Prognosis Of Myasthenia Gravis Research Articles

PROGNOSIS OF MYASTHENIA GRAVIS: A COMPREHENSIVE SYSTEMATIC REVIEW

Background: The complex disease progression and treatment challenges affect the overall prognosis in myasthenia gravis (MG) patients. Recent advancements in managing MG have brought about significant progress in attaining complete stable remission as the treatment objective. This study aims to systematically review the prognosis of MG in literatures of the last 10 years. Methods: This systematic review complied with the PRISMA 2020 standards and focused on full-text English literature published between 2014 and 2024. Articles such as editorials and review papers from the same journal, as well as submissions lacking a DOI, were excluded from consideration. Literature was sourced from online platforms like PubMed and SagePub. Result: We found 627 articles on PubMed and 810 articles on SagePub. Restricting our search to the past decade (2014-2024), PubMed presented 425 articles, whereas SagePub presented 309 articles. From these, we selected 5 papers meeting our criteria, with 2 from PubMed and 3 from SagePub. Conclusion: Significant progress has been achieved in treating MG over the past decade. While some patients experience spontaneous remission, the majority require treatment with steroids or similar drugs. Other treatment modalities such as monoclonal antibody treatment, pulmonary function testing, and thymectomy can be employed to improve the prognosis of MG.

Pediatric myasthenia gravis with a combination of AChR and RyR is associated with an earlier onset and lower CSR rate: A cohort study in southwest China

BackgroundThe clinical manifestations and prognosis of myasthenia gravis are related to antibodies, and children are affected differently than adults. The presence of ryanodine receptor and titin antibodies in adults indicates late onset and severe disease related to thymoma, but their role in children is rarely reported. MethodsThis study collected a cohort of children according to inclusion and exclusion criteria, consisting of antibody-negative, AChR-positive, and AChR with or without titin and RyR antibodies. The differences among groups in general conditions, clinical manifestation, treatment and prognosis were compared. ResultsIn total, 171 patients were included: 33 patients (19.30%) were antibody-negative, 84 patients (49.12%) were positve for AChR antibody, 22 patients (12.87%) were positve for AChR and RyR antibodies, 5 patients (2.92%) were positve for AChR and Titin antibodies, and 27 patients (15.79%) were positve for AChR, RyR and Titin antibodies.The median onset age of all the patients was 57.8 (9–177) months, and patients with AChR and RyR antibodies (p = 0.02) and AChR, RyR and Titin antibodies (p = 0.0006) had a younger onset age than patients with AChR antibodies. The rate of generalized MG and MG-ADL before treatment in the AChR-, RyR- and Titin antibody-positive groups was distinctly higher than that in the AChR antibody-positive group (p = 0.038, p = 0.0325). The rate of IVIG use in the AChR-, RyR- and Titin antibody-positive groups (p = 0.0388) was higher than that in the AChR antibody-positive group. The rate of immunosuppressant use in the AChR and RyR antibody-positive group (p = 0.0415) and in the AChR, RyR and Titin antibody-positive group (p = 0.0006) was higher than that in the AChR antibody-positive group. Plasmapheresis was performed in 1 case in the AChR-, RyR- and Titin antibody-positive groups. The CSR rate in the AChR and RyR antibody-positive group (p = 0.0423) and in the AChR, RyR and Titin antibody-positive group (p = 0.0152) was significantly lower than that in the AChR antibody-positive group. Gender, ptosis severity, and CSR time were not significantly different between groups. ConclusionsWe summarized one of the largest cohorts of pediatric MG patients and compared the clinical phenotype of patients with antibody-negative, AChR-positive, and AChR with or without titin and RyR antibodies. The results showed that patients with AChR and RyR antibodies had a younger onset age, a higher immunosuppressant use rate and a lower CSR rate.

