Ocular Myasthenia Gravis Research Articles

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.

Analysis of clinical features and outcomes in patients with ocular myasthenia gravis according to anti-acetylcholine receptor antibody-seropositivity.

To comparatively analyze the clinical characteristics of patients with ocular myasthenia gravis (OMG) referred to an ophthalmology clinic, according to anti-acetylcholine receptor antibody (AchR Ab)-seropositivity. Retrospective Cohort Study. Medical records of patients with OMG who presented to a tertiary eye care center between 2003 and 2020 were retrospectively reviewed. Demographics, ophthalmologic characteristics, response to medical treatment, presence of autoimmune thyroid disease and thyroid autoantibody were compared between the AchR Ab seropositive and seronegative groups. A total of 130 patients with OMG were identified; among them, 46 patients (35.4%) had autoantibody against acetylcholine receptors. The mean age at symptom onset was 42.4 ± 18.9 years. There were no differences in mean age at symptom onset, gender ratio, and mean follow-up period between patients with seropositive and seronegative OMG. Graves ophthalmopathy was significantly more frequent in seronegative patients (p = 0.04), while thymic disease (p < 0.01) was more frequent in seropositive patients (p < 0.01). Among patients with seropositive OMG, 52.3% showed a good response to medical treatment, while only 31.4% of the seronegative patients were classified as good responders (p = 0.01). Thyroid dysfunction was found in 27.4% patients with OMG and the proportion of thyroid dysfunction was not different according to anti-acetylcholine receptor antibody-seropositivity. Seropositivity to acetylcholine receptor antibody is associated with a better response to medical treatment and lower risk of concomitant autoimmune thyroid disease in patients with OMG.

Diagnostic accuracy of voluntary and stimulated neuromuscular jitter studies in ocular myasthenia gravis.

There is a lack of studies comparing the accuracy of neuromuscular jitter analysis during voluntary activation (v-jitter study) versus axonal stimulation (s-jitter study). The study aimed to compare these two techniques in the same population of patients with suspected ocular myasthenia gravis (OMG). Fourteen control subjects (mean age: 55.5 ± 15.2 years) and 34 patients with suspected OMG (mean age: 59 ± 13.9 years) were prospectively evaluated. Twenty spike pairs and 30 individual spikes were analyzed during v-jitter and s-jitter study, respectively. Two different criteria for abnormal individual jitter values were evaluated: ≥ or > than 10% values exceeding the upper normal limit. OMG was diagnosed in 19 patients based on clinical and laboratory findings, without considering jitter measurements. In most patients, v-jitter and s-jitter analyses provided comparable results. The maximum sensitivity (89%) was achieved with s-jitter study using the ≥10% criterion, while the maximum specificity (93%) was found with v-jitter study using the >10% criterion. Both v-jitter and s-jitter studies showed good to very good accuracy for the diagnosis of OMG, in the absence of any statistically significant difference. Therefore, the patient's cooperation level and examiner's experience should guide the choice of performing v-jitter or s-jitter analysis in patients with suspected OMG.

Type and Frequency of Misdiagnosis and Time Lag to Diagnosis in Patients with Chronic Progressive External Ophthalmoplegia.

Since ptosis is an early feature of chronic progressive external ophthalmoplegia (CPEO), patients are commonly misdiagnosed with other causes of ptosis. This study aims to report the type and frequency of misdiagnosis and time lag to diagnosis and the palpebral fissure transfer (PFT) procedure in patients with CPEO. This is a retrospective analysis of consecutive patients with CPEO who underwent PFT between 2006 and 2017. The data on previous diagnoses and treatments, age at definitive diagnosis of CPEO, and clinical manifestations were recorded. While the diagnosis of CPEO was based on clinical examination, 75% (24/32) of patients had undergone a confirmatory muscle biopsy and genetic tests. There were 32 patients (19 females) with a mean age of 24.8 years (range, 13-36) at the final diagnosis and 34.1 years (range, 15-56) at the time of PFT. Also, 78% (25/32) of patients had been initially misdiagnosed with congenital ptosis (60%; 15/25) and ocular myasthenia gravis (OMG) (40%; 10/25). The majority of patients (20/32) had one to three previous eyelid surgical procedures, of which 90% (18/20) were performed before the definitive diagnosis of CPEO. The mean time lag from the first surgical procedure to CPEO diagnosis and PFT was 6.2 and 14.7 years, respectively. In a referral center, 78% of the patients with CPEO were initially misdiagnosed with congenital ptosis and OMG, and 56% of them underwent ptosis repair before the diagnosis. While the onset of the disease was in the first or second decades of life, diagnosis was delayed up to a mean age of 25 years. Reviewing early family photos and paying attention to other signs of CPEO could prevent misdiagnosis.

