Treatment Of Ocular Myasthenia Gravis Research Articles

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

Abstract
 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.
 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.
 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.
 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.

Comparative effectiveness and safety of intravenous methylprednisolone and tacrolimus monotherapy in ocular myasthenia gravis with unsatisfactory prednisone responses: a retrospective study

BackgroundOral prednisone has been recognized as the first-line therapy for the treatment of ocular myasthenia gravis (OMG). However, its long-term use is complicated by numerous adverse effects and is ineffective for some OMG patients in reaching remission. This study aimed to evaluate the effectiveness and safety of intravenous methylprednisolone (IVMP) and tacrolimus monotherapy for OMG patients with unsatisfactory responses to conventional prednisone therapy.MethodsWe retrospectively reviewed 57 OMG patients who had not achieved satisfactory improvement after prednisone therapy and thereby received IVMP or tacrolimus monotherapy for at least 6 months. Ocular symptoms were evaluated by the ocular-quantitative MG (QMG) score at each time point. A ≥ 2-point fall in ocular QMG score was defined as the cut-off point to indicate clinical improvement. Logistic regression analysis was performed to identify factors associated with the efficacy of IVMP at discharge. Adverse events were recorded.ResultsBoth IVMP and tacrolimus monotherapy demonstrated significant clinical efficacy, with no statistical differences observed at the study endpoint. The proportions of patients who reached the cut-off point for efficacy evaluation were higher in the IVMP group than in the tacrolimus group (1, 3, and 6 months: 51.7% (15/29) vs 12.0% (3/25), p = 0.002; 69.0% (20/29) vs 40.0% (10/25), p = 0.033; 69.0% (20/29) vs 46.4% (13/28), p = 0.085, respectively). Multivariate logistics analysis showed that high ocular QMG scores at baseline indicated favourable responses to IVMP treatment (OR = 1.781; 95% CI 1.066–2.975; p = 0.028). All the adverse events were transient and tolerable.ConclusionOur findings suggest that both IVMP and tacrolimus monotherapy hold promise as viable treatment options for OMG patients with unsatisfactory responses to oral prednisone. The study supports the safety and effectiveness of both therapies, with IVMP exhibiting faster improvement and favourable efficacy in patients with high ocular QMG scores.

Ocular Myasthenia Gravis: A Current Overview.

Ocular myasthenia gravis (OMG) is a neuromuscular disease characterized by autoantibody production against post-synaptic proteins in the neuromuscular junction. The pathophysiological auto-immune mechanisms of myasthenia are diverse, and this is governed primarily by the type of autoantibody production. The diagnosis of OMG relies mainly on clinical assessment, the use of serological antibody assays for acetylcholine receptors (AchR), muscle-specific tyrosine kinase (MusK), and low-density lipoprotein 4 (LPR4). Other autoantibodies against post-synaptic proteins, such as cortactin and agrin, have been detected; however, their diagnostic value and pathogenic effect are not yet clearly defined. Clinical tests such as the ice test and electrophysiologic tests, particularly single-fiber electromyography, have a valuable role in diagnosis. The treatment of OMG is primarily through cholinesterase inhibitors (pyridostigmine), and steroids are frequently required in cases of ophthalmoplegia. Other immunosuppressive therapies include antimetabolites (azathioprine, mycophenolate mofetil, methotrexate) and biological agents such as B-cell depleting agents (Rituximab) and complement inhibitors (eculizumab). Evidence is scarce on the effect of immunosuppressive therapy on altering the natural course of OMG. Clinicians must be vigilant of a myasthenic syndrome in patients using immune-check inhibitors. Reliable and consistent biomarkers are required to assess disease severity and response to therapy to optimize the management of OMG. The purpose of this review is to summarize the current trends and the latest developments in diagnosing and treating OMG.

Immunotherapy for ocular myasthenia gravis: an observational study in Japan.