Combined effect of thymectomy on myasthenia gravis in patients with concomitant auto-immune diseases: a 22-year single-center experience

Myasthenia gravis (MG) is an autoimmune disease (AD), and patients with MG often have other types of ADs. We analyzed the prognosis of patients with MG complicated by AD after thymectomy. A retrospective analysis was performed for patients with MG complicated by ADs treated surgically in our center over the past 22 years, and their general condition and follow-up data were collected and analyzed. 33 patients were included totally. 28 patients displayed improvement or even complete recovery of MG, and 23 of 36 ADs revealed improvement or even complete recovery. The prognosis of MG is significantly correlated with the duration of postoperative follow-up time (p = 0.028), and in patients with thymoma, the larger the tumor diameter, the better the prognosis of MG (p = 0.026). Thymic hyperplasia patients were predominantly female (p = 0.049) and young (p < 0.001). The most common concomitant AD in this study was a thyroid-associated AD, which was associated with thymic hyperplasia (p < 0.001), Osserman type I MG (p < 0.001), and young age (p < 0.001). Thymectomy had a good therapeutic effect on MG complicated by AD, and there was a close correlation between surgery, thymus, MG, and ADs.

Epigenome-wide DNA methylation analysis of myasthenia gravis.

Myasthenia gravis (MG) is a common neuromuscular junction (NMJ) disorder and autoimmune disease mediated by several antibodies. Several studies have shown that genetic factors play an important role in MG pathogenesis. To gain insight into the epigenetic factors affecting MG, we report here genome-scale DNA methylation profiles of MG. DNA was extracted from 8 MG patients and 4 healthy controls for genome-wide DNA methylation analysis using the Illumina HumanMethylation 850K BeadChip. Verification of pyrosequencing was conducted based on differential methylation positions (DMPs). Subsequently, C2C12 and HT22 cell lines (derived from mouse) were treated with demethylation drugs. Transcribed mRNA of the screened differential genes were detected using quantitative Real-time PCR. The control and MG group were compared, and two key probe positions were selected. The corresponding genes were CAMK1D and CREB5 (P<0.05). Similarly, the myasthenic crisis (MC) and non-MC group were compared and four key probe positions were selected. The corresponding genes were SAV1, STK3, YAP1 and WWTR1 (P<0.05). Subsequently, pyrosequencing was performed for verification, revealing that hypomethylation of CAMK1D was significantly different between the MG and control group (P<0.001). Moreover, transcription of CREB5, PKD, YAP1 and STK3 genes in the C2C12 cells was downregulated (P<0.05) after drug treatment, but only YAP1 mRNA was downregulated in HT22 cells (P<0.05). This is the first study to investigate genome-scale DNA methylation profiles of MG using 850K BeadChip. The identified molecular markers of methylation may aid in the prevention, diagnosis, treatment and prognosis of MG.

Evaluation of clinical features and prognosis of myasthenia gravis in adults based on the age of onset: A retrospective study from a single center of 30 years MG registry

Objective: We aimed to evaluate the demographic, clinical, and immunological features of myasthenia gravis (MG) in adults according to the age of onset and to investigate the effect on prognosis. Methods: A total of 332 patients with MG were included in the study. Patients were classified into three age subgroups: early-onset MG (EOMG), late-onset MG (LOMG), and very late-onset MG. Complete stable remission, pharmacological remission, and minimal manifestations for 1 year were assessed as a good prognosis on the MGFA-PIS scale. Improved, unchanged, worse, exacerbation and death due to MG were assessed as having a poor prognosis. Results: There were 177 (53.3%) female and 155 male (46.7%) patients with a mean age of 55.3 ± 17.4. A total of 176 patients (53%) were classified as EOMG, 94 patients (28.3%) as LOMG, 62 (18.7%) as very late-onset MG, 282 patients (84.9%) as anti-AChR positive, 21 patients (6.3%) as anti-MuSK positive, and two patients (0.6%) as anti- AChR and anti-MuSK double-positive. While 95.6% of patients with a good prognosis had MGFA-1 at the onset of the disease, 40.3% of patients with a poor prognosis had MGFA-2B. At the end of the clinical follow-up, the MGFA-PIS score was evaluated, 55.1% of the patients had a good prognosis, while 44.9% had a poor prognosis. Conclusions: Age at disease onset was not associated with prognosis. Presence of generalized MG subtype and thymoma, anti-MuSK positivity, hospitalization in the intensive care unit, myasthenic crisis, IVIG administration, plasmapheresis, comorbidity, 2 or more comorbidities were found to have significant association with poor prognosis.

Overview of biomarkers in myasthenia gravis

Myasthenia gravis (MG) is a rare auto-immune neuromuscular junction (NMJ) disorder which is caused by formation of autoantibodies and destruction of NMJ components. The MG diagnosis is based on the symptoms, autoantibodies detection and paraclinical tests. Given that MG patients have so many differential diagnosis and various medication responses, choosing an accurate diagnosis and the therapy plan in MG is challenging. According to the studies, there are the immunologic, genetic, microRNAs, gut microbiome, and other established or newly proposed biomarkers for diagnosis and prognosis of MG. More studies are needed to provide better collection of biomarkers in MG patients and evaluate their role in MG pathology.