Clinical characteristics and therapeutic effect of myasthenia gravis coexisting with thyroid eye disease.

Here, we describe the clinical characteristics and therapeutic effects of myasthenia gravis (MG) coexisting with thyroid eye disease (TED). We collated clinical data from MG patients in our hospital between 2012 and 2022 and analyzed the clinical characteristics of MG patients with hyperthyroidism, MG patients with TED and ocular myasthenia gravis (OMG) patients with TED. We recruited 62 MG patients with hyperthyroidism, including 13 MG patients with TED and 10 OMG patients with TED. There were 70 MG patients without hyperthyroidism; 29 of these were OMG. Compared with patients without hyperthyroidism, patients with hyperthyroidism had an earlier age at onset and milder clinical symptoms (P < 0.05). The incidence of thymus hyperplasia in patients with hyperthyroidism and TED was significantly lower than that in patients without TED (38.5% vs. 69.4%, P < 0.05); these patients also had a significantly lower antibody titer for the acetylcholine receptor [0.72 (0.27, 14.93) nmol/L vs. 2.38 (0.28, 49.51) nmol/L, P < 0.05]. Diplopia was significantly more frequent in OMG patients with TED than in patients with OMG (84.6% vs. 44.8%, P < 0.05), and the rate of diplopia in OMG patients with TED was significantly higher after treatment with bromostigmine and glucocorticoid (69.2% vs. 3.4%, P < 0.05). MG patients with TED had a significantly lower incidence of thymus hyperplasia and a lower antibody titer for the acetylcholine receptor. Patients with OMG and TED are more likely to develop diplopia; it is very difficult to treat diplopia in these patients.

Effect of Immunosuppression in Risk of Developing Generalized Symptoms in Ocular Myasthenia Gravis: A Retrospective Cohort Study.

Early use of immunosuppression has been suggested to prevent generalization of ocular myasthenia gravis (OMG), but high-quality evidence is limited in this regard. We examined whether treatment with prednisone and other immunosuppressants reduce the risk of generalization in OMG. This is a retrospective study of consecutive adults with pure OMG who had a minimum 6 months of follow-up. The main outcome was the time to developing generalized symptoms. We used propensity scores to create matched data sets of patients treated with prednisone or any immunosuppressant vs controls. We also used unmatched models with inverse probability of treatment weights (IPTW) and variable exposure times. We used Cox proportional hazards model to estimate hazard ratio (HR) for generalization, comparing treated patients vs controls. A total of 154 patients were included, with a mean follow-up of 87.4 ± 73 months since onset. Forty-three (28%) were generalized, and mean time to generalization from diagnosis was 24.2 ± 24.1 months. Patients who received prednisone had lower risk of generalization than controls, with pooled HR 0.43 (95% CI 0.19-1.06) for the matched model, HR 0.46 (95% CI 0.21-0.89) for the IPTW model, and for HR 0.44 (95% CI 0.23-0.81) for the time-dependent exposure model. Patients who received any immunosuppressant had lower risk of generalization, with HR 0.30 (95% CI 0.11-0.77), 0.32 (95% CI 0.14-0.70), and 0.35 (95% CI 0.15-0.80) for the matched, IPTW, and IPTW-varying exposure models, respectively. Our study provides evidence that steroidal and nonsteroidal immunosuppression in patients with OMG is associated with a reduced risk of developing generalized symptoms over time. This supports the early use of immunosuppression in this population. This study provides Class III evidence that treatment of OMG with corticosteroids or nonsteroidal immunosuppressants reduces the risk of generalization.

