Treatment Of Myasthenia Gravis Research Articles

Neurology: what's new in 2024

In 2024, therapeutic and diagnostic advancements are shaping the field of neurology. Three new drugs show promise for treating myasthenia gravis and chronic inflammatory demyelinating polyneuropathy. A new classification for Parkinson's disease has been proposed, while a neuroprosthesis is improving gait in advanced stages. Revised criteria for inclusion body myositis and multiple sclerosis are refining diagnoses, and blood biomarkers are enabling early detection of Alzheimer's disease. The effectiveness of early thrombolysis in stroke has been confirmed, and new treatments are being explored for cerebral hemorrhages. In epilepsy, surgery reduces mortality by 30% in drug-resistant patients. Finally, new treatments targeting PACAP and CGRP are reducing refractory migraines with good tolerability.

Immunglobulınler ve Myastenia Gravıs: Geleneksel Derleme

Myasthenia Gravis is an autoimmune disease characterised by the development of antibodies against proteins in the neuromuscular junction, resulting in complaints such as fatigue and muscle weakness. Quality of life is adversely affected due to complaints such as droopy eyelids (ptosis), blurred or double vision, difficulty in speaking, difficulty in swallowing and breathing. It causes morbidity and mortality due to myasthenic crisis and subsequent complications. Intravenous immunoglobulin therapy, plasmapheresis, anticholinesterase drugs, immunosuppressant drugs or thymectomy surgery can be applied in the treatment of myasthenia gravis, which can lead to serious permanent damage and rarely fatal consequences. Treatment preference varies according to the course of the disease. In this review, pharmacokinetics, administration procedures, adverse events, patient variables and cost considerations of intravenous immunoglobulin (IVIg) in myasthenia gravis will be discussed.

Medical costs of treating myasthenia gravis in patients who need intravenous immunoglobulin (IVIg) – a register-based study

BackgroundSeveral innovative treatments are expected for myasthenia gravis (MG) in the coming years. Healthcare payers usually require cost-effectiveness analyses before reimbursement. We aimed to investigate resource utilization and direct medical costs for patients with MG treated with intravenous immunoglobulin (IVIg) to inform such analyses.MethodsWe identified patients with MG in the Norwegian Patient Registry based on at least two hospital encounters with an MG diagnosis (ICD-10 G70.0) from 1 Jan 2010 to 31 Dec 2021. IVIg treatment was identified by medical procedure and Anatomical Therapeutic Chemical (ATC) codes (RPGM05 and J06BA02). Using Diagnosis-Related Group (DRG) cost weights, we estimated direct medical costs for each year following the first MG diagnosis.ResultsOver the study period, 1083 patients were diagnosed with MG in Norway, of whom 155 (14.3%) were treated with IVIg. No significant differences in age or sex were observed between IVIg and non-IVIg patients. Compared with non-IVIg patients, IVIg-patients had 2.3 times higher direct medical costs during the first year after MG diagnosis (EUR 35,714 vs. EUR 15,457) and 3.1 times higher costs during the second year (EUR 19,119 vs. EUR 6256). In the fifth year after diagnosis, IVIg-patients still had higher costs and resource utilization than non-IVIg patients (EUR 9953 vs. EUR 5634).ConclusionIVIg treatment represents an important marker for high direct medical costs among patients with MG. The costs continue to be high during the first five years after MG diagnosis.

The clinical significance of open vs. minimally invasive surgical approaches in the management of thymic epithelial tumors and myasthenia gravis.

Though advancements have been made in the pharmacologic treatment of myasthenia gravis (MG), surgical resection is not only an option as a last line of defense for those patients who do not respond to medical therapy but also remains vital for those with thymic epithelial tumors (TET). While prior studies have shown the potential superiority of minimally invasive approaches via robotic- and video-assisted thoracoscopic surgery (RATS/VATS) for thymectomy compared to open surgery, in the setting of malignancies, this outcome delineation is controversial. As RATS/VATS may be associated with less post-operative complications in the treatment of TET, some surgeons argue that the open approach is necessary for complete resection (R0 resection) and to prevent potential seeding of the malignancy. In this review article, we will compare the efficacy and implications of the different surgical approaches and techniques themselves in performing a thymectomy for autoimmune and oncologic pathologies.

