OBJECTIVES

To describe the clinical profile and outcomes of a series of adult patients diagnosed with ocular myasthenia gravis and to evaluate the potential factors affecting the risk of generalization.

This retrospective cohort study involved a medical chart review of adult patients seen from 2012 to 2019 at the neuro-ophthalmology clinic of a tertiary Philippine hospital with a clinical diagnosis of ocular myasthenia gravis supported by serologic, electrophysiologic, or pharmacologic test results. Outcomes of interest were complete stable remission, pharmacologic remission, minimal manifestations, and generalization. Kaplan-Meier method and log-rank test were used to analyze the probability of generalization.

The study sample consisted of 16 patients. The female to male ratio was 3:1. Mean age at symptom onset was 39 years. All patients received pharmacologic treatment, while two patients underwent thymectomy. No patient had remission as of last follow-up. Three patients had conversion of ocular myasthenia gravis to generalized myasthenia gravis. Mean time from symptom onset to generalization was 10.7 months. The generalization curves of patients who were symptomatic for less than two years and those who were symptomatic for at least two years prior to consult were significantly different (p = 0.049).

In this single-center study, there was female predominance among adult patients diagnosed with ocular myasthenia gravis. The incidence of generalization was 4 per 100 person-years while the 2-year probability of generalization was 30%. Further study is needed in order to determine the factors affecting the risk of generalization.

Thymomas are rare tumours which generally account for only 0.2 – 1.5% of mediastinal tumours in adults. Around 40% of patients present with systemic symptoms such as motor weakness due to myasthenia gravis (MG), pure red cell aplasia, and hypogammaglobulinemia. Based on recent guidelines, management of advanced thymoma uses a multimodal approach, which is thymectomy followed by radiotherapy, but not all health care centers have radiotherapy facilities. A 52-year-old woman presented with nasal voice and had difficulty swallowing food. Patient was diagnosed with myasthenia gravis (MG). CT scan with contrast of the thorax showed a heterogenous solid mass in anterior mediastinum. Histopathological examination showed thymoma type B2. Thymectomy followed by seven cycles of platinum-based chemotherapy were done on the patient. Evaluation afterward showed complete remission of thymoma. The patient’s motor weakness improved after the chemotherapy. Post-chemotherapy period was uneventful at six months on follow-up visit. The dosage of acetylcholinesterase inhibitor drug is reduced periodically due to improvement in motor weakness. The case emphasizes how to manage an advanced thymoma with MG with limited therapeutic options, and the importance of multidisciplinary management involving oncologists, surgeons, and neurologists.
Thymoma ; Myasthenia Gravis ; Drug Therapy ; Thymectomy

INTRODUCTION

Pure red cell aplasia (PRCA) is defined as anemia in the presence of severe reticulocytopenia and absent or markedly decreased erythroid precursors in the marrow. When associated with another disease entity, it is classified as secondary acquired PRCA. A rare entity, thymolipoma, which constitutes 2% to 9% of thymic tumors has been associated with PRCA in some studies. The prevalence of thymolipoma among patients with myasthenia gravis reaches 43.8%. This paper presents the rare presentation of myasthenia gravis associated with thymolipoma and PRCA.

We present the case of a 64-year-old female who was diagnosed with myasthenia gravis and has been on maintenance pyridostigmine (Mestinon) for 12 years. She presented with symptoms of anemia and became transfusion requiring. Routine chest CT showed a thymic mass which was confirmed to be a thymolipoma during biopsy. Bone marrow studies confirmed the absence of erythroids, hence the diagnosis of secondary acquired PRCA.

There have been case reports associating PRCA with thymolipomas. Thymolipomas, which constitute 2% to 9% of thymic tumors are found among patients with myasthenia gravis, but the majority remains asymptomatic despite increasing tumor size. PRCA with associated thymic mass shows improvement of symptoms with tumor removal and immunosuppression. Oral corticosteroids result in a response rate of 39% while cyclosporine results in 77% response. This can be tapered off once response has been achieved.

This case report emphasizes the importance of early bone marrow studies among patients with myasthenia gravis presenting with sudden onset anemia.

The incidence rate of Myasthenia Gravis coexisting with other autoimmune diseases is approximately 8.7 – 25%, but it is rarely associated with premature ovarian insufficiency (POI) with only less than 1% of women affected. This is a case of premature ovarian insufficiency in a 29 year old woman diagnosed with Myasthenia Gravis, who presented with lower extremity weakness and experienced two episodes of myasthenic crisis requiring thymectomy. Three years after, she noted oligomenorrhea that quickly progressed to amenorrhea. Extensive immunologic and genetic investigative studies showed no identifiable cause for the POI, except for its close temporal relationship with the occurrence of Myasthenia Gravis. The patient has been responsive to hormone replacement and immunomodulation therapy, and has not developed any further episodes of myasthenic crisis. A review of seven other reported cases describing a similar condition was also included in the discussion.
Myasthenia Gravis