Characterizing Myasthenia Gravis Symptoms, Exacerbations, and Crises From Neurologist's Clinical Notes Using Natural Language Processing.

Background Myasthenia gravis (MG) is a rare, autoantibody neuromuscular disorder characterized by fatigable weakness. Real-world evidence based on administrative and structured datasets regarding MG may miss important details related to the clinical encounter. Examination of free-text clinical progress notes has the potential to illuminate aspects of MG care. Objective The primary objective was to examine and characterize neurologist progress notes in the care of individuals with MG regarding the prevalence of documentation of clinical subtypes, antibody status, symptomatology, and MG deteriorations, including exacerbations and crises.The secondary objectives were to categorize MG deteriorations into practical, objective states as well as examine potential sources of clinical inertia in MG care. Methods We performed a retrospective, cross-sectional analysis of de-identified neurologist clinical notes from 2017 to 2022. A qualitative analysis of physician descriptions of MG deteriorations and a discussion of risks in MG care (risk for adverse effects, risk for clinical decompensation, etc.) was performed. Results Of the 3,085 individuals with MG, clinical subtypes and antibody status identified included gMG (n = 400; 13.0%), ocular MG (n = 253; 8.2%), MG unspecified (2,432; 78.8%), seropositivity for acetylcholine receptor antibody (n = 441; 14.3%), and MuSK antibody (n = 29; 0.9%). The most common gMG manifestations were dysphagia (n = 712; 23.0%), dyspnea (n = 626; 20.3%), and dysarthria (n = 514; 16.7%). In MG crisis patients, documentation of difficulties with MG standard therapies was common (n = 62; 45.2%). The qualitative analysis of MG deterioration types includes symptom fluctuation, symptom worsening with treatment intensification, MG deterioration with rescue therapy, and MG crisis. Qualitative analysis of MG-related risks included the toxicity of new therapies and concern for worsening MG because of changing therapies. Conclusions This study of neurologist progress notes demonstrates the potential for real-world evidence generation in the care of individuals with MG. MG patients suffer fluctuating symptomatology and a spectrum of clinical deteriorations. Adverse effects of MG therapies are common, highlighting the need for effective, less toxic treatments.

Ocular versus generalized myasthenia gravis: a continuum associated with acetylcholine receptor antibody titers

The aim of this study was to evaluate clinical and serological differences between the ocular myasthenia gravis (oMG) and generalized MG (gMG). This study is a retrospective chart review, in which data was collected from patients fulfilling 2 of 3 diagnostic MG criteria (positive antibodies, evidence of neuromuscular transmission defect on neurophysiological examination, positive effect of pyridostigmine treatment). 350 patients were included and data concerning demographics and MG medical history were collected. Patients with oMG accounted for 15.7 % of the included patients. The two subgroups differed significantly in oMG having a later age at onset, lower AChR antibody-titers, longer doctor-to-diagnosis delay and less intensive MG treatment. Additionally, patients with oMG were faster at reaching a well-controlled disease state. Thymus pathology, number of antibody-positive (95.9 % of gMG and 94.5 % of oMG), sex, number of other autoimmune diseases and delay before drug stability did not differ between oMG and gMG. In conclusion, oMG is presumably a milder form of gMG characterized by lower AChR antibody-titers, a milder phenotype, and a quicker response to a less aggressive treatment. But otherwise, oMG and gMG show very similar characteristics, including the same frequency of positive AChR antibodies, which seems new compared to previous reports.

Quantification of saccadic fatigability and diagnostic efficacy for myasthenia gravis.

