Ocular Myasthenia Gravis: Diagnosis, Management, and Risk of Disease Progression

Abstract

Myasthenia gravis (MG) is an autoimmune disorder that causes muscle weakness and is categorized by four factors: the antibodies involved, the location of muscle weakness, the age of onset, and therapeutic status. Ocular myasthenia gravis (OMG) is a subtype that specifically affects the muscles of the eye and can cause droopy eyelids or double vision. 

Diagnosing OMG remains challenging due to its overlap with other neurological and ophthalmologic conditions. Common diagnostic approaches include bedside assessments such as the ice-pack test, the single-fiber EMG test (SFEMG), and the acetylcholine receptor (AChR) test. Among these, the ice-pack test offers a rapid and sensitive screening method, while SFEMG provides high diagnostic accuracy as a confirmatory test.

Management strategies focus on both symptomatic relief and prevention of disease progression. Pyridostigmine is typically used as first-line therapy, while corticosteroids such as prednisone are introduced when symptoms persist or worsen. Evidence suggests that certain factors, including adult-onset disease, abnormal repetitive nerve stimulation (RNS), AChR antibody positivity, and thymoma, are associated with an increased risk of progression from ocular to generalized MG.

Understanding these diagnostic tools and risk factors is essential for guiding clinical decision-making, optimizing treatment strategies, and reducing disease risk. This review synthesizes current evidence to provide a comprehensive overview of the diagnosis, management, and risk of progression in ocular myasthenia gravis.