Myasthenic Patients Research Articles

Comparative Analysis of Intravenous Immunoglobulins (IVIg) vs Plasmapheresis (PLEX) in the Management of Myasthenic Crisis.

Myasthenia gravis (MG) is an autoimmune disorder affecting postsynaptic membranes in neuromuscular junctions, presenting as fatigable muscle weakness. Myasthenic crisisis a life-threatening complication characterized by severe respiratory insufficiency necessitating invasive or noninvasive ventilation. Two rapid therapiesused to manage myasthenic crisesare intravenous immunoglobulins (IVIg) and plasmapheresis (PLEX). Their comparative effectiveness remains equivocal. Our article examines evidence from several clinical trials and observational studies, in order to determine the superiority of one treatment over the other. Multiple factors can complicate the choices between two treatments. We concluded that the choice between PLEX and IVIg is multifaceted, guided by individual patient characteristics, institutional resources, and clinician preference. While PLEX can be consideredasfirst-linefor rapid clinical outcomes, it is hard to pick one treatment over the other, and careful consideration of comorbiditiesand resource availability is crucial. Our article highlights the need for further research to establish definitive guidelines and enhance patient outcomes in myasthenic crisis patients.

Myasthenia Gravis Classmates Move Forward with Strength: Holistic and Perennial Care for Myasthenic Patients

<p>新光醫院有感於全方位醫療新時代來臨，神經科積極研究治療方法，為提升醫療品質及促進醫病溝通，結合病人與家屬共同參與，在1992年創院即成立「肌無力症中心」，在院長及副院長領導下，以「醫療服務、醫學研究、支持培力」三大目標呼應聯合國永續發展目標(SDGs)中之目標3「健康與福祉」、目標4「優質教育」，以及目標17「多元夥伴建立」，由神經科主持及社會服務室組織病人及家屬成立「肌無力症俱樂部」，至今服務3,415位病人，服務涵蓋率超過全國半數以上。歷年培力98位病人與家屬成為關懷幹部協力照護，將診斷治療乃至情緒管理與社會復健進行垂直整合，提供身心靈三方面完整照護，使病人可以順利回到家庭、甚至挑戰自我事業及體力高峰，增進健康生活與福祉。發行肌無力症衛教刊物，提供病人優質疾病教育；致力於聯結全國服務，培力彰化秀傳醫院成立肌無力症中心及臺灣肌無力症關懷協會，結盟高雄長庚醫院辦理醫病交流；積極與世界接軌獲邀參與中國大陸及日本醫病交流活動，建立夥伴關係鼓舞MG（重症肌無力MyastheniaGravis）同學有力向前行。</p> <p> </p><p>Since its establishment in 1992, Shin Kong Wu Ho-Su Memorial Hospital has pioneered the creation of the Center for Myasthenia Gravis, which is dedicated to providing comprehensive care for patients with myasthenia gravis. Our approach integrates diagnosis, treatment, emotional support, and social rehabilitation, ensuring holistic care for individuals coping with this condition. The center’s activities align with Sustainable Development Goals established by the United Nations, particularly Sustainable Development Goals 3 (Good Health and Well-being), 4 (Quality Education), and 17 (Partnerships). The center strives to support the successful reintegration of patients into their families, workplaces, and communities, enabling them to thrive to the fullest extent possible. The center has cared for more than 50% of patients with myasthenia gravis nationwide, providing assistance to 3,415 individuals. Moreover, the center has empowered 98 patients and their families to take on leadership roles within the center’s support network. Additionally, the center has published materials dedicated to myasthenia gravis, disseminating valuable information to those in need. Furthermore, the center has extended its reach beyond its immediate vicinity, collaborating with Changhua Show Chwan Hospital to establish a myasthenia gravis club and support group. To foster global connections, the center has organized international patient exchange activities with counterparts in China and Japan, fostering knowledge sharing and solidarity within the global myasthenia gravis community.</p> <p> </p>

Autoimmune and Non-Autoimmune Comorbidities in Myasthenic Patients of East-European Descent: A Case-Control Study.

