Clinically relevant increases in serum neurofilament light chain and glial fibrillary acidic protein in patients with Susac syndrome

Domenico Plantone, Eleonora Sabatelli, Sara Locci, Mariano Marrodan, Sini M. Laakso, Farrah J. Mateen, Amalia Feresiadou, Tom Buelens, Assunta Bianco, Marcela P. Fiol, Jorge Correale, Pentti Tienari, Paolo Calabresi, Nicola De Stefano, Raffaele Iorio

Risultato della ricerca: Contributo in rivistaArticolo in rivista

Abstract

Background and purpose: Serum levels of neurofilament light chain (sNfL) and glial fibrillary acidic protein (sGFAP) are promising neuro-axonal damage and astrocytic activation biomarkers. Susac syndrome (SS) is an increasingly recognized neurological condition and biomarkers that can help assess and monitor disease evolution are highly needed for the adequate management of these patients. sNfL and sGFAP levels were evaluated in patients with SS and their clinical relevance in the relapse and remission phase of the disease was assessed.Methods: As part of a multicentre study that enrolled patients diagnosed with SS from six international centres, sNfL and sGFAP levels were assessed in 22 SS patients (nine during a relapse and 13 in remission) and 59 age-and sex-matched healthy controls using SimoaTM assay Neurology 2-Plex B Kit.Results: Serum NfL levels were higher than those of healthy controls (p < 0.001) in SS patients and in both subgroups of patients in relapse and in remission (p < 0.001 for both), with significantly higher levels in relapse than in remission (p = 0.008). sNfL levels showed a negative correlation with time from the last relapse (r = -0.663; p = 0.001). sGFAP levels were slightly higher in the whole group of patients than in healthy controls (p = 0.046) and were more pronounced in relapse than in remission (p = 0.013).Conclusion: In SS patients, both sNFL and sGFAP levels increased compared with healthy controls. Both biomarkers had higher levels during clinical relapse and much lower levels in remission. sNFL was shown to be time sensitive to clinical changes and can be useful to monitor neuro-axonal damage in SS.
Lingua originaleEnglish
pagine (da-a)3256-3264
Numero di pagine9
RivistaEuropean Journal of Neurology
Volume30
DOI
Stato di pubblicazionePubblicato - 2023

Keywords

  • biomarkers
  • neuro-otology
  • neuro-ophthalmology
  • inflammation

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