Myasthenia Crisis Research Articles

Mortality of Guillain Barre Syndrome and Myasthenic Crisis in Haji Adam Malik Central General Hospital Medan January 2021- December 2022

Introduction: Neuromuscular disorders are disorders of the neuro system that impact the muscular system. Among them are Guillain-Barré Syndrome and Myasthenic Crisis. Guillain-Barré syndrome is an autoimmune disorder of the nervous system that is polyradiculoneuropathic, characterized by weakness that radiates from the legs to the arms, torso, and face. Myasthenic crisis is a state of acute respiratory failure due to the exacerbation of Myasthenia Gravis. These two diseases are the most common types of neurodiseases found in hospital ICU patients. Objective: Determining the mortality rate of Guillain-Barré Syndrome and Myasthenic Crisis at Haji Adam Malik Central General Hospital Medan January 2021-December 2022. Methods : This study was a descriptive study with data obtained from medical records. The subjects were inpatients and outpatients with Guillain Barré Syndrome and Myasthenia Crisis, with as many as 129 patients. The subjects were selected by purposive sampling and met the inclusion and exclusion criteria. Results: A total of 40 Guillain-Barré Syndrome patients were studied, and 6 of them died. The majority were patients aged 36-50 years, and the male and female sexes were equally significant. A total of 89 patients with Myasthenic Crisis were studied, and 1 of them died, namely patients with the age category of 36-50 years and female. Conclusion: Guillain-Barré Syndrome mortality was 15%, and Myasthenic Crisis was 1.12% at Haji Adam Malik Center General Hospital Medan January 2021-December 2022. Keyword : Guillain-Barré Syndrome, Mortality, Myasthenic Crisis

Clinical features of thymoma with and without myasthenia gravis.

To explore the clinical features of thymoma with and without myasthenia gravis (MG). This was a retrospective study. Two hundred and thirty-three patients with mediastinal masses who were initially diagnosed in People's Hospital of Shijiazhuang, China, between January 2014 and June 2022 and had complete clinical data and underwent surgical treatment at People's Hospital of Shijiazhuang were retrospectively analyzed. The age of patients with thymoma alone was significantly older than that of thymoma patients complicated with MG. The number of female patients was slightly more than males for both groups. Proportions of type A, AB, B1, B2, and B3 thymomas in Group-A were 0.77, 11.54, 11.51, 33.85, and 31.54%, respectively, and the proportions in Group-B were 9.68, 22.58, 12.90, 32.26, and 22.58%. The size of tumors in patients with thymoma alone was larger than that of patients with thymoma complicated with MG. The proportion of patients with tumor size of more than 10 cm in the thymoma alone group was significantly higher than that in the MG group. There were no relapses in patients with type A disease and relapses were noted in a few patients with type B1, B2 and B3 diseases. The same survival rates were reported for the two groups. MG rarely occurs in type A and type C diseases. The prognosis of thymoma with MG is similar to that of thymoma alone. The main causes of death may be myasthenia crisis in thymoma patients with MG and advanced tumor stage in patients with thymoma alone.

Therapeutic Plasma Exchange in AChR-Ab Positive Generalized Myasthenia Gravis: A Real World Study About Its Early Response.

Since there is no clear priority or selection principle in the guidelines for myasthenia crisis, therapeutic plasma exchange (TPE) and intravenous immunoglobulin are often administered randomly. However, it should be more prudent in taking TPE due to its higher cost and risk. Studying its early response factors is crucial for managing myasthenia crisis and can improve medical and economic benefits. A prospective observational study was conducted, and patients classified as having "impending myasthenia crisis" or experiencing a myasthenia crisis and treated by TPE were included. The primary endpoint was the response after TPE. Univariate logistic regression analysis and repeated measurement were performed to analyze factors related to TPE efficacy. A total of 30 patients who treated with TPE as their fast-acting treatments were enrolled. After TPE, those whose QMGs and/or MGCs decreased by ≥5 points or ≥30% of the baseline were judged as "response group", accounting for 66.67% (20/30). Respiratory symptoms had a response rate of 72.00% (18/25), showing the most remarkable improvement. Meanwhile, extraocular symptoms were the least sensitive, with only 8.00% (2/25) showing efficacy. Thymoma (100.00% vs 50.00%, P=0.002) and a high concentration of AChR-Ab (37.37 nmol/L vs 25.4 nmol/L, P=0.039) were common in the early response group. Repeated measures showed significant changes in AChR-Ab and CD19+ B cells before and after TPE (all with P < 0.05). After treatment, the CD19+ B cells tended to decrease in the response group. These results indicated that, for AChR-Ab positive generalized MG, TPE can quickly improve respiratory symptoms. Thymoma and a high concentration of AChR-Ab before TPE predict an early better response. Additionally, TPE may work by decreasing AChR-Ab levels and inducing immune regulation. Future prospective and randomized controlled studies are needed.

