In:
European Journal of Neurology, Wiley, Vol. 30, No. 6 ( 2023-06), p. 1600-1610
Abstract:
The objective was to assess the performance of serum neurofilament light chain (sNfL) in amyotrophic lateral sclerosis (ALS) in a wide range of disease courses, in terms of progression, duration and tracheostomy invasive ventilation (TIV). Methods A prospective cross‐sectional study at 12 ALS centers in Germany was performed. sNfL concentrations were age adjusted using sNfL Z scores expressing the number of standard deviations from the mean of a control reference database and correlated to ALS duration and ALS progression rate (ALS‐PR), defined by the decline of the ALS Functional Rating Scale. Results In the total ALS cohort ( n = 1378) the sNfL Z score was elevated (3.04; 2.46–3.43; 99.88th percentile). There was a strong correlation of sNfL Z score with ALS‐PR ( p 〈 0.001). In patients with long (5–10 years, n = 167) or very long ALS duration ( 〉 10 years, n = 94) the sNfL Z score was significantly lower compared to the typical ALS duration of 〈 5 years ( n = 1059) ( p 〈 0.001). Furthermore, in patients with TIV, decreasing sNfL Z scores were found in correlation with TIV duration and ALS‐PR ( p = 0.002; p 〈 0.001). Conclusions The finding of moderate sNfL elevation in patients with long ALS duration underlined the favorable prognosis of low sNfL. The strong correlation of sNfL Z score with ALS‐PR strengthened its value as progression marker in clinical management and research. The lowering of sNfL in correlation with long TIV duration could reflect a reduction either in disease activity or in the neuroaxonal substrate of biomarker formation during the protracted course of ALS.
Type of Medium:
Online Resource
ISSN:
1351-5101
,
1468-1331
Language:
English
Publisher:
Wiley
Publication Date:
2023
detail.hit.zdb_id:
2020241-6