Myasthenia Gravis in pregnancy: Systematic review and case series

Background Myasthenia gravis is an autoimmune disease which can impact pregnancy. Methods Six databases were systematically searched for studies with at least five subjects reporting pregnancy outcomes for women with myasthenia gravis in pregnancy. Assessment of bias was performed for all included studies. Forty-eight cases from our own centre were also included in the analysis. Results In total, 32 publications met inclusion criteria for systematic review, for a total of 33 unique data sets including 48 cases from our institution. Outcome data was available for 824 pregnancies. Spontaneous vaginal delivery occurred in 56.3% of pregnancies. Overall risk of myasthenia gravis exacerbation was 33.8% with a 6.4% risk of myasthenic crisis in pregnancy and 8.2% postpartum. The incidence risk of transient neonatal myasthenia gravis was 13.0%. Conclusions The current systematic review provides the best estimates of risk currently available to aid in counselling women with myasthenia gravis in pregnancy.

COMORBIDITIES OF MYASTHENIC CRISIS PATIENTS ACCORDING TO AGE AND GENDER IN DR. SOETOMO GENERAL ACADEMIC HOSPITAL, SURABAYA, INDONESIA IN 2017-2019

Highlight:1. Patients with myasthenia gravis (MG) can have both autoimmune and non-autoimmune comorbidities.2. MG patients were predominantly aged >50 years, females and the commonest comorbidity are hypertension and diabetes mellitus.3. Age and comorbidities were found to have significant correlation among these patients, but not between gender and comorbidities.Abstract:Background: There are limited studies on the comorbidities of myasthenic crisis (MC) associated with age and gender, specifically from Indonesia. These data might be valuable for treatment to improve the outcome of these patients. Objective: To analyze the correlation between age and gender with the comorbidities amongst the MC patients admitted in the ICU of Dr. Soetomo General Academic Hospital, Surabaya, Indonesia in 2017-2019. Materials and methods: A retrospective cross-sectional analytic study was conducted on 25 medical records of MC patients who fulfilled the inclusion and exclusion study criteria. Descriptive statistics and Chi-square tests were done to determine the potential correlation between variables (significant level of p<0.05). Results: The age of MC patients was £50 years (80%) and >50 years (20%) while the gender predominantly found were females (68%) with 32% males. A significant correlation was found between age and comorbidities (p=0.002) whilst not between gender and comorbidities (p=0.115). The most common comorbidities observed were hypertension (30%) and diabetes mellitus (20%). Conclusion: There were 40% of patients found with comorbidities. There was a significant association between age and comorbidities found amongst myasthenic crisis patients, whilst no significant association was found between gender and comorbidities.

Utilization of Decision Tree Algorithms for Supporting the Prediction of Intensive Care Unit Admission of Myasthenia Gravis: A Machine Learning-Based Approach

Myasthenia gravis (MG), an acquired autoimmune-related neuromuscular disorder that causes muscle weakness, presents with varying severity, including myasthenic crisis (MC). Although MC can cause significant morbidity and mortality, specialized neuro-intensive care can produce a good long-term prognosis. Considering the outcomes of MG during hospitalization, it is critical to conduct risk assessments to predict the need for intensive care. Evidence and valid tools for the screening of critical patients with MG are lacking. We used three machine learning-based decision tree algorithms, including a classification and regression tree, C4.5, and C5.0, for predicting intensive care unit (ICU) admission of patients with MG. We included 228 MG patients admitted between 2015 and 2018. Among them, 88.2% were anti-acetylcholine receptors antibody positive and 4.7% were anti-muscle-specific kinase antibody positive. Twenty clinical variables were used as predictive variables. The C5.0 decision tree outperformed the other two decision tree and logistic regression models. The decision rules constructed by the best C5.0 model showed that the Myasthenia Gravis Foundation of America clinical classification at admission, thymoma history, azathioprine treatment history, disease duration, sex, and onset age were significant risk factors for the development of decision rules for ICU admission prediction. The developed machine learning-based decision tree can be a supportive tool for alerting clinicians regarding patients with MG who require intensive care, thereby improving the quality of care.

