Course Of Myasthenia Gravis Research Articles

Gender features of clinical manifestations and quality of life indicators in generalized myasthenia

Objective. To study the influence of clinical indicators of myasthenia gravis severity to the quality of life of men and women with generalized myasthenia gravis.Materials and methods. The study included 41 patients aged 60.4±16.4 years with generalized myasthenia gravis: 31 (76 %) women and 10 (24 %) men. Clinical manifestations of myasthenia gravis were assessed with quantitative myasthenia gravis score, myasthenia gravis-specific activities of daily living scale and short form of specialized quality of life questionnaire. Forced vital respiratory capacity was measured in all patients with subsequent additional data calculation.Results. We revealed significant improvement of examined data during hospitalization in both males and females, but in females, significant improvement was established in all quantitative myasthenia gravis score subscales. In men worse affection of swallowing muscles and severe pain phenomena were revealed. Correlations of physical functioning parameter with total quantitative myasthenia gravis score and with score characterizing the strength of limb muscles were revealed in studying group.Conclusion. Clinical manifestations and course of myasthenia gravis have the peculiarities n men and women. Quality of life disorder in studying patients correlated with severity of clinical manifestations, mainly with limb weakness.

Tracking myasthenia gravis severity over time: Insights from the French health insurance claims database.

Few data are available on the course of myasthenia gravis (MG) regarding disease severity and stability over time in real-world settings. This study used the French National Health Insurance Database (SNDS) to assess markers of disease severity in patients with MG longitudinally. All patients with MG-related claims in the SNDS between 2013 and 2020 were identified. Patients were followed for up to 8 years after the first claim. Intensive care unit (ICU) stays, treatment with intravenous immunoglobulin (IVIg) or plasma exchange (PE), and death were documented throughout the follow-up period. Standardized mortality rates were estimated, and mortality-related variables were identified using a Cox model. In all, 14,459 individuals constituted the full study population, including 6354 incident patients. In the incident population, 2199 (34.6%) were admitted to ICUs at least once, principally during the first year after the index date (N = 1477; 23.3%). This proportion decreased progressively to reach 3.0% in the seventh year. A total of 2817 patients received IVIg and 432 PE, again principally in the first year. In the full study population, the standardized mortality rate was 1.08 (95% confidence interval [CI] 1.03-1.13), being lower in men (0.95, 95% CI 0.89-1.02) than in women (1.15, 95% CI 1.07-1.23) and in patients aged >65 years (1.06, 95% CI 1.01-1.11) than in younger patients (1.50, 95% CI 1.24-1.76). Male gender, older age and higher comorbidity were independently associated with mortality. A subgroup of patients with MG require ICU admission and rescue therapy with IVIg or PE, indicative of poor disease control. New therapies are needed to improve disease control and reduce disease burden.

Myasthenia gravis-Gender aspects and family planning

There is evidence that gender-specific differences can influence the diagnostics, treatment and long-term disease course of myasthenia gravis (MG). In women the diagnosis is often made during childbearing age. Gender-specific differences in MG and relevant aspects in routine clinical practice are presented. In addition, current studies on family planning, pregnancy and childbirth in MG are highlighted and treatment recommendations are derived. Narrative literature review. In addition to sociodemographic data, gender-specific differences encompass clinical as well as paraclinical factors, such as disease severity and antibody status. With few exceptions pregnancy is possible with good maternal and neonatal outcome. During pregnancy and peripartum, children of MG patients should be closely monitored for early detection and treatment of potential syndromes caused by diaplacental transfer of maternal antibodies. Gender-specific factors can influence the course of MG. Adequate medical counselling and multidisciplinary collaboration are essential for MG patients who wish to have children.

