Performance of serum neurofilament light chain in a wide spectrum of clinical courses of amyotrophic lateral sclerosis-a cross-sectional multicenter study.

Meyer, Thomas; Salkic, Erma; Grehl, Torsten; Weyen, Ute; Kettemann, Dagmar; Weydt, Patrick; Günther, René; Lingor, Paul; Koch, Jan Christoph; Petri, Susanne; Hermann, Andreas; Prudlo, Johannes; Großkreutz, Julian; Baum, Petra; Boentert, Matthias; Metelmann, Moritz; Norden, Jenny; Cordts, Isabell; Weishaupt, Jochen H; Dorst, Johannes; Ludolph, Albert; Koc, Yasemin; Walter, Bertram; Münch, Christoph; Spittel, Susanne; Dreger, Marie; Maier, André; Körtvélyessy, Péter

doi:10.1111/ene.15773

Klinik und Poliklinik für Neurologie (Prof. Hemmer)

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Title:: Performance of serum neurofilament light chain in a wide spectrum of clinical courses of amyotrophic lateral sclerosis-a cross-sectional multicenter study.
Document type:: Article; Multicenter Study; Journal Article; Research Support, Non-U.S. Gov't
Author(s):: Meyer, Thomas; Salkic, Erma; Grehl, Torsten; Weyen, Ute; Kettemann, Dagmar; Weydt, Patrick; Günther, René; Lingor, Paul; Koch, Jan Christoph; Petri, Susanne; Hermann, Andreas; Prudlo, Johannes; Großkreutz, Julian; Baum, Petra; Boentert, Matthias; Metelmann, Moritz; Norden, Jenny; Cordts, Isabell; Weishaupt, Jochen H; Dorst, Johannes; Ludolph, Albert; Koc, Yasemin; Walter, Bertram; Münch, Christoph; Spittel, Susanne; Dreger, Marie; Maier, André; Körtvélyessy, Péter
Abstract:: BACKGROUND AND PURPOSE: The objective was to assess the performance of serum neurofilament light chain (sNfL) in amyotrophic lateral sclerosis (ALS) in a wide range of disease courses, in terms of progression, duration and tracheostomy invasive ventilation (TIV). METHODS: A prospective cross-sectional study at 12 ALS centers in Germany was performed. sNfL concentrations were age adjusted using sNfL Z scores expressing the number of standard deviations from the mean of a control reference database and correlated to ALS duration and ALS progression rate (ALS-PR), defined by the decline of the ALS Functional Rating Scale. RESULTS: In the total ALS cohort (n = 1378) the sNfL Z score was elevated (3.04; 2.46-3.43; 99.88th percentile). There was a strong correlation of sNfL Z score with ALS-PR (p < 0.001). In patients with long (5-10 years, n = 167) or very long ALS duration (>10 years, n = 94) the sNfL Z score was significantly lower compared to the typical ALS duration of <5 years (n = 1059) (p < 0.001). Furthermore, in patients with TIV, decreasing sNfL Z scores were found in correlation with TIV duration and ALS-PR (p = 0.002; p < 0.001). CONCLUSIONS: The finding of moderate sNfL elevation in patients with long ALS duration underlined the favorable prognosis of low sNfL. The strong correlation of sNfL Z score with ALS-PR strengthened its value as progression marker in clinical management and research. The lowering of sNfL in correlation with long TIV duration could reflect a reduction either in disease activity or in the neuroaxonal substrate of biomarker formation during the protracted course of ALS.
Journal title abbreviation:: Eur J Neurol
Year:: 2023
Journal volume:: 30
Journal issue:: 6
Pages contribution:: 1600-1610
Fulltext / DOI:: doi:10.1111/ene.15773
Pubmed ID:: http://view.ncbi.nlm.nih.gov/pubmed/36899448
Print-ISSN:: 1351-5101
TUM Institution:: 1241; 1533; 303; Klinik und Poliklinik für Neurologie (Prof. Hemmer)
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mediaTUM Gesamtbestand Einrichtungen Schools TUM School of Medicine and Health Departments Clinical Medicine Klinik und Poliklinik für Neurologie (Prof. Hemmer)2023