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Journal of the Neurological Sciences Jun 2022While the clinical manifestations of myasthenia gravis (MG) are well understood, its humanistic impact is not. The objective of this systematic literature review (SLR)... (Review)
Review
BACKGROUND/OBJECTIVES
While the clinical manifestations of myasthenia gravis (MG) are well understood, its humanistic impact is not. The objective of this systematic literature review (SLR) was to provide a comprehensive understanding of the humanistic burden of MG with regards to psychological symptoms and health-related quality of life (HRQoL) according to patients and caregivers.
METHODS
A systematic search was conducted on December 27, 2019, in MEDLINE and Embase to identify English-language studies that were published from January 1, 2009-December 27, 2019 and presented relevant information on the humanistic burden among adults with MG and their caregivers. Title/abstract and full-text screening was performed by two investigators, with any discrepancies resolved by a third investigator.
RESULTS
Sixty-seven publications were included in the SLR. Compared with the general population, patients with MG experienced worse HRQoL. Studies reporting on psychological symptoms of MG, including depression, anxiety, fatigue, and sleep, were heterogeneous in terms of the scales and instruments used to assess patients, as well as the patient populations themselves. However, in general those with more severe symptoms and hospitalization days had worse depression and anxiety, and fatigue and sleep improved with disease remission and/or improvement. Scores were worse for females compared with males and where evaluated, HRQoL scores generally improved following treatment.
CONCLUSION
While the literature demonstrates that symptoms associated with MG get better with disease improvement and remission, additional options in efficacious therapy that adequately address the disease-related symptoms and also improve HRQoL may contribute to beneficial outcomes in a greater number of patients with MG.
Topics: Adult; Anxiety; Caregivers; Fatigue; Female; Humans; Male; Myasthenia Gravis; Quality of Life
PubMed: 35486970
DOI: 10.1016/j.jns.2022.120268 -
Neurology Feb 2021Introduction and validation of a phenotypic classification of neurogenic dysphagia based on flexible endoscopic evaluation of swallowing (FEES).
OBJECTIVE
Introduction and validation of a phenotypic classification of neurogenic dysphagia based on flexible endoscopic evaluation of swallowing (FEES).
METHODS
A systematic literature review was conducted, searching MEDLINE from inception to May 2020 for FEES findings in neurologic diseases of interest. Based on a retrospective analysis of FEES videos in neurologic diseases and considering the results from the review, a classification of neurogenic dysphagia was developed distinguishing different phenotypes. The classification was validated using 1,012 randomly selected FEES videos of patients with various neurologic disorders. Chi-square tests were used to compare the distribution of dysphagia phenotypes between the underlying neurologic disorders.
RESULTS
A total of 159 articles were identified, of which 59 were included in the qualitative synthesis. Seven dysphagia phenotypes were identified: (1) "premature bolus spillage" and (2) "delayed swallowing reflex" occurred mainly in stroke, (3) "predominance of residue in the valleculae" was most common in Parkinson disease, (4) "predominance of residue in the piriform sinus" occurred only in myositis, motoneuron disease, and brainstem stroke, (5) "pharyngolaryngeal movement disorder" was found in atypical Parkinsonian syndromes and stroke, (6) "fatigable swallowing weakness" was common in myasthenia gravis, and (7) "complex disorder" with a heterogeneous dysphagia pattern was the leading mechanism in amyotrophic lateral sclerosis. The interrater reliability showed a strong agreement (kappa = 0.84).
CONCLUSION
Neurogenic dysphagia is not a symptom, but a multietiologic syndrome with different phenotypic patterns depending on the underlying disease. Dysphagia phenotypes can facilitate differential diagnosis in patients with dysphagia of unclear etiology.
Topics: Deglutition Disorders; Humans; Nervous System Diseases
PubMed: 33318164
DOI: 10.1212/WNL.0000000000011350 -
Journal of Neurology Nov 2017To evaluate the efficacy and safety of tacrolimus in patients with myasthenia gravis (MG), a systematic review and meta-analysis was performed. We searched PubMed and... (Meta-Analysis)
Meta-Analysis Review
To evaluate the efficacy and safety of tacrolimus in patients with myasthenia gravis (MG), a systematic review and meta-analysis was performed. We searched PubMed and Embase for randomized controlled trials and clinical controlled trials in English language. Demographic and clinical characteristics of the MG patients were extracted. Differences in the current glucocorticoids (GC) dose in each included study were the primary outcome measure. The adverse events reported in each included study were used as safety evaluation. There were 5 trials included involving 683 patients. In this systematic review, we identified treatment with tacrolimus did not exhibit a statistically significant difference in the GC dose reduction at 6 months and 12 months compared with placebo. The standard mean differences in the GC dose reduction were -1.95 [(-4.20 to 0.30); p = 0.09] at 6 months and -1.72 [(-4.21 to 0.77); p = 0.18] at 12 months. But GC dose reduction from baseline in the tacrolimus group exceeded that in the controlled group. The weighted mean differences were -1.34 [(-2.46 to 0.23); p = 0.02] in the quantitative myasthenia gravis score and -1.10 [(-1.84 to -0.36); p = 0.004] in the myasthenia gravis activities of daily living score at 6 months. Adverse events were recorded in 80 of 347 patients (23%) treated with tacrolimus and most of them were mild. This meta-analysis proves that tacrolimus therapy is beneficial to improve clinical symptoms in MG patients. Tacrolimus may be a worthy therapy to relieve MG symptoms.