Persistent symptoms, exacerbations and drug side effects despite treatment in myasthenia gravis.

Generalized myasthenia gravis (gMG) is characterized by fluctuating muscle weakness. Exacerbation frequency, adverse events (AEs) related to immunosuppressant therapy and healthcare resource utilization (HCRU) are not well understood. Our study aimed to describe long-term clinical outcomes, drug-related AEs and estimated HCRU in gMG patients. This was a retrospective cohort analysis of clinical data from patients with gMG followed-up over eight consecutive years in a Spanish referral unit. Myasthenia Gravis Foundation of America (MGFA) clinical classification, MGFA post-interventional status (MGFA-PIS), Myasthenia Gravis Activities of Daily Living (MG-ADL) score, exacerbations, MG crises, therapies, AEs reported, specialist consultations and emergency room visits were studied biannually. An estimation of HRCU was made based on these data. Some 220 patients newly diagnosed with gMG were included. Ninety percent were seropositive (84.5% anti-acetylcholine receptor [AChR], 5.9% anti-muscle-specific kinase [MuSK]). Baseline mean MG-ADL score was 5.04 points (SD 3.17), improving to 0.7 points (SD 1.40) after 8 years. Exacerbations were more frequent in years 1-2 (30.1%) but still occurred in years 7-8 (20.2%). Myasthenic crisis frequency remained 1% in years 7-8. Eighty-nine percent achieved MGFA-PIS minimal manifestations or better at 8 years. Fifty-one percent of patients reported at least one AE during the study period, leading to drug withdrawal in approximately 20% of cases. HCRU decreased between years 1-2 to years 7-8 with an estimated cost of MG from 8074.19 € per patient/year to 1679.46 €, respectively. There is a group of MG patients that suffers from persistent symptoms and exacerbations (11%-20%) or MG crises, and drug AEs, which may increase disease burden and impact on the healthcare system.

Surgical treatment of thymic epithelial tumor and myasthenia gravis.

Thymic epithelial tumors originate from the epithelial cells of the thymus and are typically diagnosed during the 5th and 6th decades of life. The incidence is consistent between men and women, averaging 1.7 cases per year. Thymomas, neuroendocrine tumors, and thymic carcinomas are subtypes of thymic epithelial tumors, with thymomas being the most prevalent (75%-80%) and thymic carcinomas following at 15%-20%. Thymoma and thymic carcinoma exhibit distinct disease courses; thymomas grow slowly and are confined to the thymus, while thymic carcinomas demonstrate rapid growth and metastasis. Overall survival rates vary, with a 78% 5-year survival rate for thymoma and a 30% rate for thymic carcinoma. Thymic epithelial tumors may be linked to paraneoplastic autoimmune diseases, including myasthenia gravis, hypogammaglobulinemia, pure red cell aplasia, Cushing's syndrome, systemic lupus erythematosus, and polymyositis. Staging of thymic epithelial tumors can be done according to Masaoka-Koga and/or TNM 8th staging systems. The treatment algorithm is primarily determined by resectability, with surgery (Extended Thymectomy) serving as the foundational treatment for early-stage patients (TNM stage I-IIIA, Masaoka-Koga stage I-III). Adjuvant radiotherapy or chemotherapy may be considered following surgery. In advanced or metastatic cases, chemotherapy is the first-line treatment, followed by surgery and radiotherapy for local control. Myasthenia gravis, an autoimmune disease presents with progressive muscle fatigue and diplopia. Positive antibodies (Anti-AChR, Anti-MuSK, LRP4) and electromyography aid in diagnosis, and approximately 10% of myasthenia gravis patients can also have thymoma. Treatment includes cholinesterase inhibitors and immunotherapy agents, with extended thymectomy serving as an effective surgical option for drug-resistant cases. Minimally invasive approaches (video-assisted thoracoscopic surgery or robot-assisted thoracoscopic surgery) have demonstrated comparable oncological outcomes to sternotomy, highlighting their effectiveness and reliability.

