Cogan's sign in a patient with suspected post-COVID-19 vaccine-associated myasthenia gravis.

The Cogan's sign is indicative of myasthenia gravis. This is the first report of neurological signs in a patient with post-COVID-19 vaccine-associated myasthenia gravis in Brazil. In this case, a previously healthy 68-year-old woman presented with proximal limb weakness, left ptosis, and diplopia 1 month after receiving her fourth dose of the COVID-19 vaccine. Neurological examination revealed the presence of Cogan's sign, and she recovered rapidly after treatment. To our knowledge, this is the first reported case of myasthenia gravis associated with the COVID-19 vaccine in Brazil.

COVID-19 Vaccination and Late-Onset Myasthenia Gravis: A New Case Report and Review of the Literature.

Myasthenia gravis (MG) is a rare autoimmune disease that is potentially threatening for patient life. Auto-antibodies targeting structures of the neuromuscular junction, particularly the acetylcholine receptor (AchR), are often found in the serum of MG patients. New-onset MG after SARS-CoV-2 vaccination has rarely been reported since the introduction of vaccination. Infections and COVID-19 infection have also been reported as possible triggers for a myasthenic crisis. We report a case of new-onset MG after receiving the mRNA COVID-19 vaccination. The patient was a 73-year-old male initially presenting with ocular symptoms and a rapid generalization. We also performed a literature revision of 26 described cases of MG after SARS-CoV-2 immunization. The patients were a majority of males with generalized late-onset MG occurring after the first dose of vaccine, similar to our patient. Only our patient showed a thymoma. Thymic mass and the positivity of AchR antibodies suggest that vaccination might have triggered a subclinical pre-existing MG with symptoms flaring. Clinicians should be aware of possible new-onset MG after COVID-19 vaccination, particularly in at-risk patients. Even though COVID-19 vaccination should be recommended in MG patients, particularly in well-compensated patients. However, more studies need to be performed in the future.

New-Onset Myasthenia Gravis Confirmed by Electrodiagnostic Studies After a Third Dose of SARS-CoV-2 mRNA-1273 Vaccine.

ABSTRACT: Coronavirus disease 2019 vaccine-related pathology is a rare occurrence with few reported cases. We report on a case of a 60-yr-old man experiencing symptoms of dysphagia, dysarthria, diplopia, and weakness with onset 6 days after receiving a third full dose of SARS-CoV-2 vaccine (mRNA-1273 vaccine) in August 2021, which he received outside of the Center for Disease Control recommended guidelines, at 4 mos after his second dose of the Moderna vaccination course in March 2021. The Food and Drug Administration Emergency Use Authorization for mRNA-1273 booster was established in October 2021.Over the next month, the patient's symptoms progressed including his inability to swallow, requiring hospitalization due to dehydration and malnutrition. Evaluation including laboratory prompted referral for electrodiagnostic studies consisting of repetitive nerve stimulation studies and needle electromyography, confirming a case of new onset bulbar myasthenia gravis.

Clinical features and outcomes of patients with myasthenia gravis affected by COVID-19: A single-center study.

INTRODUCTION: Myasthenia Gravis (MG) is an autoimmune disorder that can exacerbate for various reasons including infections. In this study, we describe clinical symptoms, outcomes, and management of MG patients affected by COVID-19 infection. METHODS: This observational retrospective study was performed on patients previously diagnosed as MG, presenting with COVID-19 in the clinic or emergency department between April 2020 and August 2021. The clinical data, outcome, and therapeutic interventions were assessed in 83 patients with MG and COVID-19 infection. RESULTS: Seventy-seven patients performed PCR testing for COVID-19, of which 73 (94.8 %) were positive. Seven patients had the positive serologic test for COVID-19 (IgG and IgM). Fifty-seven (68.7 %) patients had lung involvement. Thirty-five (42.1 %) of patients were admitted to the hospital. Twelve (14.5 %) patients needed hospitalization in an intensive care unit (ICU), with a mean stay of 7.36 ± 5.6 days (rang: 2-20 days). Four (4.8 %) patients were intubated and required mechanical ventilation. Sixteen (19.3 %) patients experienced an exacerbation of myasthenia gravis and were treated with PLEX (n = 2), IVIG (n = 7), and intravenous (IV) methylprednisolone (n = 7). The outcome was favorable in 79 patients and fatal in four patients, three of whom had other comorbidities. One patient died due to severe COVID-19 involvement. CONCLUSION: The findings from our study demonstrated that patients with previous MG concurrence with COVID-19 have favorable clinical outcomes. Most patients did not need to be hospitalized and more than 80 % of patients did not display MG exacerbation.