Background: As the life expectancy of patients with myasthenia gravis (MG) is improving, so the number of comorbidities continues to rise, with a potentially significant impact on the overall morbidity and mortality. The main aim of the study was to assess comorbidities of MG in a group of patients of East-European descent. Methods: We retrospectively compared 185 adult myasthenic patients with 895 sex- and age-matched controls, admitted from January 2013 to December 2021. Results: Of these patients, 60% had late-onset MG (LOMG), with a clear predominance of women in both the LOMG and early-onset (EOMG) types; and 23.8% of the patients had a radiological description consistent with thymoma. All myasthenic patients had at least one comorbidity; 20 (10.8%) of the patients associated at least one autoimmune comorbidity. Obesity (p < 0.01), type 2 diabetes (p < 0.0001), cerebrovascular diseases (p < 0.0001), essential hypertension (p < 0.01), and cardiac arrythmias (p < 0.0001) were more frequent in patients than in the control group. The granulocyte-to-lymphocyte ratio was higher in the myasthenic patients compared to the controls (p < 0.01 for LOMG). Discussion: We, thus, suggest a common chronic low-grade inflammatory background as a possible connection between MG subtypes and some of these apparently unconnected comorbidities. Conclusions: The East-European origin of the patients offered a different social and cultural angle of a disease studied mainly on populations of West-European and Asian descent.

National Multicenter Study on the Comparison of Robotic and Open Thymectomy for Thymic Neoplasms in Myasthenic Patients: Surgical, Neurological and Oncological Outcomes.

Thymectomy is the gold standard in the treatment of thymic neoplasm and plays a key role in the therapeutic path of myasthenia gravis. For years, sternotomy has been the traditional approach for removing anterior mediastinal lesions, although the robotic thymectomy is now widely performed. The literature is still lacking in papers comparing the two approaches and evaluating long-term oncological and neurological outcomes. This study aims to analyze the postoperative results of open and robotic thymectomy for thymic neoplasms in myasthenic patients. Surgical, oncological and neurological data of myasthenic patients affected by thymic neoplasms and surgically treated with extended thymectomy, both with the open and the robotic approach, in six Italian Thoracic Centers between 2011 and 2021 were evaluated. A total of 213 patients were enrolled in the study: 110 (51.6%) were treated with the open approach, and 103 (48.4%) were treated with robotic surgery. The open surgery, compared with the robotic, presented a shorter operating time (p < 0.001), a higher number of postoperative complications (p = 0.038) and longer postoperative hospitalization (p = 0.006). No other differences were observed in terms of surgical, oncological or neurological outcomes. The robotic approach can be considered safe and feasible, comparable to the open technique, in terms of surgical, oncological and neurological outcomes.

Long-term mechanical ventilation in a myasthenic patient with suspected obstructive sleep apnea

Myasthenic crisis is a medical emergency with a very poor prognosis if not treated early and appropriately. In the absence of mechanical ventilation, the respiratory impairment is the most serious aspect of the condition, leading the intensive care anaesthetist to resort to intubation. The association of this disease with sleep apnea syndrome is frequent but little-known, and can make it difficult to disconnect the patient from the ventilator. Making the diagnosis of sleep apnea syndrome is difficult because of polysomnography, which is laborious to set up in intensive care, but can be facilitated by the use of a simple questionnaire when there is a strong clinical suspicion. Weaning a patient suffering from neuromuscular disease, whether or not associated with sleep apnea syndrome from mechanical ventilation can be a complex task. When extubation proves impossible, tracheostomy remains the most desirable alternative. The introduction of long-term mechanical ventilation at home is an interesting solution for shortening the long hospital stay of patients in intensive care units, and significantly reduces the risk of infection, morbidity and mortality.

Triple-seronegative myasthenia gravis: clinical and epidemiological characteristics.