Treatment for ocular myasthenia gravis (OMG) has not yet been well established. Few reports have been published on the clinical practice and outcomes of OMG. We investigated treatment of OMG and its outcomes in Japan.We investigated treatment of OMG and its outcomes in Japan. We performed a retrospective cross-sectional survey of OMG patients from eight hospitals in Japan. Clinical information, including sex, age at onset, initial symptoms, autoantibodies, clinical course, treatment history, complications, and outcomes, was obtained. In addition, we recorded the total number of patients with MG and OMG separately. In total, 135 patients with OMG (67 men, 68 women) were included. Treatment of OMG was not simple and involved various immunotherapeutic strategies. Eight patients went into remission spontaneously without immunotherapy. A total of 117 patients showed improvements after treatment, whereas 10 patients showed refractory responses to treatment. Overall outcomes were good; however, symptoms persisted in 60.7% of patients even after treatment. Among 90 patients who received immunotherapy, only two showed a refractory response. Meanwhile, for 45 patients who did not receive immunotherapy, 8 were refractory. Thus, the rate of refractory disease in the group with immunotherapy was significantly lower (p = 0.001, u-test) than in the group without immunotherapy. The proportion of generalized MG patients among all MG cases was low in medical centers where immunotherapy for OMG was frequently performed. Although the overall prognosis for patients with OMG was good, symptoms remained in more than half of the patients. Immunotherapy, including corticosteroids, may be beneficial for patients with OMG. Is immunosuppressive therapy beneficial for myasthenia gravis patients with ocular symptoms only? Patients with ocular myasthenia gravis (OMG) have only eye symptoms for more than 2 years. Whether this condition is an initial stage of the disease before eventually progressing to generalized myasthenia gravis (gMG) is still uncertain. Different from gMG, OMG is not life-threatening. But eye symptoms often cause troublesome problems in life. Doctors have treated OMG patients similarly to patients with gMG. There is no standard clinical practice for OMG. In this study, we examined how patients with OMG were treated at eight different specialist centers in Japan. In 135 patients with OMG, 8 patients became symptom free without treatment, 117 patients showed improvements after treatment, whereas 10 patients did not get well. Overall outcomes were good, but symptoms remained in 60.7% of patients even after treatment. Among 90 patients who received one or more immunotherapies, only 2 did not get well. Meanwhile, for 45 patients who did not receive immunotherapy, 8 remained ill. We found that treatment of OMG was not simple and often needed multiple immunotherapies. Administering immunotherapy, including corticosteroids, may be beneficial for patients with OMG.

Ocular myasthenia gravis

Ocular myasthenia gravis (OMG) is a relatively common subtype of myasthenia gravis (MG) in which the weakness is limited to ocular and bulbar muscles, which may lead to significant visual disability. Patients usually present with fluctuating ptosis, diplopia, and weakness of lid closure. Those patients with purely ocular weakness are at risk of developing generalized MG, especially early in the disease. This autoimmune disorder occurs due to the production of autoantibodies against nicotinic acetylcholine receptors (AChRs) at the neuromuscular junction of skeletal muscles. Examination findings may range from isolated muscle palsy to complete ophthalmoplegia. Some specific findings that support the clinical diagnosis of OMG are Cogan’s lid twitch, peek sign, and saccadic fatigue. Tests to confirm the diagnosis are edrophonium challenge test, repetitive nerve stimulation, single-fiber electromyography of the frontalis, and assays for antibody directed against the AChR. There are a variety of treatment options for OMG such as pyridostigmine, immunosuppression, intravenous immunoglobulin, plasmapheresis, thymectomy, ptosis surgery, and extraocular muscle surgery. MG is known to be great masquerade due to its varied clinical presentations. It mimics isolated cranial nerve palsies, internuclear ophthalmoplegia, gaze palsies, and sometimes blepharospasm or even may be even stroke. The aim of the review is to feature the diagnosis, investigations, and treatment of OMG.