Comorbidities worsen the prognosis of generalized myasthenia gravis post-thymectomy

BackgroundThe effect of comorbidities on the prognosis of myasthenia gravis (MG) remains unclear. In particular, the role of other autoimmune diseases (AD) is controversial. MethodsIn this retrospective single-center cohort study, we investigated 154 consecutive generalized thymectomized MG patients, with a mean follow-up time of 8.6 (±5.0) years post-thymectomy. Comorbidities diagnosed at any timepoint were retrieved from medical records and Charlson comorbidity index (CCI) scores were calculated. Patients were categorized into subgroups MG alone (n = 45) and MG with any comorbidity (n = 109); the latter was further categorized into MG with other ADs (n = 33) and MG with non-AD comorbidities (n = 76). The endpoints analyzed were complete stable remission (CSR), minimal need for medications, and need for in-hospital treatments. ResultsCSR was more frequent in MG alone than in MG with any comorbidity group (26.7% vs 8.3%, p = 0.004). Minimal need for medication was reached more often in the MG alone than in the MG with non-AD comorbidities group (p = 0.047). Need for in-hospital treatments was lower in the MG alone group than in MG patients with any comorbidity (p = 0.046). Logistic regression analysis revealed that lower CCI scores increased the likelihood of CSR (p = 0.033). Lower CCI scores were more prevalent both in patients with minimal need for medication and in patients who did not need in-hospital treatments (p < 0.001). ConclusionsPatients with generalized MG and comorbidities have a poorer prognosis than patients with MG alone during almost 9 years follow-up after thymectomy. AD comorbidities appeared not to translate into a higher risk compared to other comorbidities.

Clinical characteristics and outcomes of thymoma-associated myasthenia gravis.

Prognosis of myasthenia gravis (MG) in patients with thymoma is not well established. Moreover, it is not clear whether thymoma recurrence or unresectable lesions entail a worse prognosis of MG. This multicenter study was based on data from a Spanish neurologist-driven MG registry. All patients were aged >18years at onset and had anti-acetylcholine receptor antibodies. We compared the clinical data of thymomatous and nonthymomatous patients. Prognosis of patients with recurrent or nonresectable thymomas was assessed. We included 964 patients from 15 hospitals; 148 (15.4%) had thymoma-associated MG. Median follow-up time was 4.6years. At onset, thymoma-associated MG patients were younger (52.0 vs. 60.4 years, p<0.001), had more generalized symptoms (odds ratio [OR]: 3.02, 95% confidence interval [CI]: 1.95-4.68, p<0.001) and more severe clinical forms according to the Myasthenia Gravis Foundation of America (MGFA) scale (OR: 1.6, 95% CI: 1.15-2.21, p=0.005). Disease severity based on MGFA postintervention status (MGFA-PIS) was higher in thymomatous patients at 1year, 5years, and the end of follow-up. Treatment refractoriness and mortality were also higher (OR: 2.28, 95% CI: 1.43-3.63, p=0.001; hazard ratio: 2.46, 95% CI: 1.47-4.14, p=0.001). Myasthenic symptoms worsened in 13 of 27 patients with recurrences, but differences in long-term severity were not significant. Fifteen thymomatous patients had nonresectable thymomas with worse MGFA-PIS and higher mortality at the end of follow-up. Thymoma-associated MG patients had more severe myasthenic symptoms and worse prognosis. Thymoma recurrence was frequently associated with transient worsening of MG, but long-term prognosis did not differ from nonrecurrent thymoma. Patients with nonresectable thymoma tended to present severe forms of MG.

Interleukin-27 levels in patients with myasthenia gravis.

