Prognosis of Ocular Myasthenia Gravis: Retrospective Multicenter Analysis

Abstract

Purpose: To calculate the rate and timing of conversion from ocular myasthenia gravis to generalized myasthenia gravis.

Design: Retrospective multicenter analysis.

Subjects: Patients included in the study were diagnosed with ocular myasthenia gravis without the presence of generalized disease at onset.

Methods: We conducted a retrospective multicenter analysis. We reviewed charts of 158 patients who met diagnostic criteria for ocular myasthenia gravis. Patients were divided into 2 subgroups: an immunosuppressant treatment group and a nonimmunosuppressant treatment group. Timing of conversion to generalized disease and duration of follow-up also was evaluated. Additional data such as clinical symptoms at presentation, laboratory test results, and chest imaging results also were recorded.

Main outcome measures: Conversion rates to generalized myasthenia at 2 years, effect of immunosuppression on conversion, and timing of conversion.

Results: The 158-patient cohort included 76 patients who received immunosuppressant therapy; the remaining 82 patients did not. The overall conversion rate to generalized disease was 20.9%. At 2 years, generalized myasthenia developed in 8 of 76 patients in the treated group and in 15 of 82 patients in the nonimmunotherapy group (odds ratio, 0.52; 95% confidence interval, 0.20-1.32). Median time for conversion to generalized disease was 20 months in the nonimmunosuppressant group and 24 months in the immunosuppressant group. Conversion occurred after 2 years of symptom onset in 30% of patients.

Conclusions: Conversion rates from ocular to generalized myasthenia gravis may be lower than previously reported both in immunosuppressed and nonimmunosuppressed patients. A subset of patients may continue to convert to generalized disease beyond 2 years from onset of symptoms, and close monitoring should be continued.