Sabrina Katzdobler, Georg Nübling, Martin Klietz, Urban M. Fietzek, Carla Palleis, Alexander M. Bernhardt, Florian Wegner, Meret Huber, Sophia Rogozinski, Luisa-Sophie Schneider, Eike Jakob Spruth, Aline Beyle, Ina R. Vogt, Moritz Brandt, Niels Hansen, Wenzel Glanz, Kathrin Brockmann, Annika Spottke, Daniel C. Hoffmann, Oliver Peters, Josef Priller, Jens Wiltfang, Emrah Düzel, Anja Schneider, Björn Falkenburger, Thomas Klockgether, Thomas Gasser, Brigitte Nuscher, Christian Haass, Günter Höglinger, Johannes Levin
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MSA is characterized by an oligodendroglial α-synucleinopathy, progressive neuronal cell loss and concomitant astrocytosis. Here, we investigate glial fibrillary acidic protein (GFAP) and neurofilament light chain (NfL) as fluid biomarkers for differential diagnosis, assessment of clinical disease severity and prediction of disease progression in MSA.</p><h3 data-test=\"abstract-sub-heading\">Methods</h3><p>GFAP and NfL levels were analyzed in plasma and CSF samples of 47 MSA patients as well as 24 Parkinson’s disease (PD) and 25 healthy controls (HC) as reference cohorts. In MSA, biomarker levels were correlated to baseline and longitudinal clinical disease severity (UMSARS scores).</p><h3 data-test=\"abstract-sub-heading\">Results</h3><p>In MSA, GFAP levels in CSF and plasma predicted baseline clinical disease severity as indicated by UMSARS scores, while NfL levels predicted clinical disease progression as indicated by longitudinal changes in UMSARS scores. Cross-sectionally, NfL levels in CSF and plasma were significantly elevated in MSA compared to both PD and HC. Receiver operating curves (ROC) indicated high diagnostic accuracy of NfL for distinguishing MSA from PD (CSF: AUC = 0.97, 95% CI 0.90–1.00; plasma: AUC = 0.90, 95% CI 0.81–1.00).</p><h3 data-test=\"abstract-sub-heading\">Discussion</h3><p>In MSA, GFAP shows promise as novel biomarker for assessing current clinical disease severity, while NfL might serve as biomarker for prediction of disease progression and differential diagnosis of MSA against PD.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.8000,"publicationDate":"2024-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"GFAP and NfL as fluid biomarkers for clinical disease severity and disease progression in multiple system atrophy (MSA)\",\"authors\":\"Sabrina Katzdobler, Georg Nübling, Martin Klietz, Urban M. Fietzek, Carla Palleis, Alexander M. Bernhardt, Florian Wegner, Meret Huber, Sophia Rogozinski, Luisa-Sophie Schneider, Eike Jakob Spruth, Aline Beyle, Ina R. Vogt, Moritz Brandt, Niels Hansen, Wenzel Glanz, Kathrin Brockmann, Annika Spottke, Daniel C. Hoffmann, Oliver Peters, Josef Priller, Jens Wiltfang, Emrah Düzel, Anja Schneider, Björn Falkenburger, Thomas Klockgether, Thomas Gasser, Brigitte Nuscher, Christian Haass, Günter Höglinger, Johannes Levin\",\"doi\":\"10.1007/s00415-024-12647-z\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<h3 data-test=\\\"abstract-sub-heading\\\">Background</h3><p>Multiple system atrophy (MSA), an atypical parkinsonian syndrome, is a rapidly progressive neurodegenerative disease with currently no established fluid biomarkers available. MSA is characterized by an oligodendroglial α-synucleinopathy, progressive neuronal cell loss and concomitant astrocytosis. 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GFAP and NfL as fluid biomarkers for clinical disease severity and disease progression in multiple system atrophy (MSA)
Background
Multiple system atrophy (MSA), an atypical parkinsonian syndrome, is a rapidly progressive neurodegenerative disease with currently no established fluid biomarkers available. MSA is characterized by an oligodendroglial α-synucleinopathy, progressive neuronal cell loss and concomitant astrocytosis. Here, we investigate glial fibrillary acidic protein (GFAP) and neurofilament light chain (NfL) as fluid biomarkers for differential diagnosis, assessment of clinical disease severity and prediction of disease progression in MSA.
Methods
GFAP and NfL levels were analyzed in plasma and CSF samples of 47 MSA patients as well as 24 Parkinson’s disease (PD) and 25 healthy controls (HC) as reference cohorts. In MSA, biomarker levels were correlated to baseline and longitudinal clinical disease severity (UMSARS scores).
Results
In MSA, GFAP levels in CSF and plasma predicted baseline clinical disease severity as indicated by UMSARS scores, while NfL levels predicted clinical disease progression as indicated by longitudinal changes in UMSARS scores. Cross-sectionally, NfL levels in CSF and plasma were significantly elevated in MSA compared to both PD and HC. Receiver operating curves (ROC) indicated high diagnostic accuracy of NfL for distinguishing MSA from PD (CSF: AUC = 0.97, 95% CI 0.90–1.00; plasma: AUC = 0.90, 95% CI 0.81–1.00).
Discussion
In MSA, GFAP shows promise as novel biomarker for assessing current clinical disease severity, while NfL might serve as biomarker for prediction of disease progression and differential diagnosis of MSA against PD.
期刊介绍:
The Journal of Neurology is an international peer-reviewed journal which provides a source for publishing original communications and reviews on clinical neurology covering the whole field.
In addition, Letters to the Editors serve as a forum for clinical cases and the exchange of ideas which highlight important new findings. A section on Neurological progress serves to summarise the major findings in certain fields of neurology. Commentaries on new developments in clinical neuroscience, which may be commissioned or submitted, are published as editorials.
Every neurologist interested in the current diagnosis and treatment of neurological disorders needs access to the information contained in this valuable journal.