Factors Predicting Survival in ALS Patients - Data from a Population-Based Registry in Rhineland-Palatinate, Germany

Abstract

Background: The possibility to survive with amyotrophic lateral sclerosis (ALS) varies considerably and survival extends from a few months to several years. A number of demographic and clinical factors predicting survival have been described; however, existing data are conflicting. We intended to predict patient survival in a population-based prospective cohort of ALS patients from variables known up to the time of diagnosis. Methods: Incident ALS patients diagnosed within three consecutive years were enrolled and regularly followed up. Candidate demographic and disease variables were analysed for survival probability using the Kaplan-Meier method. The Cox proportional hazard regression model was used to assess the influence of selected predictor variables on survival prognosis. Results: In the cohort of 193 patients (mean age 65.8, standard deviation 10.2 years), worse prognosis was independently predicted by older age, male gender, bulbar onset, probable or definite ALS according to El Escorial criteria, shorter interval between symptom onset and diagnosis, lower Functional Rating Scale, diagnosis of frontotemporal dementia, and living without a partner. Conclusions: Taking into account these predictor variables, an approximate survival prognosis of individual ALS patients at diagnosis seems feasible.

Prognosis of amyotrophic lateral sclerosis patients undergoing tracheostomy invasive ventilation therapy in Japan

“…The daily lives of ALS patients with TIV may be influenced by the medical care system, nursing care system, various socioeconomic factors or family situations. There are reports that the existence of marital relationships has an impact on the prognosis of ALS patients 32 33. Collecting and analysing this information and investigating the patients’ quality of life are tasks to be addressed in the future.…”

Hospitalizations due to respiratory failure in patients with Amyotrophic Lateral Sclerosis and their impact on survival: a population-based cohort study

“…Factors such as living without a partner [31], area of residency [32], nutritional status at diagnosis or during the course of disease [33] as well as a diagnosis of frontotemporal dementia [31], have an unfavorable prognostic role in ALS. Information on clinical, psychosocial or environmental factors were not recorded in our data and we could not assess their role.…”

What causes amyotrophic lateral sclerosis?

“…For example, the p.Asp91Ala variation of the SOD1 gene is associated with very slow progression 63, 64 , while the p.Ala5Val variant is associated with aggressive disease 65 . Statistical models can be used to provide clinically useful information for patients, the strongest message being that survival is extremely unreliably predicted in individuals, even though patterns can be seen in the data 54, 57, 66– 68 .…”