Local use of dexamethasone in the treatment of ocular myasthenia gravis

BackgroundAt present, patients with ocular myasthenia gravis (OMG) are typically treated with systemic drugs. We investigated the use of dexamethasone injected in the peribulbar region or extraocular muscle to treat patients with OMG.MethodsPatients with OMG were given dexamethasone via peribulbar injection or direct injection into the main paralyzed extraocular muscles, once a week, for 4–6 weeks. The severity of diplopia, blepharoptosis, eye position, and eye movement were evaluated before and after treatment. The duration of follow-up time was ≥6 months.ResultsAmong the 14 patients with OMG who received this treatment, mean age was 38.7 ± 29.7 years. After treatment, symptoms were relieved in 12 patients (85.7%), 1 patient (7.1%) had partial response to treatment, and 1 patient (7.1%) had no response. Two patients (14.2%) experienced symptom recurrence during the follow-up period.ConclusionsDexamethasone peribulbar or extraocular muscle injection is effective in the treatment of patients with OMG and may replace systemic drug therapy.Trial registrationChinese Clinical Trial Registry, ChiCTR2000038863, October 7, 2020.Retrospectively registered.

Effect of Initial Prednisone Dosing on Ocular Myasthenia Gravis Control.

Ocular myasthenia is an autoimmune condition that results in double vision or ptosis. It often requires treatment with prednisone for immunosuppression, but there have been no prospective trials to help clinicians determine ideal dosing. This was a retrospective study comparing myasthenia symptom control at 1 month between patients treated with a maximum daily equivalent dose of prednisone less than 20 mg (low-dose group) vs 20 mg or more (medium-dose group). Thirty-nine patients were identified: 19 patients in the low-dose group with mean maximum daily dose of 10 mg and 20 patients in the medium-dose group with a mean maximum daily dose of 29 mg. The low-dose group had 75% controlled or significantly improved at 1 month, and the medium-dose group had 74% controlled or significantly improved at 1 month, P = 0.94. The overall seropositivity rate was 64%, with 84% of the antibody-positive group being controlled or significantly improved at 1 month and 57% of the antibody-negative group being controlled or significantly improved at 1 month, P = 0.07, and no difference in prednisone dosing between the 2 groups. Based on the results of this small retrospective study, it seems initial treatment for ocular myasthenia gravis with a mean maximum daily prednisone dose of 10 mg is similarly effective compared with mean maximum daily dose of 29 mg for control at 1 month.

Acupuncture for ocular myasthenia gravis: A protocol for systematic review and meta-analysis.

Background:The aim of this systematic review with meta-analysis is to determine the efficacy and security of acupuncture in treatment of ocular myasthenia gravis and find out whether or not the quick short-term efficacy of acupuncture exists.Methods:The following electronic databases will be searched by 2 independent reviewers: PubMed, Cochrane Library, EMBASE, Springer, China National Knowledge Infrastructure, Wanfang, and Chinese Biomedical Literature Database. All randomized controlled trials on acupuncture for ocular myasthenia gravis published in electronic databases from inception to March 1, 2020, with language restricted in Chinese and English will be included in the study.Methodologic quality is assessed by 2 blinded reviewers independently screen and score the articles using the PEDro scale and the Cochrane Collaboration risk of bias tool. A meta-analysis was performed when there is sufficient clinical homogeneity in at least 2 studies. The Grading of Recommendations Assessment, Development and Evaluation approach is used to rate the body of evidence in each meta-analysis. When the quantitive evaluation is not available, a qualitative description of the results of single study is provided.Results:An evidence of variety of acupuncture treatment methods for treating ocular myasthenia gravis will be illustrated using subjective reports and objective measures of performance. The primary outcomes consisted of effective rate, MGFA PIS, QMG, and MG-composite. Secondary outcomes involve clinical absolute and relative score, titers of AchR antibodies, and the side effects. The treatment frequency and courses will be measured.Conclusion:This protocol will present the evidence of whether acupuncture is an effective and safe intervention for ocular myasthenia gravis.Trial registration number:CRD42019141325

Research Advances in Ocular Myasthenia Gravis

Ocular myasthenia gravis(OMG)is an autoimmune disease caused by neuromuscular junction transmission disorders and manifested mainly as fluctuating blepharoptosis and diplopia,with the extraocular muscles as the main involveed sites.While the pathogenesis of OMG remains unclear,some antibodies,complements,and cytokines may be the contributing factors.The diagnosis and treatment of OMG have been defined in recent years.This article reviews the pathogenesis,diagnosis,and treatment of OMG.