Myasthenia gravis (MG) is an autoimmune disease usually caused by antibodies against the acetylcholine receptor (AChR-Abs), muscle-specific tyrosine kinase (MuSK-Abs), or low-density lipoprotein receptor-related protein 4 (LRP4-Abs). However, there are MG patients who do not have these antibodies and are thus said to have triple-seronegative (triple-SN) MG. This study aims to describe the frequency and clinical and epidemiological characteristics of patients with triple-SN MG. This was a retrospective cross-sectional study carried out through the analysis of medical records. Descriptive and analytical statistical analysis was performed comparing subgroups of myasthenic patients, classified according to serological profile. The sample population consisted of 93 MG patients: 85 were positive for antibodies, 80 (86%) with AChR-Abs, 5 (5.4%) with MuSK-Abs, and no MG patients with LRP4-Abs. Eight patients (8.6%) had triple-SN MG; they had a median age at disease onset of 30 years (21-45). Their most common initial symptoms were ptosis, diplopia, and generalized weakness. Most patients presented with mild symptoms at their last visit, reflecting a median MG composite scale score of 4 (0-6), and 75% of patients had an adequate response to treatment. Our study showed a low frequency of triple-SN MG in Brazilian MG patients. Triple-SN MG was predominant in females, who presented with ptosis, diplopia, and generalized weakness, and most patients had an adequate response to immunosuppressive treatment. There was no significant difference between triple-SN MG and the other subgroups.

Unique genotypic pattern in Indian DPAGT1 congenital myasthenic syndrome patients with two likely founder mutations

Unique genotypic pattern in Indian DPAGT1 congenital myasthenic syndrome patients with two likely founder mutations

Congenital myasthenic syndromes: a retrospective natural history study of respiratory outcomes in a single centre.

Respiratory problems are a major cause of morbidity and mortality in patients with congenital myasthenic syndromes, a rare heterogeneous group of neuromuscular disorders caused by genetic defects impacting the structure and function of the neuromuscular junction. Recurrent, life-threatening episodic apnoea in early infancy and childhood and progressive respiratory failure requiring ventilation are features of certain genotypes of congenital myasthenic syndromes. Robb et al. published empirical guidance on respiratory management of the congenital myasthenic syndromes, but other than this workshop report, there are little published longitudinal natural history data on respiratory outcomes of these disorders. We report a retrospective, single-centre study on respiratory outcomes in a cohort of 40 well characterized genetically confirmed cases of congenital myasthenic syndromes, including 10 distinct subtypes (DOK7, COLQ, RAPSN, CHAT, CHRNA1, CHRNG, COL13A1, CHRNE, CHRNE fast channel syndrome and CHRNA1 slow channel syndrome), with many followed up over 20 years in our centre. A quantitative and longitudinal analysis of key spirometry and sleep study parameters, as well as a description of historical hospital admissions for respiratory decompensation, provides a snapshot of the respiratory trajectory of congenital myasthenic syndrome patients based on genotype.

Propofol TCI or sevoflurane anesthesia without muscle relaxant for thoracoscopic thymectomy in myasthenia gravis patients: a prospective, observational study

BackgroundMyasthenia gravis (MG) patients interact unpredictably with anesthetic agents, including neuromuscular blocking agents. Here, we investigate the effectiveness of general anesthesia without muscle relaxants using either propofol via target-controlled infusion systems (TCI) or sevoflurane in MG patients undergoing thoracoscopic thymectomy.MethodsThis prospective, open-label, observational study was conducted in a university hospital. We included 90 myasthenic patients undergoing thoracoscopic thymectomy with general anesthesia. Patients received induction and maintenance anesthesia with propofol TCI (group P, n = 45) or induction with propofol 2–3 mg.kg−1 and maintenance anesthesia with sevoflurane (group S, n = 45). In both groups, the procedure was performed under the guidance of entropy with sufentanil but not a muscle relaxant. Intubation conditions, hemodynamic changes, respiratory function, neuromuscular transmission, arterial blood gas, and complications were evaluated.ResultsAll patients achieved good intubation conditions. Hemodynamic instability was more frequent in group S than in group P, mostly in the induction stage, and was controllable. The reduction in the intraoperative train-of-four ratio from baseline at 30 min, 60 min, and 90 min in group S was 10.3%, 14.2%, and 14.3%, respectively, significantly higher than that in group P (6.8%, 7.2%, and 8.4%, respectively), which completely recovered at the end of the surgery. All patients were extubated in the operating room without complications. No other significant differences between the groups were observed.ConclusionsAnesthesia with propofol TCI or sevoflurane without muscle relaxants in MG patients offered safe and effective conditions for thoracoscopic thymectomy. Sevoflurane achieved higher levels of intraoperative muscular relaxation than propofol TCI. Postoperative neuromuscular function was not affected by these anesthetics.

