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BMC Neurology Feb 2024Myasthenia gravis (MG) is a rare autoimmune disease characterised by muscle weakness, and progression from ocular (oMG) to generalised (gMG) symptoms results in a...
BACKGROUND
Myasthenia gravis (MG) is a rare autoimmune disease characterised by muscle weakness, and progression from ocular (oMG) to generalised (gMG) symptoms results in a substantial negative impact on quality of life (QoL). This systematic review aimed to provide an overview of the patient burden experienced by people living with gMG.
METHODS
Electronic database searches (conducted March 2022), supplemented by interrogation of grey literature, were conducted to identify studies reporting patient burden outcomes in patients with gMG in Europe, the Middle East and Africa. Results were synthesised narratively due to the heterogeneity across trials.
RESULTS
In total, 39 patient burden publications (representing 38 unique studies) were identified as relevant for inclusion in the systematic review, consisting of 37 publications reporting formal patient-reported outcome measures (PROMs), and two publications describing alternative qualitative assessments of patient experience. The studies included a variety of measures including generic and disease-specific PROMs, as well as symptom-specific PROMs focusing on key comorbidities including depression, anxiety, fatigue and sleep disturbance. The findings showed some variation across studies and PROMs; however, in general there was evidence for worse QoL in patients with gMG than in healthy controls or in patients with oMG, and a trend for worsening QoL with increasing MG severity.
CONCLUSIONS
This review highlights the importance of considering patient QoL when developing and assessing treatment and management plans for patients with gMG. However, the heterogeneity identified across studies illustrates the need for further representative and well-powered studies in large cohorts administering consistent, validated questionnaires.
TRIAL REGISTRATION
The protocol for this systematic review was registered in PROSPERO: CRD42022328444.
Topics: Humans; Quality of Life; Myasthenia Gravis; Africa; Middle East; Europe
PubMed: 38336636
DOI: 10.1186/s12883-024-03553-y -
Journal of Clinical Neuroscience :... Mar 2024Myasthenia gravis (MG) is an autoimmune neuromuscular disorder characterized by muscle weakness, posing significant challenges to patients' daily lives. Intravenous... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Myasthenia gravis (MG) is an autoimmune neuromuscular disorder characterized by muscle weakness, posing significant challenges to patients' daily lives. Intravenous immunoglobulin (IVIG) and plasmapheresis are two prominent immunomodulatory therapies used in MG management, but the choice between them remains a clinical dilemma. This systematic review and meta-analysis aim to evaluate the comparative efficacy of IVIG versus plasmapheresis in MG management.
METHODS
We adhered to PRISMA guidelines and prospectively registered the review protocol in PROSPERO. Systematic search across electronic databases identified 14 studies meeting inclusion criteria. Data from these studies were extracted, and assessed risk of bias. Primary outcomes included clinical efficacy, while secondary outcomes encompassed hospitalization, ventilation, antibody titers, and treatment-related complications. Statistical analysis was conducted using R software.
RESULTS
The pooled results indicated that patients receiving plasmapheresis had higher odds of any improvement in MG symptoms compared to IVIG. However, change in severity scores did not significantly differ between the two treatments. Hospitalization durations were similar, but IVIG-treated patients tended to have shorter stays. Antibody titers, particularly anti-MUSK antibodies, favored plasmapheresis treatment. Complication rates were comparable between two groups. However, severe complications were more common in plasmapheresis.
CONCLUSION
This comprehensive analysis suggests that plasmapheresis may offer superior short-term symptom improvement in MG compared to IVIG, while IVIG may lead to shorter hospital stays and lower complication rates. The choice between these treatments should be tailored to individual patient needs and disease characteristics. Further research is needed to explore long-term outcomes and mortality rates in MG management.
Topics: Humans; Immunoglobulins, Intravenous; Plasmapheresis; Myasthenia Gravis; Treatment Outcome; Length of Stay
PubMed: 38306763
DOI: 10.1016/j.jocn.2024.01.025 -
CNS Drugs Feb 2024The treatment of generalized myasthenia gravis (gMG) has been transformed by the development and approval of new targeted therapies. This analysis aimed to rank and... (Meta-Analysis)
Meta-Analysis
BACKGROUND
The treatment of generalized myasthenia gravis (gMG) has been transformed by the development and approval of new targeted therapies. This analysis aimed to rank and compare the new therapies for gMG using efficacy and safety data from randomized controlled trials (RCTs).
