Respiratory Dysfunction and Sleep-Disordered Breathing in Children With Myasthenia Gravis

Abstract

<jats:sec><jats:title>Objectives:</jats:title><jats:p> The purpose of this study was to prospectively evaluate sleep patterns and the presence of sleep-disordered breathing in children with myasthenia gravis. We further aimed to examine the relationship between sleep and daytime respiratory function using spirometry tests including upright and supine forced vital capacity, sniff nasal inspiratory pressure, and maximal inspiratory pressure. </jats:p></jats:sec><jats:sec><jats:title>Methods:</jats:title><jats:p> Eleven children between 3 and 18 years old with confirmed myasthenia gravis were recruited from The Hospital for Sick Children Neuromuscular Clinic in this prospective observational study. After informed consent was obtained, patients underwent a comprehensive clinical assessment with collection of anthropometric data. Following this, all subjects performed pulmonary function tests, overnight polysomnography and completed the Epworth Sleepiness Scale questionnaire. </jats:p></jats:sec><jats:sec><jats:title>Results:</jats:title><jats:p> Two of eleven children who reported no symptoms of sleep disordered breathing were diagnosed with mild to moderate obstructive sleep apnea. Pulmonary function tests showed abnormal maximal inspiratory pressure in 6 of 11 patients, whereas seated forced vital capacity as well as seated to supine forced vital capacity ratios were normal in the entire group. </jats:p></jats:sec><jats:sec><jats:title>Conclusions:</jats:title><jats:p> In our small group of pediatric myasthenia gravis subjects, there was an unexpected finding of obstructive sleep apnea in 2 of the 11 patients studied. Maximal inspiratory pressure appears to be a more sensitive method of detecting abnormalities compared to upright or seated forced vital capacity. A larger multicenter study is needed to validate our findings and to determine the impact of obstructive sleep apnea in the pediatric myasthenia gravis population as well as risk factors associated with sleep disordered breathing. </jats:p></jats:sec>

[Retracted] Analysis on the Clinical Effect of High‐Dose Glucocorticoids Combined with Immunosuppressants on Patients with Myasthenia Gravis Undergoing Video‐Assisted Thoracoscopic Surgery

“…VATS, as a minimally invasive surgery which has been widely applied for the treatment of thoracic diseases, can visualize patients' thorax clearly on a television screen through a high-definition camera, so the surgeon can conduct the surgery by viewing a television screen, with the advantages of wide range of lesion resection and high safety [ 20 , 21 ]. Some studies reveal that the thymusectomy not only removes the thymus but also effectively clears the fat of anterior mediastinum, but VATS is limited by the surgical field during the resection of the fat of anterior mediastinum, so the surgical procedure should be finished by experienced operators [ 22 , 23 ]. In this study, after the combination therapy was implemented in MG patients, the overall response rate in the experimental group was significantly higher than that in the control group ( P < 0.05), demonstrating that the combination therapy has significantly better efficacy than VATS alone in relieving myasthenic symptoms and improving prognosis.…”

MuSK+ Myasthenia Gravis Presenting with Severe Sleep Apnea, Respiratory Dysfunction and Response to Rituximab

“…In the only previously reported case of MuSK+ MG presenting with severe OSA (AHI of 31/hour), the patient had concurrent bulbar dysfunction as part of their presentation, something not observed in our case initially. An increased prevalence of sleep disordered breathing/ OSA has been reported in some studies including patients who are AChR antibody positive as well as in children with MG (8,9); however, not all studies have found this association (10). In a recent single-center study, the risk of OSA in MG patients was associated with the same risk factors as the overall population, including male sex and obesity (11).…”

Predictive factors for postoperative myasthenic crisis in patients with myasthenia gravis

“…Spirometry test is a simple but useful method for quantitative assessment and management of a variety of pulmonary conditions [ 26 ]. MG is an autoimmune disease characterized by weakness of various groups of skeletal muscles including respiratory muscles, resulting in impaired lung function [ 27 ]. Several previous studies had revealed that decreased lung function parameters were related to POMC or prolonged ventilation support [ 9 , 10 , 28 , 29 ].…”