Myasthenia Gravis Foundation Of America Post-intervention Status Research Articles

Clinicopathological predictors of postoperative long-term myasthenic status in resected thymoma with myasthenia gravis.

Surgical patients with thymoma and myasthenia gravis (MG) must have their MG status and oncological outcomes critically monitored. We aimed to identify clinicopathological predictors of the postoperative MG status. We conducted a retrospective review of 40 consecutive surgical patients with MG-related thymomas between 2002 and 2020. The quantitative myasthenia gravis score (QMGS) and Myasthenia Gravis Foundation of America post-intervention status (MGFA-PIS) were used to evaluate postoperative MG status. All patients underwent extended total thymectomy. The most common WHO type was type B2 (32%), while 65% of patients had type B1-B3 and 35% had type A-AB thymomas. Eleven patients (28%) achieved controlled MG status in MGFA-PIS 6months after surgery. This controlled status was observed more frequently in type A-AB than in B1-B3 (57% vs. 12%, p = 0.007). In a multivariate analysis, WHO type (A-AB or B1-B3) was an independent predictor of worsening episodes of MG based on the QMGS (Type B1-B3, hazard ratio: 3.23, 95% confidence interval: 1.12-9.25). At the last follow-up, 23 patients (58%) achieved controlled MG status. The 5-year overall survival rate of all patients was 93.7%. The WHO type of thymoma is an informative predictor of postoperative MG status in patients with MG-related thymoma.

The Impact Of Thymectomy In Thymomatous And Nonthymomatous Myasthenia Gravis - The Experience Of A Tertiary Center.

Thymectomy remains a mainstay of treatment in Thymomatous (T) and Nonthymomatous (nT) Myasthenia Gravis (MG), with improved clinical outcomes and reduced need for medical treatment, however, there is little research regarding long-term follow-up. We aim to assess the impact of surgery on the long-term outcome of patients with MG at our center. Retrospective analyses of MG patients submitted to thymectomy between 2007 and 2017 at the thoracic surgery department of CHUC. Clinical assessment was performed according to the MG Foundation of America (MGFA) Clinical Classification (cMGFA). The follow-up was categorized according to the MGFA Post-intervention Status (MGFA-PIS) and cMGFA. Statistical analysis was performed with SPSS, to a significance level of 5%. Thirty-seven patients underwent extended thymectomy and 67.6% were female. Median age at diagnosis was 46.68±19.2 years. Most patients (83.8%) had anti-acetylcholine receptor antibodies and 81.1% had generalized forms of MG. Many patients (67.6%) had surgery less than 12 months after the clinical diagnosis. TMG was present in 19 (51.4%) patients. Compared to nTMG, these patients were older (54.06±17.9 vs 40.17±19.4 years) and most were men (52.9% vs 16.7%). We obtained a good outcome in most patients in the first (81.1%), second (86.1%), and fifth (84.8%) year of follow-up. There was a shift towards better prognosis categories in the good outcome group: 9.1% CSR, 3.0% PR, and 66,7% MM in the fifth year. Preoperative medical treatment did not influence the long-term follow-up outcome. A shorter time to surgery (< 12 months) correlated with better outcomes at year 5 (p=0.016). Thymectomy led to a sustained clinical improvement in our cohort, allowing for a reduced need for medication. A shorter time to surgery seems to have a positive influence on long-term prognosis. We expect that an extended follow-up would improve our results.

Effectiveness and safety of telitacicept for refractory generalized myasthenia gravis: a retrospective study.