GOOD OUTCOMES OF OCULAR MYASTHENIA GRAVIS IN A YOUNG ADULT MALE: A CASE REPORT

Abstract&#x0D; Introduction : Myasthenia gravis (MG) is characterized by fluctuating weakening and fatigability of skeletal muscles. Ocular MG (OMG) occurs when the symptoms of MG are limited to the ocular muscles.&#x0D; Case Illustration : A 21-year-old male presents with a two-month history of drooping both eyelids. The drooping eyelids improved in the morning or after a nap, and got worse in the evening. Ocular examination revealed limitation towards all gazes with bilateral ptosis. Visual acuity, anterior segment, visual fields, slit lamp, and fundus examinations were unremarkable. The ice pack and rest test resulted in a significant improvement after two minutes of ice pack application and 30-minute rest, raising suspicion for the diagnosis of MG. Acetylcholine receptor antibody (AChR) test was positive for AChR binding antibody. Treatment for OMG is initiated with an oral acetylcholinesterase inhibitor, pyridostigmine, and corticosteroid. The ptosis and ophthalmoplegia improved after one week follow- up and recovered completely after eight weeks of follow-up.&#x0D; Discussion : The hallmarks of MG are fluctuating fatigable muscle weakness that worsen with activity and improve on rest. Neurological exam and clinical neurological history are essential to establish the diagnosis. Bedsite tests, electrophysiologic test, and the antibody test all support the clinical diagnosis of MG.&#x0D; Conclusion : OMG progresses to its generalized form 90% of the time during the first two years of the onset of ocular symptoms. Early detection and prompt treatment are the key which may help to prevent or minimize the occurrence of myasthenia crisis.

Effectiveness of Therapeutic Plasma Exchange in Autoimmune Neurological Diseases in a Tertiary Care Hospital of South India

ABSTRACT Background and Objectives: Therapeutic plasma exchange (TPE) is commonly used as a treatment of certain autoimmune neurological diseases, and its main target is to remove pathogenical toxins or autoantibodies. Removed plasma with toxins and autoantibodies is replaced by crystalloids, colloids, and/or normal saline. It is more effective and cost-effective than immunoglobulins. The aim of this study was to know the effectiveness of TPE in autoimmune neurological diseases. Methods: This is a prospective study involving 376 autoimmune neurological cases, and the duration of this study was 5 years. All autoimmune neurological patients irrespective of age with complaints of weakness in limbs, respiratory distress, and acute myasthenia gravis (MG) crisis cases were included in the study. The Modified Rankin Score and MG Composite Score were used to analyze the severity of the disease, and the number of TPE procedures or cycles was planned. Results: The total number of cases included was 376 (322 newly registered cases and 54 old and relapse cases), and the total number of TPE procedures done was 1491 between the years 2017 and 2021. Male patients were 138 and female patients 238. The most number of cases were of MG, followed by Guillain–Barre syndrome. The mean number of TPE procedures done was 6.1 cycles in myasthenia crisis patients. There was a significant improvement in the patients after the treatment. The Modified Rankin Score and MG Composite Score were 1/6 and 3/50, respectively (P = 0.0321 and P = 0.0298, respectively) after completion of the TPE. Conclusion: TPE is the most effective method in cases of neurological autoimmune diseases. Most of the cases show improvement immediately after the first cycle, and more than half of the cases will be able to walk or do their routine activities after 2 or 3 cycles. It is a safe and cost-effective treatment modality with minimal side effects or complications.