Interleukin-27 (IL-27), which belongs to IL-12 family, influences the function of T cells (Tregs) through regulating the expression, and function of forkhead box P3 (FoxP3). In this study, we detected the IL-27 serum levels in 59 myasthenia gravis (MG) patients and 35 healthy controls (HCs). Among them, 32 MG patients received immunoglobulin intravenous (IVIG) injections (0.4 g/kg per day for 5 consecutive days). IL-27 levels were collected before and after the treatments and subjected to a comparative study. Finally, we assessed the correlations of IL-27 levels with the clinical characteristics of MG. As a result, serum IL-27 levels were significantly higher in MG patients than those in the HCs. Meanwhile, significant reduction was detected after the IVIG treatment. IL-27 levels positively correlated with both MG activities of daily living and quantitative MG score. IL-27 may participate in the pathogenesis of MG and can be used as an early marker for the diagnosis and prognosis of MG. In addition, IL-27 can be used as a target for MG treatment through the regulation of specific immune signaling and maintaining immune homeostasis.

Emerging Roles of Dysregulated MicroRNAs in Myasthenia Gravis.

Myasthenia gravis (MG) is a rare acquired autoimmune neuromuscular disease. Autoantibodies, cellular immunity, complement, and cytokines are involved in the pathogenesis of MG. It is characterized by the dysfunction of neuromuscular junction transmission and skeletal muscle weakness. MicroRNAs (miRNAs) are non-coding small molecule ribonucleic acids that regulate various biological processes (e.g., development, differentiation, and immunity) at the transcriptional and post-transcriptional levels of gene expression. miRNAs play an important regulatory role in the pathogenesis of autoimmune diseases, including MG. In recent studies, the functional mechanisms underlying the role of miRNAs in the pathogenesis of MG have received increasing attention. miRNAs are highly stable and have high specificity in peripheral body fluids. Therefore, the miRNAs in body fluids may represent promising biomarkers for determining the prognosis of MG and the efficacy of treatment. This article reviews the role of miRNAs in the pathogenesis of MG, highlights the potential of miRNAs as new biomarkers for the diagnosis of MG, and deepens our understanding of disease processes.

History of Myasthenia Gravis Revisited.

The first description of myasthenia gravis (MG) was given by Thomas Willis in 1672. MG was the focus of attention after mid-nineteenth century and a great amount of information has been accumulated in a span of 150 years. The aim of this review is to convey this information according to a particular systematic and to briefly relate the experience of Istanbul University. MG history was examined in four periods: 1868-1930, 1930-1960, 1960-1990, and 1990-2020. In the first period (1868-1930), all the clinical characteristics of MG were defined. Physiological/pharmacological studies on the transmission at the neuromuscular junction were initiated, and the concept of repetitive nerve stimulation emerged. A toxic agent was believed to be the cause of MG which appeared to resemble curare intoxication. Association of MG with thymus was noticed. No noteworthy progress was made in its treatment. In the second period (1930-1960), acetylcholine was discovered to be the transmitter at the neuromuscular junction. Repetitive nerve stimulation was used as a diagnostic test. The autoimmune nature of MG was suspected and experiments to this end started to give results. The hallmark of this period was the use of anticholinesterases and thymectomy in the treatment of MG. The third period (1960-1990) can probably be considered a revolutionary era for MG. Important immunological mechanisms (acetylcholine receptor isolation, discovery of anti-acetylcholine receptor antibodies) were clarified and the autoimmune nature of MG was demonstrated. Treatment modalities which completely changed the prognosis of MG, including positive pressure mechanic ventilation and corticosteroids as well as plasma exchange/IVIg and azathioprine, were put to use. In the fourth period (1990-2020), more immunological progress, including the discovery of anti-MuSK antibodies, was achieved. Videothoracoscopic thymectomy reduced the morbidity and mortality rate associated with surgery. New drugs emerged and clinical trials were performed. Valuable guidelines were published. In the last part of the review, the experience in MG of Istanbul University, a pioneer in Turkey, is related.

Role of thymus on prognosis of myasthenia gravis in Turkish population.