Refractory generalized myasthenia gravis (GMG) remains a substantial therapeutic challenge. Telitacicept, a recombinant human B-lymphocyte stimulator receptor-antibody fusion protein, holds promise for interrupting the immunopathology of this condition. This study retrospectively assessed the effectiveness and safety of telitacicept in patients with refractory GMG. A single-center retrospective study. Patients with refractory GMG receiving telitacicept (160 mg/week or biweekly) from January to September in 2023 were included. We assessed effectiveness using Myasthenia Gravis Foundation of America post-intervention status (MGFA-PIS), myasthenia gravis treatment status and intensity (MGSTI), quantitative myasthenia gravis (QMG), and MG-activity of daily living (ADL) scores, alongside reductions in prednisone dosage at 3- and 6-month intervals. Safety profiles were also evaluated. Sixteen patients with MGFA class II-V refractory GMG were included, with eight females and eight males. All patients were followed up for at least 3 months, and 11 patients reached 6 months follow-up. At the 3-month evaluation, 75% (12/16) demonstrated clinical improvement with MGFA-PIS. One patient achieved pharmacological remission, two attained minimal manifestation status, and nine showed functional improvement; three remained unchanged, and one deteriorated. By the 6-month visit, 90.1% (10/11) sustained significant symptomatic improvement. MGSTI scores and prednisone dosages significantly reduced at both follow-ups (p < 0.05). MG-ADL and QMG scores showed marked improvement at 6 months (p < 0.05). The treatment was well tolerated, with no severe adverse events such as allergy or infection reported. Our exploratory investigation suggests that telitacicept is a feasible and well-tolerated add-on therapy for refractory GMG, offering valuable clinical evidence for this novel treatment option.

Long-term efficacy and safety of tacrolimus in young children with myasthenia gravis

ObjectiveThis study was performed to evaluate the efficacy and long-term safety of tacrolimus for young children with myasthenia gravis (MG). MethodsChildren with corticosteroids (CSs)-ineffective, CSs-dependent or CSs-intolerable MG treated with tacrolimus for at least one year were recruited. The Myasthenia Gravis Foundation of America (MGFA) clinical classification and MGFA post-intervention status (MGFA-PIS) were used to evaluate before tacrolimus administration and at the last visit, respectively. MG Activities of Daily Living (MG-ADL) score and the dose of prednisone were recorded. Patients were divided into responders and poor responders based on changes in MG-ADL score to investigate the factors that affected tacrolimus efficacy. Unfavorable events were recorded. ResultsTwenty-one patients with MG were enrolled. The median age of starting tacrolimus was 8.7 (range 2.2–15.1) years old. At the last visit, 15 patients (71.4%) achieved minimal manifestation (MM) or better status. The symptoms evaluated by MG-ADL improved significantly one month after initiating tacrolimus (p＜0.05) and the dose of prednisone decreased significantly three months later (p＜0.05), and it continued to improve throughout the study. Thirteen patients (61.9%) were ultimately weaned off prednisone. Compared with 16 responders, 5 poor responders had lower MG-ADL scores. MG-ADL score was the only clinical factor of tacrolimus efficacy. Intraocular pressure and transient urine microprotein were present in one patient. ConclusionA course of tacrolimus of more than one year was effective and well-tolerated in young children with MG, and tacrolimus improved MG symptoms and reduced the dose and adverse events of oral prednisone.

THYMECTOMY IN SEVERE (MGFA CLASS IV-V) GENERALIZED MYASTHENIA GRAVIS: IS THE GAME REALLY WORTH THE CANDLE?