Thymoma negatively affects the neurological outcome of myasthenia gravis after thymectomy: a propensity score matching study

BackgroundThymoma and myasthenia gravis (MG) interact with each other. This study aimed to evaluate the effects of thymoma on neurological outcome of MG patients after thymectomy using the propensity score matching (PSM) method.MethodsConsecutive patients with MG who underwent thymectomy at Beijing Hospital between January 2012 and August 2021 were retrospectively enrolled. Clinical and follow-up data were collected. Statistical analysis was performed using SPSS 23.0 software. PSM was performed to eliminate selection bias.ResultsA total of 456 patients were included in this study. Thymoma was present in 138 (30.3%) patients. The median follow-up time was 72 (range, 12–135) months. At the last follow-up, a lower proportion of thymomatous MG patients achieved complete stable remission (CSR) compared with non-thymomatous MG patients (P = 0.011), and the effective rate [CSR + pharmatologic remission (PR) + minimal manifestations (MM)] of thymomatous MG patients was also lower (P = 0.037). Considering time to CSR, Kaplan–Meier analysis showed thymomatous MG patients had lower cumulative CSR rate than non-thymomatous MG patients (log-rank, P = 0.019). After PSM, 105 pairs of patients were matched successfully. For the matched patients, thymomatous MG patients had a lower CSR rate and a lower effective rate (P = 0.002, 0.039, respectively), and K–M analysis still showed thymomatous MG patients had lower cumulative CSR rate (log-rank, P = 0.048). Multivariate Cox analysis demonstrated that thymoma (HR: 0.592, 95% CI 0.389–0.900, P = 0.014), older age at the time of surgery (HR: 0.971, 95% CI 0.953–0.990, P = 0.003), and preoperative course of MG > 12 months (HR: 0.474, 95% CI 0.317–0.708, P = 0.000) were negative predictive factors for CSR.ConclusionsThymoma had a negative effect on the neurological outcome of MG after thymectomy. MG patients with old age and a preoperative course of longer than one year had a lower probability of achieving CSR.

Pregnancy planning may impact maternal and neonatal outcomes in people with myasthenia gravis.

Myasthenia Gravis (MG) is an acquired autoimmune condition commonly diagnosed in young people of reproductive age resulting in neuromuscular junction dysfunction. The course of MG during pregnancy and its impact on maternal and neonatal outcomes is vary in the literature. Pregnancy planning is a known strategy and modifiable risk factor in obstetric practice to decrease maternal and neonatal morbidity. We aim to assess if planning a pregnancy impacts maternal and neonatal outcomes, MG exacerbation, and pregnancy-related complications. This study utilized data from an online, North American survey entitled "A Patient Centered study on Pregnancy in People with Myasthenia Gravis", distributed with the assistance of MG advocacy groups in the United States and Canada. It included individuals with MG who had at least one pregnancy in the last 10-years. Key maternal and neonatal outcomes were compared between planned and unplanned pregnancies. Out of 156 survey participants, 58 had a pregnancy following MG diagnosis, totaling 90 reported pregnancies. Of these, 56 (62.2%) were planned and 34 (37.8%) were unplanned pregnancies. The unplanned pregnancies were associated with more MG exacerbations, hospitalizations, and intensive care unit admission (37.7% vs. 13.7%, 26.5% vs. 11%, and 17.6% vs. 8.9%, respectively, p ≤ .05). The neonatal outcomes did not significantly differ between the groups. Planned pregnancies in people with MG may be associated with a reduced gestational and post-partum risk of MG exacerbation, hospitalizations, and ICU admissions. Larger studies are required to confirm this association and account for potential contributing variables.

What Is in the Neuromuscular Junction Literature?

Abstract This update covers several articles on diagnosis and misdiagnosis of myasthenia gravis (MG), the role of complement in MG, and then an impressive number of recent treatment trials. There is a negative study on any corticosteroid-sparing effect of intravenous immunoglobulin. A number of positive studies are reviewed. Open-label extension studies of phase 3 trials showed benefit regarding quality of life with efgartigimod and in functional measures with ravulizumab. The phase 3 RAISE trial of zilucoplan, a self-administered complement C5 inhibitor, is covered as well as the MyCarinG trial of rozanolixizumab. The notion of using fast-acting therapies early in the course of MG is addressed. The last sections center on MG and Lambert–Eaton myasthenic syndrome as a consequence of immune checkpoint inhibitor therapy.

