12% of Patients With ALS Survive At Least 10 Years

TOPLINEA German population-based study showed that 12% of patients with amyotrophic lateral sclerosis (ALS) survived at least 10 years from the onset of symptoms, with a median survival of 2.5 years, and predictors of longer survival were younger age, slower progression rate, absence of frontotemporal dementia, and a longer interval between symptom onset and diagnosis.METHODOLOGYResearchers analyzed data from a prospective, population-based ALS registry in Rhineland-Palatinate, Germany, which enrolled 200 patients (mean age at diagnosis, 65.8 years; 94 women) between 2009 and 2012 with a follow-up until 2022.Patients were diagnosed based on revised El Escorial criteria and classified by phenotype (bulbar, classical, flail limb, axial, or pyramidal), with the assessment of demographic and clinical variables available early in disease course.Researchers assessed survival time from symptom onset to death or invasive ventilation, as well as survival after diagnosis.Potential prognostic factors were evaluated, including age at first diagnosis, site of onset, phenotype, family status, presence of frontotemporal dementia (FTD), latency between manifestation and diagnosis, vital capacity, changes in BMI, and disease progression rate measured using the ALS Functional Rating Scale (ALSFRS) or ALSFRS-Revised (ALSFRS-R).TAKEAWAYThe annual crude incidence (per 100,000 person-years) of ALS in Rhineland-Palatinate was 1.8 (95% CI, 1.6-2.2). The median survival was 2.5 years. A total of 12% of patients survived at least 10 years from symptom onset. A total of 32% of patients died within 1 year of first diagnosis and 7% survived more than 10 years. Patients with spinal onset had significantly better survival than those with bulbar onset (10-year survival, 14% vs 3%; hazard ratio [HR], 0.55; log-rank P = .0002). Flail limb phenotype was associated with markedly improved survival compared to bulbar phenotype (10-year survival, 31% vs 3%; HR, 0.35; log-rank P = .0002).Longer latency between symptom onset and diagnosis predicted better outcomes; patients diagnosed more than 12 months after symptom onset showed greater 10-year survival than those diagnosed within 6 months (19% vs 0%; HR, 0.30; log-rank P = .0001).In multivariate analysis, four independent predictors of mortality were identified: older age at diagnosis (HR, 1.034; P = .002), presence of FTD (HR, 2.183; P = .039), shorter latency from symptom onset to diagnosis (HR, 0.949; P = .0001), and a faster progression rate (HR, 1.913; P = .0001).IN PRACTICE"12% of ALS patients are still alive ten years after the onset of symptoms, which is important for advance planning of patient care. In our analysis demographic and clinical variables available at an early stage of the disease have emerged as valuable predictive factors," the authors wrote.SOURCEThe study was led by Joachim Wolf, Bruederklinikum Julia Lanz, Mannheim, Germany. It was published online on August 27 in BMC Neurology.LIMITATIONSThe study was limited by its small sample size and missing data. Biomarkers such as neurofilaments were not examined and molecular genetic analyses were not conducted. Additionally, the retrospective calculation of ALSFRS-R from original ALSFRS scores may have been prone to error, and the study did not account for the prognostic influence of therapeutic procedures such as noninvasive ventilation or percutaneous endoscopic gastrostomy.DISCLOSURESThe study received no funding. The authors reported having no conflicts of interest.This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.