Manifestation of Ocular Myasthenia Gravis as an Initial Symptom of Coronavirus Disease 2019: A Case Report.

For a while, coronavirus disease-2019 (COVID-19) has been a major global pandemic. It primarily affects the respiratory system but has extrapulmonary manifestations such as gastrointestinal and neurological symptoms. Data on myasthenia gravis (MG), as a complication of COVID-19, are limited. We herein report the manifestation of ocular MG as an initial symptom of COVID-19. In November 2020, a 31-year-old healthy woman was referred to Firoozgar Hospital (Tehran, Iran) with left upper eyelid ptosis and diplopia as well as general weakness, myalgia, fever, and nasal congestion for four days prior to admission. Although the acetylcholine receptor antibody in her serum was negative, increased jitter in several muscles led to the diagnosis of ocular MG. Nasal swab reverse transcription-polymerase chain reaction (RT-PCR) assay tested positive for severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) infection. Computed tomography (CT) scan of the chest revealed bilateral ground-glass opacities and some foci of consolidation formation, but the thymus was normal. The patient was successfully treated with remdesivir and dexamethasone. The patient was eventually discharged in good condition and with improved neurological symptoms. A limited number of studies have suggested a possible association between MG and COVID-19. Therefore, further data are required to substantiate the proposed association. Clinicians should be aware of ocular MG during the COVID-19 pandemic to better diagnose and manage patients with SARS-CoV-2 infection.

What is in the Neuromuscular Junction Literature?

ABSTRACT: This update covers a number of treatment topics starting with Fc receptor inhibitors and the Federal Drug Administration approval of efgartigimod. Some uncertainties regarding the use of corticosteroids are addressed, namely the risk of exacerbation with initiation of treatment and how to taper. The presence and potential importance of antibody overshoot following plasmapheresis is noted and the evolving increase in usefulness of acetylcholine receptor antibodies in diagnosing ocular myasthenia. Several recent series and case reports regarding coronavirus 2019 and myasthenia gravis are reviewed. The topics of myasthenia gravis and pregnancy, and another look at thymectomy in MG are provided. Finally, a couple of case reports on Lambert-Eaton myasthenic syndrome concentrate on the ice pack test and an autoantibody association with paraneoplastic cerebellar degeneration and Lambert-Eaton myasthenic syndrome in the same patient.

Early-Onset Myasthenia Gravis Following COVID-19 Vaccination.

As coronavirus disease 2019 (COVID-19) has spread worldwide, the rate of COVID-19 vaccination uptake is encouraging. Neurological complications associated with COVID-19 vaccines such as stroke, Guillain-Barré syndrome, and Bell's palsy have been reported. Recently, late-onset myasthenia gravis (MG) following COVID-19 vaccination has been reported. To date, however, there has been no evidence of increased risk of early-onset MG following COVID-19. Here, we report a case of a patient with new-onset MG that arose after receiving a COVID-19 vaccine. A 33-year-old woman suddenly experienced generalized weakness and diplopia on the evening she had received the second dose of the Pfizer-BioNTech COVID-19 vaccine. The temporal relationship suggests that this new-onset MG is related to the vaccination. It also implies that COVID-19 vaccination could trigger early-onset MG symptoms in patients at risk of MG.

Development and validation of the Myasthenia Gravis TeleScore (MGTS).