Clinical and genetic characterisation of a large Indian congenital myasthenic syndrome cohort.

Congenital myasthenic syndromes (CMS) are a rare group of inherited disorders caused by gene defects associated with the neuromuscular junction and potentially treatable with commonly available medications such as acetylcholinesterase inhibitors and β2 adrenergic receptor agonists. In this study, we identified and genetically characterized the largest cohort of CMS patients from India to date. Genetic testing of clinically suspected patients evaluated in a South Indian hospital during the period 2014-19 was carried out by standard diagnostic gene panel testing or using a two-step method that included hotspot screening followed by whole-exome sequencing. In total, 156 genetically diagnosed patients (141 families) were characterized and the mutational spectrum and genotype-phenotype correlation described. Overall, 87 males and 69 females were evaluated, with the age of onset ranging from congenital to fourth decade (mean 6.6 ± 9.8 years). The mean age at diagnosis was 19 ± 12.8 (1-56 years), with a mean diagnostic delay of 12.5 ± 9.9 (0-49 years). Disease-causing variants in 17 CMS-associated genes were identified in 132 families (93.6%), while in nine families (6.4%), variants in genes not associated with CMS were found. Overall, postsynaptic defects were most common (62.4%), followed by glycosylation defects (21.3%), synaptic basal lamina genes (4.3%) and presynaptic defects (2.8%). Other genes found to cause neuromuscular junction defects (DES, TEFM) in our cohort accounted for 2.8%. Among the individual CMS genes, the most commonly affected gene was CHRNE (39.4%), followed by DOK7 (14.4%), DPAGT1 (9.8%), GFPT1 (7.6%), MUSK (6.1%), GMPPB (5.3%) and COLQ (4.5%). We identified 22 recurrent variants in this study, out of which eight were found to be geographically specific to the Indian subcontinent. Apart from the known common CHRNE variants p.E443Kfs*64 (11.4%) and DOK7 p.A378Sfs*30 (9.3%), we identified seven novel recurrent variants specific to this cohort, including DPAGT1 p.T380I and DES c.1023+5G>A, for which founder haplotypes are suspected. This study highlights the geographic differences in the frequencies of various causative CMS genes and underlines the increasing significance of glycosylation genes (DPAGT1, GFPT1 and GMPPB) as a cause of neuromuscular junction defects. Myopathy and muscular dystrophy genes such as GMPPB and DES, presenting as gradually progressive limb girdle CMS, expand the phenotypic spectrum. The novel genes MACF1 and TEFM identified in this cohort add to the expanding list of genes with new mechanisms causing neuromuscular junction defects.

The change of systemic inflammation response index in the treatment of patients with myasthenia gravis undergoing thymectomy: A retrospective, follow-up study

Background: This study aims to investigate the role of neutrophil-tolymphocyte ratio, platelet-to-lymphocyte ratio, monocyte-to-lymphocyte ratio, and systemic inflammation response index in patients with myasthenia gravis, thymomas and thymic hyperplasia and to identify the relationship between the inflammation response and disease activity. Methods: Between January 2010 and December 2018, a total of 97 patients (71 males, 26 females; mean age: 36.7±16.3 years; range, 15 to 76 years) who underwent extended thymectomy with the diagnosis of myasthenia gravis were retrospectively analyzed. The patients were divided into two groups as the patient group (n=42) and the control group (n=55). Neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio, monocyteto-lymphocyte ratio, and systemic inflammation response index were measured one day prior to and one month after surgery. Results: The patients with thymoma were older with a higher mean pre-systemic inflammation response index value. Preoperative systemic inflammation response index, neutrophil-to-lymphocyte ratio, and monocyte-to-lymphocyte ratio were significantly higher in patients with thymoma. A preoperative systemic inflammation response index value of less than 0.62 was accepted to indicate thymic hyperplasia and a postoperative systemic inflammation response index value higher than 2.94 was indicative of thymoma. In myasthenic patients whose steroid dose was increased and/or remained the same at the first month after surgery, postoperative monocyte-to-lymphocyte ratio and systemic inflammation response index values were found to be higher compared to preoperative values (p=0.006 and p=0.032, respectively). Patients whose pyridostigmine dose was increased and/or remained the same had significantly higher systemic inflammation response index values postoperatively (p=0.029). Conclusion: The precise cut-off values of systemic inflammation response index may be helpful for the surgeon to predict the surgical outcome and post-systemic inflammation response index may be a predictive marker for estimating postoperative treatment changes.

