Insight into Elderly ALS Patients in the Emilia Romagna Region: Epidemiological and Clinical Features of Late-Onset ALS in a Prospective, Population-Based Study

Abstract

Few studies have focused on elderly (>80 years) amyotrophic lateral sclerosis (ALS) patients, who represent a fragile subgroup generally not included in clinical trials and often neglected because they are more difficult to diagnose and manage. We analyzed the clinical and genetic features of very late-onset ALS patients through a prospective, population-based study in the Emilia Romagna Region of Italy. From 2009 to 2019, 222 (13.76%) out of 1613 patients in incident cases were over 80 years old at diagnosis, with a female predominance (F:M = 1.18). Elderly ALS patients represented 12.02% of patients before 2015 and 15.91% from 2015 onwards (p = 0.024). This group presented with bulbar onset in 38.29% of cases and had worse clinical conditions at diagnosis compared to younger patients, with a lower average BMI (23.12 vs. 24.57 Kg/m²), a higher progression rate (1.43 vs. 0.95 points/month), and a shorter length of survival (a median of 20.77 vs. 36 months). For this subgroup, genetic analyses have seldom been carried out (25% vs. 39.11%) and are generally negative. Finally, elderly patients underwent less frequent nutritional- and respiratory-supporting procedures, and multidisciplinary teams were less involved at follow-up, except for specialist palliative care. The genotypic and phenotypic features of elderly ALS patients could help identify the different environmental and genetic risk factors that determine the age at which disease onset occurs. Since multidisciplinary management can improve a patient's prognosis, it should be more extensively applied to this fragile group of patients.

Keywords: amyotrophic lateral sclerosis; elderly ALS; epidemiology; phenotype; prognosis; survival.

Figure 1

Distribution of cases by sex and 5–year age groups across different disease phenotypes: (A) classic phenotype; (B) flail phenotype, including both flail arm and flail leg phenotypes; (C) Upper Motor Neuron predominant (UMN-p) phenotype; (D) bulbar phenotype; (E) respiratory phenotype.

Figure 2

Distribution of comorbidities at diagnosis for the two age groups.

Figure 3

Kaplan-Meier analysis of length of tracheostomy-free survival from symptom onset, comparing oALS and other ALS patients.

Figure 4

Kaplan-Meier analysis of length of tracheostomy-free survival from symptom onset in oALS patients by (A) diagnostic delay (≤ or >12 months); (B) ALSFRS-R score at diagnosis (≤ or > 28/48); (C) age classes (< or ≥ 85 years).