OBJECTIVE: The aim of our study was to validate the Myasthenia Gravis TeleScore (MGTS), a scale for the evaluation of MG patients in telemedicine. INTRODUCTION: COVID-19 pandemic has boosted telemedicine in clinical practice. It could be crucial in the care of neurological patients with chronic disease. However, there is a lack of validated disease-specific tools to evaluate MG patients in telemedicine. METHODS: The MGTS included ten items divided in four districts: ocular, generalized muscular strength, bulbar, and respiratory. Patients were assessed with two different scales: the MGTS and the INCB-MG chosen as a reference from which MGTS was partially derived. Visit in presence with INCB-MG and televisit with MGTS were performed consecutively. Televisit was conducted by another neurologist between two rooms. A blind method was adopted. The strength of correlation was determined by the correlation coefficient (r); analysis of covariance (ANOVA-Kruskal-Wallis test) was used to compare subgroups. Significance was set to p < 0.05. RESULTS: One hundred thirty-one patients were included in the study, 71 females and 60 males. The Spearman correlation coefficient between the INCB-MG scale and the MGTS was 0.825 (p < 0.001), indicating a very strong correlation between them. Different items showed different correlations from low to high (0.32 to 0.80). As expected, correlation was lower between items with different evaluation modality (anamnestic vs clinical). DISCUSSION: The MGTS demonstrated a good correlation with INCB-MG, reliability and construct validity.

A Case of COVID-19 Vaccine Associated New Diagnosis Myasthenia Gravis.

An 82-year-old man presented with intermittent episodes of slurred speech during his evening meals after receiving the BNT162b2 COVID-19 vaccine. Thorough evaluation was conducted including lab work and EMG confirming a new diagnosis of late-onset myasthenia gravis. Despite treatment, the patient progressed rapidly to severe exacerbation requiring intubation and placement of a PEG tube. Infections provoking new diagnosis and exacerbations of myasthenia gravis have been reported. New diagnosis of myasthenia gravis associated with the COVID-19 vaccine is rarely reported. This case highlights the need for clinicians to be aware of the uncommon presenting symptoms in late-onset myasthenia gravis and the possibility of vaccine provoked diagnoses of immune mediated diseases.

Predictors of outcome in patients with myasthenic crisis undergoing non-invasive mechanical ventilation: A retrospective 20 year longitudinal cohort study from a single Italian center.

About 20% of patients with myasthenia gravis (MG) may develop myasthenic crisis (MC) requiring ventilation, either invasive (MV) or non-invasive (NIV) and intensive unit care (ICU). NIV failure in patients with MC can occur up to 60% of cases admitted to ICU. Moreover it is not known the outcome of MC receiving NIV. Purpose of this study was to identify predictors of outcome in MC who underwent non-invasive ventilator support outside ICU setting. We enrolled 90 patients, 53 females and 37 males admitted to University Hospital of Modena (Italy) between January 2000 and September 2020. Median age at MC was 65 years. Thirty-four patients (37.8%) required MV. Thymectomy was performed in 45 cases, associated with thymoma in 55%, with hyperplastic thymus in 33%. First-line treatment was plasmaexchange (38.8%) or intravenous immunoglobulins (45.6%). Males exhibited higher risk of MV than females .Patients in MV were treated with plasmaexchange as first-line therapy . Our in-hospital mortality rate was low. Nine patients underwent tracheostomy which was significantly related to male gender. Comorbidities had significant effect on length of ICU .Our study confirms as predictors of prognosis in our patients male gender, older age at onset, infections as trigger, pneumonia.

Myasthenia gravis and covid-19 in pregnancy: a review of the literature and case series report.