Rescue treatment with add-on efgartigimod in a patient with impending myasthenic crisis: a case report

Myasthenia gravis (MG) is an autoimmune disorder characterized by fluctuating muscle weakness. Severe patients may develop life-threatening respiratory failure and experience crisis. Plasma exchange or intravenous immunoglobulin (IVIg) is the first-line treatment option for myasthenia crisis, but some patients still poorly respond to them. Here, we first reported a generalized MG patient from China who was in a state of impending myasthenic crisis and did not respond effectively to IVIg but was successfully rescued by add-on efgartigimod. Especially, we also detected meaningful changes in T-cell and B-cell subsets after efgartigimod, promoting a potential role of efgartigimod in re-establishing immune homeostasis.

Critical Care Management of Myasthenic Crisis: A Case Report

Background: The annual incidence of myasthenia gravis is 1-2 cases in 100,000 population. 30% of all cases will develop bulbar or respiratory muscle weakness. About 15-20% of myasthenia gravis patients will develop myasthenia crisis.&#x0D; Case presentation: An 18-year-old female patient complained of difficulty swallowing two days ago difficulty swallowing solid and liquid food, and this complaint caused the patient to cough and choke. Complaints were accompanied by difficulty opening the eyelids, especially in the afternoon. Complaints were also accompanied by double vision, especially when looking to the right. There was a nasal sound, and the patient had difficulty pronouncing vocal letters. The patient was admitted to ICU, In the ICU patient was intubated and given plasma exchange therapy.&#x0D; Conclusion: Myasthenic crisis is a neurological emergency that requires prompt diagnosis and treatment. Adequate intensive care, judicious weaning, and extubation decisions, along with specifically targeted therapy, will improve the outcome of myasthenia gravis.

Plasmapheresis in Myasthenia Gravis Crisis

Introduction: Myasthenic crisis is the most lethal complication of myasthenia gravis. Referral to an intensive care unit is crucial in managing the myasthenic crisis. Hereby, we report a case of a myasthenic crisis in a 30-year-old female who underwent plasmapheresis. The patient underwent a 12-hour procedure for plasmapheresis and was discharged to a normal ward the next day. Although plasmapheresis is costly, its efficacy should be considered as the main treatment for myasthenic crisis.&#x0D; Case Illustration: Female, 30 years old, weighed 60 kgs, with myasthenia crisis. The patient came to an emergency department and was then intubated before being admitted to the intensive care unit. The physical diagnostic was normal and laboratory findings were leukocytosis. The patient was treated with normal saline, antibiotics, high-dose corticosteroids, and pyridostigmine. The patient was done plasmapheresis with synchronized intermittent mandatory ventilator mode. The patient was examined every 30 minutes. The physical examinations were relatively normal. The plasmapheresis procedure was ended in 12 hours.&#x0D; From the literature, plasmapheresis was found to have significant results for myasthenia gravis compared to conventional therapy because of its blood separation technique to remove autoantibodies. The next day patient was extubated with normal physical examinations and normal laboratory findings. The patient then moved from the intensive care unit to the normal ward and outpatient on the third day of hospital stay. The patient was given oral medicine that included antibiotics, corticosteroids, and pyridostigmine.&#x0D; Conclusion: From this case, we can see that plasmapheresis therapy has a really good outcome compared to other conventional therapy. However this therapy is expensive, so most healthcare providers don’t cover the payment. Hopefully, most hospitals and healthcare providers can cover up for this treatment to save many myasthenia gravis crisis.&#x0D; &#x0D; Keyword: Intensive Care Unit; myasthenic crisis; myasthenia gravis; plasmapheresis; treatment efficacy