Operative approaches for thymectomy: clinical characteristics and classification variant

Currently, surgical intervention is the gold standard treatment of thymus tumors. Radical removal of the thymus gland in a single block, including the tumor, adjacent lymph nodes, and surrounding tissue of the anterior mediastinum, is a prerequisite for achieving satisfactory immediate and long-term results. The need for maximum thymectomy together with anterior mediastinal fiber is attributed to the fact that, along with other long-term survival factors (stage of tumor progression and histological subtype of the tumor), 4550% of deaths in the long-term follow-up period are due to the recurrence of thymoma and 1520% are caused by a progressive course of myasthenia gravis. The results of domestic and foreign studies on the implementation of thymectomy from various surgical approaches are analyzed. These include traditional thoracotomy and sternotomy, video-assisted and robot-assisted interventions, and combined operative accesses. The data presented are based on systematic reviews, retrospective cohort studies, clinical cases, and experimental studies using text databases PubMed, Google Scholar, and eLibrary.ru. The clinical characteristics of each operative access are given with an emphasis on technical features and advantages and disadvantages of thymectomy. The immediate results of surgical treatment of patients with thymus gland neoplasms are presented. Additional methods of visualization and physical preparation of anterior mediastinal tissues are described separately, which help increase the safety and effectiveness of surgical intervention. Currently, low-traumatic video-assisted surgical interventions are widely used in thoracic surgery, gradually replacing traditional open operations. Nevertheless, the variety of proposed options for surgical access, lack of clear indications and contraindications to various interventions on the thymus gland, and discrepancies in the use of various terms determine the need to develop a modern classification of surgical accesses for performing thymectomy. Taking into account world literature data and our own experience, a classification of operative accesses for performing thymectomy is proposed.

Repetitive nerve stimulation as a predictor of treatment response in myasthenia gravis

Background and objectives. The clinical course of myasthenia gravis (MG) varies and may adversely affect quality of life and limit daily activities. Repetitive nerve stimulation (RNS) can be used to diagnose MG and may predict disease progression. We aim to establish the correlation between RNS and pharmacological treatment response. Materials and methods. We conducted a cross-sectional study evaluating the correlation between RNS result and pharmacological treatment response as evaluated using the Myasthenia Gravis-Activities of Daily Living (MG-ADL). Results. RNS positive patients possess greater severity on pre-treatment (9 [5-11] vs. 4 [2-5], p<0.001) and post-treatment (2 [1-4] vs. 1 [0-2], p=0.007) MG-ADL scores. ΔMG-ADL was also significantly greater in RNS positive patients (-6[-7 - -3) vs. -2 [-5 - -1], p=0.004). Spearman’s rank-order correlation conducted evaluating the correlation between RNS and ΔMG-ADL demonstrates a significant moderate positive correlation between RNS and ΔMG-ADL (rs=0.495, p<0.001). Conclusions. A moderate significant correlation was found between RNS and pharmacological treatment response. RNS positive patients are more responsive towards treatment compared to RNS negative patients. Higher fatigability in muscles translates to greater RNS decrements and clinical severity, lending itself to a greater opportunity for improvement following pharmacological. However, this must not be confused with long-term prognosis.

Clinical features of myasthenia gravis with neurological and systemic autoimmune diseases.

Multiple reports on the co-existence of autoimmune diseases and myasthenia gravis (MG) have raised considerable concern. Therefore, we reviewed autoimmune diseases in MG to explore their clinical presentations and determine whether the presence of autoimmune diseases affects the disease severity and treatment strategies for MG. We reviewed all the major immune-mediated coexisting autoimmune conditions associated with MG. PubMed, Embase and Web of Science were searched for relevant studies from their inception to January 2023. There is a higher frequency of concomitant autoimmune diseases in patients with MG than in the general population with a marked risk in women. Most autoimmune comorbidities are linked to AChR-MG; however, there are few reports of MuSK-MG. Thyroid disorders, systemic lupus erythematosus, and vitiligo are the most common system autoimmune diseases associated with MG. In addition, MG can coexist with neurological autoimmune diseases, such as neuromyelitis optica (NMO), inflammatory myopathy (IM), multiple sclerosis (MS), and autoimmune encephalitis (AE), with NMO being the most common. Autoimmune diseases appear to develop more often in early-onset MG (EOMG). MS coexists more commonly with EOMG, while IM coexists with LOMG. In addition, MG complicated by autoimmune diseases tends to have mild clinical manifestations, and the coexistence of autoimmune diseases does not influence the clinical course of MG. The clinical course of neurological autoimmune diseases is typically severe. Autoimmune diseases occur most often after MG or as a combined abnormality; therefore, timely thymectomy followed by immunotherapy could be effective. In addition, thymoma-associated AChR MG is associated with an increased risk of AE and IM, whereas NMO and MS are associated with thymic hyperplasia. The co-occurrence of MG and autoimmune diseases could be attributed to similar immunological mechanisms with different targets and common genetic factor predisposition. This review provides evidence of the association between MG and several comorbid autoimmune diseases.

