Problem statement: Amyotrophic Lateral Sclerosis (ALS) is a progressive and degenerative disease that affects the anterior horn motor neurons of the spinal cord and pyramidal tracts. In Brazil, there are few epidemiological data on this disease. Recently, some important findings have been reported, allowing a better understanding on the underlying processes of neuronal death, as well as the characteristics of this population. To discuss the clinical and functional profile of a convenience sample of patients with ALS in Rio de Janeiro and Neurology Department-Federal Fluminense University to compare the data with studies of other regions and countries. Approach: We used the Severity and Functional Ability Scale (SFAS) as a clinical and functional indicator for ALS. The modified El Escorial criteria were used to establish the diagnosis. The participants underwent five quarterly assessments during the study period. The research took place at