Total thymectomy in addition to medical treatment is an accepted standard therapy for myasthenia gravis. Patients with severe generalized myasthenia gravis present life-threatening events, poor prognosis and higher risk of postoperative myasthenic crisis. Aim of our study is to investigate neurological and surgical results in patients with MGFA class IV and V MG following thymectomy. Data on 76 myasthenia gravis patients with preoperative MGFA class IV and V who underwent thymectomy were retrospectively collected. Primary end-points included short-term surgical outcomes and long-term neurological results including the achievement of complete stable remission and any improvement as defined by MGFA postintervention status criteria. There were 27(35.5%) males and 49(64.5%) females; 53(69.7%) were classified as MGFA class IV and 23(30.3%) as class V. Thymectomy was performed through sternotomy in 25(32.9%) patients, by VATS in 5(6.6%) and RATS in 46(60.5%). Median operative time was 120(IQR 95; 148) min. In-hospital mortality was observed in 1(1.3%) patient, postoperative complications in 14 (18.4%). Median postoperative hospital stay was 4(IQR 3; 6) days. Pathological examination revealed 31(40.8%) thymic hyperplasia/other benign and 45(59.2%) thymomas. Cumulative complete stable remission and improvement probabilities were 20.6% and 83.7% at 5-year, and 66.9% and 97.6% at 10-year, respectively. A significant improvement rate was found in patients with age at time of thymectomy of ≤ 50 years(p = 0.0236), MGFA class V(p = 0.0154), and AchR-antibodies positivity(p = 0.0152). Thymectomy in patients with severe myasthenia gravis yields good perioperative outcomes and satisfactory long-term neurological improvement, especially for patients younger than 50 years, with MGFA class V and anti-AChR+ myasthenia gravis.

Achievement of Improved Post-Intervention Status in Patients with Generalized Myasthenia Gravis Treated with Ravulizumab during the CHAMPION MG Study (S5.008)

Achievement of Improved Post-Intervention Status in Patients with Generalized Myasthenia Gravis Treated with Ravulizumab during the CHAMPION MG Study (S5.008)

Effect of Thymectomy on Outcomes of Myasthenia Gravis Patients: A Case-Control Study at a Tertiary Care Hospital.

Background and objective Myasthenia gravis (MG) is an acquired autoimmune disease mediated by antibodies affecting the neuro-muscular junction on the postsynaptic membrane, resulting in neuromuscular transmission obstruction and, consequently, muscle weakening. It is believed that the thymus gland plays a critical role in the production of these antibodies. Screening patients for thymoma and surgical excision of the thymus gland is a crucial part of the treatment. To compare the odds of good outcomes in Myasthenia Gravis patients with or without thymectomy. Material and methods A retrospective case-control study was conducted at the Department of Medicine and Neurology, Ayub Teaching Hospital, Abbottabad, Pakistan, from October 2020 to September 2021. A purposive sampling technique was employed. Thirty-two MG patients with thymectomy and 64 MG patients without thymectomy were selected for investigation. Controls and cases were matched on the basis of sex and age (1:2). A positive EMGstudy, acetylcholine receptor antibodies, and a pyridostigmine testwere used to make the diagnosis of MG. Patients were called to the outpatient department for assessment of treatment outcomes. Primary outcome evaluation was done using the Myasthenia Gravis Foundation of America Post-Intervention Status (MGFA-PIS) tool at the last follow-up after one year. Results A sample of 96 patients was evaluated, of which 63 (65%) were females and 33 (34%) were males. The mean age for Group 1 (cases) was 35 years ±8.9 and for Group 2 (controls) was 37± 11.1. Age and Osserman stages were shown to be the two most crucial prognostic factors in our study. However, there are several other factors in our study that are linked to a poor response, such as a greater BMI, dysphagia, thymoma, older age, and a longer duration of disease. Conclusions Our findings indicate that none of the analysed groups had significantly worse outcomes as a result of the current clinical practice of thymectomy patient selection.

A retrospective study of the safety and efficacy of rituximab in Iranian patients with myasthenia gravis: A single-center experience.

