EUROPEAN JOURNAL OF NEUROLOGY. 2019

Thouvenot E, Demattei C, Lehmann S, Maceski-Maleska A, Hirtz C, Juntas-Morales R, Pageot N, Esselin F, Alphandery S, Vincent T and Camu W

Eur J Neurol. 2019 Aug 22. doi: 10.1111/ene.14063. 

Abstract

Background:

The prognostic value of serum neurofilament light chain (sNfL), a biomarker of neurodegeneration, compared to other prognostic factors of amyotrophic lateral sclerosis (ALS) at the time of diagnosis, remains unclear.

Methods/patients:

Sera from ALS patients were prospectively collected at the first diagnostic visit in our centre. sNfL levels were determined by single molecule array in 207 ALS patients and in 21 healthy controls. The prognostic value of sNfL was compared with that of other known clinical prognostic factors using a Cox regression model and multivariate analysis.

Results:

sNfL levels were higher in ALS patients than in controls (p<0.0001). Seven parameters were predictive of death in ALS: older age, bulbar onset, higher ALSFRS-R score, greater weight loss, lower maximal inspiratory pressure (MIP), forced vital capacity (FVC) and higher sNfL levels. Cox regression model showed that sNfL (p<0.0001), weight loss (p=0.040) and site at onset (p=0.048) were independentpredictive factors of death. In a sub-cohort restricted to 139 patients with complete spirometry data, sNfL (p<0.005) and FVC (p=0.022) were independent factors predictive of death. In a subgroup of 142 patients in whom ALSFRS-R score was available at several time points, sNfL levels positively correlated with ALSFRS-R rate of decline (r=0.571, p<10-12 ).

Conclusions:

higher sNfL concentration is a strong and independent prognostic factor of death in ALS as early as the time of diagnosis. This article is protected by copyright. All rights reserved.