79 - Functional weakness incongruent with acute myasthenia crisis

79 - Functional weakness incongruent with acute myasthenia crisis

High-dose Steroids Versus Standard Treatment for Myasthenic Crisis

Background: Previous literature revealed the possible benefit of high-dose steroids in myasthenic crises. Steroids are more economical than standard therapy, especially in resource-poor settings. More data are therefore needed about the utility of steroids for the myasthenic crisis. Objective: The outcomes were compared between standard therapy (intravenous immunoglobulin [IVIg] or plasmapheresis [PLEX]) and high-dose steroid (intravenous methylprednisolone) for the myasthenic crisis. The primary outcome was the length of hospital stay. The secondary outcomes were the length of intubation, intensive care unit stay, readmission rate due to crisis, posttreatment muscle score, and mortality. Methodology: This study was a single-center, longitudinal, 12-year retrospective study. Sixty-five patients were analyzed. A comparison of means was made between the outcomes of the two groups. Regression analysis was done for hospital stay and intubation outcomes. Side effects of treatment were determined. Results: Shorter hospital stay (P = 0.001), shorter intubation length (P = 0.000), and shorter intensive care (P = 0.000) were observed in the steroid group. Mortality (P = 0.187) and muscle score (P = 0.281) were comparable between the two groups. There was a trend toward lesser readmission (P = 0.097) and longer time to readmission (P = 0.069) in the standard therapy group. The IVIg group had arrhythmias, hypotension, headache, renal impairment, and hypomagnesemia. The PLEX group had hypotension and lower limb pain. The steroid group had hyperglycemia, insomnia, infection, stomach upset, facial flushing, myopathy, and sudden death. No acute worsening of the myasthenia crisis was seen in all patients in the steroid arm. Conclusion: Data on hospital stay, intensive care days, and intubation length were better with high-dose steroids. Mortality and motor outcomes were comparable between the two groups. Readmission rates due to crisis were lower in the standard therapy group.

Low-volume Plasma Exchange In Immune-mediated Neurological Diseases : A tertiary care center study

BACKGROUND The optimal cost benefit of standard plasma exchange (SPE) has not been met where most of the patients do not afford the treatment. As an alternative, low volume plasma exchange (LVPE) is cost-effective than SPE where albumin replacement is eliminated. The aim of this study was to analyse the efficacy and safety of LVPE in various Immune mediated neurological diseases (IMND).&#x0D; METHODS A hospital-based retrospective study was conducted in patients with IMND who were admitted in TUTH between October 15, 2020, and October 14, 2022. Hemodynamically stable patients over the age of 18 who met the plasma exchange criteria were eligible. Outcomes and treatment-related complications were studied separately for the different diseases.&#x0D; RESULTS Of the 29 patients enrolled, 3 had Myasthenia gravis (MG), 6 had Neuromyelitis optica spectrum disorder (NMOSD), 11 had Gullian Barre syndrome (GBS), 6 had Chronic inflammatory demyelinating polyneuropathy (CIDP), and 3 had Autoimmune encephalitis (AE). Patients with MG, GBS, and CIDP all showed statistically significant improvement in mRS scores from 2.3±0.6 to 0 (p value = 0.0198), 3.03±1.8 to 2.2±1.4 (p value=0.0046) and 2.5±1.4 to 1.8±1(p value=0.025) respectively by the time they were discharged following treatment. Two patients developed Deep vein thrombosis (DVT), and two developed anaphylaxis during the course of their treatment, although none deteriorated further while in the hospital.&#x0D; CONCLUSION LVPE was effective in MG, GBS and CIDP. Myasthenia crisis patients who re-quired mechanical ventilation demonstrated complete resolution with LVPE making it a potential life-saving alternative for those who cannot afford standard therapy.

Nomogram for the acute exacerbation of acetylcholine receptor antibody-positive generalized myasthenia gravis.

An acute exacerbation of myasthenia gravis (MG) can lead to the life-threatening myasthenia crisis which can increase the in-hospital mortality. This study aimed to clarify the correlative factor of the severity and activity of MG and the predictors of its exacerbation. A prospective study was conducted to compare the clinical characteristics of acetylcholine receptor antibody (AChR-Ab)-positive generalized MG during acute exacerbation (AE) and in a stable state (SS). Logistic regression was used to determine risk factors, and a nomogram was developed. A total of 97 AChR-Ab MG patients were enrolled, of whom 44 had AE and 53 were in SS. The concentrations of AChR-Ab were 35.24 (23.26, 42.52) nmol/L and 19.51 (8.30, 36.93) nmol/L in the AE and SS groups (P = 0.005), respectively. The receiver operating characteristic curve showed that a single AChR-Ab predicted severity and acute exacerbation, with an area under the curve (AUC) of 0.679. Logistic regression analysis showed that, in addition to AChR-Ab (P = 0.018), bulbar symptoms (P = 0.001), interleukin (IL)-6 (P = 0.025), CD4+/CD8+ T cell ratio (P = 0.031), and CD19+ B cell proportion (P = 0.019) were independent risk factors for acute exacerbation of MG. The developed nomogram had an AUC of 0.878. The Hosmer and Lemeshow chi-square test was 4.37 (P = 0.929). AChR-Ab concentration was positively correlated with the severity and activity of MG. AChR-Ab concentration, alongside bulbar symptoms, IL-6 concentration, CD4+/CD8+ T cell ratio, and CD19+ B cell proportion can predict the acute exacerbation of MG.

