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

VL - 21

JO - BMC Neurology

JF - BMC Neurology

SN - 1471-2377

IS - 1

M1 - 164

ER -