenerative disorders, with either genetic or sporadic age-related onset, characterized by the pathological accumulation of aggregated protein deposits. These mainly include Alzheimer's disease (AD), Parkinson's disease (PD), amyotrophic lateral sclerosis (ALS), Huntington's disease (HD) as well as frontotemporal lobar degeneration (FTLD). The deposition of abnormal protein aggregates in the brain of patients affected by these disorders is thought to play a causative role in neuronal loss and disease progression. Read More

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Female Urinary Retention Progressing to Possible Multiple System Atrophy-cerebellar Form after 12 Years.

Intern Med 2022 May 14. Epub 2022 May 14.

Department of Uro-Neurology, The National Hospital for Neurology and Neurosurgery, UK.

We herein report a 73-year-old Japanese woman with possible multiple system atrophy-cerebellar form (MSA-C) who suffered from urinary retention (sacral autonomic disorder) for 12 years before exhibiting cerebellar ataxia. A peculiar combination of findings on urodynamics and sphincter electromyography (EMG), e.g. Read More

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miR-222 Is Involved in the Amelioration Effect of Genistein on Dexamethasone-Induced Skeletal Muscle Atrophy.

Nutrients 2022 Apr 29;14(9). Epub 2022 Apr 29.

Department of Animal Science, College of Animal Science and Technology, Sichuan Agricultural University, Chengdu 611130, China.

Skeletal muscle atrophy is a complex degenerative disease characterized by decreased skeletal muscle mass, skeletal muscle strength, and function. MicroRNAs (miRNAs) are a potential therapeutic target, and natural products that regulate miRNA expression may be a safe and effective treatment strategy for muscle atrophy. Previous studies have shown beneficial effects of genistein treatment on muscle mass and muscle atrophy, but the mechanism is not fully understood. Read More

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Phosphorylated α-synuclein in skin Schwann cells: a new biomarker for multiple system atrophy.

Brain 2022 May 12. Epub 2022 May 12.

IRCCS Istituto delle Scienze Neurologiche di Bologna, UOC Clinica Neurologica, Italy.

Multiple system atrophy (MSA) is characterized by accumulation of phosphorylated α-synuclein (p-syn) as glial cytoplasmic inclusions in the brain and a specific biomarker for this disorder is urgently needed. We aimed at investigating if p-syn can also be detected in skin Remak non-myelinating Schwann cells (RSCs) as Schwann cell cytoplasmic inclusions (SCCi) and may represent a reliable clinical biomarker for MSA. This cross-sectional diagnostic study evaluated skin p-syn in 96 patients: 46 with probable MSA (29 MSA-P and 17 MSA-C), 34 with Parkinson's disease (PD) and 16 with dementia with Lewy bodies (DLB). Read More

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Abnormal metabolic covariance patterns associated with multiple system atrophy and progressive supranuclear palsy.

Phys Med 2022 May 7;98:131-138. Epub 2022 May 7.

Department of Nuclear Medicine, University Medical Centre Ljubljana, Zaloška cesta 7, 1000 Ljubljana, Slovenia; Department of Neurology, University Medical Centre Ljubljana, Zaloška cesta 2, 1000 Ljubljana, Slovenia; Faculty of Medicine, University of Ljubljana, Vrazov trg 2, 1000 Ljubljana, Slovenia.

Purpose: Differentiation between neurodegenerative parkinsonisms, whose early clinical presentation is similar, may be improved with metabolic brain imaging. In this study we applied a specific network analysis to 2-[F]FDG PET brain scans to identify the characteristic metabolic patterns for multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) in a new European cohort. We also developed a new tool to recognize and estimate patients' metabolic brain heterogeneity. Read More

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Spinal Cord Stimulation Improved Freezing of Gait and Hypokinetic Dysarthria of a Patient with Dopamine-Resistant Multiple System Atrophy-Parkinsonian Type.

Neurol India 2022 Mar-Apr;70(2):757-759

Trauma Center, First Affiliated Hospital of Kunming Medical University, Kunming, China.

