Epidemiology of Amyotrophic Lateral Sclerosis in the Republic of Cyprus: A 25-Year Retrospective Study

Abstract

Introduction: Amyotrophic lateral sclerosis (ALS) is a rare, rapidly progressive neurodegenerative disease. Despite wide variability in the incidence and prevalence of ALS, there is evidence of positive temporal trends and an increase in incidence with age. The aim of this study was to conduct a detailed epidemiological investigation of ALS in Cyprus. Methods: All registered Cypriot ALS patients in the Republic of Cyprus from January 1985 until December 2014 were included. Socio-demographic information was extracted from patient files. Results: The study identified 179 ALS patients, of whom 7 had a positive family history. The mean age at onset was 58.6 years and a slight male predominance was observed. Average annual crude incidence was 1.26 cases/100,000 person-years and at the beginning of 2015, prevalence of ALS was 7.9 cases/100,000 population. Both incidence and prevalence displayed an increasing trend, even after age-standardization of incidence rates. Conclusions: Incidence, prevalence and main socio-demographic characteristics of ALS in Cyprus were similar to those of other European countries, without any geographic clustering of the disease. Additionally, an increased incidence through the years was confirmed. However, observations such as a higher male prevalence and a younger mean age of onset compared to published literature require further investigation.

Retrospective longitudinal study of ALS in Cyprus: Clinical characteristics, management and survival

“…ALS patients’ clinical characteristics, as a total and stratified by gender, are presented in Table 1. As previously reported[10], there was a male predominance with a gender ratio of 1.45 M:F (106/73), disease onset was at a mean age of 58.6±10.9 years, diagnosis was at a mean age of 59.8±10.6, and only 4% (n = 7) of our sample had a family history of ALS. Clinical characteristics did not differ between gender categories (Table 1).…”

“…This study is a retrospective clinical investigation of previously identified[10] ALS patients seen at the Cyprus Institute of Neurology and Genetics (CING) between 1 January 1985 and 31 July 2015. Recruitment details of patients are outlined elsewhere[10].…”

“…This study is a retrospective clinical investigation of previously identified[10] ALS patients seen at the Cyprus Institute of Neurology and Genetics (CING) between 1 January 1985 and 31 July 2015. Recruitment details of patients are outlined elsewhere[10]. Briefly, cases were eligible for inclusion in the study if they met the El Escorial criteria (EEC) of definite or probable ALS[11–13], were of Cypriot nationality and resided in the Republic of Cyprus.…”

“…In Cyprus, average annual crude incidence over the past 25 years was 1.26 cases per100,000 person-years. Prevalence of ALS was 7.9 cases per 100,000 population at the beginning of 2015[10]. Incidence and prevalence both increased over the past 25 years, and the increase in incidence rates remained even after age-standardization[10].…”

“…Despite the previous detailed exploration of ALS epidemiology in Cyprus by our group[10], a clinical investigation of ALS in Cyprus is still lacking. The aim of this project is to determine the clinical presentation of ALS patients, the uptake of supportive therapies, and factors associated with survival, over the past 30 years in Cyprus.…”