The diagnostic challenge of myasthenia gravis (MG) is exacerbated by the variable efficacy of current testing methodologies, necessitating innovative approaches to accurately identify the condition. This study aimed to assess ocular muscle fatigue in patients with MG using video-oculography (VOG) by examining repetitive saccadic eye movements and comparing these metrics to those of healthy control participants. This prospective, cross-sectional study was conducted at a tertiary care center and involved 62 patients diagnosed with MG (48 with ocular MG and 14 with generalized MG) and a control group of 31 healthy individuals, matched for age and sex. The assessment involved recording saccadic eye movements within a ± 15° range, both horizontally and vertically, at a rate of 15 saccades per minute over a 5-min period, resulting in 75 cycles. Participants were afforded a 3-min rest interval between each set to mitigate cumulative fatigue. The primary outcome was the detection of oculomotor fatigue, assessed through changes in saccadic waveforms, range, peak velocity, latency, and the duration from onset to target, with a focus on comparing the second saccade against the average of the last five saccades. In the evaluation of repetitive saccadic movements, patients with MG exhibited a reduced saccadic range and a prolonged duration to reach the target, compared to healthy subjects. Furthermore, a significant elevation in the frequency of multistep saccades was observed among MG patients, with a marked rise observed over consecutive trials. Receiver operating characteristic (ROC) analysis revealed the discriminative performance of multistep saccade frequency, in conjunction with variations in saccadic range and duration from onset to target achievement between the second saccade and the mean of the final five saccades, as effective in distinguishing MG patients from healthy subjects. Although alterations in peak saccadic velocity and latency were less pronounced, they were nevertheless detectable. The utilization of VOG for repetitive saccadic testing in the diagnosis of MG has demonstrated considerable diagnostic precision. This methodology affords significant accuracy in evaluating ocular muscle fatigue in MG patients, providing class III evidence supportive of its clinical application.

Clinical Characteristics and Treatment of Juvenile Myasthenia Gravis-A Single-Center Experience.

Juvenile myasthenia gravis (MG) is a rare autoimmune neuromuscular disease, often treated with anticholinesterases, corticosteroids, and immunosuppressants. However, optimal treatment durations remain unclear. This study investigated the clinical characteristics and treatment of juvenile MG, including medication duration. The administration period for all drugs, immunosuppressants, and prednisolone at doses greater than 0.35 mg/kg daily was extracted retrospectively from medical records. Nineteen participants (8 boys, 11 girls) aged 8 months to 14 years (median, 2.5 years) at onset were identified. Fourteen patients (73.7%) had ocular MG and five (26.3%) had generalized MG. Drug treatment was conducted in 18 cases; however, 7 patients did not complete the treatment. Among the patients who completed drug treatment, the duration of treatment ranged from 11 to 100 months (median, 47 months). In the six patients treated with continuous administration of prednisolone or immunosuppressants, the treatment duration ranged from 33 to 99 months (median, 56 months). No severe adverse effects requiring hospitalization were reported. The patients treated with prednisolone or immunosuppressants required at least 33 months of treatment. These results will help develop protocols for juvenile MG treatment.

Ocular myasthenia gravis and sub clinical hyperthyroidism- a rare occurrence

Ocular myasthenia gravis and sub clinical hyperthyroidism- a rare occurrence

Accuracy of Repetitive Ocular Vestibular-Evoked Myogenic Potentials to Diagnose Myasthenia Gravis in Patients With Ptosis or Diplopia.

We developed repetitive ocular vestibular-evoked myogenic potentials (roVEMP) as an electrophysiologic test that allows us to elicit the characteristic decrement of extraocular muscles in patients with ocular myasthenia gravis (OMG). Case-control studies demonstrated that roVEMP reliably differentiates patients with OMG from healthy controls. We now aimed to evaluate the diagnostic accuracy of roVEMP for OMG diagnosis in patients with ptosis and/or diplopia. In this blinded prospective diagnostic accuracy trial, we compared roVEMP in 89 consecutive patients presenting with ptosis and/or diplopia suspicious of OMG with a multimodal diagnostic approach, including clinical examination, antibodies, edrophonium testing, repetitive nerve stimulation of accessory and facial nerves, and single-fiber EMG (SFEMG). We calculated the roVEMP decrement as the ratio between the mean of the first 2 responses compared with the mean of the sixth-ninth responses in the train and used cutoff of >9% (unilateral decrement) in a 30 Hz stimulation paradigm. Following a complete diagnostic work-up, 39 patients (44%) were diagnosed with ocular MG, while 50 patients (56%) had various other neuro-ophthalmologic conditions, but not MG (non-MG). roVEMP yielded 88.2% sensitivity, 30.2% specificity, 50% positive predictive value (PPV), and 76.5% negative predictive value (NPV). For comparison, SFEMG resulted in 75% sensitivity, 56% specificity, 55.1% PPV, and 75.7% NPV. All other diagnostic tests (except for the ice pack test) also yielded significantly higher positive results in patients with MG compared with non-MG. The study revealed a high sensitivity of 88.2% for roVEMP in OMG, but specificity and PPV were too low to allow for the OMG diagnosis as a single test. Thus, differentiating ocular MG from other neuro-ophthalmologic conditions remains challenging, and the highest diagnostic accuracy is still obtained by a multimodal approach. In this study, roVEMP can complement the diagnostic armamentarium for the diagnosis of MG. This study provides Class I evidence that in patients with diplopia and ptosis, roVEMP alone does not accurately distinguish MG from non-MG disorders. ClinicalTrials.gov: NCT03049956.

