Author:
Kjældgaard Anne-Lene,Pilely Katrine,Olsen Karsten Skovgaard,Jessen Anders Hedegaard,Lauritsen Anne Øberg,Pedersen Stephen Wørlich,Svenstrup Kirsten,Karlsborg Merete,Thagesen Helle,Blaabjerg Morten,Theódórsdóttir Ásta,Elmo Elisabeth Gundtoft,Møller Anette Torvin,Bonefeld Lone,Berg Mia,Garred Peter,Møller Kirsten
Abstract
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.
Publisher
Springer Science and Business Media LLC
Subject
Neurology (clinical),General Medicine
Cited by
23 articles.
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