Prevalence of amyotrophic lateral sclerosis in Latium region, Italy.

Maria Puopolo,Nicola Vanacore,Maurizio Inghilleri,Marialaura Santarelli,Ilaria Bacigalupo,Antonio Petrucci,Paola Piscopo,Alessandra Di Pucchio,Eleonora Lacorte,Mario Sabatelli

doi:10.1002/brb3.2378

Abstract

ObjectivePrevalence estimate of amyotrophic lateral sclerosis (ALS) ranged between 1.1/100,000 and 11.2/100,000 inhabitants with different design of the study (prospective or retrospective) and sample size. The aim of this study is to conduct for the first time an estimate of the ALS prevalence in the Latium region.Materials and methodsThe study was performed in Latium, a region located in the center of Italy, with a population, as of January 1, 2016, of 5888.472 inhabitants. In this region, a network of 15 clinical centers (of which 4 referral ALS centers are located in Rome) and 10 local health authorities involved in the diagnosis and treatment of ALS patients has been identified. Each patient was classified according to the El Escorial revised criteria.ResultsThe prevalence study in 2016 identified 353 ALS cases (200 males). By considering population aged >=20 years, the total crude prevalence rate resulted 7.33 (CI95% 6.59–8.14) × 100,000 and 8.75 and 6.05 in males and females, respectively. Age‐specific prevalence rates did not differ among males and females in the population aged less than 49 years. The difference emerged in population aged > 50 years. This type of diagnosis was recorded for 343 patients (11 missing). 68% of these patients have a definite diagnosis, 14% likely, 11% possible, and 12% defined as suspect.ConclusionsThe estimate of prevalence rates observed in this study is probably in line with the values reported in the literature for prospective prevalence studies.

Highlights

According to numerous studies, amyotrophic lateral sclerosis (ALS) incidence and prevalence widely varies between geographical areas worldwide, presuming that several factors play an important role in the occurrence of the disease
Age-specific prevalence rates did not differ among males and females in the population aged less than 49 years
The prevalence study identified 353 ALS cases (200 males and 153 females) who (i) received within December 31, 2016, a diagnosis di ALS according to El Escorial criteria; (ii) lived at least one day in the Latium region during the study period; and (iii) who were resident in the Latium region

Summary

Introduction

Amyotrophic lateral sclerosis (ALS) incidence and prevalence widely varies between geographical areas worldwide, presuming that several factors (ancestral, genetic, environmental, and lifestyle) play an important role in the occurrence of the disease. A meta-analysis of studies covering geographical areas and 11 sub-continents, first reported in 2017, pooled estimates of ALS incidences worldwide of 1.68/100,000 PYFU (number of cases per 100,000 person-years of follow-up) (1.50−1.85), 1.96 (1.75–2.18) for men and 1.39 (1.21–1.56) for women, with a standardized sex ratio of 1.41 [1]. Heterogeneity was observed between Northern Europe [1.89 (1.46−2.32)/100,000 PYFU] and East Asia [0.83 (0.42−1.24)/100,000 PYFU, China and Japan] or South Asia [0.73 (0.58−0.89)/100,000/PYFU Iran], all statistically significant (Marin et al, 2017). This study, reported homogeneous rates between populations from Europe, North America, and New Zealand with a pooled ALS incidence of 1.81 (1.66–1.97)/100,000 PYFU. No data regarding prevalence rates were reported (Marin et al, 2017)

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Conclusion