Impact of Thymectomy on Crisis Incidence and Quality of Life amongst Generalised Myasthenia Gravis Patients.

Thymectomy, combined with corticosteroids, immunosuppressive agents, and cholinesterase inhibitors, has been accepted as the standard treatment for myasthenia gravis (MG) patients. Data on the effect of thymectomy on occurrence of myasthenic crisis are few. To assess the long-term impact of thymectomy in patients with generalized Myasthenia gravis (GMG) in terms of occurrence of myasthenia crisis and quality of life. A retrospective analysis of 274 clinical records of patients diagnosed with myasthenia gravis (MG) in Nizam's institute of medical sciences (NIMS), a tertiary level teaching hospital between January 2000 and December 2015 was done. Severity of the disease was assessed using Myasthenia Gravis Foundation of America (MGFA) classification and quantitative myasthenia gravis (QMG) score. Myasthenia crisis was diagnosed in our patients when they required ventilator assistance due to respiratory failure caused by muscle weakness (MGFA class V). Quality of life (QoL) was assessed. Of 230 cases included in the final analysis, 108 (46.9%) underwent thymectomy. Posttreatment crisis occurred in 53.3% of the nonthymectomy subjects, and 25.9% of thymectomy group (P < 0.001). In multivariate logistic regression analysis, after controlling for the effect of gender, age at diagnosis and grade of the disease, the odds ratio of myasthenic crisis in people with thymectomy was 0.186.(95% CI 0.087 to 0.387, P = 0.001). No statistically significant differences were observed in quality of life scores between thymectomy and nonthymectomy groups, either before (P = 0.86) or after surgery (P = 0.939). The odds of myasthenia crisis was lesser in people, who underwent thymectomy even after controlling for MGFA grade and other potential confounders but no significant differences in quality of life were found with thymectomy.

Characteristics of neurologic manifestations in COVID-19 patients at Sanglah Hospital, Denpasar, Indonesia

Background/aim. Coronavirus Disease 2019 (COVID-19) is an infectious disease caused by SARS-CoV-2. Although the main symptoms of this virus are in the respiratory system, neurological clinical manifestations in the form of the central nervous system (CNS), peripheral nervous system (PNS) and musculoskeletal system are often found. This study aims for data on the characteristics of neurological manifestations in COVID-19 patients. Research methods. A retrospective cohort study with medical record from June 2020 to June 2021 which was analyzed descriptive, Chi-Square test and survival using SPSS program. Research result. There were 136 patients with PCR swab results (+), 80 (58.8%) male and 56 (41.2%) female, age &gt; 50 years 92 (67.6%), 47 (34.6%) died. Neurological manifestations in the CNS that stroke 72 (52.9%) RR 4.8 (CI 95% 2.1-10.6; p&lt;0.001), seizures 19 (14%) RR 14.7 (95% CI 4-54.3; p &lt;0.001), headache 32 (23.5%) RR 5.7 (95% CI 2, 4-13.4; p&lt;0.001), encephalopathy 35 (25.7%) RR 41.1 (95% CI 12.7-132.7; p&lt;0.001), in the PNS myasthenic crisis 6 (4.4%) RR 10.4 (95% CI 1.2-92.5; p = 0.035) anosmia 73 (53.7%) RR 0.2 (95% CI 0.1-0.5; p&lt;0.001) while musculoskeletal myalgia 25 (18.4%) and low back pain 18 (13.2%) was not significant. Conclusion. Most neurological clinical manifestations in the CNS (stroke, headache, seizures and encephalopathy) followed by the PNS (myasthenia crisis and anosmia). Neuroinvasive complications are thought to play a role as one of the causes of respiratory failure and death in patients with COVID-19.

