Prognosis of ocular myasthenia in Korea: a retrospective multicenter analysis of 202 patients.
OBJECTIVE
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<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<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, 1.60-17.8) 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.
Hong
Yoon-Ho
YH
Seoul National University Boramae Hospital, South Korea.
Kwon
Seok-Beom
SB
Kim
Byung-Jo
BJ
Kim
Byoung Joon
BJ
Kim
Seung Hyun
SH
Kim
Jong Kuk
JK
Park
Kyung-Seok
KS
Park
Ki-Jong
KJ
Sung
Jung-Joon
JJ
Sohn
Eun Hee
EH
Lee
Yeong-Bae
YB
Jeong
Dushin
D
Joo
In Soo
IS
Choi
Byung-Ok
BO
Choi
Young-Chul
YC
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000375:Aging / Q000473:pathology*; D000704:Analysis of Variance; D005260:Female; D006801:Humans; D007723:Korea / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000175:diagnosis* / Q000453:epidemiology*; D011379:Prognosis; D011950:Receptors, Cholinergic / Q000276:immunology / Q000502:physiology; D012189:Retrospective Studies; D018709:Statistics, Nonparametric
D011950:Receptors, Cholinergic