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  • Title: Clinical features and incidence trends of amyotrophic lateral sclerosis in Navarre, Spain, 2007-2018: a population-based study.
    Author: Jericó I, Elizalde-Beiras I, Pagola I, Torné L, Galbete A, Delfrade-Osinaga J, Vicente E.
    Journal: Amyotroph Lateral Scler Frontotemporal Degener; 2021 Aug; 22(5-6):401-409. PubMed ID: 33641502.
    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.
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