{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T21:43:41Z","timestamp":1791323021480,"version":"4.3.1"},"reference-count":43,"publisher":"Oxford University Press (OUP)","issue":"12","license":[{"start":{"date-parts":[[2022,7,29]],"date-time":"2022-07-29T00:00:00Z","timestamp":1659052800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100018889","name":"MSA Coalition","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100018889","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Medical Research Council"},{"name":"Wellcome Trust equipment and strategic awards"},{"name":"French Clinical Research Programme"},{"name":"PSP Association"},{"name":"Horizon 2020 research and innovation programme"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,12,19]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Disease-modifying treatments are currently being trialled in multiple system atrophy. Approaches based solely on clinical measures are challenged by heterogeneity of phenotype and pathogenic complexity. Neurofilament light chain protein has been explored as a reliable biomarker in several neurodegenerative disorders but data on multiple system atrophy have been limited. Therefore, neurofilament light chain is not yet routinely used as an outcome measure in multiple system atrophy. We aimed to comprehensively investigate the role and dynamics of neurofilament light chain in multiple system atrophy combined with cross-sectional and longitudinal clinical and imaging scales and for subject trial selection.<\/jats:p><jats:p>In this cohort study, we recruited cross-sectional and longitudinal cases in a multicentre European set-up. Plasma and CSF neurofilament light chain concentrations were measured at baseline from 212 multiple system atrophy cases, annually for a mean period of 2 years in 44 multiple system atrophy patients in conjunction with clinical, neuropsychological and MRI brain assessments. Baseline neurofilament light chain characteristics were compared between groups. Cox regression was used to assess survival; receiver operating characteristic analysis to assess the ability of neurofilament light chain to distinguish between multiple system atrophy patients and healthy controls. Multivariate linear mixed-effects models were used to analyse longitudinal neurofilament light chain changes and correlated with clinical and imaging parameters. Polynomial models were used to determine the differential trajectories of neurofilament light chain in multiple system atrophy. We estimated sample sizes for trials aiming to decrease neurofilament light chain levels.<\/jats:p><jats:p>We show that in multiple system atrophy, baseline plasma neurofilament light chain levels were better predictors of clinical progression, survival and degree of brain atrophy than the neurofilament light chain rate of change. Comparative analysis of multiple system atrophy progression over the course of disease, using plasma neurofilament light chain and clinical rating scales, indicated that neurofilament light chain levels rise as the motor symptoms progress, followed by deceleration in advanced stages. Sample size prediction suggested that significantly lower trial participant numbers would be needed to demonstrate treatment effects when incorporating plasma neurofilament light chain values into multiple system atrophy clinical trials in comparison to clinical measures alone.<\/jats:p><jats:p>In conclusion, neurofilament light chain correlates with clinical disease severity, progression and prognosis in multiple system atrophy. Combined with clinical and imaging analysis, neurofilament light chain can inform patient stratification and serve as a reliable biomarker of treatment response in future multiple system atrophy trials of putative disease-modifying agents.<\/jats:p>","DOI":"10.1093\/brain\/awac253","type":"journal-article","created":{"date-parts":[[2022,7,29]],"date-time":"2022-07-29T04:49:11Z","timestamp":1659070151000},"page":"4398-4408","source":"Crossref","is-referenced-by-count":70,"title":["Neurofilament light levels predict clinical progression and death in multiple system atrophy"],"prefix":"10.1093","volume":"145","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7797-0756","authenticated-orcid":false,"given":"Viorica","family":"Chelban","sequence":"first","affiliation":[{"name":"Department of Neuromuscular Diseases, Queen Square Institute of Neurology, University College London , London WC1N 3BG , UK"},{"name":"Neurobiology and Medical Genetics Laboratory, \u201cNicolae Testemitanu\u201d State University of Medicine and Pharmacy , MD 2004 Chisinau , Republic of Moldova"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elham","family":"Nikram","sequence":"additional","affiliation":[{"name":"Peninsula Technology Assessment Group (PenTAG), University of Exeter , Exeter EX 2LU , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra","family":"Perez-Soriano","sequence":"additional","affiliation":[{"name":"Movement Disorders Unit, Neurology Service, Hospital Cl\u00ednic de Barcelona , Barcelona 08036 , Spain"},{"name":"Parkinson's Disease and Movement Disorders Unit, Neurology Department, Institute of Biomedical Research August Pi i Sunyer (IDIBAPS) , Barcelona 08036 , Spain"},{"name":"Parkinson's Disease and Movement Disorders Unit, Neurology Department, Centro de Investigaci\u00f3n Biom\u00e9dica en Red sobre Enfermedades Neurodegenerativas , Madrid 28029 , Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7250-8597","authenticated-orcid":false,"given":"Carlo","family":"Wilke","sequence":"additional","affiliation":[{"name":"Division Translational Genomics of Neurodegenerative Diseases, Hertie-Institute for Clinical Brain Research and Center of Neurology, University of T\u00fcbingen , 72074 T\u00fcbingen , Germany"},{"name":"German Center for Neurodegenerative Diseases (DZNE) , 72074 T\u00fcbingen , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra","family":"Foubert-Samier","sequence":"additional","affiliation":[{"name":"CRMR AMS, Service de Neurologie \u2013 Maladies Neurod\u00e9g\u00e9n\u00e9ratives, CHU de Bordeaux , F-33000 Bordeaux , France"},{"name":"Universit\u00e9 de Bordeaux, CNRS, IMN, UMR 5293 , F-33000 Bordeaux , France"},{"name":"Universit\u00e9 de Bordeaux, INSERM, BPH, U1219 , F-33000 Bordeaux , France"},{"name":"Inserm, CIC 1401 Bordeaux, Clinical Epidemiology Unit , F-33000 Bordeaux , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9671-0212","authenticated-orcid":false,"given":"Nirosen","family":"Vijiaratnam","sequence":"additional","affiliation":[{"name":"Department Clinical and Movement Neuroscience, UCL Queen Square Institute of Neurology, University College London , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tong","family":"Guo","sequence":"additional","affiliation":[{"name":"Department Clinical and Movement Neuroscience, UCL Queen Square Institute of Neurology, University College London , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6844-882X","authenticated-orcid":false,"given":"Edwin","family":"Jabbari","sequence":"additional","affiliation":[{"name":"Department Clinical and Movement Neuroscience, UCL Queen Square Institute of Neurology, University College London , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simisola","family":"Olufodun","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Diseases, Queen Square Institute of Neurology, University College London , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariel","family":"Gonzalez","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Diseases, Queen Square Institute of Neurology, University College London , London WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Konstantin","family":"Senkevich","sequence":"additional","affiliation":[{"name":"Neurogenomics and Precision Medicine (NAP-Med) Laboratory, The Neuro (Montreal Neurological Institute-Hospital) , Montreal, QC H3A 2B4 , Canada"},{"name":"Department of Neurology & Neurosurgery, McGill University , Montreal, QC H3A 2B4 , Canada"},{"name":"Laboratory of Human Genetics, Petersburg Nuclear Physics Institute named by B.P. 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