Topics: Humans; Immunosuppressive Agents; Myasthenia Gravis; Tacrolimus
PubMed: 28921038
DOI: 10.1007/s00415-017-8616-7 -
Brain and Behavior Jan 2023Myasthenia gravis (MG) people experience adverse psychiatric outcomes, which may impact on their life and disturb their daily activity. Depression and anxiety are... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
Myasthenia gravis (MG) people experience adverse psychiatric outcomes, which may impact on their life and disturb their daily activity. Depression and anxiety are identified as significant psychiatric problems that MG people face. However, there is no sufficient epidemiological information about depression and anxiety-based publication. Due to this limitation, the aim of this study was to review the prevalence of depression and anxiety in MG patients.
METHODS
Original and international databases were searched to find papers about the estimation of anxiety and depression. Random-effects analysis was used for calculating the proportions of anxiety and depression. For estimating anxiety and depression based the severity, instruments, type of studies, and study regions, subgroup analysis was performed.
RESULTS
38 studies met inclusion criteria and entered study. The pooling of the prevalence of depression was found at 36%, (95% CI 28% to 45%). Also, prevalence of anxiety was found at 33%, (95% CI 25% to 42%). Prevalence of depression based on mild, moderate, and severe level was 27%, 14%, and 9%, respectively.
CONCLUSIONS
Anxiety and depression are a major concern among MG individuals. The estimation of both anxiety and depression are high even when compared to other autoimmune diseases. It seems depression and anxiety are important issues and more attention needs to be paid to these psychiatric disorders.
Topics: Humans; Depression; Prevalence; Anxiety; Anxiety Disorders; Myasthenia Gravis
PubMed: 36495116
DOI: 10.1002/brb3.2840 -
The Association Between Asthma and Risk of Myasthenia Gravis: A Systematic Review and Meta-analysis.Lung Jun 2021This study aimed to investigate the association between asthma and risk of myasthenia gravis (MG) using the method of systematic review and meta-analysis. (Meta-Analysis)
Meta-Analysis
PURPOSE
This study aimed to investigate the association between asthma and risk of myasthenia gravis (MG) using the method of systematic review and meta-analysis.
METHODS
Potentially eligible studies were identified from Medline and EMBASE databases from inception to July 2020 using search strategy that comprised terms for "Asthma" and "Myasthenia Gravis". Eligible cohort study must consist of one cohort of individuals with asthma and another cohort of individuals without asthma. Then, the study must report relative risk (RR) with 95% confidence intervals (95% CIs) of incident MG between the groups. Eligible case-control studies must include cases with MG and controls without MG. Then, the study must explore their history of asthma. Odds ratio (OR) with 95% CIs of the association between asthma status and MG must be reported. Point estimates with standard errors were retrieved from each study and were combined together using the generic inverse variance method.
RESULTS
A total of 6,835 articles were identified. After two rounds of independent review by five investigators, two cohort studies and three case-control studies met the eligibility criteria and were included into the meta-analysis. Pooled analysis showed that asthma was significantly associated with risk of MG with the pooled risk ratio of 1.38 (95% CI 1.02-1.86). Funnel plot was symmetric, which was not suggestive of publication bias.
CONCLUSION
The current study found a significant association between asthma and increased risk of MG.
Topics: Asthma; Global Health; Humans; Incidence; Myasthenia Gravis; Risk Assessment; Risk Factors
PubMed: 33987703
DOI: 10.1007/s00408-021-00444-8 -
Annals of Indian Academy of Neurology 2017This study was designed to determine whether treatments with tacrolimus would provide benefit for patients with myasthenia gravis (MG). (Review)
Review
AIMS
This study was designed to determine whether treatments with tacrolimus would provide benefit for patients with myasthenia gravis (MG).