Advances in Understanding and Managing Myasthenia Gravis: Current Trends and Future Directions.

Myasthenia gravis (MG) is an autoimmune illness characterized by autoantibodies against the acetylcholine receptor (AChR), muscle-specific tyrosine kinase (MuSK), and an increasing number of extra postsynaptic proteins. Pathogenic autoantibodies reduce the number of functional AChRs in the neuromuscular junction's (NMJ)muscle end plate. The cause of the autoimmune response is unknown, but thymic abnormalities and immune regulatory deficiencies are significant. The disease's incidence is likely influenced by genetic predisposition, with sex hormones and exercise playing a role. MGcan affect any age, race, or gender and can be caused by any stressor, with infections being the most frequent cause. Treatment focuses on airway support and the triggering incident. MG is a rare autoimmune disease causing fatigue-inducing weakness in the axial, respiratory, leg, and bulbar muscles. Initially affecting the eyes, most MGpatients experience at least one worsening symptom during their illness. The disease is mainly caused by antibodies against the AChR, dependence on the immune system within cells, and engagement of the complement system. The complement system plays a significant role in MG, and complement inhibition can both prevent the onset and slow its development. Ocular MG affects around 15% of people, with most patients having blocking antibodies against the cholinergic receptor. There may be correlations between thymoma and other autoimmune conditions, especially thyroid illness. Treatment and management for MGinvolve removing autoantibodies from circulation or blocking effector mechanisms using techniques such as complement inhibition, plasmapheresis, and B-cell elimination.

Taste disorders and alopecia in myasthenia gravis

BackgroundNon-motor symptoms in myasthenia gravis (MG) are rarely confirmed. Although there are some small cohort studies, a large-systemic survey has not yet been performed.MethodsWe investigated the incidence and clinical characteristics of patients with MG who had taste disorders and alopecia using data of 1710 patients with MG enrolled in the Japan MG Registry 2021.ResultsAmong them, 104 (6.1%) out of 1692 patients and 138 (8.2%) out of 1688 patients had histories of taste disorders and alopecia, respectively. Among the patients with MG, taste disorders were significantly more common in women, those with severe symptoms, refractory MG, or thymoma-associated MG, and were less common in those with ocular MG. The taste disorders often occurred after the onset of MG and often responded to MG treatments. Alopecia was more common in MG patients with a history of bulbar palsy and thymoma, and it often occurred before the onset of MG and sometimes responded to MG treatments. Multivariate logistic regression analysis revealed taste disturbance was associated with worst quantitative MG score and thymoma-associated MG; and alopecia was associated with thymoma-associated MG.ConclusionClinicians should be aware of the non-motor symptoms in MG, especially in patients with severe myasthenic symptoms and thymoma-associated MG.

Pearls & Oy-sters: Thyroid-Associated Ophthalmopathy and Transient Neuromuscular Junction Disorder Due to Graves Disease.

The concomitant presentation of thyroid-associated ophthalmopathy (TAO) and ocular myasthenia gravis is well documented. In the course of Graves disease (GD), symptomatic transient neuromuscular junction disorder may occur due to the effect of thyroid hormones at the neuromuscular synapse. Diagnostic clues are the clinical and electrophysiologic remission synchronous with restoration of euthyroidism. Furthermore, the occurrence of thymic hyperplasia in GD poses further diagnostic and therapeutic considerations. These points are discussed in the case report of a 43-year-old male patient suffering from TAO and transient neuromuscular junction disorder due to GD.