OBJECTIVES: The aim of our study was to investigate the interrelations of symptoms, clinical outcomes and treatment regimens in pregnant women, diagnosed with myasthenia gravis and superimposed COVID-19 infection. METHODS: We conducted an observational retrospective study between August, 2020 and July, 2021. Five patients with preexisting MG and superimposed COVID- infection were included in our study. We investigated the duration of MG, the antibody patient status, any present comorbidities, MG baseline treatment and MG severity class prior to the COVID-19 infection, MG severity class and treatment during the COVID-infection, and last but not least, the maternal and fetal clinical outcome. RESULTS: None of the participants were hospitalized as they were treated under quarantine at their homes. The most frequently reported complaints were anosmia, headache and fever, which were observed in 3 out of 5 patients. The MG severity was evaluated twice - before and after the quarantine period. Progression to a more advanced stage was found in 2 of our 5 patients. Three of the patients did not require any changes in the prescribed baseline MG treatment. In 2 patients the pyridostigmine dosage had to be increased. One patient received azithromycin and 4 patients were given LMWH (nadroparin) as specific anti-COVID measures. All patients fully recovered and gave birth to healthy newborns. CONCLUSION: To our knowledge, this is the first study on pregnant MG patients with superimposed COVID-19 infection. Based on our observations in this study it would seem that the coexistence of MG and COVID-19 infection in pregnancy does not elicit exacerbation in neither of those conditions. Further research is needed to confirm or challenge these findings, especially with the prospects of new virus variants emerging in the future.

Myasthenia gravis and telemedicine: a lesson from COVID-19 pandemic.

COVID-19 pandemic has induced an urgent reorganization of the healthcare system to ensure continuity of care for patients affected by chronic neurological diseases including myasthenia gravis (MG). Due to the fluctuating nature of the disease, early detection of disease worsening, adverse events, and possibly life-threatening complications is mandatory. This work analyzes the main unresolved issues in the management of the myasthenic patient, the possibilities offered so far by digital technologies, and proposes an online evaluation protocol based on 4 simple tests to improve MG management. Telemedicine and Digital Technology might help neurologists in the clinical decision-making process of MG management, avoiding unnecessary in presence consultations and allowing a rational use of the time and space reduced by the pandemic.

Transient acetylcholine receptor-related myasthenia gravis, post multisystem inflammatory syndrome in children (MIS-C) temporally associated with COVID-19 infection.

We report on a unique case of a 7-year-old girl with new onset ocular myasthenia gravis shortly after recovery from multisystem inflammatory syndrome in children (MIS-C) temporally associated with SARS-CoV-2 infection. The diagnosis of myasthenia gravis was based on suggestive symptoms of fatigable bilateral orbital ptosis, diplopia, positive ocular cold compression test and serum acetylcholine receptor antibody positivity, as well as a favourable treatment response to pyridostigmine. The addition of corticosteroids and methotrexate resulted in complete resolution of the ocular signs.

Temporal association between SARS-CoV-2 and new-onset myasthenia gravis: is it causal or coincidental?

Several case reports of COVID-19 in patients with myasthenia gravis (MG) have been documented. However, new-onset autoimmune MG following COVID-19 has been reported very rarely. We report one such case here. A 65-year-old man presented to us with dysphagia 6 weeks following mild COVID-19. He was evaluated and diagnosed as antiacetylcholine receptor antibody (AchR) positive, non-thymomatous, generalised MG. He subsequently developed myasthenic crisis and improved after treatment with intravenous immunoglobulin, prednisolone and pyridostigmine. Systematic literature review showed eight more similar cases. Analysis of all cases including the one reported here showed these features: mean age 55.8 years, male gender (5), time interval between COVID-19 and MG (5-56 days), generalised (5), bulbar and/or ocular symptoms (4), anti-AchR antibodies (7) and antimuscle-specific kinase antibodies (2). All have improved with immunotherapy. Although, many hypothesis are proposed to explain causal relationship between the two, it could as well be sheer coincidence.

COVID-19 in patients with myasthenia gravis: Epidemiology and disease course.