A Case Report of Korean Medical Intervention in a Patient with Myasthenia Gravis after Myasthenia Crisis

Objective: This study reports on the management of a patient with myasthenia gravis (MG) following myasthenic crisis (MC) using Korean medicine.Methods: A 77-year-old Class Ⅴ MG patient was treated with acupuncture, moxibustion, and Korean herbal medicine. A manual muscle test was used to derive a Medical Research Council (MRC) score and the patient’s subjective view of his dyspnea was measured to assess MG symptoms.Results: The MRC grade indicated significant deterioration after 65 days of treatment, although there was no change in the patient’s subjective dyspnea assessment. But no other MG or MC complications occurred.Conclusion: This study suggests that Korean medicine could be used for the management of Class Ⅴ MG patients.

Lymphoplasma Exchange Improves Myasthenia Gravis Exacerbations: A Retrospective Study in a Chinese Center.

BackgroundLymphoplasma exchange (LPE), a technique combining plasma exchange with leukapheresis, is emerging as promising treatment for autoimmune diseases. Data on the efficacy and safety of LPE in myasthenia gravis (MG) therapy are scarce. In this study, we aimed to comprehensively review the clinical efficacy, safety, and immunological characteristics of LPE therapy in MG patients.Study Design and MethodsA Chinese cohort of 276 generalized MG patients in state of exacerbation, including impeding crisis, myasthenia crisis, and preparation for thoracic exsection between January 2014 and December 2020, were evaluated in this study.ResultsA total of 276 patients with a median age of 45.5 ± 16.7 years underwent a total of 635 LPE sessions. Clinical scales of Quantitative Myasthenia Gravis (QMG) scores, Myasthenia Gravis Specific Manual Muscle Testing (MMT) scores, activities of daily living (ADL) scores, and quality of life (QOL) scores were improved during 4 weeks’ follow-up. Adverse effects occurred in 20 out of 276 patients, with 14 patients having one adverse event each. Independent predictive factors for good response to LPE therapy were symptom onset before LPE therapy ≤3 days and age on LPE therapy <50 years of age. LPE decreased the serum levels of antibodies, immunoglobulins, and complements 4 weeks after the first replacement, with decreased levels of interleukin (IL)-17A and interferon (IFN)-γ and increased level of IL-10.ConclusionLPE is an effective treatment for MG patients in state of exacerbation and preparation for thymectomy. Early use of LPE on early-onset MG may have good therapeutic effects. The potential mechanism for LPE is the polarization of cytokines from IL-17A, IFN-γ, into IL-10.

Clinical characteristics and outcomes of myasthenia gravis patients with double positive antibodies against acetylcholine receptor and muscle-specific tyrosine kinase

Objective: To investigate the clinical manifestations, treatment characteristics and outcomes of myasthenia gravis (MG) dually positive for anti-acetylcholine receptor antibody (AChR-Ab) and anti-muscle-specific tyrosine kinase antibody (MuSK-Ab). Method: MG patients hospitalized in the First Affiliated Hospital of Sun Yat-sen University from August 2017 to November 2020 were retrospectively collected. Thirty-four MuSK-Ab positive MG (MuSK-MG) patients, 11 double-antibodies positive MG (DP-MG) patients, and 80 AChR-Ab positive MG (AChR-MG) patients were included and allocated to three different groups. The clinical data of patients in the three groups were collected, and the differences of demographic characteristics, clinical manifestations and treatment outcomes between DP-MG patients and AChR-MG and MuSK-MG patients were analyzed. Result: The proportion of female and male patients in DP-MG group was 7/11 and 4/11 respectively, and the onset age of DP-MG was (41±27) years.The difference in gender distribution between DP-MG and AChR-MG groups was statistically significant (P<0.05). The proportion of extraocular muscle involvement in the DP-MG and MuSK-MG groups (8/11 and 52.9%) was lower than that in the AChR-MG group (83.8%), and the difference was statistically significant (P<0.05). The incidence of myasthenia crisis in DP-MG and MuSK-MG groups (54.5% and 61.8%) were higher than that in AChR-MG group (20.0%), with astatistically significant difference(P<0.05). The positive rate of neostigmine test in DP-MG and MuSK-MG groups(8/11 and 74.2%) were lower than that of AChR-MG group (96.8%), and the positive rate of low frequency repetitive nerve stimulation (RNS) in DP-MG group (5/10) was lower than that in AChR-MG group (85.1%), with statistically significant differences (all P<0.05). MuSK-Ab titer was positively correlated with the course of disease (r=0.466, P<0.05), and antibody titer decreased after symptom improvement (P<0.05). The response of patients in DP-MG and MuSK-MG groups to cholinesterase inhibitors (2/11 and 9.1%) was worse than that in the AChR-MG group (66.3%), and the incidence of side effects in the two groups (5/11 and 39.4%) was higher than that in the AChR-MG group (15.0%), with statistically significant differences (all P<0.05). There were 4 DP-MG patients underwent thymectomy, and the pathological results detected two cases of thymoma and two cases of thymic hyperplasia. Subsequent follow-up showed that 5 (5/11) DP-MG patients achieved minimal manifestation status or better status. Conclusion: The gender distribution, age of onset, pharmacological characteristics and electrophysiological examination of DP-MG patients were similar to those of MuSK-MG patients, but the severity of DP-MG patients was between that of AChR-MG and MuSK-MG patients.