Diagnosis and therapy of myasthenia gravis-the patients' perspective: a cross-sectional study.

The survey aimed to explore patients' perspectives with myasthenia gravis (MG) toward the diagnosis made and the therapy used to treat MG. The survey was conducted with a quantitative method, using the CAWI technique. A total of 321 people participated in the survey. More than half of the respondents (56.4%) had suffered from MG for less than 10 years. In three out of 10 cases (30.9%), the diagnosis of MG lasted 3 years or longer. The diagnostic delay was significantly longer in female respondents than in the males (p = 0.029). Cholinergic drugs were used in 92.9% of cases initially, and as maintenance therapy in 84.3% of cases. Corticosteroids were used in initiating therapy (45.8%) and as maintenance therapy (46.4%). One in four respondents (25.5%) reported experiencing very strong and strong side effects after using steroids. The side effects from steroid therapy very strong or strong affected overall physical health in 55.9% of respondents, very strong or strong affected self-acceptance in 52%, to a very large or large extent on mental health in 47.1%, and to a very strong or strong extent influenced the performance of daily activities in 28.2%. More than half of the respondents (57.0%) had had a thymectomy. Seven out of 10 respondents (72.0%) declared that the therapy they were on at the time of the survey allowed them (to varying degrees) to control their course of MG. Low therapy acceptance and less well controlled MG was associated with a preference for non-tablet therapies (p = 0.045). Regular follow-up and cooperation with the specialist health care system should improve MG symptoms, activities of daily living, and quality of life.

Plasma C18:0-ceramide is a novel potential biomarker for disease severity in myasthenia gravis.

Myasthenia gravis (MG) is an antibody-mediated autoimmune disorder characterized by fluctuation of fatigue and weakness of muscle. Due to the heterogeneity of the course of MG, available biomarkers for prognostic prediction are urgently needed. Ceramide (Cer) was reported to participate in immune regulation and many autoimmune diseases, but its effects on MG remain undefined. This study aimed to investigate the ceramides expression levels in MG patients and their potential as novel biomarkers of disease severity. Levels of plasma ceramides were determined by ultra performance liquid chromatography-tandem mass spectrometry (UPLC-MS/MS). Severity of disease was assessed by quantitative MG scores (QMGs), MG-specific activities of daily living scale (MG-ADLs) and 15-item MG quality of Life (MG-QOL15). The concentrations of serum interleukin-1β (IL-1β), IL-6, IL-17A, and IL-21 were determined by enzyme-linked immunosorbent assay (ELISA), and the proportions of circulating memory B cells and plasmablasts were detected by flow-cytometry assay. Four plasma ceramides levels we studied were detected higher in MG patients. And three of them (C16:0-Cer, C18:0-Cer, and C24:0-Cer) were positively associated with QMGs. In addition, receiver operating characteristic (ROC) analysis suggested that plasma ceramides have a good ability of differentiating MG from HCs. Importantly, only C18:0-Cer was shown to be positively associated with the concentration of serum IL and circulating memory B cells, and the decrease in plasma C18:0-Cer paralleled the clinical improvement of patients with MG. All together, our data suggest that ceramides may play an important role in the immunopathological mechanism of MG, and C18:0-Cer has the potential to be a novel biomarker for disease severity in MG.