COVID-19 AND MYASTHENIA: A CASE REPORT AND REVIEW OF MANAGEMENT STRATEGIES

Background: The COVID-19 pandemic poses unique challenges in managing patients with pre-existing autoimmune disorders, such as myasthenia gravis. This article presents a case report of a myasthenic patient who contracted COVID-19, providing insights into the clinical presentation, diagnostic workup, treatment approaches, and disease course. Methods: A comprehensive review of the literature was conducted to examine the current understanding of the interaction between myasthenia gravis and COVID-19. The focus was on the challenges faced in managing these patients and the optimal therapeutic strategies. Results: The case report describes a 42-year-old male with confirmed myasthenia gravis who presented with sudden-onset respiratory distress and flu-like symptoms. The patients clinical course, including the utilization of immunomodulatory therapies and respiratory support, is discussed. The review of the literature highlights the increased risk of severe infection in myasthenic patients, the potential exacerbation of myasthenic symptoms, and the impact of immunosuppressive treatments on the course of COVID-19. Conclusion: The management of myasthenia gravis patients with COVID-19 requires a tailored approach that considers the delicate balance between controlling the viral infection and preserving neuromuscular function. Early recognition, prompt diagnosis, and the integration of multidisciplinary care are crucial for optimizing outcomes in this patient population. Further research is needed to enhance our understanding of the underlying mechanisms and refine management strategies for these complex cases.

Comparing the Impact of COVID-19 on Vaccinated and Unvaccinated Patients Affected by Myasthenia Gravis.

We evaluated 13 patients affected by myasthenia gravis (MG) who had coronavirus disease 2019 (COVID-19) before vaccination and 14 myasthenic patients who contracted severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) infection after vaccination to evaluate factors related to different COVID-19 outcomes. We compared the two groups' previous stability of MG and the severity of SARS-CoV-2 infection. Vaccinated and non-vaccinated patients were comparable in terms of severity of the previous MG course (mean maximum myasthenia gravis Foundation of America-MGFA-Class III) and during SARS-CoV-2 infection (mean MGFA Class II). In non-vaccinated patients, the hospitalization and severe course percentages were 61.5%, while the mortality reached 30.8%. The hospitalization, severe course, and mortality percentages in vaccinated patients were 7.1%. In deceased, non-vaccinated patients, greater myasthenia severity in the past clinical history, but not at the time of infection, was observed. Similarly, older age at MG onset and at the time of infection correlated with a more severe COVID-19 course in non-vaccinated patients (p = 0.03 and p = 0.04), but not in the group of vaccinated patients. In summary, our data support a protective role of vaccination in myasthenic patients, even if anti-CD20 therapy might be associated with a poor immune response to vaccines.

Rare case of double seronegative ocular myasthenia gravis and non-functional pituitary macroadenoma

Chronic autoimmune neuromuscular disorder known as Myasthenia Gravis (MG). The relationship of myasthenia gravis with pituitary adenomas is relatively uncommon, despite the fact that it is widely known that people with the condition have a higher prevalence of other autoimmune diseases. Here, we describe the case of a 45-year-old Indian man who had complained of severe cephalgia, associated with ocular pain, dizziness, diplopia, ptosis of the unilateral right eye. Although he has a history of severe headaches that started prior to 3 months, his complaints became more obvious at the end of the day. An MRI of the brain revealed an increased pituitary gland that measured 13.7 × 15.5 x 16. 9 mm. Furthermore, post admission histological examinations supported the diagnosis of a double seronegative ocular myasthenia gravis and non-functional pituitary macroadenoma. Following treatment, it was discovered that Pyridostigmin and Prednisolone considerably reduced his myasthenia symptoms. Finally, it should be noted that pituitary tumours are one potential underlying cause of headache in myasthenic patients.