INTRODUCTION/AIMS: Coronavirus disease 2019 (COVID-19), a disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, has become a global pandemic. Patients with myasthenia gravis (MG), often treated with immunosuppressants, might be at higher risk of developing COVID-19 and of demonstrating a severe disease course. We aimed to study prevalence and describe features of COVID-19 in MG patients. METHODS: In May 2020, we conducted telephonic interviews with MG patients followed at our referral center. We collected structured data regarding MG and COVID-19, which was diagnosed as probable or confirmed according to the European Centre for Disease Prevention and Control case definition. We compared confirmed-COVID-19 prevalence calculated from the beginning of the pandemic in MG patients with that of the overall Pavia district. RESULTS: We interviewed 162 MG patients (median age, 66 y; interquartile range 41-77; males 59.9%), 88 from the Pavia district. Three patients had SARS-CoV-2-confirmed by polymerase chain reaction and eight had probable-COVID-19. In the Pavia district, the prevalence of confirmed-COVID-19 among MG patients (1/88, 1.14%) and overall population (4777/546 515, 0.87%) did not differ (P = .538). Higher Myasthenia Gravis Foundation of America clinicalclass and the need for recent rescue treatment, but not ongoing immunosuppressive treatments, were associated with COVID-19 risk. Of 11 MG patients with probable/confirmed-COVID-19, 3 required ventilator support, and 2 elderly patients died of COVID-19 respiratory insufficiency. Only 1 of11 patients experienced worsening MG symptoms, which improved after increasing their steroid dose. DISCUSSION: The risk of COVID-19 in MG patients seems to be no higher than that of the general population, regardless of immunosuppressive therapies. In our cohort, COVID-19 barely affected MG course.

Telemedicine visits in myasthenia gravis: Expert guidance and the Myasthenia Gravis Core Exam (MG-CE).

INTRODUCTION/AIMS: Telemedicine may be particularly well-suited for myasthenia gravis (MG) due to the disorder's need for specialized care, its hallmark fluctuating muscle weakness, and the potential for increased risk of virus exposure among patients with MG during the coronavirus disease 2019 (COVID-19) pandemic during in-person clinical visits. A disease-specific telemedicine physical examination to reflect myasthenic weakness does not currently exist. METHODS: This paper outlines step-by-step guidance on the fundamentals of a telemedicine assessment for MG. The Myasthenia Gravis Core Exam (MG-CE) is introduced as a MG-specific, telemedicine, physical examination, which contains eight components (ptosis, diplopia, facial strength, bulbar strength, dysarthria, single breath count, arm strength, and sit to stand) and takes approximately 10 minutes to complete. RESULTS: Pre-visit preparation, remote ascertainment of patient-reported outcome scales and visit documentation are also addressed. DISCUSSION: Additional knowledge gaps in telemedicine specific to MG care are identified for future investigation.

Telephone consultation for myasthenia gravis care during the COVID-19 pandemic: Assessment of a novel virtual myasthenia gravis index.

INTRODUCTION/AIMS: The aim of the study was to determine the association between the virtual Myasthenia Gravis Impairment Index (vMGII) with other patient-reported outcomes (PROs) of myasthenia gravis (MG) and its usefulness in telephone consultations with MG patients. METHODS: This was a retrospective case series in which vMGII score along with virtual Single Simple Question (vSSQ), virtual Patient-Acceptable Symptom State PASS (vPASS) response, and patient disease status based on Myathenia Gravis Foundation of America postintervention status were collected during telephone consultation along with the MGII, SSQ, and PASS responses during the preceding in-person clinic visits. RESULTS: In 214 patients, the mean difference of vMGII between the vPASS "Yes" and "No" groups was -14.2 ± 1.4 (95% confidence interval, -16.9 to -11.3; P < .001) with mean vMGII for vPASS "Yes" group being 6.4 ± 7.7 and vPASS "No" being 20.5 ± 11.5. A vMGII of 11.5 or higher predicted vPASS "yes" response with a sensitivity of 78.7% and specificity of 81.4%. A strong negative correlation was found between the vMGII and vSSQ (r = -.667; P < .001). The mean vMGII was 0.48 ± 1.42 for patients in remission, and 9.31 ± 10.93 for improved, 9.32 ± 8.79 for stable, and 22.58 ± 14.04 for worsened groups (P < .001). These associations were the same as those obtained during the preceding in-person clinic visit and the direction of change in MGII scores also indicated change in disease status. DISCUSSION: vMGII is an effective measure to assess an MG patient's disease status in telephone consultations and relates well with other PRO measures. The vMGII remains reliable for assessing MG disease status even with removal of the physical examination component.