OBJECTIVE:Myasthenia gravis (MG) is an autoimmune disease that may cause a disorder in transmission at the neuromuscular junction. Antibodies directed against acetylcholine receptors are responsible. The thymus is the place that that production of these antibodies mainly occurs. The thymus gland abnormalities and abnormal production of these antibodies are associated with MG. Consequently, thymectomy is a common treatment for MG. The nature of the disease makes it difficult to plan prospective, controlled trials; therefore, there is no current consensus among clinicians on a single algorithm of treatment, and the approach is frequently based on the observations and experiences of experts. The contributions to the literature largely consist of retrospective studies examining an approach to treatment and the effects of thymectomy on prognosis. In this retrospective study, evaluation of Turkish patients with myasthenia gravis was carried out for the importance of thymectomy and effects on prognosis.METHODS:In this study, 93 patients with myasthenia gravis whose followed up at Neuromuscular outpatient clinic between 1998–2018 were evaluated retrospectively. Type of disease, antibody status, treatment, thymectomy, thymus pathology and prognosis were assessed.RESULTS:Thymectomy had been a positive effect on the prognosis of the disease independent of the duration of disease and thymic pathology. The best results had been obtained with early thymectomy with short disease duration, younger age and patients with thymic hyperplasia. Success of therapy was limited with thymoma. With advanced age need for thymectomy was decreased.CONCLUSION:In the present study, evaluation of 93 patients with myasthenia gravis was done retrospectively and it was concluded that thymectomy had a positive effect on prognosis, especially in young patients when performed as early as possible. The most successful results were obtained in cases with thymic hyperplasia.

Anti-acetylcholine receptor antibodies as a diagnostic marker for myasthenia gravis

Background Myasthenia gravis (MG) is a common primary disorder of neuromuscular transmission, which is characterized by fluctuating weakness of a certain voluntary muscles, particularly those innervated by motor nuclei of the brain stem, that is, ocular and mastication muscles, and caused by antibodies binding to components in the neuromuscular junction. Most MG cases demonstrate elevated serum levels of acetylcholine receptor (ACh-R) antibodies, which cause partial or complete inhibition of receptor function and complement-mediated focal lysis of the postsynaptic membrane. ACh-R antibodies are detected in the serum of more than 80–90% patients with generalized MG, in ~50% with ocular myasthenia, and rarely in healthy people. Aims The aim of our study is to determine the value of anti-ACh-R antibodies in diagnosis and prognosis of MG and to evaluate their sensitivity and specificity in comparison between different types of myasthenia. Patients and methods All patients were recruited from the outpatient clinic of Mataria Teaching Hospital on their first visit to neuropsychiatry clinic with suspicious symptoms of MG within 3 months. Our study included 90 women, of whom 60 patients had clinical symptoms suspicious of MG. The patients were classified into five groups: four groups according to Osserman classification and the fifth group included the seronegative patients. Their age ranged from 20 to 30 years. There was another group, the sixth group, which included 30 age-matched healthy controls. All patients were subjected to full history taking, general examination, and neurological examination to facilitate clinical assessment and staging of the disease according to Osserman classification and detection of ACh-R antibodies (binding, modulating and blocking antibodies), prostigmine test, repetitive nerve stimulation, and single-fiber electromyography stimulation tests. Result There was no significant difference between studied groups regarding age. Moreover, we found significant differences between them and the control group regarding the levels of ACh-R antibodies (binding, blocking, and modulating antibodies), but there were no significant differences between them regarding the extent of MG. There is no correlation between the studied groups and the level of acetylcholine, except for a weak positive correlation between the Osserman class and level of blocking receptor antibodies. Receiver operating characteristic curves showed that all the three types of antibodies had poor value for discrimination between MG subgroups. Moreover, there is a significant difference between the studied groups regarding prostigmine, repetitive nerve stimulation, and single fiber electromyography tests. Conclusion ACh-R antibody levels are of major importance as a noninvasive sensitive and specific diagnostic test for different types of myasthenia. However, they had no role in predicting the prognosis of different classes of the disease.

Different neurologic outcomes of myasthenia gravis with thymic hyperplasia and thymoma after extended thymectomy: A single center experience

This study aimed to reveal the clinical course and outcomes of myasthenia gravis (MG) in patients with thymic lymphoid hyperplasia and thymoma undergoing extended thymectomy and to identify the clinical prognostic factors of remission for MG. In total, 73 patients undergoing extended thymectomy were divided into two groups: group A with lymphoid hyperplasia (n=39) and group B with thymoma (n=34). According to the MG Foundation of America (MGFA) post-intervention status, the primary endpoint was a composite measure defined as achievement of complete stable remission (CSR), pharmacologic remission (PR), minimal manifestations (MM) or improvement (IM). The secondary endpoint was CSR. The cumulative probabilities of reaching the primary endpoint were 71.8% in group A and 85.3% in group B (p=0.164), respectively. Using Kaplan-Meier survival analysis, the probability of reaching the primary endpoint in group B was remarkably greater than group A (p=0.036). Cox multivariate analysis indicated that pre-operative MGFA class I (HR: 3.019, 95% CI: 1.084–8.410) and MGFA II (2.665, 95% CI: 1.033–6.873) compared to MGFA III and presence of thymoma (HR: 2.229, 95% CI: 1.079–4.606), showed the most consistent association with remission of MG after thymectomy. Finally, thymic lymphoid hyperplasia and severe symptoms may negatively affect prognosis of MG following thymectomy.