Treatment of Ocular Myasthenia Gravis.

Myasthenia gravis is a relatively common neuromuscular disorder, with ocular myasthenia gravis being a subset defined as myasthenia gravis limited to the orbicularis, levator, and extraocular muscles. Patients with ocular myasthenia gravis can have disabling diplopia or functional blindness from ptosis and in most cases treatment is required. Like generalized myasthenia gravis, there are a variety of treatments available that include pyridostigmine, immunosuppression, intravenous immunoglobulin, plasmapheresis, thymectomy, lid crutches, ptosis surgery, and extraocular muscle surgery. Unfortunately, there is limited data on the use of individual treatments in ocular myasthenia gravis and no data comparing treatments. Using a combination of available data on treatment of generalized myasthenia gravis, data on treatment of ocular myasthenia gravis, best practices, and clinical experience we will provide a rational framework for treatment of ocular myasthenia gravis.

Pediatric ocular myasthenia gravis: Case report and literature review

Objective: The objective was to describe a case of ocular myasthenia gravis (MG) in a pediatric patient. Design: This is a descriptive, retrospective study and case report. Methodology: For the purpose of the case report, we searched and analyzed the literature referring to the diagnosis and treatment of ocular MG (OMG) in children. Description: A previously healthy 5-year-old girl presented to the emergency room because of sudden-onset ptosis of the right upper lid without other symptoms. Her examination demonstrated fluctuating ptosis and limitation to downgaze in the right eye. Because of the fluctuation of the ptosis, OMG was suggested, and the patient was tested with ice-pack test and neostigmine test with positive results. Treatment with pyridostigmine was initiated with a good response. Discussion: OMG has a very low incidence in pediatric patients. It is a disorder characterized by impairing neurotransmission at the neuromuscular junction that generates extraocular muscle weakness. It is important that ophthalmologists have knowledge about this disease because it is a differential diagnosis when there is a patient who has ptosis, strabismus, or limitation of movements. There are no standard diagnostic criteria for this disease; the diagnosis is based on clinical presentation and pharmacologic, serologic, and electrophysiologic tests. The treatment is based on steroids and anticholinesterase drugs. Some patients will require plasmapheresis or immunomodulators.

Efficacy and Safety of Low-to-Moderate Dose Oral Corticosteroid Treatment in Ocular Myasthenia Gravis.

To evaluate the response to corticosteroid therapy as a primary treatment for ocular myasthenia gravis. Patients diagnosed as having ocular myasthenia gravis by an acetylcholine receptor binding antibody test between January 2011 and September 2015 were included in the study and started receiving treatment with a corticosteroid. Patients with a blowout fracture, hyperthyroidism, diabetes mellitus, hypertension, cardiovascular disease, or history of strabismus surgery were excluded. Disappearance of diplopia and ptosis were considered a response to treatment. Methylprednisolone therapy was administered to 29 patients (19 men and 10 women; average age: 49 ± 16.5 years) as an initial treatment. A total of 6 patients were lost to follow-up. Twenty-three of 29 patients (82.6%) were regarded as having presented a response to treatment. The average treatment duration was 3 weeks for patients responding to primary treatment. Eight patients complained of adverse effects from steroid therapy such as heartburn, insomnia, weight gain, and myalgia. A corticosteroid could be considered as an initial treatment for patients diagnosed as having ocular myasthenia gravis by an acetylcholine receptor binding antibody test. [J Pediatr Ophthalmol Strabismus. 2018;55(5):339-342.].

