Very late-onset amyotrophic lateral sclerosis in a Portuguese cohort

Objective: Although amyotrophic lateral sclerosis (ALS) incidence has been stable among Western countries, population-ageing effect will probably increase the proportion of very-old ALS patients. We aim to study this population. Methods: A retrospective study was performed, including 1083 ALS patients followed longitudinally in our ALS unit from January 1995 to December 2017. The patients were divided in two groups, according to age at disease onset (</>= 80 years). Demographic, clinical, and survival data were compared between groups. Results: Fifty out of 1083 (4.62%) patients were aged 80 or over. Mean onset age in this group was 82.9 +/- 2.59 years and 28 (56%) were men. Contrasting with the younger group, bulbar-onset was remarkably the most common presentation form (54%, p < 0.001), but with no gender preference (p = 0.52) and so was significantly shorter disease duration before first visit (13.41 +/- 9.42 versus 18.84 +/- 21.66 months, p = 0.001). Survival after disease onset (31.87 +/- 25.45 versus 45.61 +/- 39.93 months, respectively for older and younger groups, p = 0.001) was significantly dependent on age of onset, disease duration and onset form in the younger group while it was only dependent on onset form in the older group. No significant differences were observed regarding cognitive dysfunction, ALS/FTD familial history, non-invasive ventilation (NIV) or riluzole prescription. Conclusions: Very-old patients represent a minor but distinctive ALS group. A predominant bulbar presentation was disclosed and it could probably explain the shorter disease duration before first visit as well as survival. Older age was not an exclusion factor for good health care practices, in particular NIV and riluzole prescription.