Background: This retrospective cohort study was conducted to evaluate the efficacy and tolerance of rituximab (RTX) for the management of myasthenia gravis (MG). Methods: This retrospective cross-sectional study was conducted on 61 patients with refractory and non-refractory MG who received RTX. The Myasthenia Gravis Activities of Daily Living (MG-ADL) profile was used to assess MG symptoms and their effects on daily activities at the start of RTX and in the last follow-up. The Myasthenia Gravis Foundation of America Post-Intervention Status (MGFA-PIS) scale has been used as an outcome measure after treatment with RTX in the 12th month and the last follow-up. Results: The mean age of the patients was 40.31 ± 13.53 years (range: 15-78 years). Of 61 patients, eight (13.1%) were double seronegative, 29 (47.5%) had anti-acetylcholine receptor (AChR+) antibody, and 24 (39.3%) had anti-muscle-specific tyrosine kinase antibody (MuSK+). According to the mean rank table, the results of this study showed that the drug was more effective in improving the symptoms of MuSK+ patients compared to the other two groups (P = 0.006). The mean MG-ADL was 4.86 ± 1.83 before treatment and 1.51 ± 2.02 in the last follow-up visit. Paired t-test showed a significant association between MG-ADL before and after treatment in the last visit [t(55): 11.30, 95% confidence interval (CI): 2.79-3.99, P = 0.001)]. Conclusion: This retrospective study showed a considerable effect of RTX as induction therapy in patients with MG, especially those with MuSk+ MG.

Rituximab in myasthenia gravis: efficacy, associated infections and risk of induced hypogammaglobulinemia

The aim of this study is to evaluate the long-term efficacy, safety, and impact on immunoglobulin G (IgG) levels of rituximab in patients with myasthenia gravis (MG). A retrospective, observational study of drug-refractory MG patients treated with rituximab was done. The MG Foundation of America postintervention status (MGFA-PIS) was used to evaluate clinical response. Serum IgG levels were determined at baseline and post-treatment. Hypogammaglobulinemia was defined as IgG<7g/L. Thirty patients were included, 12 with anti-MuSK and 18 with anti-AChR antibodies. Mean (SD) follow-up was 85.5 (48) months. All 12 MuSK+ patients but only six (33%) AChR+ patients achieved minimal manifestations or remission (p<0.01). Nine severe infections were observed in five patients (17%). One patient was diagnosed with progressive multifocal leukoencephalopathy. At baseline, two patients (2/24; 8%) had hypogammaglobulinemia. During follow-up, hypogammaglobulinemia was observed in 60% (3/5) of patients who developed an infection and in 33% (7/21) who did not. Two of these patients died of infection-related complications. This study supports the effectiveness of rituximab in patients with MG, especially those with anti-MuSK antibodies. Severe infections may appear after rituximab treatment and hypogammaglobulinemia might play a role on it. A standard protocol would be needed to closely monitor IgG levels in MG patients treated with rituximab.

Efficacy and Safety of Tacrolimus Therapy for a Single Chinese Cohort With Very-Late-Onset Myasthenia Gravis.

Background and PurposePrevious studies have found tacrolimus to be a favorable drug for treating different types of myasthenia gravis (MG), but few have focused on very-late-onset MG (VLOMG). This study evaluated the efficacy and safety of tacrolimus for VLOMG therapy.MethodsThis was a retrospective single-center cohort study of 70 patients with VLOMG (onset ≥65 years) who visited Peking University First Hospital in 2019. Participants were divided into the tacrolimus (Tac) group and the control group based on tacrolimus usage. We further divided the Tac group into patients treated without corticosteroids and with corticosteroids. Sociodemographic features, clinical profiles, and outcomes were compared between different therapies and further analyzed by multivariate regression. Details of tacrolimus treatment, comorbidities, and adverse drug reactions (ADRs) were described.ResultsAmong 70 patients, the median (interquartile range) age at onset was 71 (68–77) years, and the follow-up duration was 27 (27-29) months. Most patients were types I (28%) and III (40%) according to the MG Foundation of America (MGFA) classification. In the Tac group, tacrolimus treatment was maintained for 36 (27-38) months. The dosage at the final evaluation was 1.0 (1.0–1.75) mg/day, and the last blood concentration test was 4.25 (2.85–5.7) ng/ml. A total of 43% reached remission, and 37% improved based on MGFA postintervention status (MGFA-PIS). For the 9 patients, newly diagnosed at enrollment within this group, MG activities of daily living (MG-ADL) decreased significantly from 3 (2-5) to 2 (1-2) (p = 0.041). Regarding the 13 patients, coadministering Wuzhi capsules the tacrolimus concentration increased from 2.75 (1.4–3.8) ng/ml to 5.95 (5.1–7.0) ng/ml (p = 0.012). No significant differences in outcomes were observed between tacrolimus treatment without and with corticosteroids or between the Tac group and the control group. A total of 93% had at least one comorbidity. ADRs related to tacrolimus emerged in 25% (9/36) of patients, most of which were not serious and reversible.ConclusionsTacrolimus is effective and safe in treating VLOMG. Tacrolimus monotherapy without corticosteroids can be used as an initial and maintenance treatment for VLOMG. Wuzhi capsules work well in elevating tacrolimus concentrations in this population.

