Sleep study, lung function, respiratory muscle strength and validation of two instruments of clinical outcome in patients with myasthenia gravis: Myasthenia gravis Quantitative testing form and MG Composite Scale.

• Grant number: 15/11738-6
• Support Opportunities: Scholarships in Brazil - Doctorate
• Start date: September 01, 2015
• End date: November 30, 2017
• Field of knowledge: Interdisciplinary Subjects
• Principal Investigator: Claudia Santos Oliveira
• Grantee: Ezequiel Fernandes de Oliveira
• Host Institution: Universidade Nove de Julho (UNINOVE). Campus Vergueiro. São Paulo , SP, Brazil

Abstract

The Myasthenia gravis (MG) is one of the most common diseases that affects the neuromuscular junction. Its prevalence has been reported as 2-7 / 10 000 in the UK, and about 1 / 10,000 american citizens between the ages of 20 and 40, being the most committed women than men at a ratio of 3:2. The manifestations in the respiratory system are generally attributed to the weakness of the diaphragm and other accessory muscles of ventilation. The weakness of these muscles initially leads to breathlessness efforts, progressing to rest dyspnea. Among these manifestations, we highlight the emergence of sleep-disordered breathing (SDB) due to the weakening of the muscles of the oropharynx. It is already described in the literature that there is a correlation between health related quality of life (HRQOL) and severity of MG. The comments on HRQOL of patients may influence therapeutic decisions paving the way for a proper clinical support. The more specific evaluation instrument, it becomes more relevant in the evaluation and monitoring of patients. Currently, according to our knowledge, were found in the international scientific literature, four specific instruments for MG, two about HRQOL and two assessment evaluation of disease severity. In these instruments, only the "Quality of Life Questionnaire specific for Myasthenia Gravis - 15 items" was translated into Portuguese from Brazil. Therefore justifies a study as the goal of translation and validation of two specific tools to assess the clinical course and severity of MG. Objectives: To investigate the pulmonary function and respiratory muscle strength through spirometry and manometer; To investigate the physiological sleep pattern through the full-night polysomnography; verify the quality of sleep, the risk for OSA and the presence of excessive daytime sleepiness through scales and specific questionnaires; To perform the translation, cultural adaptation and validation of two specific tools to assess the clinical course of patients with MG, called "Quantitative Myasthenia gravis test form (QMG) and MG composite scale (MGC). We hypothesized that patients with MG have a poor sleep due to the presence of DRS, committed by the weakness of respiratory muscles resulting in worsening of the clinical status, the appearance of respiratory complications and, consequently, worsening clinical condition of MG.

Scientific publications

(4)

(References retrieved automatically from Web of Science and SciELO through information on FAPESP grants and their corresponding numbers as mentioned in the publications by the authors)

OLIVEIRA, EZEQUIEL FERNANDES; OLIVEIRA VALERIO, BERENICE CATALDO; CAVALCANTE, VALERIA; URBANO, JESSICA JULIOTI; SILVA, ANDERSON SOARES; POLARO, MELISSA NUNES; NACIF, SERGIO ROBERTO; OLIVEIRA, CLAUDIA SANTOS; DUTRA RESENDES, MARIA BEMADETE; BUTTE OLIVEIRA, ACARY SOUZA; et al. Teste Quantitativo para Miastenia Gravis: estudo multicêntrico brasileiro para tradução, adaptação cultural e validação. Arquivos de Neuro-Psiquiatria, v. 75, n. 7, p. 457-463, JUL 2017. (15/11738-6)

MARIA FERNANDA HUSSID; FELIPE XEREZ CEPEDA; CAMILA P. JORDÃO; RAFAELA R. P. LOPES-VICENTE; LESLIE VIRMONDES; KEYLA Y. KATAYAMA; EZEQUIEL F. DE OLIVEIRA; LUIS V. F. OLIVEIRA; FERNANDA MARCIANO CONSOLIM-COLOMBO; IVANI CREDIDIO TROMBETTA. Obesidade Visceral e Hipertensão Sistólica como Substratos da Disfunção Endotelial em Adolescentes Obesos. Arquivos Brasileiros de Cardiologia, v. 116, n. 4, p. 795-803, 2021-04-16. (15/03274-0, 16/16831-7, 15/11738-6)

OLIVEIRA, EZEQUIEL FERNANDES; NACIF, SERGIO ROBERTO; URBANE, JESSICA JULIOTI; SILVA, ANDERSON SOARES; OLIVEIRA, CLAUDIA SANTOS; PEREZ, EDUARDO ARAUJO; POLARO, MELISSA NUNES; OLIVEIRA VALERIO, BERENICE CATALDO; STIRBULOV, ROBERTO; INSALACO, GIUSEPPE; et al. Sleep, lung function, and quality of life in patients with myasthenia gravis: A cross-sectional study. Neuromuscular Disorders, v. 27, n. 2, p. 120-127, FEB 2017. (15/11738-6)

OLIVEIRA, EZEQUIEL FERNANDES; LIMA, VALERIA CAVALCANTE; PEREZ, EDUARDO ARAUJO; POLARO, MELISSA NUNES; OLIVEIRA VALERIO, BERENICE CATALDO; PEREIRO, JOAO R.; NACIF, SERGIO ROBERTO; OLIVEIRA, CLAUDIA SANTOS; BULLE OLIVEIRA, ACARY SOUZA; FRANCO OLIVEIRA, LUIS VICENTE. Tradução,adaptação cultural e validação da escala composta de Miastenia Grave para a lingua portuguesa do Brasil. Estudo multicêntrico. Arquivos de Neuro-Psiquiatria, v. 74, n. 11, p. 914-920, NOV 2016. (15/11738-6)