Clinically relevant increases in serum neurofilament light chain and glial fibrillary acidic protein in patients with Susac syndrome
Year: 2023
Type: article
Study design: Observational Study
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.
Source: European Journal of Neurology
DOI: 10.1111/ene.15939
Institutions: University of Siena, Agostino Gemelli University Polyclinic, Istituti di Ricovero e Cura a Carattere Scientifico, Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia, University of Helsinki, Helsinki University Hospital, Massachusetts General Hospital, Uppsala University, Uppsala University Hospital, Centre Hospitalier Universitaire Brugmann, Centre Hospitalier Universitaire de Saint-Pierre, Consejo Nacional de Investigaciones Científicas y Técnicas, Universidad de Buenos Aires, Instituto de Química y Fisicoquímica Biológicas, Università Cattolica del Sacro Cuore
Language: English
Subfield: Pathology and Forensic Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $4,940
OpenAlex ID: W4381190283