METHODS
We searched PubMed, Embase, the Cochrane Central Register of Controlled Trials (CENTRAL), and ClinicalTrials.gov (up to November 2022) for RCTs of targeted drugs for gMG. We used a Bayesian random-effects network meta-analysis (NMA) model and a Markov chain Monte Carlo (MCMC) model for statistical analysis. The primary outcome was the change in quantitative myasthenia gravis score (QMGS) from baseline, while the secondary outcome was the risk ratio (RR) of adverse events (AEs) during treatment. The surface under the cumulative ranking curve (SUCRA) was used to rank these targeted drugs, with higher SUCRA values indicating better efficacy or lower likelihood of AEs.
RESULTS
In total, 13 studies (872 subjects) were included in this analysis evaluating 10 targeted drugs (batoclimab, belimumab, CFZ533, eculizumab, efgartigimod, nipocalimab, rituximab, ravulizumab, rozanolixizumab, and zilucoplan). With regards to the primary outcome, batoclimab [standardized mean difference (SMD), - 1.61; 95% credible interval (CrI), - 2.78, - 0.43] significantly reduced QMGS in patients with gMG when compared with placebo and was ranked as the most efficacious drug. Ranked second and third were eculizumab (SMD, - 0.67; 95% CrI, 1.43, 0.01) and zilucoplan (SMD, - 0.54; 95% CrI, - 1.56, 0.46), respectively. Nipoclimab (SMD, - 0.02; 95% CrI, - 1.04, 1.00) had the worst efficacy and ranked last among all targeted drugs. In our study, except for batoclimab, there was no statistically significant difference in the reduction of patient QMGS for the remaining targeted agents compared with placebo. With regards to the secondary outcomes, only batoclimab (RR, 0.19; 95% CrI, 0, 0.97) led to a significant reduction in the incidence of AEs when compared with the placebo. Belimumab (RR, 0.85; 95% CrI, 0.57, 1.19), CFZ533 (RR, 0.95; 95% CrI, 0.72, 1.25), eculizumab (RR, 0.99; 95% CrI, 0.85, 1.21), and efgartigimod (RR, 0.93; 95% CrI, 0.76, 1.15) also led to a lower incidence of AEs, although these effects were not significantly different from the placebo.
CONCLUSIONS
Batoclimab had the best efficacy and safety for the treatment of gMG and was ranked first out of the 10 targeted drugs included in this study. Eculizumab was ranked second, and nipocalimab had the worst efficacy. With the exception of batoclimab, the incidence of AEs for the remaining drugs was not statistically significantly different from placebo. We note, however, that wide CrIs reflect the uncertainty in this analysis owing to the small number of available studies and low numbers of study participants; moreover, batoclimab had the widest CrI of all drugs in this analysis. More well-designed studies with long-term follow-up are needed to further evaluate and compare the efficacy and safety of these drugs in the future.
Topics: Humans; Network Meta-Analysis; Drug Delivery Systems; Myasthenia Gravis
PubMed: 38300476
DOI: 10.1007/s40263-024-01062-7 -
Cureus Dec 2023Myasthenia gravis (MG), a rare disease, is the most common neuromuscular junction problem. It's the quintessential autoimmune disease with ocular, bulbar, respiratory,... (Review)
Review
Myasthenia gravis (MG), a rare disease, is the most common neuromuscular junction problem. It's the quintessential autoimmune disease with ocular, bulbar, respiratory, axial, and limb muscles exhibiting a typical fatigable weakening due to the development of antibodies against the acetylcholine receptor (AChR). Infections, stress, surgeries, thymus gland anomalies, and pharmaceutical side effects can also cause it. Ocular symptoms are initially experienced by most of the sufferers. The majority of the sufferers will go through at least one episode of symptom exacerbation during their illness. The immune system in MG interferes with nerve-muscle communication, causing muscles to become weak and tired quickly. The actual cause is not yet known, but a problem in the thymus gland may be the cause. In a person suffering from this disease, the size of the thymus becomes larger than normal, which is also called thymic hyperplasia. It is more common for women to have early-onset MG (EOMG) than for males to have late-onset MG (LOMG). Merely clinical evidence, encompassing the patients' medical history and physical indications of fluctuating muscle weakness in a specific region, is utilized to diagnose MG. Complementary diagnostic procedures and lab techniques aid in confirming the synaptic dysfunction and characterizing its kind and degree. Early diagnosis and the availability of effective treatments have reduced the burden of severe impairment and high mortality previously associated with MG. Current immunomodulation-based therapies come with side effects brought on by persistent immune suppression. Improved knowledge of this relatively uncommon but curable condition is required among primary carers. The objective of this review is to provide information about MG and to help people recognize its symptoms and start treatment without panic so that the progression of this disease can be stopped and complications can be avoided.