Immunomodulatory effect of compound Xuelian capsule on experimental autoimmune myasthenia gravis in mice

IntroductionThis study aimed to explore the immunomodulatory effects of Xuelian capsules in experimental autoimmune myasthenia gravis (EAMG) mice. MethodsFemale C57BL/6 mice were immunized with AChR-α subunit R97–116 peptide to induce EAMG. Mice were randomized into five groups: EAMG model (n = 8), prednisone (n = 7), and low-, middle-, and high-dose Xuelian capsule groups (n = 7, 7, 8). Hematological parameters and NLR were assessed using an automatic hematology analyzer. Serum AchR-Ab, IFN-γ, IL-4, IL-10 levels were measured by ELISA. Thymus pathology was evaluated by HE staining. Spleen mRNA levels of TGF-β, IL-17, IL-6, IFN-γ were quantified by qPCR, and spleen cell populations by flow cytometry. ResultsXuelian capsules significantly reduced the ratio of NLR in peripheral blood, the concentration of AchR-Ab, IFN-γ, and IL-4 in serum, the expression of TGF-β, IL-17, IL-6, and IFN-γ in the spleen, and the proportion of CD4+/IL-4+ and CD4+/IFN-γ+ cells in the spleen, while increasing the concentration of IL-10 in the serum and the proportion of CD4+/Treg cells in the spleen. It also improved pathological changes in the thymus tissue of mice. DiscussionOne of the possible mechanisms of Xuelian capsules in the treatment of myasthenia gravis is to improve the pathological structure of the thymus of EAMG mice by inhibiting inflammatory factors and improving immunity, which then affects the occurrence and development of inflammation to achieve anti-inflammation, thus improving myasthenia gravis (MG).

Efficacy and safety of rituximab in the treatment of pediatric myasthenia gravis

Objective: To evaluate the efficacy and safety of rituximab in pediatric myasthenia gravis (MG). Methods: Case series study. The clinical manifestations, laboratory tests, treatment plans and prognosis of 27 pediatric MG patients treated with rituximab from June 2013 to June 2023 at Children's Medical Center of Peking University First Hospital were retrospectively collected. Results: There were 5 males and 22 females in 27 MG children. The onset age was 2.1 (1.6, 4.8) years, ranging from 8 months to 11 years. The clinical classification included 20 children (74%) of ocular MG and 7 children (26%) of generalized MG. Seventeen children (63%) had positive MG-related pathogenic antibodies, including 17 children of anti-AchR antibody and 1 of them also had anti-MuSK antibody. Rituximab was used as first-line immunosuppressant in 13 children, second-line immunosuppressant in 13 children and third-line immunosuppressant in 1 child. Immunosuppressants used before rituximab including 8 children of cyclosporine, 3 children of tacrolimus, 1 child of azathioprine, 1 child of mycophenolate mofetil and 1 child of cyclosporine combined with azathioprine. Rituximab was used for at least half a year with a follow-up period of more than 12 months. At the last follow-up after rituximab treatment, all children achieved improved or above, 14 children (52%) achieved complete stable remission, 7 children (26%) achieved pharmacologic remission, 1 child (4%) achieved minimal manifestations, and 5 children (18%) improved. After rituximab treatment, 27 children all could reduce the immunomodulation therapy and shorten the course of glucocorticoid therapy, and 22 children (81%) had stopped the glucocorticoid therapy. Among the 14 children with poor efficacy of other immunosuppressants, rituximab had complete stable remission of 7 children. The most common adverse reaction was respiratory infection (4 children (15%)). Only 2 children had allergic reaction to rituximab and got better after symptomatic treatment. Conclusions: Rituximab has good efficacy and tolerance in pediatric MG. Early application of rituximab can improve the prognosis and shorten the course of glucocorticoid treatment.