Effect of Jianpiyiqi granule on ocular myasthenia gravis in children prepared with a formula from Traditional Chinese Medicine

ObjectiveTo investigate the effect of Jianpiyiqi granule, prepared according to a Traditional Chinese Medicine (TCM) formula, on the ocular type of myasthenia gravis (MG) caused by a defect in synaptic transmission at the neuromuscular junction. MethodsA total of 155 children with ocular MG were recruited from January 2008 to January 2015. All individuals received ineffective glucocorticoid treatment prior to admission, and were given Jianpiyiqi granules (two doses per day) for 12 months. Plasma levels of acetylcholine receptor autoantibodies (AChR-Ab), cytokines, immune parameters and clinical score were analyzed. ResultsAfter intervention with Jianpiyiqi granule for 12 months, the levels of cytokines [including interleukin (IL)-6, IL-12, IL-17], immune parameters [including immunoglobulin G (IgG), IgM, free triiodothyronine, free thyroxine] and AChR-Ab were significantly decreased (P = 0.042, P = 0.049, P = 0.011, P = 0.02, P = 0.04, P = 0.03, P = 0.01; F = 21.60, P = 0.000). In contrast, IL-4 levels were significantly increased (P = 0.049). The absolute clinical score after treatment declined significantly compared with before treatment (F = 33.24, P = 0.000). The effective cure rate and the total effective rate gradually increased to a maximum of 149 (96.2%) and 151 (97.4%) in MG patients after 12 months of treatment, respectively. ConclusionThe Jianpiyiqi granule treatment lowered AChR-Ab levels and improved cytokine and other immune parameter levels, which suggests that the granule could be an ancillary treatment for ocular MG in children caused by a defect in synaptic transmission at the neuromuscular junction.

Ocular myasthenia gravis: a review.

Myasthenia gravis (MG) is a disease that affects the neuro-muscular junction resulting in classical symptoms of variable muscle weakness and fatigability. It is called the great masquerader owing to its varied clinical presentations. Very often, a patient of MG may present to the ophthalmologist given that a large proportion of patients with systemic myasthenia have ocular involvement either at presentation or during the later course of the disease. The treatment of ocular MG involves both the neurologist and ophthalmologist. Thus, the aim of this review was to highlight the current diagnosis, investigations, and treatment of ocular MG.

Sixty cases of ocular myasthenia gravis treated with acupuncture and moxibustion at extra-meridian and collateral

ObjectiveTo explore effective therapeutic method for the treatment of ocular myasthenia gravis. MethodsSixty cases of ocular myasthenia gravis were selected. The acupuncture was applied at Dàzhuī ▪ GV 14), B▪ihuì (▪) GV 20), Shàngxīng (▪ GV 23) of Governor Vessel, and its crossing points with Yinqiao and Yangqiao Meridian, such as Jīngmíng (▪ BL 1), shēnmài (▪ BL 62), Dìcāng (▪ ST 4), Jūliáo (▪ ST 3), Chéngqì (▪ ST 1), etc. Besides, ginger-partition moxibustion was applied at Yángbái (▪ GB 14) and Sìbái (▪ ST 2). The plum-blossom needle was applied at upper limb distribution of lung channel of hand taiyin, heart channel of hand shaoyin and pericardium channel of hand jueyin. The patients’ clinical symptoms, potential degradation percentages of repetitive nerve stimulation (RNS) in low frequency were observed. Results8 cases were clinically cured, 20 cases were basically cured, 12 cases were remarkably effective, 15 cases were effective and 5 cases were failed. The total effective rate was 91.7%. Compared with before the treatment, the potential degradation percentages of repetitive nerve stimulation in low frequency were lowered [(19.44±6.28)% vs (8.78±3.12)%, (P<0.05)]. ConclusionAcupuncture and moxibustion at extra-meridian and collateral for ocular myasthenia gravis could achieve positive efficacy.

