Spontaneous Coronary Artery Dissection (SCAD) and Autoimmunity
SCADAddison Disease28 moreThis case control study aims to determine whether spontaneous coronary artery dissection (SCAD) is associated with autoimmune diseases and to update the incidence of SCAD in a population-based cohort.
Early Versus Late Extubation in Myasthenia Gravis Patients
Myasthenia Gravis Associated With ThymomaMYASTHENIA GRAVIS (MG) is an autoimmune disease characterized by varying degrees of muscle weakness and fatigability worsened with exertion and relieved with rest。Thymectomy plays an important role in the management of these patients because a consistent association between myasthenic and thymic pathology has been recognized.The need for prolonged mechanical ventilation in these patients after thymectomy is determined by their preoperative condition and various perioperative risk factors. Leventhal et al proposed a preoperative scoring system to predict the need for postoperative mechanical ventilation in myasthenic patients undergoing thymectomy based on the following 4 criteria: duration of MG, chronic respiratory disease, dose of pyridostigmine, and vital capacity. However, some investigators discovered that the Leventhal criteria may not be the sole benchmark and that other criteria such as severity of myasthenia,history of myasthenic crisis, and presence of thymoma may be more important in predicting the necessity for prolonged mechanical ventilation after thymectomy. Naguib et al described multivariate determinants of the need for postoperative ventilation after thymectomy in MG patients predominantly on the basis of pulmonary function tests. In addition, the prevalence and presentation of MG may be variable among different ethnic groups. However, there are very few large studies investigating the determinants of prolonged mechanical ventilation after thymectomy. The authors describe the parameters associated with prolonged mechanical ventilation after trans-sternal thymectomy at their institution.
Help Build an A.I. Model to Predict Myasthenia Gravis Symptom Patterns and Flares
Myasthenia GravisThere are limited objective measurements of MG symptoms as well as a dearth of data at a granular level of MG (myasthenia gravis) symptoms and triggers occurring longitudinally. This study is designed to use the strengths of mobile smartphones which enable participant-driven real time capture of data manually and through augmented sensors such as video and audio, in order to better characterize MG symptoms and flares. The study aims to enroll approximately 200 participants for approximately 9 months until analyzable data is available from at least 100 participants. Participants will complete in-app surveys for 3 months with, audiovisual recording of symptoms. This will take approximately 35 minutes per week after the initial survey.
Follow-up of a Cohort of Patients With Myasthenic Syndrome and COVID-19 Infection
Myasthenia GravisCoronavirus disease 2019 (COVID-19), declared by the World Health Organization (WHO) as a "public health emergency of international concern" (January 31, 2020), has posed a significant threat to global health. This infectious disease, caused by the 'severe acute respiratory syndrome coronavirus-2'(SARS-CoV-2), was first reported in China at the end of 2019. As other coronaviruses, SARS-CoV-2 primarily targets the human respiratory system. The most common symptoms are fever, fatigue, and dry cough. During the second week of the disease, part of patients may progress to shortness of breath, then hypoxemia and severe pneumonia. Acute respiratory distress syndrome (ARDS), linked to some risk factors such as advanced age and underlying comorbidities (hypertension, diabetes, cardiovascular disease, and cerebrovascular disease), may be fatal and needs early supportive therapy and monitoring. Some patients with COVID-19 experienced neurological complications including headache, dizziness, hypogeusia and/or anosmia, altered level of consciousness, strokes, seizures, and ataxia, less frequently neuromuscular disorders (NMD) such as acute inflammatory polyradiculoneuropathy. Among NMD, myasthenia gravis (MG) patients, particularly susceptible to infections causing crises, could be of special risk of COVID-19 ARDS. Some general recommendations were established for the management of NMD during the COVID-19 pandemic,with also specific recommendations for MG. However, only data on a small number of patients who were managed in hospital are currently available;in addition, only two cases of myasthenic crisis following COVID-19 were reported. For this reason, the French neuromuscular rare disease network (FILNEMUS: 'FILière NEuroMUSculaire') has created the 'CO-MY-COVID register' to describe the clinical course and prognosis of patients with COVID-19 and pre-existing myasthenic syndrome.
Burden of Disease in Myasthenia Gravis
Myasthenia GravisQuestionnaire-based survey addressed to german patients with the chronic autoimmune muscle disease called myasthenia gravis regarding quality of life, socioeconomic impact, social support, course of disease, complications of therapy and psychological comorbidities.
Ocular Mysathenia Gravis Generalization
Ocular Myasthenia GravisSecondary ocular myasthenia gravis (OMG) generalization represents a pejorative evolution and no validated generalization prevention strategy exists. The aim of this observational study was to determine the percentage of patients with OMG generalization and identify factors predictive of that pejorative evolution. Data from patients with OMG registered in the Fondation Hospital A. de Rothschild database between January 1990 and January 2017 were collected. Among the 183 patients registered in this database, 151 patients with available informations were analyzed.
Translation, Cross-cultural Adaptation and Validation of the MGQOL-15-F
Myasthenia GravisThe purpose of this study is to translate and culturally adapt the MGQOL-15 (health-related quality of life scale for myasthenia gravis) for use with French-speaking patients in France and to evaluate its psychometric properties to ensure reliability and validity.
Exercise in Autoimmune Myasthenia Gravis and Myasthenic Syndromes
Autoimmune Myasthenia GravisLambert-Eaton Myasthenic Syndrome2 moreA few recent observational studies show that despite the lack of clear recommendations, many individuals with myasthenia participate in exercise. However, no link has been found between exercise and the severity of myasthenia symptoms, measured by the Muscle Myasthenia Score or the Myasthenia Gravis Composite Score. This suggests that there are other factors that may limit or prevent some individuals from being active, or factors which may facilitate participation in exercise. Studies in other diseases have shown that there are internal (beliefs, motivation, etc.) and external (cost, accessibility, etc.) factors unrelated to the disease which may play a role in exercise participation. The aim of this study is to identify factors that facilitate or limit exercise in individuals with autoimmune myasthenia gravis, congenital myasthenia syndrome and Lambert-Eaton syndrome.
Tissue Repository for Studies of Myasthenia Gravis
Myasthenia GravisThis is a study during which the investigators collect plasma and cells from patients with myasthenia gravis for the purpose of finding new antibodies.
Variables Predicting Reintubation After Thymectomy in Patients With Myasthenia Gravis
Myasthenia GravisMyasthenia gravis (MG) is an autoimmune disease that is characterized by muscle weakness and fatigue. The role of the thymus in MG has been suggested by the evidence that 10% to 15% of patients present with a thymoma and at least 60% with thymus hyperplasia or dysplasia.Beneficial effects of thymectomy in patients with MG have been described in 40% to 90%.Few studies have looked at the incidence of reintubation (not just within 24 hours after extubation), the factors associated with reintubation, and patient outcome. Premature extubation may lead to hypercarbia, hypoxemia, pulmonary hypertension, right heart failure, and myocardial ischemia. Additionally, it subjects the patient to the physical risks of reintubation, including esophageal intubation, laryngeal trauma, and pulmonary aspiration. The purpose of the present study was to determine the incidence of reintubation, the variables associated with reintubation, and patient outcome