Background: Multiple system atrophy parkinsonian type (MSA-P) patients with resistance to dopamine have highly limited treatment options. This calls for further study of spinal cord stimulation (SCS) as a potential nondopaminergic therapy to improve motor and speech functions of patients with dopamine-resistant parkinsonism.

Case Presentation: A 58-year-old male with MSA-P had hypokinetic dysarthria, freezing of gait (FOG), and spinal disc herniation with refractory back pain. Read More

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Pharyngolaryngeal semiology and prognostic factors in multiple system atrophy.

Eur Arch Otorhinolaryngol 2022 May 5. Epub 2022 May 5.

Department of ENT in Toulouse, Hospital of Larrey, Toulouse, France.

Introduction: Multiple system atrophy (MSA) is a rare degenerative neurological disorder in adults. It induces parkinsonian and/or cerebellar syndrome associated with dysautonomia. Pharyngolaryngeal symptoms are common. Read More

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Marked response to levodopa in a patient with multiple system atrophy presenting with orthostatic hypotension: should reduced DAT uptake on DaTSCAN be a criterion for response to levodopa?

Neurol Sci 2022 May 5. Epub 2022 May 5.

Neurology Clinic, Diskapi Yildirim Beyazit Training and Research Hospital, Şehit Ömer Halisdemir Street, No: 20 Altındag, 06110, Ankara, Turkey.

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Eye tracking identifies biomarkers in α-synucleinopathies versus progressive supranuclear palsy.

J Neurol 2022 Apr 30. Epub 2022 Apr 30.

Centre for Neuroscience Studies, Queen's University, 18 Stuart Street, Kingston, ON, K7L 3N6, Canada.

Objectives: This study (1) describes and compares saccade and pupil abnormalities in patients with manifest alpha-synucleinopathies (αSYN: Parkinson's disease (PD), Multiple System Atrophy (MSA)) and a tauopathy (progressive supranuclear palsy (PSP)); (2) determines whether patients with rapid-eye-movement sleep behaviour disorder (RBD), a prodromal stage of αSYN, already have abnormal responses that may indicate a risk for developing PD or MSA.

Methods: Ninety (46 RBD, 27 PD, 17 MSA) patients with an αSYN, 10 PSP patients, and 132 healthy age-matched controls (CTRL) were examined with a 10-min video-based eye-tracking task (Free Viewing). Participants were free to look anywhere on the screen while saccade and pupil behaviours were measured. Read More

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Safety, tolerability and pharmacokinetics of the oligomer modulator anle138b with exposure levels sufficient for therapeutic efficacy in a murine Parkinson model: A randomised, double-blind, placebo-controlled phase 1a trial.

EBioMedicine 2022 Apr 29;80:104021. Epub 2022 Apr 29.

MODAG GmbH, Wendelsheim, Germany; Center for Neuropathology and Prion Research, Ludwig-Maximilians-University Munich, Germany. Electronic address:

Background: Synucleinopathies such as Parkinson ́s disease (PD), Dementia with Lewy bodies (DLB) and Multiple System Atrophy (MSA) are characterized by deposition of misfolded and aggregated α-synuclein. Small aggregates (oligomers) of α-synuclein have been shown to be the most relevant neurotoxic species and are targeted by anle138b, an orally bioavailable small molecule compound which shows strong disease-modifying effects in animal models of synucleinopathies.

Methods: Anle138b was studied in a single-centre, double-blind, randomised, placebo-controlled single ascending dose (SAD) and multiple ascending dose (MAD) study in healthy subjects. Read More

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[Medically unexplained or not? Be aware of a somatic cause of Medically Unexplained Symptoms].

Ned Tijdschr Geneeskd 2022 02 24;166. Epub 2022 Feb 24.

GGZ Breburg, Tilburg.

In 2 cases of men aged 26 and 55 respectively we illustrate the complexity of MUS patients since in both cases a somatic diagnosis was missed. The first patient presented with an inability to walk and persistent pain. Because of his symptoms he lost his job, had depressive moods and used alcohol and cannabis. Read More

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February 2022

Disease-Modifying Therapies for Multiple System Atrophy: Where Are We in 2022?

