Myasthenic crisis

BACKGROUND: Noninvasive ventilation (NIV) plays an important role in avoiding endotracheal intubation during myasthenic crisis, yet there are few published data concerning long-term home NIV in stable out-patients with myasthenia gravis (MG). The aim of this study was to describe the prevalence of NIV in a cohort of subjects with stable MG and to analyze contributing factors that could predict the need of NIV. METHODS: We performed a cross-sectional study that included subjects diagnosed with MG managed in the respiratory care unit over the previous year...

Myasthenic crisis (MC) requiring mechanical ventilation is a serious complication of myasthenia gravis (MG). Here we analyze the frequency and risk factors of weaning- and extubation failure as well as its impact on the clinical course in a large cohort. We performed a retrospective chart review on patients treated for MC in 12 German neurological departments between 2006 and 2015. Weaning failure (WF) was defined as negative spontaneous breathing trial, primary tracheostomy, or extubation failure (EF) (reintubation or death)...

We report the case of a 62-year-old male patient with a previous diagnosis of myasthenia gravis who experienced a myasthenic crisis due to a lower respiratory tract infection caused by Burkholderia cepacia . The patient was admitted to the neuro-intensive care unit and received ventilatory support to address respiratory insufficiency. Treatment included tigecycline and piperacillin-tazobactam for the suspected bacterial infection, as well as targeted management for the myasthenic crisis. Following a successful recovery and favorable clinical response, this case report aims to contribute to the literature by discussing the patient's presentation and exploring the incidence and characteristics of B...

Congenital myasthenia syndromes (CMS) are a heterogeneous group of hereditary disorders of the neuromuscular junction. The symptoms include fatigue, muscle weakness, ptosis, mastication or swallowing problem, respiratory distress. We present a 42-year-old male patient who was admitted with complaints of paroxysmal limb weakness for 25 years and got repeated apnea crisis due to using AchE inhibitors. We considered this patient to be COLQ -related CMS because of two types characteristics. One is the symptom will deteriorate or non-responsive after giving AchE inhibitors and the other is repeated compound action potentials may appear after one current stimulation...

Thymoma-associated myasthenia gravis (TMG) had more severe symptoms and worse prognoses in comparison to non-thymoma-associated MG. Thymoma recurrence was frequently associated with transient worsening of MG and even acute respiratory failure, namely myasthenic crisis (MC). However, little is known about the clinical features and outcomes of MC in thymoma-associated MG patients. We performed a retrospective cohort study in MG patients recruited from 9 independent tertiary neuromuscular centers in China from Jan 2015, through Oct 2022...

When it comes to thyroid disorders, Graves' disease (GD) is the most prevalent autoimmune thyroid disorder in which antibodies are formed against thyroid receptors. Myasthenia gravis (MG) is a rare autoimmune neuromuscular junction disorder. Autoimmune antibodies are formed against postsynaptic neuromuscular junctions in MG, interrupting neuromuscular transmission and causing variable muscle weakness and tiredness. It affects older men and young women. The two disorders may coexist in a patient, or either of them may develop first...

This single-center retrospective exploratory analysis evaluated the effects of sugammadex compared with neostigmine on postoperative recovery in patients with myasthenia gravis (MG) who underwent video-assisted thoracoscopic surgery (VATS)-thymectomy. This retrospective study included 180 patients with MG, aged >18 years, who received sugammadex (sugammadex group, n = 83) or neostigmine-glycopyrrolate (neostigmine group, n = 88) after VATS-thymectomy between November 2007 and December 2020. Inverse probability of treatment weighting (IPTW) adjustment was performed to balance the baseline characteristics between the two groups...

OBJECTIVE: To evaluate the risk of myasthenia gravis (MG) associated with immune checkpoint inhibitors (ICI). METHODS: Adverse event (AE) reports related to MG, myasthenic syndrome, and MG crisis for durvalumab, atezolizumab, pembrolizumab, nivolumab, avelumab, and ipilimumab in the US FDA Adverse Event Reporting System (FAERS) from Q1 2004 to Q3 2022 were collected. The proportional reporting odds ratio (PRR) method was used to evaluate the correlation between the six drugs and the three AEs...

BACKGROUND: Myasthenia gravis is an autoimmune condition affecting the neuromuscular junction and causing muscle weakness along with fatigue (myasthenia). When the clinical manifestations of myasthenia gravis are isolated to the eye muscles, only causing weak eye movements, it is referred to as ocular myasthenia gravis, which can mimic a 1 and ½ syndrome. CASE PRESENTATION: An African-American female in her fifties with past medical history of hypertension presented to our outpatient clinic with complaints of blurred vision for two weeks...

BACKGROUND: Myasthenia gravis (MG) is the most prevalent autoimmune disorder affecting the neuromuscular junction. A rapid deterioration in respiratory muscle can lead to a myasthenic crisis (MC), which represents a life-threatening condition with high mortality in MG. Multiple CD4+ T subsets and hypercytokinemia have been identified in the peripheral pro-inflammatory milieu during the crisis. However, the pathogenesis is complicated due to the many types of cells involved, leaving the underlying mechanism largely unexplored...