PND10 Conceptualization and Novel Budget Impact Analysis Framework for Treatments of Myasthenia Gravis

Myasthenia gravis (MG) is a rare chronic autoimmune neuromuscular disorder, which causes debilitating and potentially life-threatening exacerbations of muscle weakness. The objective of this study was to develop a novel United States (US) budget impact analysis framework to assess innovative MG treatments. A targeted literature review was conducted to identify previous economic evaluations of MG. Clinical experts were consulted to ensure model components reflected current clinical practice and understanding. US payers were engaged to provide feedback on the structure and outputs, and how to optimize the utility of the model framework for informing formulary decision-making. Few US economic analyses of MG treatments were identified in the literature. Recent data on direct pharmacy and medical costs were sparse. A novel model framework was developed for assessing the budget impact of an innovative MG therapy, which includes treatment-related costs and medical costs offsets. Modeled costs in the framework include direct pharmacy and medical costs of chronic MG treatments, chronic therapy adverse effects, MG exacerbations and myasthenic crisis events. Myasthenia gravis exacerbations and crisis costs include acute inpatient, emergency department and outpatient care as well as temporary treatment with immunoglobulin or plasma exchange following MG exacerbations. Clinical experts recommended that treatment comparators should include all combinations of conventional and add-on therapies to reflect the individualized treatment pathways observed in practice. Conventional therapies include combinations of corticosteroids, non-steroidal immunosuppressive drugs (e.g. azathioprine, mycophenolate mofetil) and acetylcholinesterase inhibitors. US payers recommended flexibility to adapt treatment market shares and time horizons to conduct alternative scenario analyses. Future budget impact analysis for MG treatments should capture both direct pharmacy and medical costs including those related to disease exacerbations and treatment adverse effects. The framework includes all the identified core components essential to assessing the full budget impact of novel MG treatments on direct pharmacy and medical costs.

Myasthenia gravis and pregnancy.

Myasthenia gravis is an autoimmune disease characterised by fluctuating muscle weakness, which worsens during activity. It affects particularly scapular and pelvic girdles, axial and bulbar muscles. Myasthenia gravis is twice more frequent in women and symptoms often appear in the second and third decade of life. Thus, a growing number of women affected by this condition become pregnant. To minimise the effects of myasthenia gravis on pregnancy and the newborn, and to avoid myasthenia crisis in the post-partum, the pregnancy must be planned as far as possible. During pregnancy, treatment must be reviewed due to the threat of teratogenic effects (mycophenolate mofetil, rituximab), and the follow-up must be multidisciplinary.

Difficulties in Diagnosing a Case of Myasthenia Crisis

A 26 year old, female presented to EMD in severe respiratory distress. Patient had history of breathlessness since past one month with h/o intrauterine death 1month back .On further investigation it was found that anti acetylcholine receptor antibody was above normal limits and was started on cholinesterase inhibitors, initially patient showed signs of improvement but later patient condition worsened and patient was diagnosed with Myasthenia gravis in crisis. Patient was started on pulse steroid therapy but patient did not show significant response, patient attenders were advised for IVIg but they could not afford the same, later patient was transferred to a center with plasmapheresis facility where plasmapheresis was done and now patient doing well and is on oral cholinesterase inhibitors. Keywords: Myasthenia crisis, anti-acetylcholine receptor antibodies