J Parkinsons Dis 2022 Feb 27. Epub 2022 Feb 27.

Department of Neurology, Medical University ofInnsbruck, Innsbruck, Austria.

Multiple system atrophy is a rapidly progressive and fatal neurodegenerative disorder. While numerous preclinical studies suggested efficacy of potentially disease modifying agents, none of those were proven to be effective in large-scale clinical trials. Three major strategies are currently pursued in preclinical and clinical studies attempting to slow down disease progression. Read More

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February 2022

Multiple system atrophy: Inspiratory sighs as a key polysomnographic sign to early diagnosis?

Pulmonology 2022 Apr 28. Epub 2022 Apr 28.

Sleep Medicine Center, Centro Hospitalar e Universitário de Coimbra, R. do Museu 2, Coimbra 3045-081, Portugal; Neurology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra 3004-561, Portugal.

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Effect of the odour compound from Periploca sepium Bunge on the physiological and biochemical indices, photosynthesis and ultrastructure of the leaves of Humulus scandens (Lour.) Merr.

Ecotoxicol Environ Saf 2022 Jun 27;238:113556. Epub 2022 Apr 27.

State Key Laboratory of Functions and Applications of Medicinal Plants, Guizhou Medical University, Guiyang 550014, China; The Key Laboratory of Chemistry for Natural Products of Guizhou Province and Chinese Academy of Sciences/Guizhou Provincial Engineering Research Center for Natural Drugs, Guiyang 550014, China.

Natural odour compounds could be a potential alternative to synthetic herbicides. The odour compound of Periploca sepium Bunge, named 2-hydroxy-4-methoxy-benzaldehyde (HMB), is a herbicidal compound. However, its herbicidal mechanism is unclear. Read More

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Advanced diffusion imaging to track progression in Parkinson's disease, multiple system atrophy, and progressive supranuclear palsy.

Neuroimage Clin 2022 26;34:103022. Epub 2022 Apr 26.

Laboratory for Rehabilitation Neuroscience, Department of Applied Physiology and Kinesiology, University of Florida, Gainesville, FL, USA; J. Crayton Pruitt Family Department of Biomedical Engineering, University of Florida, Gainesville, FL, USA; Department of Neurology and the Norman Fixel Institute for Neurological Diseases, College of Medicine, University of Florida, Gainesville, FL, USA. Electronic address:

Advanced diffusion imaging which accounts for complex tissue properties, such as crossing fibers and extracellular fluid, may detect longitudinal changes in widespread pathology in atypical Parkinsonian syndromes. We implemented fixel-based analysis, Neurite Orientation and Density Imaging (NODDI), and free-water imaging in Parkinson's disease (PD), multiple system atrophy (MSAp), progressive supranuclear palsy (PSP), and controls longitudinally over one year. Further, we used these three advanced diffusion imaging techniques to investigate longitudinal progression-related effects in key white matter tracts and gray matter regions in PD and two common atypical Parkinsonian disorders. Read More

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Unique seeding profiles and prion-like propagation of synucleinopathies are highly dependent on the host in human α-synuclein transgenic mice.

Acta Neuropathol 2022 Jun 30;143(6):663-685. Epub 2022 Apr 30.

Department of Neuroscience, College of Medicine, University of Florida, Gainesville, FL, 32610, USA.

α-synuclein (αSyn) is an intrinsically disordered protein which can undergo structural transformations, resulting in the formation of stable, insoluble fibrils. αSyn amyloid-type nucleation can be induced by misfolded 'seeds' serving as a conformational template, tantamount to the prion-like mechanism. Accumulation of αSyn inclusions is a key feature of dementia with Lewy bodies (DLB) and multiple system atrophy (MSA), and are found as additional pathology in Alzheimer's disease (AD) such as AD with amygdala predominant Lewy bodies (AD/ALB). Read More

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Tracheostomy invasive ventilation for stridor in multiple system atrophy.

