Clinical features and incidence trends of amyotrophic lateral sclerosis in Navarre, Spain, 2007–2018: a population-based study. Issue 5 (3rd July 2021)

• Record Type: Journal Article
• Authors: Jericó, Ivonne; Elizalde-Beiras, Itsaso; Pagola, Inmaculada; Torné, Laura; Galbete, Arkaitz; Delfrade-Osinaga, Josu; Vicente, Esther
• Is Part Of: Amyotrophic lateral sclerosis and frontotemporal degeneration. Volume 22:Issue 5/6(2021)
• Page Start: 401
• Page End: 409
• Publication Date: 2021-07-03
• Subjects: Amyotrophic Lateral Sclerosis -- epidemiology -- incidence -- diagnosis delay -- survival; 616.839
• DOI: 10.1080/21678421.2021.1891249
• Languages: English
• ISSNs: 2167-8421

Abstract

Abstract: Objective : Amyotrophic Lateral Sclerosis (ALS) is a heterogeneous neurodegenerative disorder with a median survival of 3 years. The aim of our study is to analyze the incidence, age-related phenotype and clinical onset, geographical distribution, survival and diagnostic delay of ALS in Navarre. Methods : This is a population-based observational retrospective study, including all residents of Navarre (a northern Spanish region) from 2007 to 2018, who were followed until 30th September 2020. Results : We observed a global incidence 2.47/100, 000 person-years, with an upward trend throughout the study, with the highest being in the age group of 70–74 years old. Point prevalence in December 2018 was 6.64/100, 000 inhabitants (95%CI: 4.52–8.45). Upper limbs weakness onset was the most frequent in young people (<60 years), and bulbar, lower limbs weakness, generalized and respiratory associated with older age. Bulbar phenotype is the most frequent in women and in 80+ group. The median survival from clinical onset was 27.7 months (95%CI: 24.0–31.4), higher in spinal phenotype and younger onset age, and the diagnosis delay was 10.0 months (95%CI: 8.9–11.2) from clinical onset. Conclusions : We have observed a trend of increasing incidence in older people where the bulbar phenotype and female predominance