OBJECTIVES: To investigate the clinical effect of different immunosuppressive treatment regimens in children with ocular myasthenia gravis (OMG). METHODS: A retrospective analysis was conducted on 130 children with OMG who were treated in the Department of Neurology, Jiangxi Children's Hospital, from February 2018 to February 2023. According to the treatment regimen, they were divided into four groups: glucocorticoid (GC) group (n=29), mycophenolate mofetil (MMF) group (GC+MMF; n=33), methotrexate (MTX) group (GC+MTX; n=30), and tacrolimus (FK506) group (GC+FK506; n=38). Treatment outcomes and adverse reactions were compared among the groups. RESULTS: After 3 months of treatment, the FK506 group had significantly lower scores of Myasthenia Gravis Quantitative Scale and Myasthenia Gravis-Specific Activities of Daily Living than the other three groups (P<0.05). After 3 months of treatment, the FK506 group had a significantly lower dose of prednisone than the GC group, and after 6 and 9 months of treatment, the MMF, MTX, and FK506 groups had a significantly lower dose of prednisone than the GC group (P<0.05). After 12 months of treatment, the MMF, MTX, and FK506 groups had a significantly lower incidence rate of GC-related adverse reactions than the GC group (P<0.05). CONCLUSIONS For children with OMG, the addition of various immunosuppressants can reduce the dosage of GC and adverse reactions. Among them, FK506 shows superior efficacy compared to other immunosuppressants in the early treatment of OMG.
Humans ; Child ; Prednisone/adverse effects* ; Tacrolimus/adverse effects* ; Retrospective Studies ; Activities of Daily Living ; Immunosuppressive Agents/adverse effects* ; Myasthenia Gravis/drug therapy* ; Glucocorticoids/therapeutic use* ; Mycophenolic Acid/adverse effects*

Autoantibodies ; Calcium Channels ; Humans ; Myasthenia Gravis ; Receptors, Cholinergic/metabolism*

OBJECTIVE: To compare the clinical efficacy between acupuncture combined with western medication and simple western medication for ocular myasthenia gravis (OMG), and to explore its possible mechanism. METHODS: A total of 60 patients of ocular myasthenia gravis were randomized into an acupuncture combined with western medication group (30 cases, 1 case dropped off) and a western medication group (30 cases, 2 cases dropped off). Oral pyridostigmine bromide tablet and prednisone acetate tablet were given in the western medication group. On the basis of the treatment in the western medication group, Tongdu Tiaoqi acupuncture (acupuncture for unblocking the governor vessel and regulating qi ) was applied at Baihui (GV 20), Fengfu (GV 16), Hegu (LI 4), Zusanli (ST 36), etc. in the acupuncture combined with western medication group, once a day, 6 days a week. The treatment was given 8 weeks in both groups. Before and after treatment, the OMG clinical absolute score was observed, electrophysiological indexes of orbicularis oculi (value of mean jitter, percentage of jitter >55 μs and percentage of blocks) were measured by single-fiber electromyography (SFEMG), serum levels of acetylcholine receptor antibody (AChR-Ab), interferon-gamma (IFN-γ) and interleukin-4 (IL-4) were detected by ELISA method. RESULTS: After treatment, the OMG clinical absolute scores, values of mean jitter, percentages of jitter >55 μs, percentages of blocks and serum levels of AChR-Ab, IFN-γ and IL-4 were decreased compared before treatment in both groups (P<0.05), and those in the acupuncture combined with western medication group were lower than the western medication group (P<0.05). CONCLUSION Acupuncture combined with western medication can effectively improve ptosis, palpebra superior fatigability, eye movement disorder and neuromuscular junction dysfunction in patients with ocular myasthenia gravis, the therapeutic effect is superior to simple western medication. Its mechanism may be related to down-regulating serum levels of AChR-Ab, IFN-γ and IL-4 and promoting the recovery of orbicularis oculi function.
Acupuncture Therapy ; Facial Muscles ; Humans ; Interferon-gamma ; Interleukin-4 ; Myasthenia Gravis/drug therapy*

Through collecting the relevant provisions and medical cases of
Acupuncture ; Acupuncture Points ; Acupuncture Therapy ; Humans ; Meridians ; Moxibustion ; Myasthenia Gravis/therapy*

Immune checkpoint inhibitors (ICIs) have been changing the paradigm of cancer treatment. However, immune-related adverse effects (irAEs) have also increased with the exponential increase in the use of ICIs. ICIs can break up the immunologic homeostasis and reduce T-cell tolerance. Therefore, inhibition of immune checkpoint can lead to the activation of autoreactive T-cells, resulting in various irAEs similar to autoimmune diseases. Gastrointestinal toxicity, endocrine toxicity, and dermatologic toxicity are common side effects. Neurotoxicity, cardiotoxicity, and pulmonary toxicity are relatively rare but can be fatal. ICI-related gastrointestinal toxicity, dermatologic toxicity, and hypophysitis are more common with anti- CTLA-4 agents. ICI-related pulmonary toxicity, thyroid dysfunction, and myasthenia gravis are more common with PD-1/PD-L1 inhibitors. Treatment with systemic steroids is the principal strategy against irAEs. The use of immune-modulatory agents should be considered in case of no response to the steroid therapy. Treatment under the supervision of multidisciplinary specialists is also essential, because the symptoms and treatments of irAEs could involve many organs. Thus, this review focuses on the mechanism, clinical presentation, incidence, and treatment of various irAEs.
Autoimmune Diseases ; Cardiotoxicity ; Homeostasis ; Incidence ; Myasthenia Gravis ; Organization and Administration ; Specialization ; Steroids ; T-Lymphocytes ; Thyroid Gland

Myasthenia gravis(MG)is a B cell-mediated,T cell-dependent,complements-involved autoimmune disease.Ocular myasthenia gravis(OMG)is a typical MG,with its symptoms limited to the extraocular muscles.The occurrence and development of a variety of autoimmune diseases including OMG are closely associated with the imbalanced expression of follicular regulatory T cells(Tfr cells).Therefore,Tfr cells may be a new research topic for OMG.
Complement System Proteins ; Humans ; Myasthenia Gravis ; Oculomotor Muscles ; T-Lymphocytes, Regulatory