Profile of patients who have died with multiple sclerosis in Brazil

Abstract

Background: Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease. Loss of pyramidal and anterior horn motor neurons leads to progressive limb weakness, disability, dysarthria, dysphagia and respiratory insufficiency with a progressive fatal course. Objective: To evaluate the epidemiological and clinical characteristics of amyotrophic lateral sclerosis (ALS) in the Republic of Moldova (3,559,497 inhabitants) during a 5-year period (2009)(2010)(2011)(2012)(2013). Materials and methods: To ensure complete case ascertainment, multiple sources of information were used, including neurologists, other medical specialties, neurophysiology laboratories. ALS diagnosis was based on El Escorial criteria. Although all patients with motor neuron disease were enrolled, only probable and definite cases were included in the study. Results: A total of 94 cases was collected. The average annual incidence of ALS was 0.42 per 100,000 inhabitants. On December 31, 2013 the prevalence was 2.64 per 100,000. Mean age at onset was 55.7 years, with no sex difference. The peak incidence was in the age group 51-60 years. The male: female ratio was 2,03:1. The mean duration of the disease at diagnosis was 13.6 months. The clinical spectrum comprised spinal onset, beginning in the upper or lower limbs, or bulbar onset. Conclusion: The clinical features of ALS in Moldova are similar to those of other epidemiological studies in Europe. Incidence and prevalence are lower than in other European countries. This could be explained by under ascertainment of cases due to misdiagnosis and late neurological consultation.