Profiles of disease progression and predictors of mortality in Colombian patients with amyotrophic lateral sclerosis: a comprehensive longitudinal study.

Amyotroph Lateral Scler Frontotemporal Degener

Inserm U955, Institut Mondor de Recherche Biomédicale, NeuroPsychologie Interventionnelle, Créteil, France.

Published: September 2024

AI Article Synopsis

  • The study evaluated the effectiveness of the ALS Functional Rating Scale-Revised (ALSFRS-R) in predicting mortality and understanding disease progression in ALS patients in Colombia, analyzing data from 528 patients over 14 years.
  • Results showed that a one-point increase in the ALSFRS-R score significantly increased the risk of mortality, and initial assessments provided more reliable predictions than scores from multiple evaluations.
  • The research identified three different disease progression patterns (slow, intermediate, and rapid), highlighting the need for tailored treatment strategies based on individual patient trajectories.

Article Abstract

: This study aimed to assess the prognostic value of the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) in predicting mortality and characterizing disease progression patterns in ALS patients in Colombia. : We conducted a retrospective longitudinal analysis of 537 ALS patients from the Roosevelt Institute Rehabilitation Service between October 2008 and October 2022. The study excluded nine patients due to incomplete data, resulting in 528 individuals in the analysis. ALS diagnoses were confirmed using the revised El Escorial and Gold Coast criteria. Disease progression was assessed using the ALSFRS-R, and mortality data were sourced from follow-up calls and a national database. Statistical analysis included Cox proportional hazards models to identify mortality predictors and Growth Mixture Modeling (GMM) to explore ALS progression trajectories. : The majority of the cohort (63.8%) deceased within the 84-month follow-up period. Survival analysis revealed that each point increase in the ALSFRS-R rate was associated with a 2.22-fold (95% CI =1.99-2.48,  < 0.001) increased risk of mortality. In the population with data from two clinical visits, the ALSFRS-R rate based on initial assessments predicted mortality more effectively over 36 months than the rate based on two evaluations. GMM identified three distinct progression trajectories: slow, intermediate, and rapid decliners. : The ALSFRS-R rate, derived from self-reported symptom onset, significantly predicts mortality, underscoring its value in clinical assessments. This study highlights the heterogeneity in disease progression among Colombian ALS patients, indicating the necessity for personalized treatment approaches based on individual progression trajectories. Further studies are needed to refine these predictive models and improve patient management and outcomes.

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Source
http://dx.doi.org/10.1080/21678421.2024.2405587DOI Listing

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