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. 2021 Apr 17;21(1):164.
doi: 10.1186/s12883-021-02187-8.

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

Anne-Lene Kjældgaard  1   2 , Katrine Pilely  3 , Karsten Skovgaard Olsen  4 , Anders Hedegaard Jessen  5 , Anne Øberg Lauritsen  4 , Stephen Wørlich Pedersen  6 , Kirsten Svenstrup  6   7 , Merete Karlsborg  7 , Helle Thagesen  8 , Morten Blaabjerg  8 , Ásta Theódórsdóttir  9 , Elisabeth Gundtoft Elmo  6 , Anette Torvin Møller  10 , Lone Bonefeld  9 , Mia Berg  8 , Peter Garred  3   11 , Kirsten Møller  4   11
Affiliations

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

Anne-Lene Kjældgaard et al. BMC Neurol. .

Abstract

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.

Keywords: ALSFRS-R slope; Amyotrophic lateral sclerosis; Median survival time; Prognostic biomarker.

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Conflict of interest statement

None declared.

Figures

Fig. 1
Fig. 1
Kaplan-Meier survival plots of the ALS cohort (n = 90). a The survival plot in accordance with progression rate (ΔFS’) from the onset of symptoms until the primary end point divided by the median ΔFS’ value. b The survival plot in accordance with progression rate from onset of symptoms until primary end point divided by two arbitrary cut-off values. c The survival plot from onset of symptoms until primary end point in accordance with age at onset of symptoms
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