Clinical aspects of amyotrophic lateral sclerosis in Rio de Janeiro/Brazil

Objective: The clinical and epidemiological profile of sporadic amyotrophic lateral sclerosis (ALS), a chronic, degenerative, progressive motor neuron disease of unknown etiology, was described and evaluated in the city of Rio de Janeiro. Method: Patients with a diagnosis definite of ALS according to the revised criteria of the El Escorial World Federation of Neurology were included in this retrospective, descriptive study (n =227). Demographic data, clinical variables, mortality and survival of these patients were assessed. Results: Of the 227 included cases, 143 (63%) were male and 84(37%) were female, resulting in a male/female ratio of 1.7:1. Mean age at onset of the disease was 53.6 +/- 12.1 years, overall median survival time was 49 months (95%CI: 42.4-55.5) and the majority of patients (71.4%) were white, black patients 15.9% and mulattos 12.8%. The most common forms of the disease were classic and bulbar ALS. Conclusion: Taking classic and bulbar ALS together, the disease was more common in white, male patients of 50 to 70 years of age. When analyzed separately, the bulbar form was more common in women and in older patients. Survival of patients with bulbar ALS was shorter compared to that of patients with classic ALS. (C) 2012 Elsevier B.V. All rights reserved.