MATERIALS AND METHODS
The databases of Medline, EMBASE, the Cochrane Library, and four Chinese databases were searched for eligible studies. Weighted mean differences and standardized mean differences (SMD) with corresponding 95% confidence intervals (CIs) were used to summarize the primary outcome, namely, steroid-sparing effect of tacrolimus in maintaining minimal manifestations, and the secondary outcome, namely, the effect of tacrolimus in reducing the severity of MG, respectively.
RESULTS
After systematic retrieval, 13 researches with two randomized controlled trials (RCTs) and 11 prospective open-label single-arm clinical trials were included in the study. For the primary outcome of two RCTs, one RCT which was followed up for 1 year showed a positive effect and the other RCT which was associated with treatment duration of 28 weeks showed a negative result. For the secondary outcome, meta-analyses of other 11 trials showed a benefit effect, overall. For the quantitative MG (QMG) score, there were significant differences with high heterogeneity (SMD: 2.93; 95% CI: 1.14-4.73; = 86%). In contrast, for MG activities of daily living (MGADL) score, it was reduced by tacrolimus with significant SMD and less heterogeneity (SMD: 0.59; 95% CI: 0.33-0.85; = 7%). Adverse effects were mentioned as mild.
DISCUSSION
The opposite results of two RCTs showed that tacrolimus with enough treatment duration might have positive steroid-sparing effect. The most possible cause of heterogeneity in the outcome of QMG score between trials was the baseline severity of MG.
CONCLUSION
The above finding suggests that there might be a potential beneficial role with no serious side effects of tacrolimus, and additional better RCTs including larger sample sizes and long-term study are needed to confirm or refute the results.
PubMed: 29184334
DOI: 10.4103/aian.AIAN_97_17 -
European Journal of Neurology Jul 2010The clinical course of myasthenia gravis (MG) is variable, and spontaneous remission is still uncommon. Knowledge of the prognostic factors may help understand the... (Review)
Review
The clinical course of myasthenia gravis (MG) is variable, and spontaneous remission is still uncommon. Knowledge of the prognostic factors may help understand the course of MG and thus optimize its management. A systematic review search was conducted in MEDLINE and EMBASE for English language studies from 1985 through 2009. We identified additional studies by reviewing bibliographies of retrieved articles and hand search main journal of neurology. Studies evaluating variables associated with or predictive of remission in adult patients with MG were included. Because of methodological heterogeneity, we refrained from statistical pooling, instead, a best evidence synthesis was used for summarizing the results. From 1810 potentially relevant studies, 13 cohort studies met the inclusion criteria. The included studies were heterogeneous considerably in sample size, disease duration, follow-up years, definition of remission, and analysis. Study quality was limited by retrospective design in most studies and lack of multivariate analysis. Time of diagnosis from onset (<1 year) showed strong evidence of predicting a better remission. In studies using completely stable remission outcomes, there was strong evidence that age at onset (<40 years) was of prognostic importance. Furthermore, gender showed no association with remission. Time of diagnosis from onset and age at onset were found to be predictors of remission. Gender does not seem to predict the course of MG. Our findings should be interpreted with caution because of the clinical and methodological heterogeneity of included studies.
Topics: Age of Onset; Disease Progression; Early Diagnosis; Humans; Myasthenia Gravis; Prognosis; Review Literature as Topic; Sex Distribution
PubMed: 20402761
DOI: 10.1111/j.1468-1331.2010.03017.x -
Medicine Oct 2023Myasthenia Gravis (MG), a chronic neuromuscular junction disorder, emerged as one of the serious side effects of the Coronavirus Disease 2019 (COVID-19) vaccination. We...
BACKGROUNDS
Myasthenia Gravis (MG), a chronic neuromuscular junction disorder, emerged as one of the serious side effects of the Coronavirus Disease 2019 (COVID-19) vaccination. We aimed to summarize the findings of studies on the clinical features and outcomes of COVID-19 vaccination-associated MG.
METHODS
We performed a systematic search on 3 databases, Medline, Embase, and Scopus, using the query "COVID-19 vaccine" and "Myasthenia Gravis." Patients' data, including clinical data, MG subtype, vaccine type, and vaccine dose number, were extracted from the eligible studies.
RESULTS
A total of 20 COVID-19 vaccination-related MGs have been reported worldwide. The median (interquartile range) age was 64 (51, 75) years; 85% (17/20) of them were male, and 70% (14/20) of patients had received messenger RNA-based vaccines. The most common symptoms, in order of frequency, were binocular diplopia (8/11) and ptosis (4/11); the median (interquartile range) time from vaccine to MG symptoms was 6 (2, 7.5) days. Repetitive nerve stimulation showed abnormal decrement in 85% (11/13) of patients, and all 4 patients getting single-fiber electromyography showed an abnormal finding. Nine out of twelve patients with data on clinical outcomes experienced partial/complete improvement of symptoms within 1 month.