Physical and mental fatigue in myasthenia gravis and its correlation with other symptoms.

Muscle weakness and easy fatigability are the clinical hallmarks of myasthenia gravis (MG). However, fatigue perception, which can be seen quite often in myasthenic patients, and its effect on the quality of life, irrespective of motor deficit, has not been elucidated yet. The aim is to evaluate the frequency of fatigue in myasthenic patients with nearly full muscle strength and the effect of fatigue on quality of life by assessing its correlation with other symptoms. Fifty-three patients with ocular or mild generalized MG in remission or minimal manifestations completed the questionnaires measuring the severity of MG and quality of life (MG Composite Scale and MG-Activities of Daily Living Profile). Both patient group and control group (53 healthy volunteers)completed the scales assessing fatigue [Fatigue Assessment Scale (FAS) and Fatigue Impact Scale (FIS)], depression [Beck Depression Inventory (BDI)] and sleep (Epworth Sleepiness Scale). Disease severity was assessed using MG Foundation of America (MGFA) and MGFA Post-Intervention Status classifications. FAS, FIS physical and BDI scores were significantly higher in patients compared to the control group (p = 0.003, p = 0.001, and p = 0.003, respectively) and fatigue was associated with depression and daytime sleepiness. Inpatient group, depressive symptoms and daytime sleepiness were higher in females (p = 0.019 and p = 0.013). The mean values of FIS total and cognitive scores were higher in patients with generalized MG (p = 0.033 and p = 0.045). Fatigue scores correlated with motor signs. Fatigue can be seen in MG independently from muscle weakness and is an important symptom worsening the quality of life.

Rituximab in Myasthenia Gravis- Experience from a Low- and Middle-Income Country (LMIC) Setting.

Myasthenia gravis (MG) is an immune-mediated disorder of the neuromuscular junction. About 10% are refractory to immunosuppressive therapy. To analyze the response of patients with generalized MG to rituximab. A retrospective review of patients with MG who received rituximab was carried out (n = 13, M:F = 6:7, mean age: 44.84 ± 15.73 years). Myasthenia Gravis Foundation of America (MGFA), MGFA post-intervention status (MGFA-PIS), and Myasthenia Gravis Status and Treatment Intensity (MGSTI) were assessed before and after rituximab. The duration of MG was 104.07 ± 92.25 months. Before rituximab, the MGFA was IIA/IIB/IIIA/IIIB/IVB/V in 1/1/2/6/2/1 patients and MGSTI was four in eight patients and six in three patients. The mean duration of follow up was 20.92 ± 14.06 months (range, 4 to 42 months). Dose reduction or discontinuation of cholinesterase inhibitors could be achieved 12 patients. Complete stable remission (CSR) and pharmacologic remission (PR) were achieved in one and four patients respectively and five patients had minimal manifestations. Most patients attained level 0, 1 or 2 MGSTI at last follow up. No rituximab infusion-related adverse events were noted. Three patients had exacerbation of MG between one to five weeks after rituximab administration. Three patients died, one each due to a cardiac event unrelated to MG or treatment, complications related to myasthenic crisis, and coronavirus disease. Rituximab was effective in bringing about remission in MG and can be considered as a first-line agent. However, it has to be administered under close supervision as some patients develop exacerbation of MG akin to steroid-induced worsening.