Parkinsonism Relat Disord 2022 Apr 25;97:105-106. Epub 2022 Apr 25.

CHU Bordeaux, Service de Neurologie - Maladies Neurodégénératives, CRMR AMS, IMNc, F-33000, Bordeaux, France; Univ. Bordeaux, CNRS, IMN, UMR 5293, F-33000, Bordeaux, France; Dept. Medicine, University of Otago, Christchurch, New Zealand; New Zealand Brain Research Institute, Christchurch, New Zealand. Electronic address:

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Tau protein quantification in skin biopsies differentiates tauopathies from alpha-synucleinopathies.

Brain 2022 Apr 29. Epub 2022 Apr 29.

Laboratory for Biomedical Neurosciences, Neurocenter of Southern Switzerland, Ente Ospedaliero Cantonale, Lugano, Switzerland.

Abnormal accumulation of microtubule-associated protein tau (τ) is a characteristic feature of atypical parkinsonisms with tauopathies such as Progressive Supranuclear Palsy (PSP) and Corticobasal Degeneration (CBD). However, pathological τ has also been observed in α-synucleinopathies like Parkinson's Disease (PD) and Multiple System Atrophy (MSA). Based on the involvement of peripheral nervous system in several neurodegenerative diseases, we characterized and compared τ expression in skin biopsies of patients clinically diagnosed with PD, MSA, PSP, CBD, and in healthy control subjects. Read More

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Clinical advances of RNA therapeutics for treatment of neurological and neuromuscular diseases.

RNA Biol 2022 31;19(1):594-608. Epub 2021 Dec 31.

Center for RNA Medicine, Department of Clinical Medicine, Aalborg University, A.C. Meyers Vænge 15, 2450 Copenhagen, Denmark.

RNA therapeutics comprise a diverse group of oligonucleotide-based drugs such as antisense oligonucleotides (ASOs), small interfering RNAs (siRNAs), and short hairpin RNAs (shRNAs) that can be designed to selectively interact with drug targets currently undruggable with small molecule-based drugs or monoclonal antibodies. Furthermore, RNA-based therapeutics have the potential to modulate entire disease pathways, and thereby represent a new modality with unprecedented potential for generating disease-modifying drugs for a wide variety of human diseases, including central nervous system (CNS) disorders. Here, we describe different strategies for delivering RNA drugs to the CNS and review recent advances in clinical development of ASO drugs and siRNA-based therapeutics for the treatment of neurological diseases and neuromuscular disorders. Read More

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Multimodal Biomarkers of Repetitive Head Impacts and Traumatic Encephalopathy Syndrome: A Clinico-Pathological Case Series.

J Neurotrauma 2022 Apr 28. Epub 2022 Apr 28.

University of California San Francisco, Neurology, San Francisco, California, United States;

Traumatic encephalopathy syndrome (TES) criteria were developed to aid diagnosis of chronic traumatic encephalopathy (CTE) pathology during life. Interpreting clinical and biomarker findings in patients with TES during life requires autopsy-based determination of the neuropathological profile. We report a clinico-pathological series of 9 patients with prior repetitive head impacts (RHI) classified retrospectively using the TES research framework (100% male and white/Caucasian, age at death 49-84) who completed antemortem neuropsychological evaluations, T1-weighted MRI, diffusion tensor imaging (N=6), (18)F-fluorodeoxyglucose-PET (N=5), and plasma measurement of neurofilament light (NfL), glial fibrillary acidic protein (GFAP), and total tau (N=8). Read More

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Neuropathology of Multiple System Atrophy, a Glioneuronal Degenerative Disease.

Cerebellum 2022 Apr 26. Epub 2022 Apr 26.

Department of Neuropathology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, 5 Zaifu-cho, Hirosaki, 036-8562, Japan.

Multiple system atrophy (MSA) is a fatal disease characterized pathologically by the widespread occurrence of aggregated α-synuclein in the oligodendrocytes referred to as glial cytoplasmic inclusions (GCIs). α-Synuclein aggregates are also found in the oligodendroglial nuclei and neuronal cytoplasm and nuclei. It is uncertain whether the primary source of α-synuclein in GCIs is originated from neurons or oligodendrocytes. Read More

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Freezing of Gait in Multiple System Atrophy.

