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

JOURNAL OF THE NEUROLOGICAL SCIENCES

卷:273

Article

Hong, Yoon-Ho¹  Kwon, Seok-Beom²  Kim, Byung-Jo³  Kim, Byoung Joon⁴  Kim, Seung Hyun⁵  Kim, Jong Kuk⁶  Park, Kyung-Seok⁷  Park, Ki-Jong⁸  Sung, Jung-Joon⁹  Sohn, Eun Hee¹⁰  Lee, Yeong-Bae¹¹  Jeong, Dushin¹²  Joo, In Soo¹³  Choi, Byung-Ok¹⁴  Choi, Young-Chul¹⁵ 

[1] Seoul Natl Univ, Boramae Hosp, Seoul 151, South Korea

[2] Hollym Univ Hosp, Gyong San, South Korea

[3] Korea Univ, Med Ctr, Seoul, South Korea

[4] Sungkyunkwan Univ, Samsung Med Ctr, Seoul, South Korea

[5] Hanyang Univ Hosp, Seoul, South Korea

[6] Kosin Univ, Gospel Hosp, Pusan, South Korea

[7] Seoul Natl Univ, Bundang Hosp, Seoul 151, South Korea

[8] Gyeongsang Natl Univ Hosp, Jinju, South Korea

[9] Seoul Natl Univ Hosp, Seoul, South Korea

[10] Chungnam Natl Univ Hosp, Taejon, South Korea

[11] Gachon Univ Med & Sci, Inchon, South Korea

[12] Soonchunhyang Univ, Cheonan Hosp, Chungcheongnam Do, South Korea

[13] Ajou Univ Hosp, Suwon, South Korea

[14] Ewha Womans Univ, Dongdaemun Hosp, Seoul, South Korea

[15] Yonsei Univ, Youngdong Severance Hosp, Seoul 120749, South Korea

关键词: ocular myasthenia gravis;    prognosis;    AChR antibody;    thymoma;    repetitive nerve stimulation test;   

DOI  :  10.1016/j.jns.2008.05.023

来源: Elsevier

【 摘 要 】

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 < 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. (c) 2008 Elsevier B.V. All rights reserved.

【 授权许可】

Free