CONCLUSION
MG cases after the COVID-19 vaccine are more likely to occur among males and adults older than 50 years. Our pooled cohort data suggest MG symptoms appear within 2 weeks after receiving the vaccine. The presenting symptoms in MG cases associated with COVID-19 vaccine are possibly similar to non-vaccination related MGs. Most patients are expected to experience partial/complete improvement within 1 month.
Topics: Adult; Humans; Male; Female; COVID-19 Vaccines; COVID-19; Myasthenia Gravis; Diplopia; Vaccines; Vaccination
PubMed: 37800781
DOI: 10.1097/MD.0000000000034890 -
European Journal of Neurology Jan 2023Although myasthenia gravis (MG) is recognized as an immunoglobulin G autoantibody-mediated disease, the relationship between autoantibody levels and disease activity in... (Review)
Review
BACKGROUND AND PURPOSE
Although myasthenia gravis (MG) is recognized as an immunoglobulin G autoantibody-mediated disease, the relationship between autoantibody levels and disease activity in MG is unclear. We sought to evaluate this landscape through systematically assessing the evidence, testing the impact of predefined variables on any relationship, and augmenting with expert opinion.
METHODS
In October 2020, a forum of leading clinicians and researchers in neurology from across Europe (Expert Forum for Rare Autoantibodies in Neurology in Myasthenia Gravis) participated in a series of virtual meetings that took place alongside the conduct of a systematic literature review (SLR).
RESULTS
Forty-two studies were identified meeting inclusion criteria. Of these, 10 reported some correlation between a patient's autoantibody level and disease severity. Generally, decreased autoantibody levels (acetylcholine receptor, muscle-specific kinase, and titin) were positively and significantly correlated with improvements in disease severity (Quantitative Myasthenia Gravis score, Myasthenia Gravis Composite score, Myasthenia Gravis Activities of Daily Living score, Myasthenia Gravis Foundation of America classification). Given the limited evidence, testing the impact of predefined variables was not feasible.
CONCLUSIONS
This first SLR to assess whether a correlation exists between autoantibody levels and disease activity in patients with MG has indicated a potential positive correlation, which could have clinical implications in guiding treatment decisions. However, in light of the limited and variable evidence, we cannot currently recommend routine clinical use of autoantibody level testing in this context. For now, patient's characteristics, clinical disease course, and laboratory data (e.g., autoantibody status, thymus histology) should inform management, alongside patient-reported outcomes. We highlight the need for future studies to reach more definitive conclusions on this relationship.
Topics: Humans; Activities of Daily Living; Myasthenia Gravis; Autoantibodies; Immunoglobulin G; Biomarkers
PubMed: 36094738
DOI: 10.1111/ene.15565 -
Orphanet Journal of Rare Diseases Jun 2018The objective of this study is to evaluate by means of a systematic review, the efficacy of thymectomy as compared to medical treatment for non-thymomatous myasthenia... (Meta-Analysis)
Meta-Analysis
BACKGROUND
The objective of this study is to evaluate by means of a systematic review, the efficacy of thymectomy as compared to medical treatment for non-thymomatous myasthenia gravis (MG).
METHODS
Medline, Embase, and Lilacs were searched for experimental and observational studies that compared non-surgical (drug therapy) and surgical treatment of non-thymomatous MG (thymectomy performed by the transsternal approach). Inclusion criteria were: studies that compared the two types of treatment and had at least 10 adult patients in each group. Exclusion criteria were articles published before 1970, as well as those that included patients treated before 1950. The outcomes evaluated were: remission, and improvement rates. RevMan 5.3 software provided by the Cochrane Collaboration was used. When the heterogeneity between the studies was greater than 75%, a meta-analysis was not performed according to RevMan guidelines.
RESULTS
The total number of patients evaluated in 19 articles selected was 5841 (2911 surgical and 2930 non-surgical). Two included randomized clinical trials showed superiority of the surgical treatment over the non-surgical. Four retrospective studies with 379 patients paired by gender, age, and other confounders, also showed superiority of surgical treatment (OR 4.10, 95% CI 2.25 to 7.44; I = 20%). In meta-analyses, remission assessed in 17 studies (5686 patients) was greater in patients who underwent surgical treatment (OR 2.34, 95% CI 1.79 to 3.05; I = 56%). For improvement assessed in 13 studies (3063 patients) were not appropriate to carry out the meta-analysis due to the high heterogeneity among the studies in the outcome (87%).
CONCLUSION
Thymectomy may be considered effective in the treatment for non-thymomatous MG, with remission rate higher than for non-surgical treatment.
Topics: Female; Humans; Male; Myasthenia Gravis; Thymectomy
PubMed: 29940999
DOI: 10.1186/s13023-018-0837-z