Outcomes after robotic thymectomy in nonthymomatous versus thymomatous patients with acetylcholine-receptor-antibody-associated myasthenia gravis

The aim of this study was to investigate the surgical and long-term neurological outcomes of patients with acetylcholine-receptor-antibody-associated myasthenia gravis (AChR-MG) who underwent robotic thymectomy (RATS). We retrospectively analyzed the clinical-pathological data of all patients with AChR-MG who underwent RATS using the DaVinci® Robotic System at the MUMC+ between April 2004 and December 2018. Follow-up data were collected from 60 referring Dutch hospitals. In total, 230 myasthenic patients including 76 patients with a thymoma (33.0%) were enrolled in this study. Mean follow-up time, procedure time and hospitalization were, respectively 65.7 ± 43.1 months, 111±52.5 min and 3.3 ± 2.2 days. Thymomatous patients had significantly more frequently and more severe complications than nonthymomatous patients (18.4% vs. 3.9%, p<0.001). Follow up data was available in 71.7% of the included patients. The Myasthenia Gravis Foundation of America postintervention score showed any kind of improvement of MG-symptoms after RATS in 82.4% of the patients. Complete stable remission (CSR) or pharmacological remission (PR) of MG was observed in 8.4% and 39.4% of the patients, respectively. Mean time till CSR/PR remission after thymectomy was 26.2 ± 29.2 months. No statistical difference was found in remission or improvement in MGFA scale between thymomatous and nonthymomatous patients. RATS is safe and feasible in patients with MG. The majority of the patients (82.4%) improved after thymectomy. CSR and PR were observed in 8.4% and 39.4% of the patients, respectively, with a mean of 26.2 months after thymectomy. Thymomatous patients had more frequently and more severe complications compared to nonthymomatous patients.

Hypothyroidism and myasthenia gravis: A rare association

The prevalence of endocrinopathies during myasthenia is relatively common. The association of myasthenia with dysthyroidism can make the diagnosis of these two conditions difficult, because of the similaritý of some clinical signs. Several disorders of specifically thyroid immunologic origin have beeń reported in myasthenic patients. This relationship remains poorly elucidated, but an immunologic cross-reaction between the neuromuscular junction and thyroid components has been found in myasthenia and Graves' disease. It is generally accepted that the association between hyperthyroidism and myasthenia is much more frequent than that between myasthenia and hypothyroidism. However, no clear explanation has been proposed for this difference. We report the case of a 28-year-old patient who was initially followed for myasthenia under anticholinesterase therapy and whose evolution was marked by the discovery of hypothyroidism during follow-up. The patient responded well to replacement therapy with a good evolution.

Myasthenia gravis in the family doctor’s practice

Myasthenia gravis is an autoimmune disorder of the neu­ro­mus­cu­lar junction, mediated by autoantibodies against the nicotinic acetylcholine receptor and muscle-specific ty­ro­sine kinase. A characteristic feature of myasthenia gravis is muscle weakness/fatigue, which worsens on physical exer­tion, sometimes minimal, sometimes prolonged. The cli­ni­cal forms, symptomatology, associated diseases, cli­ni­cal evolution and treatment are important aspects, wor­thy of the family doctor’s attention, given the fact that myas­the­nia is a real diagnostic trap, sometimes even for the neurologist. This presentation summarizes the main as­pects that any family doctor must take into account when dea­ling with a myasthenic patient.

Guideline for the management of myasthenic syndromes.