Invited Commentary

We read with interest the study by Liu and associates [1Liu Z. Feng H. Yeung S.-C.J. et al.Extended transsternal thymectomy for the treatment of ocular myasthenia gravis.Ann Thorac Surg. 2011; 92: 1993-1999Abstract Full Text Full Text PDF PubMed Scopus (47) Google Scholar]. Optimal treatment of ocular myasthenia gravis (MG) is still a controversial issue with no agreement among surgeons and neurologists. There is no doubt that patients with thymoma should undergo surgery, whatever the MG stage is; however, many doubts still persist regarding the indication for surgery for those with pure ocular MG. This dilemma is related to several factors. At this stage, some natural remissions (approximately 30% of cases) occur without surgery. In addition, most of the patients with ocular MG remain at this stage with symptoms easily controlled by medical treatment. Furthermore, the variability in diagnosis, type of medical treatment, length of time before referral for surgery, preparation for surgery, and type of surgical approach has made MG (in particular ocular MG) difficult to study. In the last two decades, an increasing number of reports have sustained the potential role of surgery, even at the early stage of MG. The results are even more crucial in the era of thoracoscopic and robotic thymectomy. Proponents of thymectomy generally agree that the duration of symptoms before surgery is a predictor for outcome, with better results in patients reporting symptoms for less than 1 year. However, this does not match with the guidelines proposed by neurologists: they suggest a period of medical treatment not shorter than 1 year in the absence of thymoma. The results of surgical treatment of ocular MG should be popularized among neurologists to encourage the debate and eventually increase referrals to thoracic surgery units. It could also help to alleviate neurologists' historical reluctance to withdraw medications after surgery in patients without symptom; this is usually reported as one of the factors contributing to lower cure rates. We must admit that phase II studies are interesting and encouraging, but they are not persuasive from the scientific point of view. We now need phase III studies in patients with ocular MG involving surgeons and neurologists; this will be the only way to identify the best treatment for ocular MG. Extended Transsternal Thymectomy for the Treatment of Ocular Myasthenia GravisThe Annals of Thoracic SurgeryVol. 92Issue 6PreviewThe optimal treatment for ocular myasthenia gravis (OMG) remains controversial. We conducted a review of the long-term clinical outcomes of Chinese patients with OMG after extended transsternal thymectomy (ETT) to determine the efficacy of this procedure as a treatment for OMG. Full-Text PDF

Extended Transsternal Thymectomy for the Treatment of Ocular Myasthenia Gravis

The optimal treatment for ocular myasthenia gravis (OMG) remains controversial. We conducted a review of the long-term clinical outcomes of Chinese patients with OMG after extended transsternal thymectomy (ETT) to determine the efficacy of this procedure as a treatment for OMG. We reviewed the cases of 115 consecutive patients with OMG who underwent ETT at our Myasthenia Gravis Research Center between January 2006 and December 2008. Extended transsternal thymectomy was done in patients who had thymoma, resistance to pyridostigmine therapy, or relapse after immunosuppressive therapy. The patients' postoperative responses were defined as strict complete remission (SCR), consisting of an asymptomatic status without medication for more than 12 months; general complete remission (GCR), consisting of an asymptomatic status with low-dose single-drug therapy or without medication for more than 12 months; or improvement, consisting of fewer symptoms or less of a need for medication than before surgery. The overall complication rate was 7.8%. None of the patients experienced a myasthenic crisis, progression to generalized myasthenia gravis, or mortality. Hyperplasia of the thymus was present in 106 of the 115 patients (92.2%). Among 110 patients on whom follow-up was done postoperatively, 29 (26.4%) were in SCR, 64 (58.2%) showed improvement, 7 (6.4%) remained unchanged, and 10 (9.1%) had a worsening of their conditions. Kaplan-Meier analysis revealed rates of GCR of 41.8% at 24 months and 47.3% at 48 months after surgery, and rates of SCR of 24.5% at 24 months and 26.4% at 48 months. Both univariate analysis and multivariate Cox regression analysis revealed that only preoperative duration of illness was positively associated with GCR (p < 0.001). The results of the review indicate that ETT is a safe and effective treatment for OMG, especially in patients with illness of shorter duration.