Effects of rituximab on prognosis in myasthenia gravis: A single-center experience from Turkey

Management of treatment-resistant patients with myasthenia gravis (MG) remains an important issue. This study aimed to evaluate the effects of rituximab (RTX) treatment on the prognosis of patients with acetylcholine receptor autoantibody-positive (AChR-Ab+), muscle-specific kinase autoantibody-positive (MuSK-Ab+), or seronegative or double seropositive MG. Nineteen patients treated with RTX between 2015 and 2020 were included in this study. Demographic and clinical characteristics, prognosis, and prognostic predictors of MG were evaluated retrospectively. The Myas-thenia Gravis Foundation of America Post-Inter-vention Status (MGFA-PIS) before RTX treatment (pre-RTX) and after RTX treatment (post-RTX) were recorded. A total of 10 patients (52.6%) were AchR Ab+, 6 patients (31.6%) were MuSK Ab+, 1 patient (5.3%) was seronegative, and 2 patients (10.5%) were double seropositive. Steroid dose was pre-RTX 38.9±5.7 (25-45), it was post-RTX 10.5±10.3 (0-30) (p<0.001). Post-RTX steroid treatment was discontinued in 6 of 19 patients (p=0.041). Only three patients received intravenous immunoglobulin at the post-RTX follow-up (p<0.001). In post-RTX 12th month, the MGFA-PIS score was as minimally manifestation or better in 9 patients (47.3%) and improved or was better in 18 patients (94.7%) (p-value 0.004; <0.001, respectively). The improvement in MGFA-PIS scores post-RTX was similar in MuSK-Ab+ and AChR-Ab+ patients. The data are insufficient in seronegative and double seropositive patients and RTX must be considered in the treatment of suitable patients with MuSK-Ab+ and AChR-Ab+ refractory MG.

Retrospective analysis of safety and outcomes of rituximab for myasthenia gravis in patients ≥65years old.

Optimal management of myasthenia gravis (MG) in individuals ≥65 y old is unknown and patient factors may limit therapeutic choices. Safety and efficacy of rituximab in older patients with MG has not been well-studied. This retrospective study examined 40 patients (14 patients ≥65 y old) treated with rituximab for MG. The primary efficacy outcome was the proportion of patients reaching "Improved" or better on Myasthenia Gravis Foundation of America (MGFA) Post-Intervention Status (PIS) at 12 mo, compared between younger and older patients. Ninety-two percent of patients ≥65 y old achieved MGFA PIS Improved or better at 12 mo compared to 69% of those <65 y old (P=.11). Median prednisone dose for the cohort decreased in the year following rituximab initiation (20 mg [interquartile range, 10-35] to 10mg [0-13], P=.01). Non-refractory MG was predictive of favorable outcome, whereas age was not. Serious adverse events (SAEs) were similar between older and younger patients (21.4% vs. 30.8%, P=.715). No patients ≥65 y old required discontinuation of rituximab due to SAE. One death occurred in a patient <65 y old due to systemic inflammatory response syndrome. At 12 mo following initiation of rituximab for MG, patients ≥65 y old experienced similarly high rates of improvement in their myasthenic symptoms as younger patients, without an increased risk of experiencing SAEs. Rituximab should be considered in the treatment paradigm in older patients and in non-refractory MG patients of any age.

Clinical profile and impact of comorbidities in patients with very-late-onset myasthenia gravis.