The impact of Myasthenia Gravis antibody status on pregnancy and neonatal outcomes (P1-8.007)

<h3>Objective:</h3> The objective of this study to assess the impact of Myasthenia Gravis (MG) serological status on pregnancy-related and neonatal outcomes. <h3>Background:</h3> Pregnancy has a variable effect on the course of MG. Current knowledge describes an increased likelihood of flares in the first trimester and the postpartum period, an increased risk of caesarean section and ICU admission, transient neonatal MG and arthrogryposis. It’s unknown if serological status impacts pregnancy and neonatal outcomes. <h3>Design/Methods:</h3> An online survey was developed in conjunction with patient representatives from the Myasthenia Gravis Foundation of America. Respondents who have (or had) ovaries, uterus, and/or cervix with a diagnosis of MG. For the current analysis, those with at least one pregnancy in prior 10 years were included. Information on disease course, pregnancy complications, antibody status, fetal and neonatal outcomes was collected. <h3>Results:</h3> 157 opened the survey, and 142 met entry criteria for a response rate of 90%. 96 pregnancies were analyzed. MG symptoms appeared to worsen regardless of antibody status in the first trimester and postpartum. Antibody negative vs AchRAB and MUSK participants respectively, reported a greater risk of ICU admission (23% vs 16% and 12%, P =0.058 ) intubation ( 50% vs 4% and 17% P value 0.007) C-section ( 92% vs 23% and 42%, put p-0.001), neonatal MG rates (67% vs 8% and 0%, P value 0.001), and unplanned pregnancy (75% vs 21% and 50%, put p-value 0.004). <h3>Conclusions:</h3> This is the first know study of the association between negative serological status and poor pregnancy outcomes and although limited in size, may influence future preconception counselling and pregnancy monitoring. Highlighting the importance of interdisciplinary preconception and antenatal care including Neurology, Maternal-Fetal-Medicine and Neonatology. Findings of transient neonatal myasthenia gravis and arthrogryposis in seronegative individuals suggests that another, currently unknown antibody or biological process may be present in the seronegative group. Future, larger studies are warranted. <b>Disclosure:</b> Dr. Al Hussona has nothing to disclose. Dr. Izenberg has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Izenberg has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Izenberg has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genzyme. Dr. Izenberg has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Aaron Izenberg. Dr. Barnett Tapia has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alexion. Dr. Barnett Tapia has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. Dr. Barnett Tapia has received personal compensation in the range of $0-$499 for serving as a Consultant for Sanofi. Dr. Barnett Tapia has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. The institution of Dr. Barnett Tapia has received research support from Department of defense. The institution of Dr. Barnett Tapia has received research support from Muscular dystrophy canada. The institution of Dr. Barnett Tapia has received research support from MgNet. The institution of Dr. Barnett Tapia has received research support from NIH. Dr. Barnett Tapia has received intellectual property interests from a discovery or technology relating to health care. Dr. Berndl has nothing to disclose.

The relationship of cognitive impairment and clinical manifestations of myasthenia gravis

BACKGROUND. There are many studies in the literature suggesting that cognitive impairment is more pronounced in patients with myasthenia gravis compared with healthy people, but there is no explanation of the reasons and mechanisms. However, the reverse character the influence of intellectual-mnestic disorders on the course of myasthenia gravis has not been studied enough.&#x0D; AIM. To compare clinical, anamnestiс, laboratory and instrumental data in patients with myasthenia gravis according to the presence or absence of cognitive impairment.&#x0D; MATERIAL AND METHODS. 61 patients with a confirmed diagnosis of myasthenia gravis were divided into 2 groups according to the Montreal cognitive test. Patients scoring 25 or less were assigned to the first group, those scoring 26 points or more to the second group.&#x0D; A comparative assessment was made according to the myasthenia gravis impairment index, age of onset, duration of the disease, duration of hospitalization, severity of myasthenia gravis according to clinical scales, body mass index, electroneuromyography data, levels of antibodies to the acetylcholine receptor and skeletal muscles, and daily doses of the drugs received.&#x0D; RESULTS. In the 1st group, there was a significantly higher score on the Generalised Disorders subscale of the Myasthenia Gravis Impairment Index; the severity of Quantitative myasthenia gravis score was significantly higher in the 1st group than in the 2nd both for the total score and for the Musculoskeletal Lesions subscale. On average, 49 mg more pyridostigmine bromide was required in the first group, while the average body mass index was significantly lower in the first group.&#x0D; CONCLUSION. The presence of a cognitive deficit in patients with myasthenia gravis may worsen the clinical picture of the disease, which should be taken into account by clinicians, especially in elderly patients, who are at increased risk of developing cognitive disorders.