Grilled nux vomica alleviates myasthenia gravis by inhibiting the JAK2/STAT3 signaling pathway: a study in a mice model

BackgroundGrilled Nux Vomica (GNV) is a promising traditional Chinese medicine to treat myasthenia gravis (MG), but its effects and mechanisms need further exploration.MethodsExperimental autoimmune MG (EAMG) model was established by muscle-specific kinase (MuSK) induction on C57BL/6 J mice. Mice were treated with GNV and/or ruxolitinib (JAK2 inhibitor) or AG490 (STAT3 inhibitor) for 30 days via gavage after modeling and randomized into 7 groups: control, model, low-dose GNV, middle-dose GNV, high-dose GNV, GNV + ruxolitinib, GNV + AG490. Body weight, muscle strength, clinical score, MuSK level, neuromuscular junction integrity (agrin and acetylcholine receptor [AChR] levels), inflammatory factors (IL-2 and IL-6), and the activation of the JAK2/STAT3 pathway were measured and compared between groups.ResultsGNV significantly improved body weight and muscle strength, as well as reduced clinical scores, MuSK levels, and inflammatory markers (IL-2 and IL-6) levels compared with untreated EAMG mice. GNV also protected the neuromuscular junction and increased agrin and AChR co-expression in a dose-dependent manner. In addition, GNV attenuated the levels of p-JAK2 and p-STAT3, which are aberrantly upregulated in EAMG mice. After co-treatment with ruxolitinib or AG490, the effect of GNV on body weight, muscle strength, clinical score, MuSK level, neuromuscular junction integrity, levels of inflammatory factors, and JAK2/STAT3 pathway was further amplified in EAMG mice.ConclusionsGNV improves MG by inhibiting the JAK2/STAT3 pathway, which might be an effective therapeutic strategy for MG.

Patient Preferences for Generalized Myasthenia Gravis Treatment Profiles: Results of a Web-based Survey

Patient Preferences for Generalized Myasthenia Gravis Treatment Profiles: Results of a Web-based Survey

Possible sex and racial disparities in myasthenia gravis care.

Given the importance of early diagnosis and treatment of myasthenia gravis (MG), it is critical to understand disparities in MG care. We aimed to determine if there are any differences in testing, treatment, and/or access to neurologists for patients of varying sex and race/ethnicity with MG. We used a nationally representative healthcare claims database of privately insured individuals (2001-2018) to identify incident cases of MG using a validated definition. Diagnostic testing, steroid-sparing agents, intravenous immunoglobulin (IVIG), plasma exchange (PLEX), and thymectomy were defined using drug names or CPT codes. Steroid use was defined using AHFS class codes. We also determined whether an individual had a visit to a neurologist and the time between primary care and neurologist visits. Logistic regression determined associations between sex and race/ethnicity and testing, treatments, and access to neurologists. Female patients were less likely to get a computed tomography (CT) chest (odds ratio (OR) 0.73, 95% confidence interval (CI): 0.64-0.83), receive steroids (OR: 0.85, 95% CI: 0.75-0.97), steroid-sparing agents (OR: 0.84, 95% CI: 0.72-0.97), and IVIG or PLEX (OR: 0.80, 95% CI: 0.67-0.95). Black patients were less likely to receive steroids (OR: 0.78, 95% CI: 0.63-0.96). No significant disparities were seen in access to neurologists. We found healthcare disparities in MG treatment with female and Black patients receiving less treatment than men and those of other races/ethnicities. Further research and detailed assessments accounting for individual patient factors are needed to confirm these apparent disparities.