Therapeutic Potential of Docetaxel plus Cisplatin Chemotherapy for Myasthenia Gravis Patients with Metastatic Thymoma.

The prognosis of myasthenia gravis (MG) in association with invasive or metastatic thymoma is usually worse, and therapeutic options are quite limited. Here, we retrospectively reported the therapeutic effect of docetaxel plus cisplatin (docetaxel/cisplatin) chemotherapy in 7 MG patients with metastatic thymoma. Previously, all patients underwent thymectomy at the first onset of thymoma. After the metastasis of thymoma, none of the patients received thymectomy due to unresectable conditions after surgeon's evaluation for great risk of myasthenic crisis (n = 5) or patients' refusal (n = 2). All patients received docetaxel (75 mg/m2) and cisplatin (70 mg/m2) on day 1 (d1) every 21 days, with the cycle ranging from 1 to 4. After docetaxel/cisplatin chemotherapy, one patient achieved partial response, and 6 with stable disease of the tumors. The clinical symptoms of MG were alleviated in all patients, 2 with complete remission and the other 5 with marked improvement. Myelosuppression was the major adverse event, occurring in 2 patients (grade II and IV). MG relapse occurred in one patient during the follow-up. Our study presented a series of MG patients with metastatic thymoma who underwent docetaxel/cisplatin chemotherapy. Besides the improved/stabilized thymoma, markedly improvement of MG with the tolerable adverse events was achieved. Docetaxel/cisplatin chemotherapy appears to be an effective treatment for selected patients with MG in association with unresectable metastatic thymoma. Further follow-up of these patients and additional subjects will be needed to determine whether the therapeutic benefits are durable.

Clinical features of myasthenia gravis with thyroid disease with 106 patients

To report the presentation, clinical course and prognosis of myasthenia gravis (MG) with thyroid disease. Retrospective data analysis was conducted.Between 2004 and 2013, we reviewed a total of 106 patients with MG. We analyzed the clinical features, the relationship between the thyroid function, antibodies and the clinical course, prognosis. (1) In our study, 20/106 (18.87%) patients were thyroid function-abnormal, 37/106 (34.91) were thyroglobulin antibodies (TGAb) and/or thyroid microsomal antibody (TMAb)-positive, and abnormality was observed in 46 (43.40%) of the thyroid gland. Thyroid antibody positive rate was higher than abnormal thyroid function rate, and the difference was significant (P=0.036). (2) The thyroid stimulating hormone (TSH) level ((2.9±4.0) mIU/L) of ocular MG was higher than the level ((1.5±1.1) mIU/L) of generalized MG (P=0.01). (3) The transformation time of 52 ocular type to generalized type was longer in higher antibody group than in normal group (P=0.04). And there were no significant differences between the elevated TSH type and the normal TSH type, the abnormal thyroid function type and normal thyroid function type, the abnormal thyroid type and the normal thyroid type. (4) Comparing the TSH level, total antibody level, TGAb, and TMAb level between the ease group and the unease group in the course of 1 year, 2 years, 5 years, there were no significant differences (all P>0.05). MG is often companied with thyroid abnormalities. MG patients are more susceptible to hashimoto thyroiditis and other autoimmune thyroid diseases. Ocular type patients are more likely to suffer from thyroid function decrease than the generalized type. MG patients with hashimoto thyroiditis and other autoimmune thyroid diseases are more sensitive to respond to therapy means like glucocorticoid therapy, and the short-term prognosis is relatively good. There are no significant correlations between the MG remission rate and TSH level, total antibody level, TGAb and TMAb level.

