• Anne-Lene Kjældgaard, Katrine Pilely, Karsten Skovgaard Olsen, Anders Hedegaard Jessen, Anne Øberg Lauritsen, Stephen Wørlich Pedersen, Kirsten Svenstrup, Merete Karlsborg, Helle Thagesen, Morten Blaabjerg, Ásta Theódórsdóttir, Elisabeth Gundtoft Elmo, MøllerAnette TorvinATDepartment of Neurology, Aarhus University Hospital, Aarhus, Denmark., Lone Bonefeld, Mia Berg, Peter Garred, and Kirsten Møller.
• Laboratory of Molecular Medicine, Department of Clinical Immunology Section 7631, Diagnostic Centre, Rigshospitalet, Ole Maaloesvej 26, DK-2200, Copenhagen N, Denmark. anne-lene.kjaeldgaard@regionh.dk.
• Bmc Neurol. 2021 Apr 17; 21 (1): 164.

IntroductionAmyotrophic 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.ObjectiveThis 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.MethodsThe 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 ConclusionsBoth 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.

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