Background Semantic dementia (SD) is a neurodegenerative disorder characterised by progressive language problems falling within the clinicopathological spectrum of frontotemporal lobar degeneration (FTLD). The development of diseasemodifying 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. Methods This 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). Results CSF 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 (r s =−0.32, p=0.002) and with smaller grey matter volume of the parahippocampal gyri (r s =−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. Conclusion CSF NfL is a promising biomarker in the diagnostic process of SD, although it has limited crosssectional monitoring or prognostic abilities.

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Persistent URL dx.doi.org/10.1136/jnnp-2018-319784, hdl.handle.net/1765/120543
Journal Journal of Neurology, Neurosurgery and Psychiatry: an international peer-reviewed journal for health professionals and researchers in all areas of neurology and neurosurgery
Citation
Meeter, L.H.H, Steketee, R.M.E., Salkovic, D., Vos, M.E., Grossman, M, McMillan, C.T., … van Swieten, J.C. (2019). Clinical value of cerebrospinal fluid neurofilament light chain in semantic dementia. Journal of Neurology, Neurosurgery and Psychiatry: an international peer-reviewed journal for health professionals and researchers in all areas of neurology and neurosurgery, 90(9), 997–1004. doi:10.1136/jnnp-2018-319784