Clinical characteristics and prognosis of very late-onset myasthenia gravis in China

Alteration in onset-age distribution in myasthenia gravis (MG) and its increasing prevalence among the elderly underscores the need for a better understanding of the clinical course of MG and the establishment of personalized treatment. In this study we reviewed the demographics, clinical profile, and treatment of MG. Based on onset age, eligible patients were classified as early-onset MG (onset age ≥18 and <50 years), late-onset MG (onset age ≥50 and <65 years), and very late-onset MG (onset age ≥65 years). Overall, 1160 eligible patients were enrolled. Patients with late- and very late-onset MG showed a male predominance (P=0.02), ocular MG subtype (P=0.001), and seropositivity for acetylcholine receptors and titin antibodies (P<0.001). In very late-onset MG, a lower proportion of patients retained minimal manifestations status or better, a higher proportion of patients had MG-related deaths (P<0.001), and a shorter maintenance time of minimal manifestation status or better was seen at the last follow-up (P=0.007) than that in patients with early- and late-onset MG. Non-immunotherapy may associated with a poor prognosis in patients in the very late-onset group. Further studies on very late-onset MG patients should be performed to evaluate the relationship between immunotherapy and prognosis.

Severity and antibodies profile of seropositive myasthenia gravis

Background: Myasthenia gravis (MG), an autoimmune disease, affects the neuromuscular junction. The impaired neuromuscular transmission results in fatigable muscle weakness among MG patients. Positive antibodies are found among MG patients. Aims: The main aim of this study is to evaluate the correlation of MG severity with their anti-muscle-specific kinase (MUSK) and anti-acetylcholine receptor (AChR) antibody status. Moreover, the study also identifies the correlation between the antibodies profile and the severity of a seropositive MG. Setting and Design: This retrospective cross-sectional study was conducted in the tertiary center of the western region of Saudi Arabia named King Abdullah Medical City (Makkah) over 8 years, from January 2011 to December 2019. Materials and Methods: This retrospective cross-sectional study was conducted in Saudi Arabia in King Abdullah Medical City (Makkah) over 8 years, from January 2011 to December 2019 was reviewed. Seventeen patients out of 27 MG patients came out to be seropositive. The correlation was investigated between severity and antibody status. Statistical Analysis: Data were collected in an Excel sheet, and statistical analysis was performed using SPSS 21 software. Results: The results showed that most patients (87.5%) were positive for AChR antibodies only, while others (12.5%) were positive for anti-MUSK and anti-AChR. Thymoma and crisis were observed in 100% with dual seropositivity and 21% with single seropositivity. Conclusion: The study concluded that a severely aggressive course of MG is present among patients. However, detailed clinical trial studies are required for conforming MG severity and dual seropositivity.

Myasthenia gravis complicated by the development of COVID-19: an analysis of case series