Efficacy and safety of low-dose rituximab in the treatment of myasthenia gravis: a systemic review and meta-analysis.

Rituximab (RTX) is a monoclonal antibody that has been increasingly used in the treatment of myasthenia gravis (MG). In most studies, the therapeutic protocol of RTX has been similar to that adopted for B cell lymphoma, with an increasing number of studies aimed at exploring the efficacy of low-dose RTX in MG. However, the beneficial effects of low-dose RTX in MG remain a subject of critical debate. This study was conducted following the PRISMA (Preferred Reporting Items for Systematic Review and Meta-Analysis) guidelines. Two reviewers (Xishuai Yang and Bingxia Li) independently conducted searches across multiple databases, including PubMed, MEDLINE, EMBASE, Web of Science, Cochrane Library, and China National Knowledge Infrastructure (CNKI). A meta-analysis, utilizing representative forest plots, was performed to assess "Improved clinical status" and changes in the Quantitative Myasthenia Gravis (QMG) score before and after treatment. A total of 17 studies involving 292 patients were included in the meta-analysis. A noticeable improvement in clinical status was observed in 91% of patients at the final follow-up after therapy (95% CI: 84-96%, P < 0.001). The QMG score showed a significant reduction following the treatment, with a standardized mean difference (SMD) of -1.69 (95% CI: -2.21 to -1.16, Z = 6.29, P < 0.001). In the acetylcholine receptor antibody-positive myasthenia gravis (AChR-MG) group, 90% of patients achieved improved clinical status (95% CI: 80-97%, P < 0.001) and the QMG score significantly decreased after low-dose RTX treatment, with an SMD of -1.51 (95% CI: -0.80 to -2.21, Z = 4.50, P < 0.001). In the muscle-specific kinase antibody-positive myasthenia gravis (MuSK-MG) group, 97% of patients achieved improved clinical status (95% CI: 89-100%, P < 0.001). The QMG score also significantly decreased following low-dose RTX treatment, with an SMD of -2.31 (95% CI: -2.99 to -1.62, Z = 6.60, P < 0.001). Adverse effects were reported in 29 out of 207 patients (14%, including infusion reactions in 22 patients (10.1%), infections in three patients (1.45%), cytopenia in two patients (0.96%), eosinophilia in one patient (0.48%), and hemiplegia in one patient (0.48%). Additionally, one patient (0.48%) succumbed to complications from invasive thymoma. Our meta-analysis shows that low-dose RTX is both effective and safe for treating MG. PROSPERO, identifier: CRD42024509951.

Treatment of juvenile myasthenia gravis with tacrolimus: A cohort study.

We investigated the proper timing, efficacy and safety of tacrolimus for juvenile myasthenia gravis (JMG). We conducted a retrospective cohort study for JMG patients treated with tacrolimus at Xiangya Hospital, Central South University, Changsha, China from 2010 to 2023. The clinical information of patients with a follow-up of more than 1 year was collected. Comparisons of clinical features between groups of patients who achieved therapeutic goal and those who did not achieve therapeutic goal as well as between groups of patients treated with tacrolimus within or after 1 year from JMG onset was carried out. Forty-three patients were enrolled, of whom 28 achieved therapeutic goal. Tacrolimus reduced glucocorticoids (GC) dosages for the 28 cases and 15 cases discontinued GC completely. Generalized myasthenia gravis (GMG) subtype had an association with a group of patients who achieved therapeutic goal (p = 0.001). Median duration from JMG onset to tacrolimus use was 10.50 months for those who achieved therapeutic goal and 36.00 months for those who did not achieve therapeutic goal (p = 0.010). The median Myasthenia Gravis Activities of Daily Living (MG-ADL) score improved significantly (p = 0.003). The initiation of tacrolimus within 1 year of JMG onset showed an association with achievement of therapeutic goal (p = 0.026). GMG subtype showed an association with a group of patients who received tacrolimus within 1 year (p = <0.001). Tacrolimus side effects were tolerable. The provision of tacrolimus within 1 year of JMG onset is effective and safe.

