TY - JOUR
T1 - Prediction of survival in amyotrophic lateral sclerosis
T2 - a nationwide, Danish cohort study
AU - Kjældgaard, Anne Lene
AU - Pilely, Katrine
AU - Olsen, Karsten Skovgaard
AU - Jessen, Anders Hedegaard
AU - Lauritsen, Anne Øberg
AU - Pedersen, Stephen Wørlich
AU - Svenstrup, Kirsten
AU - Karlsborg, Merete
AU - Thagesen, Helle
AU - Blaabjerg, Morten
AU - Theódórsdóttir, Ásta
AU - Elmo, Elisabeth Gundtoft
AU - Møller, Anette Torvin
AU - Bonefeld, Lone
AU - Berg, Mia
AU - Garred, Peter
AU - Møller, Kirsten
PY - 2021
Y1 - 2021
N2 - 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.
AB - 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.
KW - ALSFRS-R slope
KW - Amyotrophic lateral sclerosis
KW - Median survival time
KW - Prognostic biomarker
U2 - 10.1186/s12883-021-02187-8
DO - 10.1186/s12883-021-02187-8
M3 - Journal article
C2 - 33865343
AN - SCOPUS:85104539323
SN - 1471-2377
VL - 21
JO - BMC Neurology
JF - BMC Neurology
IS - 1
M1 - 164
ER -