• Lieke H H Meeter, Rebecca M E Steketee, Dina Salkovic, Maartje E Vos, Murray Grossman, Corey T McMillan, David J Irwin, Adam L Boxer, Julio C Rojas, Nicholas T Olney, Anna Karydas, Bruce L Miller, PijnenburgYolande A LYALAlzheimer Center and Department of Neurology, Amsterdam Neuroscience, Vrije Universiteit Amsterdam, Amsterdam UMC, Amsterdam, The Netherlands., Frederik Barkhof, Raquel Sánchez-Valle, Albert Lladó, Sergi Borrego-Ecija, Janine Diehl-Schmid, Timo Grimmer, Oliver Goldhardt, Alexander F Santillo, Oskar Hansson, Susanne Vestberg, Barbara Borroni, Alessandro Padovani, Daniela Galimberti, Elio Scarpini, Jonathan D Rohrer, WoollacottIone O CIOCDementia Research Centre, Department of Neurodegenerative Diseases, UCL Institute of Neurology, London, UK., Matthis Synofzik, Carlo Wilke, Alexandre de Mendonca, Rik Vandenberghe, Luisa Benussi, Roberta Ghidoni, Giuliano Binetti, Wiro J Niessen, Janne M Papma, Harro Seelaar, Lize C Jiskoot, Frank Jan de Jong, Laura Donker Kaat, Marta Del Campo, Charlotte E Teunissen, Esther E Bron, Esther Van den Berg, and John C Van Swieten.
• Alzheimer Center and Department of Neurology, Erasmus MC, Rotterdam, The Netherlands.
• J. Neurol. Neurosurg. Psychiatr. 2019 Sep 1; 90 (9): 9971004997-1004.

BackgroundSemantic dementia (SD) is a neurodegenerative disorder characterised by progressive language problems falling within the clinicopathological spectrum of frontotemporal lobar degeneration (FTLD). The development of disease-modifying agents may be facilitated by the relative clinical and pathological homogeneity of SD, but we need robust monitoring biomarkers to measure their efficacy. In different FTLD subtypes, neurofilament light chain (NfL) is a promising marker, therefore we investigated the utility of cerebrospinal fluid (CSF) NfL in SD.MethodsThis large retrospective multicentre study compared cross-sectional CSF NfL levels of 162 patients with SD with 65 controls. CSF NfL levels of patients were correlated with clinical parameters (including survival), neuropsychological test scores and regional grey matter atrophy (including longitudinal data in a subset).ResultsCSF NfL levels were significantly higher in patients with SD (median: 2326 pg/mL, IQR: 1628-3593) than in controls (577 (446-766), p<0.001). Higher CSF NfL levels were moderately associated with naming impairment as measured by the Boston Naming Test (rs =-0.32, p=0.002) and with smaller grey matter volume of the parahippocampal gyri (rs =-0.31, p=0.004). However, cross-sectional CSF NfL levels were not associated with progression of grey matter atrophy and did not predict survival.ConclusionCSF NfL is a promising biomarker in the diagnostic process of SD, although it has limited cross-sectional monitoring or prognostic abilities.© Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY. Published by BMJ.

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