Myasthenia gravis (MG) is an autoimmune disease characterized by increased dynamic muscleweakness. Patients with myasthenia gravis are united by the phenomenon of deterioration of the clinicalcondition after infection, refusal of treatment or taking certain medications, surgical intervention, exposureto heat and stress. In the context of the COVID‑19 (Coronavirus disease 2019) pandemic, the study ofpatients with myasthenia gravis and a new infectious disease may reveal new pathogenetic patterns andchange the therapeutic strategy.&#x0D; Objective — to identify clinical and paraclinical, therapeutic regularities in patients with MG and COVID‑19.&#x0D; Methods and subjects. From April 2021 to November 2021, the course of MG against the background ofCOVID‑19 in 11 patients was analyzed. The control group consisted of 7 patients with COVID‑19, butwithout MG. General clinical, neurological, instrumental, laboratory and statistical examination methods,scales Myasthenia Gravis Foundation of America (MGFA), The Quantitative Myasthenia Gravis Score (QMGS), Myasthenia Gravis Activities of Daily Living (MG‑ADL), The National Early Warning Score 2 (NEWS2) and questionnaires were used.&#x0D; Results. In the experimental and control groups, the level of SpO2 when breathing atmospheric air wascorrelated with the presence of bronchial asthma (BA) (r = –0.791), diabetes mellitus (DM) (r = –0.553),hypertension (r = –0.301). A positive correlation (r = 0.271) was found between the presence of MG and the level of SpO2 when breathing atmospheric air, which may be associated with the intake of pyridostigmine anda decrease in muscle mass in patients with MG. Presence of a relationship between the NEWS2 indicatorwith DM (r = 0.501), BA (r = 0.483), obesity (r = 0.376), hypertension (r = 0.352), multinodular goiter (r = 0.204), hydrothorax (r = 0.204) and MG (r = 0.120). In the myasthenia group, a relationship was established between the duration of treatment for COVID‑19 and body mass index (BMI) (r = 0.523), age (r = 0.504), pyridostigmine intake (r = –0.243) and weight (r = 0.228). NEWS2 in the experimental group was correlated with pyridostigmine intake (r = –0.386), weight (r = 0.355) and BMI (r = 0.256). Duration of treatment for COVID‑19 was associated with duration of MG (r = 0.570), obesity (r = 0.572), and BMI (r = 0.526). NEWS2 is related to the level of SpO2 when breathing atmospheric air (r = — 0.907), hemoglobin (r = –0.847) and vital capacity of the lungs (VC) (r = –0.699). Obesity (r = 0.787), anemia (r = 0.684) and BA were correlated with NEWS2. The finding of an inverse correlation between NEWS2 and pyridostigmine intake (r = –0.684) was promising. Soft palate paresis (r = –0.614), dysphagia (r = –0.614) and nasality (r = –0.545) were correlated with a decrease in VC. A correlation was found between VC and the NEWS2 (r = –0.699). The duration of COVID‑19 (r = –0.646) and patient age (r = –0.626) were correlated with VC.&#x0D; Conclusions. MG with the addition of COVID‑19 tends to worsen the course, progress of muscle weakness,development of respiratory failure and hypoxia. Aggravating factors are age, duration of MG, duration ofCOVID‑19, BMI, concomitant pathology (DM, hypertension, BA, obesity, anemia). Constitutional features(lower BMI and weight) may contribute to shortening the duration of treatment. Taking pyridostigmineallows to reduce not only the duration of treatment, but also the risk of worsening of the condition, whichmay be associated with the suppression of the inflammatory process when taking an anticholinesteraseagent.&#x0D;

Fecal Incontinence as a Symptom of Myasthenia Gravis

I report three cases of fecal incontinence during the clinical course of myasthenia gravis. Fecal incontinence developed after withdrawal from the pyridostigmine and improved after re-administration of the pyridostigmine. Myasthenia gravis could affect external anal sphincter and puborectalis function. Myasthenia gravis is presumably one of the causes of fecal incontinence. We must check for fecal incontinence in the patients with myasthenia gravis.

Клініко-нейрофізіологічні особливості у хворих на міастенію залежно від структурних змін тимуса

