Dados do Trabalho

Título

AMYOTROPHIC LATERAL SCLEROSIS ATTENDED IN A REFERENCE CENTER IN THE CITY OF RIO DE JANEIRO

Resumo

INTRODUCTION
Amyotrophic lateral sclerosis (ALS) is, to date, one of the incurable progressive neurological diseases with the worst clinical prognosis. The Neuromuscular Diseases Service of the Pedro Ernesto University Hospital (HUPE/UERJ) is a reference center that receives patients referred from the primary and secondary care levels of the SUS, which provides the meeting of rarer and more complex neurological cases, such as ALS.

OBJECTIVE
To carry out a retrospective epidemiological study on confirmed cases of ALS treated at the Neuromuscular Diseases Outpatient Clinic at HUPE/UERJ between 2004 and 2022 to identify the locoregional profile of the patients.

METHODS
Review of care records of patients with suspected or confirmed cases of ALS and treated at the Neuromuscular Diseases Outpatient Clinic of HUPE/UERJ between 12/17/2004 (first record found) and 05/31/2022, identifying and organizing information related to gender, age, etiology and clinical phenotype of ALS cataloged cases.

RESULTS
Ninety-five suspected cases of ALS were identified in the 17 years and 5-month interval considered, of which, after reviewing the consultations, 16 cases were discarded, making a total of 79 ALS records. In this series, 45 were men (57%), and 34 were women (43%). The vast majority of cases were idiopathic (76 cases, 96% of the total), while only 3 cases showed genetic/family inheritance (4%). The most affected age group was 50-69 years-old (46 cases, 58%), with a mean age of 55 years-old, and the most common phenotype was classic ALS (65 cases, 82%), although were found 4 bulbar ALS, 4 cases of progressive muscular atrophy, 2 cases of primary lateral sclerosis, 2 cases of monomelic atrophy, 1 flail-arm syndrome and 1 Mills variant.

CONCLUSION
The data collected on a locoregional scale, despite the small number of patients considered (because it is a rare disease), are in line with the epidemiological characteristics of ALS already demonstrated in other studies of national and international scope, showing a predominance of men, sporadic cases as opposed to hereditary cases, and average age at onset of the typical condition from the sixth decade of life.

Palavras Chave

Amyotrophic Lateral Sclerosis; ALS; Motor Neuron Disease; Neurology; Rio de Janeiro; Brazil

Área

Doenças do Neurônio Motor – Esclerose Lateral Amiotrófica