Prediction of survival in amyotrophic lateral sclerosis: a nationwide, Danish cohort study

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  • Anne Lene Kjældgaard, University of Copenhagen
  • ,
  • Katrine Pilely, University of Copenhagen
  • ,
  • Karsten Skovgaard Olsen
  • Anders Hedegaard Jessen, University of Copenhagen
  • ,
  • Anne Øberg Lauritsen, University of Copenhagen
  • ,
  • Stephen Wørlich Pedersen, University of Copenhagen
  • ,
  • Kirsten Svenstrup, University of Copenhagen
  • ,
  • Merete Karlsborg, University of Copenhagen
  • ,
  • Helle Thagesen, University of Copenhagen
  • ,
  • Morten Blaabjerg, University of Copenhagen
  • ,
  • Ásta Theódórsdóttir, University of Southern Denmark
  • ,
  • Elisabeth Gundtoft Elmo, University of Copenhagen
  • ,
  • Anette Torvin Møller
  • Lone Bonefeld, University of Southern Denmark
  • ,
  • Mia Berg, University of Copenhagen
  • ,
  • Peter Garred, University of Copenhagen
  • ,
  • Kirsten Møller, Department of Neuroanaesthesiology, University of Copenhagen

Introduction: Amyotrophic lateral sclerosis (ALS) is a progressive motor neuron disease with great heterogeneity. Biological prognostic markers are needed for the patients to plan future supportive treatment, palliative treatment, and end-of-life decisions. In addition, prognostic markers are greatly needed for the randomization in clinical trials. Objective: This study aimed to test the ALS Functional Rating Scale-Revised (ALSFRS-R) progression rate (ΔFS) as a prognostic marker of survival in a Danish ALS cohort. Methods: The ALSFRS-R score at test date in association with duration of symptoms, from the onset of symptoms until test date, (defined as ΔFS’) was calculated for 90 Danish patients diagnosed with either probable or definite sporadic ALS. Median survival time was then estimated from the onset of symptoms until primary endpoint (either death or tracheostomy). ΔFS’ was subjected to survival analysis using Cox proportional hazards modelling, log-rank test, and Kaplan-Meier survival analysis. Results and conclusions: Both ΔFS’ and age was found to be strong predictors of survival of the Danish ALS cohort. Both variables are easily obtained at the time of diagnosis and could be used by clinicians and ALS patients to plan future supportive and palliative treatment. Furthermore, ΔFS’, is a simple, prognostic marker that predicts survival in the early phase of disease as well as at later stages of the disease.

Original languageEnglish
Article number164
JournalBMC Neurology
Publication statusPublished - Dec 2021

Bibliographical note

Publisher Copyright:
© 2021, The Author(s).

Copyright 2021 Elsevier B.V., All rights reserved.

    Research areas

  • ALSFRS-R slope, Amyotrophic lateral sclerosis, Median survival time, Prognostic biomarker

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