Incidence of amyotrophic lateral sclerosis in Europe

Abstract

Background Geographical differences in amyotrophic lateral sclerosis (ALS) incidence have been reported in the literature, but comparisons across previous studies are limited by different methods in case ascertainment and by the relatively small size of the studied populations. To address these issues, the authors undertook a pooled-analysis of European population-based ALS registries. Methods All new incident ALS cases in subjects 18 years old and older were identified prospectively in six population-based registries in three European countries (Ireland, United Kingdom, Italy) in the two year period 1998-1999 with a reference population of almost 24 million. Results Based on 1,028 identified incident cases, the crude annual incidence rate of ALS in the general European population was 2.16 per 100,000 person-years; 95% CI 2.0-2.3), with similar incidence rates across all registries. The incidence was higher among men (3.0 per 100,000 person-years; 95% CI = 2.8 to 3.3) than among women (2.4 per 100,000 person-years; 95% CI=2.2 to 2.6). Spinal onset ALS was more common among men compared to women, particularly in the 70-80 year age group. Disease occurrence decreases rapidly after 80 years of age. Conclusions ALS incidence is homogeneous across Europe. Sex differences in incidence may be explained by the higher incidence of spinal onset ALS among males and the age-related disease pattern suggests that ALS occurs within a susceptible group within the population rather than being a disease of aging.

Amyotrophic lateral sclerosis: A higher than expected incidence in people over 80 years of age

“…Prospective, population-based methodology has been shown to be useful in defining clinical characteristics and prognostic indicators. Reported incidence rates are remarkably uniform across all registries among Caucasian populations, ranging from 1.7 to 2.3 cases per 100 000 person-years [1][2][3] .…”

“…Several reasons have been put forward, such as difficulty in diagnosing ALS among the elderly because of comorbidity, difficulty in access to specialised care or a more rapid and aggressive disease and shorter survival that cause elderly patients to die before the diagnosis of ALS is established 3,7,9,10 . Identification of subpopulations at risk is likely to help to our understanding of the disease pathogenesis.…”

Age-period-cohort analysis of trends in amyotrophic lateral sclerosis incidence

“…The crude incidence of ALS for those aged 18 or over is estimated to be approximately 2.7 per 100,000 person-years in Europe [1]. The annual incidence in Ireland is about 2.6 per 100,000 [2], with approximately 110 new cases diagnosed annually.…”

Esclerosis lateral amiotrófica: actualización

“…En un estudio de países europeos se encontró mayor incidencia en hombres, con dos picos de edad de presentación: 58 a 63 años para los casos esporádicos y 47 a 52 años para los familiares; la enfermedad fue muy rara después de los 80 años (28). Cronin y colaboradores hicieron una revisión sistemática de la literatura (1966 a 2006), acerca de la variación étnica en ELA y encontraron que la incidencia es más baja en etnias africanas, asiáticas e hispánicas con respecto a caucásicos europeos y norteamericanos (29).…”