Buzhong Yiqi decoction attenuates acquired myasthenia by regulating the JAK2/STAT3/AKT signaling pathway, inhibiting inflammation, and improving mitochondrial function.

Acquired myasthenia (AM), a debilitating autoimmune disease, is typically characterized by skeletal muscle fatigue and weakness. Despite advances in myasthenia gravis treatment, current approaches remain unsatisfactory and many result in unexpected side effects. Traditional Chinese medicine has shown great potential in the treatment of myasthenia gravis, including relieving myasthenic symptoms, improving patients' quality of life, and reducing Western medicine side effects. This study investigates the protective effects and mechanism of BZYQD in mice with acquired myasthenia. BZYQD alleviates the reduced grip strength and increased expression of MAFbx and MuRF-1 in mice with acquired myasthenia. It also reduces levels of pro-inflammatory factors IL-1β, IL-6, and TNF-α in the mouse serum. In addition, BZYQD reduces ROS accumulation and the mitochondrial ROS production rate, while increasing ATP levels and mitochondrial membrane potential in mice with acquired myasthenia. Moreover, BZYQD decreases the expression of p-JAK2, p-STAT3, and p-AKT in the skeletal muscle of mice with acquired myasthenia. In summary, BZYQD reduces inflammation, enhances mitochondrial function, and regulates the JAK2/STAT3/AKT signaling pathway to treat acquired myasthenia.

Patient and care partner perspectives and preferences related to myasthenia gravis treatment: A qualitative study.

Due to the high symptom and treatment burden in myasthenia gravis (MG), understanding patient and care partner perspectives and preferences is crucial. This study used voice analysis and virtual focus groups to understand patient and care partner experiences with MG-related symptoms, treatments, and preferences. The voice analysis via social media listening used artificial intelligence-powered tools to gather and structure public digital conversations on MG. Focus groups included people living with MG and care partners who completed a questionnaire and participated in a 1-h virtual session facilitated using a semi-structured interview guide. Qualitative data were aggregated, transcribed, and thematically analyzed. The voice analysis examined 11,554 posts from 8321 individuals, discussing MG symptoms, treatments, and burden. Of 7563 symptom-related posts, 5902 (78%) conveyed negative, 1427 (19%) neutral, and 234 (3%) positive sentiment. The most frequently mentioned symptoms were categorized as dysarthria, muscle weakness, and dysphagia. MG treatment sentiment analysis identified 6667 posts (67%) as neutral, 2887 (29%) as negative, and 350 (4%) as positive. For the focus groups, 15 individuals (12 patients and 3 care partners) completed the questionnaire and 14 participated in the virtual focus group sessions. The 15 participants who completed the questionnaire prioritized treatment convenience, symptom control for improved quality of life, and preventing potential MG crises in their current treatment. New treatment expectations included increased effectiveness, less frequent dosing, faster onset, and fewer side effects. Participants were also receptive to wearable medication delivery systems placed on the body and valued direct involvement in treatment decisions. Patients and care partners are often negatively impacted by MG symptoms and value convenient and fast-acting treatments that control symptoms with minimal side effects. Considering patient preferences may help optimize treatment decisions and improve patients' overall well-being and satisfaction in their care.

Targeted Treatments for Myasthenia Gravis in Children and Adolescents.

Myasthenia gravis (MG) is an antibody-mediated disorder of the neuromuscular junction affecting children and adults. MG is a treatable condition with most patients requiring immunosuppression for disease control and/or remission. Juvenile myasthenia gravis (JMG) is rare in comparison with adult-onset MG but given the same underlying pathophysiology, treatment strategies are similar to those in adults. Until recently, there were only a few randomised controlled trials (RCTs) for MG treatments in adults and none in children, and management strategies were primarily based on expert consensus. In addition, treatment options for refractory MG cases have been severely limited, resulting in poor long-term quality of life in such patients due to the significant disease burden. Recently, there have been several RCTs focussing on novel therapeutic strategies with potentially promising outcomes, suggesting a change in MG management over the coming years and access to more effective and faster-acting drugs for MG patients. This paper will review current and new MG treatments including efgartigimod, eculizumab, rozanolixizumab, ravulizumab, and zilucoplan, with a focus on juvenile myasthenia gravis.