Для вивчення особливостей клінічного перебігу й порушення нервово-м’язової передачі в пацієнтів з міастенією залежно від структурних змін тимуса були проаналізовані результати клініко-нейрофізіологічного обстеження 78 хворих. Матеріали та методи. Залежно від структурних змін тимуса всі хворі були поділені на 3 групи. Перша група — 29 пацієнтів з міастенією без змін структури тимуса. Друга група — 30 пацієнтів, у яких міастенія супроводжувалася гіперплазією тимуса. Третя група — 19 хворих з міастенією на фоні тимоми. Ступінь тяжкості захворювання оцінювався за допомогою кількісної шкали тяжкості клінічних проявів міастенії (QMGS) та клінічної класифікації Myathenia Gravis Foundation of America (MGFA), 2001. Оцінка нервово-м’язової передачі проводилася на підставі аналізу результатів декремент-тесту та амплітуди негативної фази М-відповіді при низькочастотній непрямій супрамаксимальній стимуляції m. abductor digiti minimi та m. orbicularis oculi. Результати та висновки. Зміни структури тимуса негативно впливають на перебіг міастенії, про що свідчать показники за шкалою QMGS та класифікацією MGFA. Найбільша тяжкість захворювання (з переважанням ураження бульбарної групи м’язів) поєднувалася із наявністю у хворих тимоми, а найменша відзначалась у хворих з нормальною структурою тимуса. Переважання пацієнтів жіночої статі в групі хворих з міастенією на тлі гіперплазії тимуса може свідчити про вплив гормонального статусу як на структуру вилочкової залози з розвитком її гіперплазії, так і на перебіг міастенії, що потребує подальшого вивчення. Простежена пряма залежність між структурними змінами в тимусі та зниженням амплітуди негативної фази М-відповіді, що свідчить про негативний вплив морфологічних порушень тимуса на нервово-м’язову передачу імпульсів. Значне зниження амплітуди негативної фази М-відповіді на фоні високих показників декремент-тесту можна вважати маркером тяжкого перебігу захворювання.

Anti‐lipoprotein receptor‐related protein 4 antibody titer correlates with the clinical course of myasthenia gravis

Anti‐lipoprotein receptor‐related protein 4 antibody titer correlates with the clinical course of myasthenia gravis

Lečenje stečene autoimune mijastenije gravis - gde smo danas?

Acquired autoimmune myasthenia gravis (MG) is a prototype of autoimmune disease in which autoantibodies directed against various antigens of the neuromuscular junction, leading to impaired neuromuscular transmission with clinical presentation of fluctuating weakness and excessive fatigue of transverse striated skeletal muscles. In 85% of patients with MG there are antibodies to the nicotine acetylcholine receptor of the postsynaptic muscle membrane (nAChR), in 5-8% of patients there are antibodies to muscle-specific tyrosine kinase (MuSK), in 1-2% of antibodies to low-density lipoprotein receptor-related protein 4 (LRP4), while in the remaining 10% none of the mentioned autoantibodies can be detected, and these patients are classified in the group of so-called "seronegative" MG. It is well known that this antigenic specificity affects both the clinical presentation and the therapeutic response, course and prognosis of the disease, which is why the early serological confirmation of the diagnosis is important. In this way, determining the exact profile of autoantibodies can provide a significantly more efficient, personalized therapeutic approach for each patient, and thus improve the prognosis and quality of life of these patients. In general, when a patient is diagnosed with MG, the goal is to alleviate the symptoms of the disease as soon as possible, as well as to induce remission or minimal manifestations of the disease in the following period. MG therapy can be roughly divided into symptomatic therapy, which includes drugs that alleviate the symptoms of weakness and fatigue, causal therapy, which immunomodulates the pathogenetic process that affects the course of MG and temporary therapeutic procedures to achieve rapid improvement in patients to avoid or treatment of the most severe clinical manifestations, the so-called "crisis" of MG. Symptomatic MG therapy involves the use of acetylcholinesterase inhibitors, represented by pyridostigmine bromide. It works by relieving the symptoms of MG in a short period of time, 4-8 hours. Causal therapy acts on the pathogenetic process on the basis of MG and it includes corticosteroids as first-line drugs and immunosuppressive drugs: azathioprine, cyclosporine A, mycophenolate mofetil, etc, while temporary therapeutic procedures include therapeutic plasma changes and intravenous immunoglobulins. All the above mentioned MG therapies are is effective in most patients with this disease, but it should be emphasized that, on the one hand, to achieve its full effect usually takes several months, and on the other hand, that such prolonged therapy is often followed by the occurrence of various side effects that require its reduction or complete cessation. With advances in immunology, molecular biology, and rapid drug development, new agents are being developed today that have much more selective immune targets, save the rest of the immune system, have lower toxicity, and a much faster onset of action MG. Some of these drugs are already being used successfully today, but we can freely say that the door to highly selective immunotherapy in MG has just been opened and that this is certainly the therapy of the future.