Abstract:
OBJECTIVE:To clarify the prevalence and clinical characteristics of myasthenia gravis (MG) in Japan. METHODS:We performed a nationwide epidemiological survey of MG in Japan. The clinical features were compared among five groups of patients, divided according to onset age. A generalized additive model (GAM) was used to assess the linearity of these relationships. RESULTS:A total of 8542 patients were reported, and detailed data were analyzed for 3141 patients. The estimated number of MG patients in Japan was 15,100, giving a prevalence of 11.8 per 100,000. Elderly-onset MG (≥ 65 years) accounted for 7.3% in 1987 (adjusted for population in 2005), but this had increased to 16.8% in 2006. Infantile-onset MG (0-4 years) accounted for 10.1% in 1987, and was still as high as 7.0% in 2006. The rate of ocular MG was highest (80.6%) in infantile-onset and lowest (26.4%) in early-onset disease, but the rate rose again in the late-onset group. GAM analysis of the ocular form showed a U-shaped curve, with a dip in the 20s. Anti-acetylcholine receptor antibodies were positive in only 50% of infantile-onset, but nearly 90% of elderly-onset patients. GAM analyses assessing the concurrence of thymoma and hyperplasia both showed reversed U-shapes, with peaks in the 50s and 20s-40s, respectively. CONCLUSIONS:Persistent high incidence of infantile-onset disease and clinical heterogeneity according to onset age are characteristic features of MG in Japan.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Murai H,Yamashita N,Watanabe M,Nomura Y,Motomura M,Yoshikawa H,Nakamura Y,Kawaguchi N,Onodera H,Araga S,Isobe N,Nagai M,Kira Jdoi
10.1016/j.jns.2011.03.004subject
Has Abstractpub_date
2011-06-15 00:00:00pages
97-102issue
1-2eissn
0022-510Xissn
1878-5883pii
S0022-510X(11)00113-4journal_volume
305pub_type
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