An unusual presentation of thymoma: dysgeusia, ulcerative colitis, keratoconjunctivitis sicca, autoimmune retinopathy and myasthenia gravis

A 41-year-old female presented with dysgeusia, dry eyes, nyctalopia with progressive visual field constriction (due to autoimmune retinopathy) and gastrointestinal symptoms (due to ulcerative colitis). She was subsequently admitted to intensive care with a myasthenic crisis, and CT of the thorax demonstrated a thymoma.Following thymectomy and adjuvant radiotherapy, she has remained in complete remission from her ulcerative colitis and myasthenia gravis, her retinopathy has stabilised and there has been no thymoma recurrence over a 10-year postoperative period. There was a brief relapse of her dysgeusia (causing weight loss) and dry eye symptoms 3 years after her surgery, which resolved 8 months later. While the association of thymomas with paraneoplastic syndromes (PNS) is well established, it is unusual to present with multiple PNS, and some of these have only been documented in sparse case reports to date. Thymectomy played a crucial role in improvement and stabilisation of her PNS.

Manajemen Perawatan Neurointensif pada Miastenia Gravis

Myasthenia gravis (MG) is an autoimmune disorder that affects neuromuscular transmission, causing generalized or localized weakness characterized by fatigue. Myasthenia gravis is most commonly associated with antibodies to the acetylcholine receptor (AChR) on the motor end plate in the postsynaptic neuron. This article aims to determine the appropriate neurointensive management in patients with myasthenia gravis with complications of myasthenic crisis. The writing of this article includes various sources originating from scientific journals and government guidelines and related agencies. Source searches were carried out on online portals for journal publications such as MedScape, Google Scholar (scholar.google.com) and the National Center for Biotechnology Information (ncbi.nlm.nih.gov), with the keyword “Myasthenia Gravis”. The management of myasthenia gravis can be done in various ways, namely, mechanical intubation and ventilation, non-invasive ventilation, pridostigmine as an anticholinesterase inhibitor, immunosuppressant therapy, short term immunotherapy, intravenous immunoglobulin, and surgical therapy. In the treatment of myasthenia gravis, the main goal is to restore muscle condition, especially patient productivity where the management of myasthenia gravis consists of management of myasthenic crisis, cholinergic crisis, symptoms, immunosuppressant therapy, and thymectomy surgical therapy if a tumor is indicated.

Patients With Myasthenia Gravis With Acute Onset of Dyspnea: Predictors of Progression to Myasthenic Crisis and Prognosis

Background: Life-threatening myasthenic crisis (MC) occurs in 10–20% of the patients with myasthenia gravis (MG). It is important to identify the predictors of progression to MC and prognosis in the patients with MG with acute exacerbations.Objective: This study aimed to explore the predictors of progression to MC in the patients with MG with acute onset of dyspnea and their short-term and long-term prognosis.Methods: This study is a retrospective cohort study. We collected and analyzed data on all the patients with MG with acute dyspnea over a 10-year period in a single center using the univariate and multivariate analysis.Results: Eighty-six patients with MG were included. In their first acute dyspnea episodes, 36 (41.9%) episodes eventually progressed to MC. A multivariate analysis showed that the early-onset MG (adjusted OR: 3.079, 95% CI 1.052–9.012) and respiratory infection as a trigger (adjusted OR: 3.926, 95% CI 1.141–13.510) were independent risk factors for the progression to MC, while intravenous immunoglobulin (IVIg) treatment prior to the mechanical ventilation (adjusted OR: 0.253, 95% CI 0.087–0.732) was a protective factor. The prognosis did not significantly differ between the patients with and without MC during the MG course, with a total of 45 (52.3%) patients reaching post-intervention status better than minimal manifestations at the last follow-up.Conclusion: When treating the patients with MG with acute dyspnea, the clinicians should be aware of the risk factors of progression to MC, such as early-onset MG and respiratory infection. IVIg is an effective treatment. With proper immunosuppressive therapy, this group of patients had an overall good long-term prognosis.