A case report of elevated bromide levels from pyridostigmine bromide for treatment of myasthenia gravis.

In an effort to expedite the publication of articles, AJHP is posting manuscripts online as soon as possible after acceptance. Accepted manuscripts have been peer-reviewed and copyedited, but are posted online before technical formatting and author proofing. These manuscripts are not the final version of record and will be replaced with the final article (formatted per AJHP style and proofed by the authors) at a later time. Elevated serum bromide levels can cause dermatological, gastrointestinal, and neurological abnormalities. As bromide and chloride are both halogens, bromide may interfere with chloride assays, causing a falsely high serum chloride concentration and a low or negative anion gap. There is a paucity of data describing bromide toxicity from high doses of pyridostigmine bromide (PB). This case report describes a patient with an elevated bromide level with neurological symptoms from a therapeutic dose of PB. A 37-year-old male with myasthenia gravis secondary to type B2 thymoma status following thymectomy presented in myasthenic crisis. He required mechanical ventilation and was managed with steroids, intravenous immune globulin, plasmapheresis, and PB. On day 9, the patient experienced acute agitation. He had an anion gap of 2 mEq/L and a chloride concentration of 109 mEq/L. The plasma creatinine concentration was 0.63 to 1.15mg/dL and urine output was 0.76 to 1.79mL/kg/h throughout his admission. All other laboratory values were normal. The daily dose of PB was 660mg on day 9, but the patient received 76mg of intravenous PB over the first few days of his admission with the largest dose in 24 hours equal to 48mg. On day 10, the patient's bromide level was 37 μg/mL. His agitation was initially managed with quetiapine, followed by PB dose reduction. To our knowledge, there are 2 cases in the literature of bromide toxicity secondary to PB. These patients experienced neurological symptoms with bromide levels of 88 to 90 μg/mL. Bromide concentrations of more than 12 μg/mL are associated with a higher risk of neuronal dysfunction demonstrated as disturbances on an electroencephalogram, and levels greater than 50 μg/mL are considered toxic. While our patient's bromide level was not as high as those previously reported, no other causes for his agitation were identified. Elevated bromide levels from therapeutic PB can occur, and monitoring of these levels should be considered.

Evaluation of feasibility and safety of non-intubated anesthesia video-assisted thoracoscopic thymectomy for myasthenia gravis

Video-assisted thoracoscopic surgery (VATS) thymectomy is a well-established treatment for Myasthenia gravis (MG). However, traditional VATS thymectomy uses general anesthesia with endotracheal intubation, which can worsen MG symptoms. This study evaluated the feasibility and safety of non-intubated anesthesia VATS for patients with MG to solve this issue. It was a prospective, descriptive study involving six patients aged 18 to 60, ASA I, II, who underwent VATS thymectomy under non-intubated anesthesia and local anesthesia at Vietduc University Hospital between May 2022 and December 2023. The induction time was short, ranging from 3 to 4.5 minutes. The median of Peak PaCO2 and EtCO2 were 42.5 mmHg (range 33-49 mmHg) and 46.4 mmHg (range 44-48 mmHg), respectively. The median values of PaO2 and SpO2 during one-lung ventilation were 161.1 mmHg (range 82-206.9 mmHg) and 98% (range 93-99%). The lungs collapsed well, and breathing and circulation remained stable. No complicationarose, and all patients recovered well. These initial findings suggest that non-intubated anesthesia is a safe and straightforward option for MG thymectomy