PubMed: 38186498
DOI: 10.7759/cureus.50017 -
PloS One 2024Randomized controlled trials (RCTs) of acupuncture for myasthenia gravis (MG) were searched and the efficacy of acupuncture in the treatment of MG was evaluated by... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Randomized controlled trials (RCTs) of acupuncture for myasthenia gravis (MG) were searched and the efficacy of acupuncture in the treatment of MG was evaluated by meta-analysis.
METHODS
We searched for RCTs in six main electronic databases, and collected RCTs of acupuncture treatment for MG from database creation to 28 February 2023. The main outcome was the effective rate and the secondary outcome was the Traditional Chinese Medicine (TCM) relative clinical score, absolute clinical score (ACS) of MG, Quantitive myasthenia gravis score (QMG), quality of life, and adverse events. Odds ratios (ORs) and weighted mean differences (WMD) and 95% confidence intervals (CI) were used to assess pooled effect estimates using Review Manager software.
RESULTS
A total of 14 RCTs were included. Meta-analysis showed that the effective rate in the acupuncture group was significantly improved compared with conventional Western medicine alone [OR = 4.28, 95% CI (2.95, 6, 22), P<0.005]. The pooled WMDs revealed that TCM relative clinical score [WMD = -2.22, 95% CI = (-2.53, -1.90), P<0.005], ACS of MG [WMD = -3.14, 95% CI = (-3.67, -2.62), P<0.005], and QMG [WMD = -0.88, 95% CI = (-1.46, -0.29), P<0.005] in the acupuncture group was lower than the control group. Adverse reactions related to acupuncture and quality of life were less mentioned among included RCTs.
CONCLUSION
This meta-analysis demonstrated that acupuncture as an auxiliary may play a positive role in treating MG. It can improve the effective rate of treatment, and reduce TCM relative clinical score, ACS of MG, and QMG. However, the quality of included studies was generally low and caution should be exercised when considering this treatment option. In the future, more rigorous study designs and high-quality RCTs are needed to verify the efficacy of acupuncture in the treatment of MG, because the results of high-quality RCTs are more reliable and accurate.
Topics: Humans; Acupuncture Therapy; Medicine, Chinese Traditional; Myasthenia Gravis; Research Design; Quality of Life
PubMed: 38165870
DOI: 10.1371/journal.pone.0291685 -
Biomedicines Nov 2023Currently used rescue interventions to prevent rapid myasthenic deterioration are plasma exchange (PLEX) and intravenous immunoglobulin (IVIG). We investigated the... (Review)
Review
Currently used rescue interventions to prevent rapid myasthenic deterioration are plasma exchange (PLEX) and intravenous immunoglobulin (IVIG). We investigated the evidence to determine whether the two methods were interchangeable or whether one was superior to the other. This review was registered on PROSPERO (CRD42021285985). Only randomized controlled trials (RCTs) comparing the efficacy and safety of PLEX and IVIG in patients with moderate-to-severe myasthenia gravis (MG) were included. Five major databases were systematically searched (PubMed, CENTRAL, Embase, Scopus, and Web of Science). Odds ratios (OR) with 95% confidence intervals (CI) were calculated for adverse events and mean differences (MD) for changes in quantitative myasthenia gravis scores (QMG). Three RCTs met the inclusion criteria. Two investigating 114 patients in total were eligible for meta-analysis to analyze efficacy and safety. For the change in QMG score, the MD was -2.8 (95% CI: -5.614-0.113), with PLEX performing better. For adverse events, an OR of 1.04 was found (95% CI: 0.25-4.27). This study demonstrated a low risk of bias in evaluating treatment efficacy but indicated a high risk of bias in assessing procedural safety outcomes. Although the results did not show any significant difference, there was a tendency indicating faster efficacy of PLEX in the first two weeks of treatment. In such a critical clinical condition, this tendency may be clinically meaningful, but further studies should clarify this benefit.