The purpose of this study was to evaluate the clinical profile of myasthenia gravis (MG) in older patients and determine the impact of medical comorbidities on their MG status and outcome. This was a retrospective chart review of patients with a symptom onset of MG at or after 65 years of age. Correlations were made between demographics, clinical characteristics, the Myasthenia Gravis Foundation of America (MGFA) severity scale scores, and Myasthenia Gravis Impairment Index (MGII) scores with two outcome measures: MGFA Post-Intervention Status (MGFA-PIS) and Simple Single Question (SSQ). The study population included 109 patients, with 90 of them having more than one follow-up visit. Their mean age was 75.3 ± 6.9 years and sex distribution was even. Of these patients, 67.7% had generalized MG. Nine-one percent of patients had one comorbidity. None of the demographic factors or comorbidities showed an association with MGFA-PIS, SSQ, or MGII after correction for multiple comparisons. Seventy-one percent of the patients improved with treatment, 12.4% remained unchanged, and 16.6% showed worsening at their last follow-up visit. Our study shows that patients with very-late-onset MG had a good prognosis and treatment response. None of the comorbidities had an impact on the severity of myasthenic symptoms or on outcome in these patients.

Clinical characteristics and outcomes of thymoma-associated myasthenia gravis.

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

Clinical features and treatment status of antiacetylcholine receptor antibody-positive ocular myasthenia gravis.

To clarify the factors predictive of progression from ocular myasthenia gravis (OMG) to generalized MG (GMG) among patients with positive antiacetylcholine receptor antibodies (AChRAb) and to evaluate the efficacy of our OMG treatment protocol including tacrolimus (TAC). Retrospective study. Fifty-two patients with AChRAb positivity and ptosis and/or eye movement disturbance showing diurnal fluctuation and fatigability were included. First, we compared the clinical characteristics of patients without OMG progression (p-OMG) with those of patients whose OMG transformed to GMG (TMG). Second, we subdivided the p-OMG patients according to the time of approval of TAC into the Before group and the After group. We compared the dosage and period of prednisolone administration (PSL) of the 2 groups. Third, we evaluated the responses to OMG treatment using the Myasthenia Gravis Foundation of America Postintervention Status (MGFA-PS) scale. Forty-one patients were classified into the p-OMG group, and 11, into the TMG group. The AChRAb level (P = 0.0006) and prevalence of thymoma (P = 0.001) were significantly higher in the TMG group than in the p-OMG group. In the p-OMG group, the MG composite score (P ≤ 0.0001) and AChRAb level (P = 0.005) improved after treatment. The periods of PSL administration at ≥ 20mg/day (P = 0.009) and at 10 to 19mg/day (P = 0.002) were significantly shorter in the After group. At the last follow-up, 78.0% of the p-OMG group patients had achieved MGFA-PS minimal manifestations or better status with PSL ≤ 5mg/day. The TMG cases were characterized by higher AChRAb levels and presence of thymoma. The P-OMG patients could be managed with our treatment protocol. Combined use of TAC was efficacious in patients with steroid-dependent p-OMG.

Prognostic predictors of remission in ocular myasthenia after thymectomy.

BackgroundIdentifying prognostic predictors can assist in making clinical decisions. This study aimed to identify the potential predictors of remission in patients with ocular myasthenia gravis (OMG) after thymectomy.MethodsOMG patients who had thymectomy between 2011 and 2017 were reviewed retrospectively. Clinical outcomes were assessed according to the Myasthenia Gravis Foundation of America Post-Intervention Status (MGFA-PIS). Kaplan-Meier analysis was used to estimate the cumulative probability of complete stable remission (CSR). Univariate and multivariable analyses with Cox proportional hazards regression were used to identify predictors of CSR.ResultsFifty-one patients (23 male, 28 female) with a median age at OMG onset of 40 (range, 5–79) years were eligible for inclusion. Patients with thymomatous OMG (n=9) had a statistically older median age at disease onset [61 (range, 32–78) vs. 33.5 (range, 5–79) years, P=0.001], shorter duration from disease onset to thymectomy [3 (range, 2–24) vs. 10 (range, 1–132) months, P=0.004], and a higher rate of postoperative complication (44.4% vs. 9.5%, P=0.025), compared with non-thymomatous OMG (n=42). The estimated cumulative probability of CSR in the whole cohort was 41.8% (95% CI, 28.6–58.2%) 5 years after surgery. Age at onset of 40 years or younger (P=0.00016), female sex (P=0.069), and thymic hyperplasia (P=0.0061) were potential predictors under univariate analysis. However, only age at onset of 40 years or younger (HR: 4.117, 95% CI, 1.177–14.399, P=0.027) remained significant after multivariable analysis.ConclusionsCSR could be achieved in about 40% of OMG patients 5 years after thymectomy and is likely to be predicted by age at onset of 40 years or younger.