Correlation Analysis of Myasthenia Gravis Thymic Disease with Quantitative Score of Myasthenia Gravis Swallowing Function and Respiratory Function

Introduction: The pathophysiology of myasthenia gravis (MG), an autoimmune disease affecting the neuro-muscular junction, is closely related to thymic disorders (thymic hyperplasia, thymic involution, thymic neoplasms). Myasthenia gravis often causes or exacerbates thymus disease, which often occurs together with myasthenia gravis. Further studies are needed to determine the relationship between thymus properties and quantitative myasthenia gravis (QMG), swallowing, and respiratory function in patients with myasthenia gravis. Methods: From 2021 to 2022, 93 MG patients with definite thymic disease were found by thymic CT or histopathological biopsy in the Myasthenia Gravis Treatment Center of the First Affiliated Hospital of Guangzhou University of Traditional Chinese Medicine. The Quantitative Myasthenia Gravis (QMG) score was used by physicians at the deputy high level and above to measure patients' swallowing and respiratory function. The purpose of this study was to observe the relationship between myasthenia gravis thymic disease and QMG score, swallowing function score, and respiratory function score. Results：The poor swallowing function score (3 points) accounted for 58.9%, 4.8%, and 13.6% in the thymoma group, thymic hyperplasia group, and thymus without abnormality group, respectively, and the difference between the groups was statistically significant (P&lt;0.05). The respiratory function score was poor (3 points), and the thymus group accounted for 58.7%, which was significantly higher than the thymic hyperplasia group and the thymus without abnormality group. There was significant difference between groups (P&lt;0.05). Myasthenia gravis quantitative score (QMG)&gt;20 points (severe), the thymoma group accounted for 77%, which was significantly larger than the thymic hyperplasia group, and the thymus group had no abnormality, and the difference between the groups was significant (P&lt;0.05). After multiple comparison test results, it was found that the QMG score of the thymoma group was 1.07298 higher than that of the normal thymus, and the difference was significant. The swallowing score of the thymoma group was 1.461 higher than that of the normal thymus group, and the difference was significant. The respiratory function score of the thymoma group was 0.832 higher than that of the normal thymus group, and the difference was significant. The lung function score of the thymoma group was significantly higher than that of the thymic hyperplasia group by 1.509.

Efgartigimod in refractory autoimmune myasthenia gravis.

Efgartigimod, a neonatal Fc-receptor inhibitor, has recently been approved as treatment for myasthenia gravis (MG). In this retrospective cohort study, we aimed to systematically assess short- and long-term effectiveness of efgartigimod in patients with refractory MG. Sixteen patients with refractory autoimmune acetylcholine receptor MG were treated with efgartigimod. Data were collected from January 2021 to March 2023 on Myasthenia Gravis Activities of Daily Living (MG-ADL), Quantitative Myasthenia Gravis score (QMG), Myasthenia Gravis Composite score (MGC) and the 15-item revised version of the Myasthenia Gravis Quality of Life questionnaire (MG-QoL15r). A favorable outcome was seen in 56% of patients at the last measurement. Out of 16 patients, 50% were an MG-ADL responder after the first treatment cycle. After 4 weeks, a clinically meaningful improvement compared to baseline was seen on the MG-ADL, QMG, and MGC. There was a statistically significant improvement on the MGQoL15r from baseline to week 4. The improvement was maintained until the last measurement for the MGC and the MGQoL15r. At the last visit, all patients had discontinued 4-weekly dosages, shifting to administration frequencies of 1, 2, or 3 weeks. Drug doses could be decreased for prednisolone (n = 7), azathioprine (n = 2), and intravenous immunoglobulin (n = 9). Frequency of plasma exchange was decreased in nine patients. In patients with refractory MG, efgartigimod was effective for at least half of all patients. Patients required more frequent dosing compared to the ADAPT phase 3 trial. In 80% of the patients concurrent medication could be reduced or discontinued.