PubMed: 38137401
DOI: 10.3390/biomedicines11123180 -
European Radiology Jul 2024It is uncertain whether modern iodine-based or gadolinium-based contrast media (CM) administration can lead to increased symptoms in patients with myasthenia gravis. (Review)
Review
OBJECTIVES
It is uncertain whether modern iodine-based or gadolinium-based contrast media (CM) administration can lead to increased symptoms in patients with myasthenia gravis.
METHODS
A systematic search in Medline was conducted for studies describing the symptomatology of myasthenia gravis patients before and after receiving intravenous (IV) CM and having a matched control group of myasthenia gravis patients who did not receive IV CM.
RESULTS
Three retrospective studies were selected with a total of 374 myasthenia gravis patients who received iodine-based CM and a total of 313 myasthenia gravis patients who underwent unenhanced CT and served as controls. Pooling of the data from the three retrospective studies showed that in 23 of 374 patients, increased symptoms after iodine-based CM administration were described (6.1%). Increased symptomatology also occurred in 11 of 313 patients after unenhanced CT (3.5%). When looking more deeply into the data of the three studies, conflicting results were found, as two articles did not find any relationship between CM and myasthenia gravis symptoms. The remaining study only found a significant increase in symptomatology within 1 day after CT scanning: seven patients (6.3%) in the contrast-enhanced CT group and one patient (0.6%) in the unenhanced CT group (p = 0.01).
CONCLUSIONS
There is limited evidence on the relationship between CM and myasthenia gravis symptoms. In the vast majority of myasthenia gravis patients, CM are safe. Probably, in less than 5% of the patients, iodine-based CM administration may lead to increased severity of the symptoms within the first 24 h after administration.
CLINICAL RELEVANCE STATEMENT
Be aware that intravenous administration of iodine-based contrast media can lead to an increase of symptoms in patients with myasthenia gravis within the first 24 h. This can probably happen in less than 5% of the patients.
KEY POINTS
• It is unclear whether modern contrast media can lead to increased symptoms in myasthenia gravis patients after intravenous administration. • There seems to be a small risk of increased myasthenia gravis symptoms within 24 h after intravenous administration of iodine-based contrast media, probably in less than 5% of the administrations. • Gadolinium-based contrast media are safe for patients with myasthenia gravis.
Topics: Myasthenia Gravis; Humans; Contrast Media; Tomography, X-Ray Computed; Gadolinium; Practice Guidelines as Topic; Europe; Iodine
PubMed: 38092951
DOI: 10.1007/s00330-023-10463-z -
BMC Neurology Dec 2023Neuromuscular diseases (NMD) emerged as one of the main side effects of the COVID-19 vaccination. We pooled and summarized the evidence on the clinical features and...
BACKGROUND
Neuromuscular diseases (NMD) emerged as one of the main side effects of the COVID-19 vaccination. We pooled and summarized the evidence on the clinical features and outcomes of NMD associated with COVID-19 vaccination.
METHODS
We comprehensively searched three databases, Medline, Embase, and Scopus, using the key terms covering "Neuromuscular disease" AND "COVID-19 vaccine", and pooled the individual patient data extracted from the included studies.
RESULTS
A total of 258 NMD cases following COVID-19 have been reported globally, of which 171 cases were Guillain-Barré syndrome (GBS), 40 Parsonage-Turner syndrome (PTS), 22 Myasthenia Gravis (MG), 19 facial nerve palsy (FNP), 5 single fiber neuropathy, and 1 Tolosa-Hunt syndrome. All (100%) SFN patients and 58% of FNP patients were female; in the remaining NMDs, patients were predominantly male, including MG (82%), GBS (63%), and PTS (62.5%). The median time from vaccine to symptom was less than 2 weeks in all groups. Symptoms mainly appeared following the first dose of vector vaccine, but there was no specific pattern for mRNA-based.