Treatment of Ocular Myasthenia Gravis

Myasthenia gravis (MG) is an autoimmune disorder that is characterized by variable weakness and fatigability. Often, MG presents with only ocular symptoms such as ptosis and diplopia. Treatment of ocular MG is aimed at relieving the symptoms of ptosis and diplopia, as well as preventing the development of generalized MG symptoms. Immune suppression with steroids is often the main therapy. Steroid doses must be increased slowly because of a risk of precipitating myasthenic crisis. After achieving the highest target dose, steroids are then slowly tapered down to the lowest effective dose. Often, acetylcholinesterase inhibitors such as pyridostigmine and neostigmine are also employed to help control symptoms. When steroids are contraindicated, acetylcholinesterase inhibitors can be tried as the primary therapy. Steroid-sparing agents such as azathioprine and mycophenolate may also have a role in treating ocular MG. Other treatments for MG include plasmapheresis, intravenous immunoglobulin, and other immunosuppressive agents, but these are rarely required for ocular MG. Patients should also be evaluated for thymoma. Thymoma should be resected surgically. Ocular MG without thymoma is not usually treated with thymectomy. Topical agents may be useful as additional therapy for mild or moderate ptosis. Nonpharmacologic treatments include occlusive devices, prisms, eyelid supports, contact lenses, and (in long-standing, stable cases) strabismus surgery or eyelid elevation surgery.

Medical treatment options for ocular myasthenia gravis

To update our current concepts of ocular myasthenia gravis medical management and to provide a short overview of upcoming treatments. Cholinesterase inhibitors and corticosteroids have been the first-line treatment for ocular myasthenia gravis. Several studies on other immunosuppressants, either as a steroid-sparer, steroid adjuvant or initial monotherapy, have demonstrated significant clinical efficacy. Preventing progression to generalized myasthenia gravis is still under debate and needs to be further studied. Novel techniques that target specific components of the autoimmune cascade are forthcoming. Currently, limited evidence favors the use of corticosteroids, azathioprine, and mycophenolate mofetil in ocular myasthenia gravis. There is a need for rigorous clinical trials on the efficacy and safety of these medical therapeutic options in improving ocular symptoms and decreasing the risk of developing generalized myasthenia gravis. Studies on emerging immunomodulators that dampen autoreactivity without affecting general immunity should be pursued.

Corticosteroids for myasthenia gravis.

Although widely accepted as an appropriate immunosuppressive therapy, the efficacy of glucocorticosteroid treatment has only rarely been tested in controlled studies. To assess the efficacy of glucocorticosteroids or adrenocorticotrophic hormone (ACTH) medication in autoimmune myasthenia gravis. We searched the Cochrane Neuromuscular Disease Group Trials Register in July 2004, MEDLINE (from January 1966 to June 2004) and EMBASE (from January 1980 to June 2004). We also checked the bibliographies in reviews and the randomised trials and contacted their authors to identify additional published and unpublished data. From the articles identified we selected those open or controlled studies which allowed us to assess the outcome of treated and untreated patients at definite endpoints. Types of studies: quasi-randomised or randomised controlled trials. patients with myasthenia gravis of all ages and all degrees of severity. Types of interventions: any form of glucocorticosteroids or adrenocorticotrophic hormone treatment. Types of outcome measures:Primary outcome(1) improvement after at least three months in either the weakest muscles or all muscles. Secondary outcomes(1) proportion of patients improved after at least six months(2) proportion of patients in remission(3) number of episodes of worsening during the first six months(4) acetylcholine receptor antibody titres after at least three months of therapy. Three authors extracted the data from the selected articles and one other checked them. A trial of adrenocorticotrophic hormone (43 patients) did not show any advantage compared with placebo for the treatment of ocular myasthenia gravis. Two double-blind trials compared prednisone with placebo for generalised myasthenia gravis. In the first (13 patients), the improvement was slightly greater in the prednisone group at six months. In the second (20 patients) which was a short-term trial, the improvement was significantly greater at two weeks. Two trials compared glucocorticosteroids with azathioprine (41 and 10 patients respectively). In one of these the rate of treatment failure was greater in the prednisone group. In a trial of glucocorticosteroids versus intravenous immunoglobulin (33 patients) no differences in treatment responses were encountered during a treatment period of 14 days. An open trial (39 patients) evaluating different corticosteroid doses revealed a shorter time to improvement in the high-dose group. However only limited evidence can be drawn from the available randomised controlled trials due to numerous and important methodological flaws. Limited evidence from randomised controlled trials suggests that corticosteroid treatment offers significant short-term benefit in myasthenia gravis compared with placebo. This supports the conclusions of observational studies and expert opinion. Limited evidence from randomised controlled trials does not show any difference in efficacy between corticosteroids and either azathioprine or intravenous immunoglobulin.