Ocular myasthenia gravis

Fortin, Élizabeth; Cestari, Dean M.; Weinberg, David H.

doi:10.1097/icu.0000000000000526

Cited by 45 publications

(33 citation statements)

References 63 publications

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“…While ptosis and diplopia are common presenting symptoms including in patients who will eventually evolve into generalized myasthenia, only 20% of patients will turn out to have pure ocular MG-signifying that these patients will never develop generalized disease) (56,57). The diagnostic difficulty with this entity is that only 50% have detectable antibodies to the AChR (57). Single fiber EMG studies support the diagnosis of neuromuscular transmission failure in patients without detectable antibodies, including ocular myasthenia (58).…”

Section: Ocular Mgmentioning

confidence: 99%

“…The ceiling steroid dose in ocular myasthenia is deemed to be lower than that used in generalized myasthenia (16,57). One usually aims for a maximal steroid dose (prednisolone/prednisone) of around 25 mg daily (or equivalent of 50 mg alternate days) but in some instances higher doses may need to be considered particularly if there is a delay in the correction of the ocular motility disturbance and if diplopia remains a persistent symptom.…”

Section: First-line Pharmacological Therapy In Ocular Mgmentioning

confidence: 99%

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A Practical Approach to Managing Patients With Myasthenia Gravis—Opinions and a Review of the Literature

Farrugia

Goodfellow

2020

Front. Neurol.

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When the diagnosis of myasthenia gravis (MG) has been secured, the aim of management should be prompt symptom control and the induction of remission or minimal manifestations. Symptom control, with acetylcholinesterase inhibitors such as pyridostigmine, is commonly employed. This may be sufficient in mild disease. There is no single universally accepted treatment regimen. Corticosteroids are the mainstay of immunosuppressive treatment in patients with more than mild MG to induce remission. Immunosuppressive therapies, such as azathioprine are prescribed in addition to but sometimes instead of corticosteroids when background comorbidities preclude or restrict the use of steroids. Rituximab has a role in refractory MG, while plasmapheresis and immunoglobulin therapy are commonly prescribed to treat MG crisis and in some cases of refractory MG. Data from the MGTX trial showed clear evidence that thymectomy is beneficial in patients with acetylcholine receptor (AChR) antibody positive generalized MG, up to the age of 65 years. Minimally invasive thymectomy surgery including robotic-assisted thymectomy surgery has further revolutionized thymectomy and the management of MG. Ocular MG is not life-threatening but can be significantly disabling when diplopia is persistent. There is evidence to support early treatment with corticosteroids when ocular motility is abnormal and fails to respond to symptomatic treatment. Treatment needs to be individualized in the older age-group depending on specific comorbidities. In the younger age-groups, particularly in women, consideration must be given to the potential teratogenicity of certain therapies. Novel therapies are being developed and trialed, including ones that inhibit complement-induced immunological pathways or interfere with antibody-recycling pathways. Fatigue is common in MG and should be duly identified from fatigable weakness and managed with a combination of physical therapy with or without psychological support. MG patients may also develop dysfunctional breathing and the necessary respiratory physiotherapy techniques need to be implemented to alleviate the patient's symptoms of dyspnoea. In this review, we discuss various facets of myasthenia management in adults with ocular Farrugia and Goodfellow Practical Approaches to Myasthenia Management and generalized disease, including some practical approaches and our personal opinions based on our experience.

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Section: Ocular Mgmentioning

confidence: 99%

Section: First-line Pharmacological Therapy In Ocular Mgmentioning

confidence: 99%

A Practical Approach to Managing Patients With Myasthenia Gravis—Opinions and a Review of the Literature

Farrugia

Goodfellow

2020

Front. Neurol.

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“…The CNE jitter is abnormal in 98-100% in generalized MG and above 90% in ocular MG. These results have been replicated numerous times [7,14,34,52,53,68]. The most sensitive are the facial muscles around the eye, especially in ocular MG or ocular onset MG. Like in RNS, one should focus on a clinically involved muscle (the masseter or the tongue in bulbar onset, the cervical paraspinals in dropping head/axial onset, deltoid in generalized fatigue).…”

Section: Neuromuscular Jitter Studymentioning

confidence: 65%

“…A degree of diagnostic suspicion is appropriate regarding the rare cases of antibody-positive subjects with other disorders [11] or human and technical error [12]. The observation that some MG patients have increased titer years before the clinical onset (as retrospectively found on preserved sera) may explain some of the apparent false-positive results [13].The RIPA for binding Ab is abnormal in up to 85% of adult patients with generalized MG, but only about 50% of patients with ocular MG [2,5,6,14]. Detection of modulating and blocking AChR-Ab through RIPA is commercially available but adds little diagnostic value [5], although others recently emphasized their significance [15].…”

Section: Anti-acetylcholine Receptor Antibodiesmentioning

confidence: 99%

Diagnosis of Myasthenia Gravis

Rousseff

2021

JCM

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The diagnosis of autoimmune Myasthenia Gravis (MG) remains clinical and rests on the history and physical findings of fatigable, fluctuating muscle weakness in a specific distribution. Ancillary bedside tests and laboratory methods help confirm the synaptic disorder, define its type and severity, classify MG according to the causative antibodies, and assess the effect of treatment objectively. We present an update on the tests used in the diagnosis and follow-up of MG and the suggested approach for their application.

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“…In different studies, positivity rates ranged from 16.7 to 44% (19,(60)(61)(62), consistently associated with high specificity (63). SF-EMG, when performed in the orbicularis oculi muscle, was found to be 79-100% sensitive for the detection of OMG (19,(63)(64)(65)(66), but it is time consuming and not largely available. An altered SF-EMG is commonly found in other disorders of NMT like Lambert-Eaton myasthenic syndrome (LEMS) and botulism, and both low-rate RNS and SF-EMG are frequently positive in congenital myasthenic syndromes (CMS).…”

Section: Electrophysiological Studiesmentioning

confidence: 99%

Controversies in Ocular Myasthenia Gravis

Evoli

Iorio

2020

Front. Neurol.

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Myasthenia gravis (MG) with symptoms limited to eye muscles [ocular MG (OMG)] is a rare disease. OMG incidence varies according to ethnicity and age of onset. In recent years, both an increase in incidence rate, particularly in the elderly, and a lower risk for secondary generalization may have contributed to the growing disease prevalence in Western countries. OMG should be considered in patients with painless ptosis and extrinsic ophthalmoparesis. Though asymmetric muscle involvement and symptom fluctuations are typical, in some cases, OMG can mimic isolated cranial nerve paresis, internuclear ophthalmoplegia, and conjugate gaze palsy. Diagnostic confirmation can be challenging in patients negative for anti-acetylcholine receptor and anti-muscle-specific tyrosine kinase antibodies on standard radioimmunoassay. Early treatment is aimed at relieving symptoms and at preventing disease progression to generalized MG. Despite the absence of high-level evidence, there is general agreement on the efficacy of steroids at low to moderate dosage; immunosuppressants are considered when steroid high maintenance doses are required. The role of thymectomy in non-thymoma patients is controversial. Prolonged exposure to immunosuppressive therapy has a negative impact on the health-related quality of life in a proportion of these patients. OMG is currently excluded from most of the treatments recently developed in generalized MG.

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Ocular myasthenia gravis

Cited by 45 publications

References 63 publications

A Practical Approach to Managing Patients With Myasthenia Gravis—Opinions and a Review of the Literature

A Practical Approach to Managing Patients With Myasthenia Gravis—Opinions and a Review of the Literature

Diagnosis of Myasthenia Gravis

Controversies in Ocular Myasthenia Gravis

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