Prognosis of ocular myasthenia in Korea: A retrospective multicenter analysis of 202 patients

Abstract

Objectives: The aims of this study were to obtain data on the frequency with which Korean patients with autoimmune myasthenia gravis (MG) present solely with ocular disturbances and progress to develop generalized disease and to identify the prognostic factors associated with secondary generalization. Methods: We conducted a retrospective multicenter survey in which a total of 376 adult patients who were newly diagnosed with MG from 2000 through 2005 were reviewed for analysis. Patients with ocular MG at the time of symptom presentation (n = 202, 53.7%) were divided into two subgroups according to their prognosis: the patients whose disease remained ocular throughout the follow-ups were placed in the OMG-R group, and the patients who progressed to develop generalized disease were placed in the OMG-G group. Clinical characteristics and laboratory findings were compared between the two subgroups. Results: Secondary generalization developed in 47 (23.3%) of the 202 study subjects, mostly within the first 6 months after symptom presentation, while the disease remained ocular throughout the follow-up duration (median 11.8 months) in the remaining 155 patients (76.7%). AChR antibody, abnormal repetitive nerve stimulation tests (RNST) and thymoma were more frequently observed in the patients in the OMG-G group than in those in the OMG-R group (p b 0.01 in all). In seropositive cases, the titers of AChR antibody were also significantly higher in the OMG-G group than in the OMG-R group (median, 3.8 nM vs. 6.4 nM; p b 0.05). Cox proportional hazards regression analyses showed that early oral prednisolone treatment significantly reduced the risk of secondary generalization (HR, 0.24; 95% CI, 0.11-0.56), whereas abnormal AChR antibody (HR, 5.34; 95% CI,) and thymoma (HR, 2.32; 95% CI, 1.21-4.45) were predictive of the development of secondary generalization. Conclusions: Our findings suggest that several factors, including the AChR antibody, thymoma, early corticosteroid treatment, and possibly latent neuromuscular abnormality revealed by RNST, may have an impact on the risk of developing generalized disease in Korean patients presenting with ocular myasthenia.

Prognosis of Ocular Myasthenia Gravis

“…Sommer et al 6 Previous studies have shown overall conversion rates of OMG to GMG of 50% to 64%. 2,4,12,13 Our series of OMG patients showed a much lower overall conversion rate of 20.9%, consistent with more recent retrospective studies reporting overall conversion rates ranging from 23% to 31%, 6,8,14,15 and this low conversion rate was observed in subjects with or without immunosuppressant therapy. Prior studies have emphasized the possible effect of immunotherapy on conversion rates.…”

Prognosis of Ocular Myasthenia Gravis in an Argentinian Population

“…This proportion was significantly higher than that shown in other series [9, 10, 12-14]. This apparent worse prognosis observed in our patients could be attributed, in part, to the long follow-up in the present study (mean 8.58 years), making it unlikely that what patients interpreted as ocular MG, later evolved to gMG.…”

Different molecular expression in thymoma with ocular or generalized myasthenia gravis

“…Myasthenia gravis (MG) is a prototypical antibody-mediated autoimmune disease characterized by production of autoantibodies against the skeletal muscle acetylcholine receptor (AChR) at the neuromuscular junction (NMJ) [1]. Ocular MG (OMG) is the most common initial presentation of MG, occurring in about 85% of MG patients.…”