Myasthenia gravis (MG) is a disorder of the neuromuscular junction that can deteriorate into myasthenic crisis, involving weakness of bulbar and respiratory muscles. In this study, we describe the clinical manifestations of myasthenic crisis, identify risk factors, and examine treatments and outcomes. All 95 patients with generalized MG treated at our center during the last 10 years were included in this retrospective study. We collected data from the patients' records, including clinical follow-ups, muscle antibodies, thymic status, and treatments...

Acute exacerbations of Myasthenia Gravis (MG) may be triggered by infections and certain drugs. No consensus has been reached on vaccines and the risk for developing myasthenic crisis. During the COVID-19 pandemic, MG patients are considered at high risk for severe illness, and vaccination is strongly recommended. We report the case of a 70-year-old woman with MG, diagnosed 2 years earlier, that developed myasthenic crisis 10 days after the second dose of the BNT162b2 mRNA COVID-19 vaccine (Pfizer-BioNTech)...

BACKGROUND: Approximately 10% to 20% of myasthenia gravis (MG) patients have experienced a myasthenic crisis (MC), which contributes to morbidity and mortality. MC triggered by infection is associated with poor outcomes. However, there is a lack of prognostic factors that clinicians can utilize to target interventions for preventing recurrent infection-triggered MC. This study aimed to characterize clinical manifestations, comorbidities, and biochemical profiles associated with recurrent infection-triggered MC in MG patients...

In this article, we present the case of a patient with previously undiagnosed myasthenia gravis who underwent an endoscopic procedure for Zenker's diverticulum. The patient was readmitted due to ongoing dysphagia and severe respiratory distress caused by myasthenic crisis. This case demonstrates that myasthenia gravis, although rare, can occur in elderly patients and present with other sequelae that may mask the underlying diagnosis.

BACKGROUND: Myasthenia gravis (MG) is a rare neuromuscular disorder. Symptoms can range from ptosis only to life threatening myasthenic crisis. Thymectomy is recommended for anti-acetylcholine receptor-antibody positive patients with early-onset MG. Here, we investigated prognostic factors shaping therapeutic outcomes of thymectomy to improve patient stratification. METHODS: We retrospectively collected single-center data from a specialized center for MG from all consecutive adult patients that underwent thymectomy from 01/2012 to 12/2020...

INTRODUCTION: Myasthenia gravis is a long-lasting autoimmune neuromuscular disease caused by antibodies attacking the neuromuscular junction, which can result in muscle weakness, fatigue, and respiratory failure in severe cases. Myasthenic crisis is a life-threatening event that requires hospitalization and treatments with intravenous immunoglobulin or plasma exchange. We reported the case of an AChR-Ab-positive myasthenia gravis patient with refractory myasthenic crisis, in which starting eculizumab as rescue therapy led to a complete resolution of the acute neuromuscular condition...

Myasthenia gravis is an autoimmune disorder caused by the production of antibodies that block either acetylcholine receptors or structural receptors of the neuromuscular junction. There is expanding evidence that novel coronavirus (2019-nCoV) disease can lead to the development of an autoimmune response. Myasthenic crisis, a life-threatening respiratory muscle weakness severe enough to necessitate intubation or tracheostomy, can be a potential complication of myasthenia gravis. In this report, we describe the case of a 57-year-old man with acute respiratory insufficiency requiring emergency tracheostomy...

Myasthenia gravis (MG) is a common neuromuscular junction (NMJ) disorder and autoimmune disease mediated by several antibodies. Several studies have shown that genetic factors play an important role in MG pathogenesis. To gain insight into the epigenetic factors affecting MG, we report here genome-scale DNA methylation profiles of MG. DNA was extracted from 8 MG patients and 4 healthy controls for genome-wide DNA methylation analysis using the Illumina HumanMethylation 850K BeadChip. Verification of pyrosequencing was conducted based on differential methylation positions (DMPs)...

OBJECTIVES: To determine the relationship between the number of plasma exchanges and clinical outcome in patients experiencing myasthenic crisis. METHODS: We retrospectively reviewed all episodes of myasthenia gravis exacerbation/crisis who received plasmapheresis in patients admitted to a single-center tertiary care referral center from July 2008 to July 2017. We performed statistical analyses to determine whether the increased number of plasma exchanges improves the primary outcome (hospital length of stay) and the secondary outcome (disposition to home, skilled nursing facility, long-term acute care hospital, or death)...

Presynaptic congenital myasthenic syndromes (CMS) are a group of genetic disorders affecting the presynaptic side of the neuromuscular junctions (NMJ). They can result from a dysfunction in acetylcholine (ACh) synthesis or recycling, in its packaging into synaptic vesicles, or its subsequent release into the synaptic cleft. Other proteins involved in presynaptic endplate development and maintenance can also be impaired.Presynaptic CMS usually presents during the prenatal or neonatal period, with a severe phenotype including congenital arthrogryposis, developmental delay, and apnoeic crisis...