Myasthenia gravis (MG), Lambert-Eaton myasthenic syndrome (LEMS), and congenital myasthenic syndromes (CMS) represent an etiologically heterogeneous group of (very) rare chronic diseases. MG and LEMS have an autoimmune-mediated etiology, while CMS are genetic disorders. A (strain dependent) muscle weakness due to neuromuscular transmission disorder is a common feature. Generalized MG requires increasingly differentiated therapeutic strategies that consider the enormous therapeutic developments of recent years. To include the newest therapy recommendations, a comprehensive update of the available German-language guideline 'Diagnostics and therapy of myasthenic syndromes' has been published by the German Neurological society with the aid of an interdisciplinary expert panel. This paper is an adapted translation of the updated and partly newly developed treatment guideline. It defines the rapid achievement of complete disease control in myasthenic patients as a central treatment goal. The use of standard therapies, as well as modern immunotherapeutics, is subject to a staged regimen that takes into account autoantibody status and disease activity. With the advent of modern, fast-acting immunomodulators, disease activity assessment has become pivotal and requires evaluation of the clinical course, including severity and required therapies. Applying MG-specific scores and classifications such as Myasthenia Gravis Activities of Daily Living, Quantitative Myasthenia Gravis, and Myasthenia Gravis Foundation of America allows differentiation between mild/moderate and (highly) active (including refractory) disease. Therapy decisions must consider age, thymic pathology, antibody status, and disease activity. Glucocorticosteroids and the classical immunosuppressants (primarily azathioprine) are the basic immunotherapeutics to treat mild/moderate to (highly) active generalized MG/young MG and ocular MG. Thymectomy is indicated as a treatment for thymoma-associated MG and generalized MG with acetylcholine receptor antibody (AChR-Ab)-positive status. In (highly) active generalized MG, complement inhibitors (currently eculizumab and ravulizumab) or neonatal Fc receptor modulators (currently efgartigimod) are recommended for AChR-Ab-positive status and rituximab for muscle-specific receptor tyrosine kinase (MuSK)-Ab-positive status. Specific treatment for myasthenic crises requires plasmapheresis, immunoadsorption, or IVIG. Specific aspects of ocular, juvenile, and congenital myasthenia are highlighted. The guideline will be further developed based on new study results for other immunomodulators and biomarkers that aid the accurate measurement of disease activity.

Cholesterol Management in Neurology: Time for Revised Strategies?

Statin therapy has been extensively evaluated and shown to reduce the incidence of new or recurrent vascular events, ischemic stroke included. As a consequence, each published guideline pushes for lower low-density cholesterol levels in the population at large, recommending increased statin doses and/or adding new cholesterol-lowering molecules. Neurologists find it sometimes difficult to apply these guidelines, having to confront situations such as (1) ischemic strokes, mainly cardioembolic ones, in patients with already low LDL-cholesterol levels; (2) myasthenic patients, whose lifespan has been extended by available treatment, and whose age and cholesterol levels put them at risk for ischemic stroke; (3) patients with myotonic dystrophy, whose disease often associates diabetes mellitus and heart conduction defects, and in whom blood cholesterol management is also not settled. As such, further trials are needed to address these issues.

Characteristics of Repetitive Nerve Stimulation (Rns) and Correlation with Severity and Quality of Life of Myasthenia Gravis Patients

Background: Myasthenia gravis is a rare autoimmune disease due to neuromuscular junction (NMJ) disorders. The diagnosis of myasthenia gravis is based on cardinal clinical symptoms, including fluctuating weakness, worsening with activity, and improving with rest. In addition to clinical signs and antibody tests, other tests that are also crucial for diagnosis are electrodiagnostic examination (EDX) such as repetitive nerve stimulation (RNS) and single fibre electromyography (SFEMG). Recent studies have focused on assessing patients' severity and quality of life, essential for determining the prognosis and subsequent treatment plans. Objective: To evaluate the characteristic of RNS and its correlation with rigor and quality of life of myasthenia gravis patients. Methods: Inclusion criteria: patients diagnosed with myasthenia gravis, aged 18-65 years, willing to participate in the study. Exclusion criteria include a history of neuropathy, motor neuron disease, or myopathy and dropping out of the study. Patient demographic data were collected. The results of the RNS examination were divided into two categories, normal and abnormal. MG severity was assessed by the Myasthenia gravis foundation of America (MGFA) classification, and the patient's quality of life was assessed by Myasthenia Gravis Quality of Life 15 (MG-QOL 15). Results: Of the 24 samples, 69.1% of RNS were positive. The most sensitive muscle for assessing RNS is the anconeus muscle, followed by the trapezius and nasal. There was no significant relationship between RNS features and severity and the quality of life of myasthenic Gravis patients. Conclusion: Onconeus muscle is the best location for assessing RNS. Although the number of patients with normal RNS had lower severity and better quality of life, there was no statistically significant relationship. Further studies with larger samples and longer follow-ups are needed.