Myasthenia Gravis and Its Comorbidities

Background: Myasthenia gravis (MG) is an unpredicted neurologic disorder that may cause death; previous reports have failed to find a prognostic marker for the disease. Aim: To determine the impact of comorbidities in patients with Myasthenia gravis. Material and Methods: From January of 2002 to February of 2008 a database was created for patients with Myasthenia gravis (MG). The following variables were studied: age at onset of MG, gender, diabetes mellitus, dyslipidemia, arterial hypertension, dysthyroidism, autoimmune disease, thymectomy procedure and histopathologic result, myasthenic crisis, emergency room visits due to weakness, use and maximum dose of pyridostigmine, prednisone and azathioprine. Results: In a total of 253 patients, we found comorbidities in 73%. The most frequent associated disorders were dyslipidemia, thyroid disease, diabetes, hypertension and other autoimmune conditions. Patients with MG and thymoma, diabetes, dyslipidemia, dysthyroidism and/or hypertension had higher rates of ER visits, myasthenic crises, and required higher doses of drugs. Conclusions: This study demonstrates that comorbidities are frequent in patients with MG (73%) and that they might worsen the prognosis of MG.

Once myasthenic, always myasthenic? observations on the behavior and prognosis of myasthenia gravis in a cohort of 100 patients.

The natural history of myasthenia gravis [MG] is unpredictable: In the first few years the disease course is worst with subsequent gradual disease stabilization. However, some patients tend to have continued disease activity or resurgence of the disease many years into the illness. The factors correlating with disease course need further evaluation. To study the patterns of remissions, worsening and exacerbations in patients with MG and correlate various factors affecting them. Retrospective, Institute Review Board (IRB) approved study in tertiary referral neurology center. Hundred patients with acquired MG confirming the inclusion criteria were studied. Pharmacological remissions, complete stable remissions, exacerbations, worsening episodes were analyzed with respect to age of onset, disease extent, disease severity at onset and worst of illness, acetyl choline receptor antibody positivity, thymectomy status, period of disease, pharmacotherapy and crisis episodes. In this cohort the percentage of new remission rates per year steadily declined after the first year. Ocular myasthenia had lesser clinical worsening episodes and high chance of complete stable remission. Generalized disease had less chance drug free remission. The risk of episodes of worsening persisted at a steady rate over a period of time, being maximum in the first year. The risk of exacerbations was unpredictable and could occur after prolonged clinical quiescence, often was related to reduction of immunosuppression. The disease course did not differ significantly in the juvenile and adult age-groups. There was a strong case for permanent immunomodulation in MG.

Myasthenia gravis – treatment of acute severe exacerbations in the intensive care unit results in a favourable long‐term prognosis

Acute severe exacerbations of myasthenia gravis (MG) are common in both early and late onset MG. We wished to examine the current management in the intensive care unit (ICU) of severe exacerbations of MG and to study the long-term prognosis of MG following discharge from the ICU. We retrospectively reviewed the medical records of all patients admitted to a specialist neuro-ICU with acute exacerbations of MG over a 12-year period. We identified 38 patients. Over 60% were over the age of 50 years, and MG was newly diagnosed in over 40%. Intubation was required in 63%, and over 90% of patients were treated with prednisolone and/or intravenous immunoglobulin. Four patients died in hospital. The remainder of patients were followed up for a mean of 4 years, and the majority were either asymptomatic or had mild symptoms of MG at clinical review. Despite the significant morbidity and mortality associated with severe exacerbations of MG, specialized neurointensive care can result in a good long-term prognosis in both early- and late-onset MG.

Myasthenia, Spondylitis and Enterococcus faecalis Endocarditis

Parasympathomimetics, immunosuppression and plasmapheresis have considerably improved management and prognosis of myasthenia gravis. Side effects of these measures, however, may complicate the course of the disease. In a 66-year-old male with myasthenia gravis and lower back pain, blood cultures, echocardiography and magnetic resonance imaging led to the diagnosis of endocarditis and spondylodiscitis. Enterococcus faecalis grew in the blood cultures as well as on the aortic and tricuspid valve vegetations which were resected during cardiac surgery. Possible sources of the infection might be E. faecalis infections of catheter tips during a 46-day stay in the intensive care unit 11months earlier where he had undergone plasmapheresis, hemodiafiltration and mechanical ventilation, or recurrent diarrheas since 18months. Infection was favored by immunosuppression with glucocorticoids and azathioprine which received the patient because of myasthenia gravis and hypothyroidism. Patients with myasthenia gravis require close follow up, including infection parameters, especially when they receive immunosuppressive therapy and when microorganisms known to cause endocarditis, are identified.