Robotic-Extended Rethymectomy for Refractory Myasthenia Gravis: A Case Series

To assess the safety and efficacy of robotic-extended rethymectomy in selected refractory myasthenia gravis (MG) patients with suspected residual thymic tissue. Robotic-extended rethymectomy was performed in 6 MG patients with seropositive acetylcholine receptors (AChR) antibody who had undergone a previous thymectomy (1 cervicotomy, 2 video-assisted thoracoscopic surgeries, and 3 sternotomies). The median observation time before robotic rethymectomy was 108 (24-171) months. The main outcomes were perioperative morbidity, mortality, conversion to open surgery, and clinical outcomes according to the Myasthenia Gravis Foundation of America Post-Intervention Status (MGFA-PIS). Before rethymectomy, all patients required immunosuppressants and 5 patients (83.3%) required intravenous immune globulin and/or plasma exchange to control the symptoms. The median specimen weight was 24.5 (14-144) g after rethymectomy, and residual thymic tissue was found in 5 patients (83.3%). No conversion to open surgery or perioperative morbidity and mortality was observed. With a median follow-up time of 46.5 (13-155) months, 3 patients (50%) achieved "improved" and 3 (50%) were "unchanged" according to the MGFA-PIS. Compared with preoperative use, the median daily dose of corticosteroids statistically decreased (25 [7.5-60] vs 0 [0-5] mg, P = 0.002) without significant change in azathioprine use (100 [0-200] vs 50 [0-150] mg, P = 0.360). AChR antibody positive MG patients with a treatment refractory long-term course after thymectomy might have remaining thymic tissue with the 2 commonly associated thymus pathologies, thymoma, and follicular hyperplasia. Robotic-extended rethymectomy might be considered as a safe and beneficial treatment option in these patients.

Thymectomy in ocular myasthenia gravis before generalization results in a higher remission rate.

This study aimed to compare the outcomes of patients with ocular myasthenia gravis (OMG) who underwent thymectomy before generalization with the outcomes of those who underwent thymectomy after generalization. We retrospectively reviewed patients who underwent robotic thymectomy for myasthenia gravis between January 2003 and February 2018. Patients who presented with purely ocular symptoms at myasthenia gravis onset were eligible for inclusion. Exclusion criteria were patients who were lost to follow-up and patients who underwent re-thymectomy. Patients with OMG who developed generalization before thymectomy were categorized into gOMG group and those who did not were categorized into OMG group. The primary outcome was complete stable remission according to the Myasthenia Gravis Foundation of America Post-Intervention Status (MGFA-PIS). One hundred and sixty-five (66 males and 99 females) out of 596 patients with myasthenia gravis were eligible for inclusion. Of these, there were 73 and 92 patients undergoing thymectomy before and after the generalization of OMG, respectively. After propensity score matching, a data set of 130 patients (65 per group) was formed and evaluating results showed no statistical differences between the 2 groups. The estimated cumulative probabilities of complete stable remission at 5 years were 49.5% [95% confidence interval (CI) 0.345-0.611] in the OMG group and 33.4% (95% CI 0.176-0.462) in the gOMG group (P = 0.0053). Similar results were also found in patients with non-thymomatous subgroup [55 patients per group, OMG vs gOMG, 53.5% (95% CI 0.370-0.656) vs 28.9% (95% CI 0.131-0.419), P = 0.0041]. Thymectomy in OMG before generalization might result in a higher rate of complete stable remission than thymectomy after generalization.