Front Aging Neurosci 2022 8;14:833287. Epub 2022 Apr 8.

Department of Radiology, Renmin Hospital of Wuhan University, Wuhan, China.

Background And Purpose: Freezing of gait (FOG) is a common gait disturbance phenomenon in multiple system atrophy (MSA) patients. The current investigation assessed the incidence FOG in a cross-sectional clinical study, and clinical correlations associated with it.

Methods: Ninety-nine MSA patients from three hospitals in China were consecutively enrolled in the study. Read More

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MicroRNA as Candidate Biomarkers in Atypical Parkinsonian Syndromes: Systematic Literature Review.

Authors:
Anastasia Bougea

Medicina (Kaunas) 2022 Mar 26;58(4). Epub 2022 Mar 26.

1st Department of Neurology, Eginition Hospital, Medical School, National and Kapodistrian University of Athens, 72-74 Vassilisis Sofia's Avenue, 11528 Athens, Greece.

Multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) are rare atypical parkinsonian syndromes, characterized by motor and cognitive symptoms. Their clinical diagnosis is challenging because there are no established biomarkers. Dysregulation of microRNAs (miRNAs/miRs) has been reported to serve an important role in neurodegenerative diseases. Read More

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RT-QuIC and Related Assays for Detecting and Quantifying Prion-like Pathological Seeds of α-Synuclein.

Biomolecules 2022 04 14;12(4). Epub 2022 Apr 14.

Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Hamilton, MT 59840, USA.

Various disease-associated forms or strains of α-synuclein (αSyn) can spread and accumulate in a prion-like fashion during synucleinopathies such as Parkinson's disease (PD), Lewy body dementia (DLB), and multiple system atrophy (MSA). This capacity for self-propagation has enabled the development of seed amplification assays (SAAs) that can detect αSyn in clinical samples. Notably, α-synuclein real-time quaking-induced conversion (RT-QuIC) and protein misfolding cyclic amplification (PMCA) assays have evolved as ultrasensitive, specific, and relatively practical methods for detecting αSyn in a variety of biospecimens including brain tissue, CSF, skin, and olfactory mucosa from synucleinopathy patients. Read More

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The Movement Disorder Society Criteria for the Diagnosis of Multiple System Atrophy.

Mov Disord 2022 Apr 21. Epub 2022 Apr 21.

Department of Neurology, Dysautonomia Center, Langone Medical Center, New York University School of Medicine, New York, New York, USA.

Background: The second consensus criteria for the diagnosis of multiple system atrophy (MSA) are widely recognized as the reference standard for clinical research, but lack sensitivity to diagnose the disease at early stages.

Objective: To develop novel Movement Disorder Society (MDS) criteria for MSA diagnosis using an evidence-based and consensus-based methodology.

Methods: We identified shortcomings of the second consensus criteria for MSA diagnosis and conducted a systematic literature review to answer predefined questions on clinical presentation and diagnostic tools relevant for MSA diagnosis. Read More

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Altered voxel-level whole-brain functional connectivity in multiple system atrophy patients with depression symptoms.

BMC Psychiatry 2022 Apr 20;22(1):279. Epub 2022 Apr 20.

Department of Radiology, Renmin Hospital of Wuhan University, Wuhan, 430060, China.

Background: It is yet unknown if the whole-brain resting-state network is altered in multiple system atrophy with symptoms of depression. This study aimed to investigate if and how depression symptoms in multiple system atrophy are associated with resting-state network dysfunction.

Methods: We assessed the resting-state functional network matric using Degree centrality (DC) coupling with a second ROI-wise functional connectivity (FC) algorithm in a multimodal imaging case-control study that enrolled 32 multiple system atrophy patients with depression symptoms (MSA-D), 30 multiple system atrophy patients without depression symptoms (MSA-ND), and 34 healthy controls (HC). Read More

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