Progressive Bilateral Ptosis in Adduction Deficit: True Internuclear Ophthalmoplegia (INO) or Pseudo-INO?

Abstract&#x0D; Introduction : Bilateral INO is a rare disease caused by a lesion in the medial longitudinal fasciculus (MLF) in the pons, resulting in adduction deficit. Progression to ptosis is rare and may confuse with pseudo-INO as in ocular myasthenia gravis (OMG). This case aims to report progressive ptosis in bilateral INO that mimics pseudo-INO.&#x0D; Case Illustration : A 36-year-old woman complained sudden binocular diplopia 4 days before admission. Bilateral adduction deficits (-3 and -2 of right and left eye, respectively) with nystagmus were seen. There was no ptosis. The patient was diagnosed with bilateral INO. One month later, the patient returned with bilateral ptosis. The marginal reflex distance 1 (MRD1) were 0 on both eyes. Post fatigue and ice pack test showed 2mm difference of MRD1. The single fiber electromyography (EMG) result came out negative with jitter &lt;10%. Brain MRI revealed lesions in the posterior pons and periaqueductal on the level of midbrain.&#x0D; Discussion : The MLF controls 6th and contralateral 3rd cranial nerve nucleus for horizontal gaze. Ptosis in INO can occurred due to central caudal subnucleus involvement in midbrain that innervates levator palpebra. The result of fatigue and ice pack test may be misleading, as those are not pathognomonic for OMG. The single-fiber EMG are valuable modalities to exclude pseudo-INO in this case.&#x0D; Conclusion : Progressive bilateral ptosis may occur in bilateral INO. Fatigue and ice pack test may give false- positive results leading to confusion with pseudo-INO, rather than true INO. Supporting examinations are needed to confirm the diagnosis.

Peribulbar Corticosteroids for Ocular Myasthenia Gravis.

Ocular myasthenia gravis is treated predominantly by oral medications, with the potential for systemic adverse effects. Successful treatment has been achieved using peribulbar dexamethasone. We assessed the effect of peribulbar dexamethasone or triamcinolone (40-mg Triesence), a longer-acting corticosteroid, targeting the peribulbar area as opposed to directly injecting the affected extraocular muscle. This more convenient and secure approach holds the potential for straightforward integration within clinical environments. Retrospective chart review. Five patients were identified that were treated with peribulbar corticosteroids. In 4 of the 5 cases, ophthalmoparesis was unilateral. One case had isolated ptosis, and 4 had both ptosis and ophthalmoparesis. Three of these 4 cases reported complete resolution of symptoms within weeks of a single injection. Improvement lasted between 5 to 6 months, and all patients responded to repeated injections. Peribulbar corticosteroids can be effective in ocular myasthenia gravis. We suggest that longer-acting agents such as triamcinolone are preferable, to reduce injection frequency.

Prevalences of Other Non-Thyroid Autoimmune Diseases and Factor Associated with Their Presence in Ocular Myasthenia Gravis.

