Evaluation of clinical, epidemiological and electrophysiological variables for early diagnosis of amyotrophic lateral sclerosis

Joana Carvalho Dias, Camila Castelo Branco Pupe, Tania Maria Escada, Eduardo Rodrigues Davidovich, Bruno Mattos Coutinho, Osvaldo J.M. J.M. Nascimento


Motor neuron disease (MND) is a systemic disease with a broad clinical spectrum. It is characterized by primary involvement of the lower or upper motor neuron (UMN), or both, simultaneously, represented by the most common form, amyotrophic lateral sclerosis (ALS). ALS is rapidly progressive and fatal disease that evolve to death due to respiratory failure, on average, in three to five years since the onset of symptoms. This fact attends to the early and correct diagnosis of the disease. Objective: To evaluate clinical, epidemiological and electrophysiological variables for the early diagnosis of ALS. Methods: This is an observational, descriptive and retrospective study, conducted from the collect of the database, in which the variables were submitted to statistical analysis: Mann-Whitney test and Fisher's exact test. Results: When correlating clinical, epidemiological and electrophysiological findings of patients with ALS and other forms of MND, the variables: age of onset of symptoms (P=0,02) hyperreflexia (P=0,001), presence of bulbar symptoms/signs (P<0,001), pathological reflexes (P=0.001), and presence of fasciculation in electromyography (P=0,001) presented statistical significance for the diagnosis of ALS. Conclusion: Despite the small sample size, the findings reinforce the importance of well- done neurological examination, to search for signs of involvement of the UMN, in the first evaluation of patients with suspected MND. And that more research is needed to better understand the different phenotypes of the disease in order to obtain an increasingly early diagnosis to offer improvements in the quality of life of thesepatients.



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