CONCLUSION
COVID-19 vaccines might induce some NMDs, mainly in adults. The age distribution and gender characteristics of affected patients may differ based on the NMD type. About two-thirds of the cases probably occur less than 2 weeks after vaccination.
Topics: Adult; Humans; Female; Male; COVID-19 Vaccines; COVID-19; Neuromuscular Diseases; Myasthenia Gravis; Guillain-Barre Syndrome; Bell Palsy; Facial Paralysis
PubMed: 38082244
DOI: 10.1186/s12883-023-03486-y -
Le Infezioni in Medicina 2023Listeriosis is a known cause of community acquired bacterial meningitis/meningoencephalitis. It occurs more frequently in neonates, older people and immunocompromised...
BACKGROUND
Listeriosis is a known cause of community acquired bacterial meningitis/meningoencephalitis. It occurs more frequently in neonates, older people and immunocompromised hosts. Rarely, brain abscesses can complicate the course of infection, which poses a difficulty in the management and elevates morbidity and mortality. Myasthenia gravis is an autoimmune disease that often requires immunosuppressive treatment, which increases the risk for invasive infections.
CASE DESCRIPTION
A 75-year-old myasthenic patient, treated with prednisone and pyridostigmine, was diagnosed with invasive listeriosis. He presented with muscle weakness, dyspnea, hemiparesis and altered mental status. Brain imaging revealed multiple abscesses and blood cultures were positive for . Despite combination antibiotic therapy, he died 6 weeks after admission.
SYSTEMATIC REVIEW
Ninety-six cases of brain abscesses from 1968 to 2023 were reviewed; the majority of the patients were men, 54 years-old on average, and had at least one risk factor for invasive listeriosis. The mortality exceeded 22%. Blood cultures and CSF/abscess cultures were positive in only 79.2% and 54.6%, respectively. The most common therapeutic approach was a combination regimen that included amoxicillin or ampicillin. Only 8 patients underwent surgery, of which one died.
CONCLUSION
This case highlights the importance of as a cause of brain abscesses in populations at risk, including myasthenic patients. The challenge of diagnosing and treating this condition is aggravated by the paucity of literature and the high mortality rate.
PubMed: 38075415
DOI: 10.53854/liim-3104-16 -
The British and Irish Orthoptic Journal 2023The conversion of ocular myasthenia gravis (OMG) to generalised myasthenia gravis (GMG) is reported to differ depending on the presence of generalisation risk factors...
PURPOSE
The conversion of ocular myasthenia gravis (OMG) to generalised myasthenia gravis (GMG) is reported to differ depending on the presence of generalisation risk factors (Mazzoli et al. 2018). Thymic pathology has been recognised as a potential risk factor for generalisation in the literature (Teo et al. 2017). Thymoma and thymic hyperplasia have yet to be examined as a risk factor for generalisation of OMG independently of other risk factors in the literature. Thus, the purpose of this review is to examine the literature to identify whether thymoma and thymic hyperplasia do increase the risk of OMG progressing to GMG.
METHODS
A literature search was carried out which employed a systematic approach. The search was undertaken using the following academic libraries: MEDLINE, Embase and Starplus. The search was limited to publications between the years 2001 to 2021. The search yielded 82 studies, which after the screening of titles and abstracts, left 62 studies for further analysis against the inclusion and exclusion criteria.
RESULTS
The review found thymoma to be associated with an increased risk of GMG development. However, there was a scarce amount of literature which investigated thymic hyperplasia. Therefore, a firm conclusion could not be made with regards to thymic hyperplasia and the risk of GMG development.
CONCLUSIONS
This review provides evidence for the consideration of thymectomy early after thymomatous OMG diagnosis to prevent GMG conversion. As the review did not collect enough evidence to support the influence of thymic hyperplasia on OMG conversion, further research is required.
PubMed: 38046270
DOI: 10.22599/bioj.315