To report the prevalences of other non-thyroid autoimmune diseases and identify factors associated with their presence in ocular myasthenia gravis (OMG) subjects. A total of 208 subjects with OMG diagnosis were included. Demographic data, clinical characteristics, the ice-pack test, the acetylcholine receptor (AChR) antibody test, electrophysiology tests (single-fiber electromyography and repetitive nerve stimulation), the presence of thymoma, generalized myasthenia gravis conversion, and the presence of other non-thyroid autoimmune diseases (defined as the presence of at least one other non-thyroid autoimmune disease) were retrospectively reviewed. Factors associated with the presence of other non-thyroid autoimmune diseases were analyzed by univariate and multivariate logistic regression. Of the total 208 subjects, 21 (10.10%) exhibited the presence of other non-thyroid autoimmune diseases (19 subjects (9.14%) and 2 subjects (0.96%) had one and two other non-thyroid autoimmune diseases, respectively), and systemic lupus erythematosus (SLE) was diagnosed in 9 subjects, followed by Sjogren's syndrome (7 subjects), rheumatoid arthritis (6 subjects), and ankylosing spondylitis (1 subject). Therefore, the prevalences of SLE, Sjogren's syndrome, rheumatoid arthritis, and ankylosing spondylitis in OMG subjects were estimated to be 4.33% (95% confidence interval (CI): 2.29-8.02%), 3.37% (95% CI: 1.64-6.79%), 2.88% (95% CI: 1.33-6.14%), and 0.48% (95% CI: 0.08-2.67%), respectively. Positivity of the AChR antibody was the only significant factor associated with the presence of other non-thyroid autoimmune diseases (odds ratio 4.10, 95% CI: 1.11-15.21, p = 0.035). The presence of other non-thyroid autoimmune diseases was found in approximately 10% of OMG patients, with SLE displaying the highest prevalence. We recommend screening and monitoring for other non-thyroid autoimmune diseases in OMG patients, particularly those with positivity of the AChR antibody.

Intravenous methylprednisolone therapy for ocular myasthenia gravis: A retrospective study

AbstractBackgroundAlthough intravenous methylprednisolone pulse (IVMP) therapy has been recommended for ocular myasthenia gravis (OMG), the evidence is limited.AimWe aimed to investigate the efficacy and safety of IVMP for OMG.MethodsThe patients with OMG were chosen retrospectively from May 2010 to December 2022. The therapeutic effects between IVMP‐treated and non‐IVMP‐treated groups were determined by ∆ocular quantitative MG (QMG) score and the ∆ocular MG activities of daily living profile (MG‐ADL) score, which is the disparities of the scores between before and after 1 month of drug administration; and Myasthenia Gravis Foundation of America post‐intervention status at 1, 3, 6, and 12 months.ResultsThere were 26 patients with OMG included, with the mean age of 67.2 ± 13.1 years. 13 of the 26 people with OMG received IVMP and subsequent low‐dose immunotherapies. The ∆ocular QMG and ∆ocular MG‐ADL scores were significantly higher in the IVMP group of 3 (1.5, 4) and 3 (2, 4.5) than in the non‐IVMP group of 1.5 (0, 3) and 1 (0, 2), p = 0.038 and p = 0.0027, respectively. The rates of minimal manifestation or better status in post‐intervention status at 1 and 12 months were also considerably higher in the IVMP group of 77% and 92% compared with the non‐IVMP group of 0% and 23%, p = 0.0001 and 0.001. There were no serious side effects discovered.ConclusionIVMP induction therapy can quickly and safely improve symptoms of OMG, and subsequent low‐dose immunotherapy can keep symptoms at bay for 12 months.

Clinical features of double seronegative ocular myasthenia gravis.

To clarify the clinical features of patients with Double seronegative (DS) ocular myasthenia gravis (OMG). Sixty-one patients diagnosed with DS OMG at the Department of Ophthalmology, Hyogo Medical University Hospital over a 5-year period from 2017 were included. Patients were classified into three groups based on the initial examination findings: group P (ptosis alone), group M (ocular motility disorder alone), and group PM (combination of both). We retrospectively reviewed the patients and clarified their clinical features. There were 32 males and 29 females, with a mean age of 49.8 ± 20.9:1-82years. Twenty-one patients (34.4%) were in group P, 23 (37.7%) in group M, and 17 (27.8%) in group PM. The proportion of males (73.9%) was significantly higher in group M compared with the other two groups. The diagnosis was proven by detection of neuromuscular junction (NMJ) disorder in 73.8%, oral pyridostigmine trial test in 13.1%, and eight patients (13.1%) in group M were diagnosed after surgical treatment. The clinical symptoms were resolved by oral pyridostigmine treatment in 54.1% of cases. About 30% of patients with DS OMG had no obvious NMJ disorder, and an oral pyridostigmine trial test was necessary to diagnose these patients. Although DS OMG is often considered as the mildest form of MG, its prognosis is not optimistic and it requires aggressive therapeutic intervention. Trial registration number: 202104-750, "2016/4/18," retrospectively registered.