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1.
Muscle Nerve ; 2022 Jun 08.
Article in English | MEDLINE | ID: covidwho-1877659

ABSTRACT

INTRODUCTION/AIM: Given the lack of information on safety of COVID-19 vaccination in myasthenia gravis (MG) patients, we aimed to review our experience after surveying patients, as part of routine clinical practise, to ensure that advice on safety is accurate. METHODS: We performed a retrospective chart review of MG patients from the Prosserman Family Neuromuscular Clinic at the Toronto General Hospital who received two injections of any COVID-19 vaccine from February to August 2021. Demographic data were abstracted from the patient medical records. We assessed changes in the severity of MG using the virtual myasthenia gravis impairment index (vMGII), the simple single question (SSQ) and patient acceptable symptom state (PASS). We also assessed adverse effects after vaccination. RESULTS: We included 200 patients with a mean age of 64.3 ± 13.9 years, 51.5% were men, and 82% had generalized MG. The vMGII, SSQ and PASS scores remained stable after each vaccine dose, and at last follow-up. 60% of the patients reported an adverse reaction after the first injection, and 56% after the second. The most common adverse reactions reported were local pain at the injection site, fatigue, headache, and fever. DISCUSSION: COVID-19 vaccinations were well tolerated in MG patients and were not associated with worsening severity of their MG. The prevalence of vaccine-related adverse reactions was the same as in the general population. This article is protected by copyright. All rights reserved.

2.
Lancet Neurol ; 21(2): 189-202, 2022 02.
Article in English | MEDLINE | ID: covidwho-1625864

ABSTRACT

Myasthenia gravis and Lambert-Eaton myasthenic syndrome are antibody-mediated autoimmune diseases of the neuromuscular junction that usually present with weakness in ocular muscles and in proximal muscles of the limb and trunk. Prognosis regarding muscle strength, functional abilities, quality of life, and survival is generally good. However, some patients do not respond to treatment. Symptomatic drugs, corticosteroids, and steroid-sparing immunosuppressive drugs remain the cornerstone of treatment. In the past few years, new biological agents against complement, the FcRn receptor, or B-cell antigens have been tested in clinical trials. These new therapies extend the possibilities for targeted immunotherapies and promise exciting new options with a relatively rapid mode of action. Challenges in their use might occur, with barriers due to an increase in cost of care and additional considerations in the choice of drugs, and potential consequences of infection and vaccination due to the COVID-19 pandemic.


Subject(s)
Autoimmune Diseases , Neuromuscular Junction Diseases , Autoimmune Diseases/therapy , Humans , Lambert-Eaton Myasthenic Syndrome/immunology , Lambert-Eaton Myasthenic Syndrome/therapy , Myasthenia Gravis/immunology , Myasthenia Gravis/therapy , Neuromuscular Junction Diseases/immunology , Neuromuscular Junction Diseases/therapy
3.
Orphanet J Rare Dis ; 16(1): 204, 2021 05 06.
Article in English | MEDLINE | ID: covidwho-1219017

ABSTRACT

BACKGROUND: The global spread of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection causing the ongoing coronavirus disease 2019 (COVID-19) pandemic has raised serious concern for patients with chronic disease. A correlation has been identified between the severity of COVID-19 and a patient's preexisting comorbidities. Although COVID-19 primarily involves the respiratory system, dysfunction in multiple organ systems is common, particularly in the cardiovascular, gastrointestinal, immune, renal, and nervous systems. Patients with amyloid transthyretin (ATTR) amyloidosis represent a population particularly vulnerable to COVID-19 morbidity due to the multisystem nature of ATTR amyloidosis. MAIN BODY: ATTR amyloidosis is a clinically heterogeneous progressive disease, resulting from the accumulation of amyloid fibrils in various organs and tissues. Amyloid deposition causes multisystem clinical manifestations, including cardiomyopathy and polyneuropathy, along with gastrointestinal symptoms and renal dysfunction. Given the potential for exacerbation of organ dysfunction, physicians note possible unique challenges in the management of patients with ATTR amyloidosis who develop multiorgan complications from COVID-19. While the interplay between COVID-19 and ATTR amyloidosis is still being evaluated, physicians should consider that the heightened susceptibility of patients with ATTR amyloidosis to multiorgan complications might increase their risk for poor outcomes with COVID-19. CONCLUSION: Patients with ATTR amyloidosis are suspected to have a higher risk of morbidity and mortality due to age and underlying ATTR amyloidosis-related organ dysfunction. While further research is needed to characterize this risk and management implications, ATTR amyloidosis patients might require specialized management if they develop COVID-19. The risks of delaying diagnosis or interrupting treatment for patients with ATTR amyloidosis should be balanced with the risk of exposure in the health care setting. Both physicians and patients must adapt to a new construct for care during and possibly after the pandemic to ensure optimal health for patients with ATTR amyloidosis, minimizing treatment interruptions.


Subject(s)
Amyloid Neuropathies, Familial , COVID-19 , Amyloid , Humans , Pandemics , Prealbumin , SARS-CoV-2
4.
Muscle Nerve ; 63(6): 831-836, 2021 06.
Article in English | MEDLINE | ID: covidwho-1162898

ABSTRACT

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.


Subject(s)
COVID-19/epidemiology , Myasthenia Gravis/diagnosis , Myasthenia Gravis/therapy , Severity of Illness Index , Telemedicine/methods , Telephone , Aged , COVID-19/prevention & control , Female , Humans , Male , Middle Aged , Retrospective Studies
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