Treatment and outcome of myasthenia gravis: retrospective multi-center analysis of 470 Japanese patients, 1999–2000

Kawaguchi, Naoki; Kuwabara, Satoshi; Nemoto, Yasuo; Fukutake, Toshio; Satomura, Yoichi; Arimura, Kimiyoshi; Osame, Mitsuhiro; Hattori, Takamichi

doi:10.1016/j.jns.2003.09.016

Cited by 64 publications

(51 citation statements)

References 22 publications

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“…[5][6][7][8] In particular, paediatric cases are frequent in China, Taiwan and Japan and purely ocular MG is relatively common in children. Since there are also differences in human leucocyte antigen associations between Japanese, Chinese and Caucasian populations, [9][10][11][12] these observations may provide clues to the immunopathogenesis of MG.…”

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confidence: 99%

Clinical and serological study of myasthenia gravis in HuBei Province, China

Zhang

Yang²,

Xu³

et al. 2006

Journal of Neurology, Neurosurgery & Psychiatry

133

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Background: Ocular and childhood myasthenia gravis (MG) cases seem relatively more common in Oriental than in Caucasian populations, but there have been no comprehensive serological studies on patients from mainland China. Methods: 391 unselected patients with MG attending Tongji Hospital in WuHan (the largest hospital in the province of HuBei, China) were studied during a 15-month period; most had already received treatment for their condition. Results: The male to female ratio was 0.8. 50% of the patients were children (,15 years), and age at onset showed a single peak at between 5 and 10 years of age. 64% of the children and 66% of the adults were positive for acetylcholine receptor (AChR) antibodies but the antibody titres were lower than in similar Caucasian studies, although this was partly due to the high incidence of ocular MG. Of the 43 patients with generalised MG without AChR antibodies, only 1 had muscle-specific kinase antibodies (2.5%) and 2 had voltage-gated calcium channel antibodies indicating probable Lambert-Eaton myasthenic syndrome. 75% of the children, compared with only 28% of the adults, had ocular MG. Thymoma was evident by MRI in 1.5% of children and in 20% of adults. Despite most patients having received prednisone, very few had obtained full clinical remission. Conclusion: This study emphasises the frequency of early childhood onset with ocular symptoms and shows that many of these patients have AChR antibodies. By contrast, patients presenting in later age seem to be very uncommon in comparison with recent studies in Caucasian populations.

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confidence: 99%

Clinical and serological study of myasthenia gravis in HuBei Province, China

Zhang

Yang²,

Xu³

et al. 2006

Journal of Neurology, Neurosurgery & Psychiatry

133

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“…[1][2][3][13][14][15] This study probably for the first time documented that year to year remission rates decrease with time, maximum in the first year. Earlier studies suggested that remission rates increase with the duration of illness and spontaneous clinical remissions are likely from second year onwards.…”

Section: Discussionmentioning

confidence: 99%

Once myasthenic, always myasthenic? Observations on the behavior and prognosis of myasthenia gravis in a cohort of 100 patients

et al. 2014

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In this cohort the percentage of new remission rates per year steadily declined after the first year. Ocular myasthenia had lesser clinical worsening episodes and high chance of complete stable remission. Generalized disease had less chance drug free remission. The risk of episodes of worsening persisted at a steady rate over a period of time, being maximum in the first year. The risk of exacerbations was unpredictable and could occur after prolonged clinical quiescence, often was related to reduction of immunosuppression. The disease course did not differ significantly in the juvenile and adult age-groups. There was a strong case for permanent immunomodulation in MG.

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“…Prednisone, azathioprine, mycophenolate mofetil, and rarely cyclosporine are the immunosuppressive medications prescribed for juvenile MG. All of these medications have significant adverse effects, especially in children. The potential adverse effects of steroid medications that are particularly concerning in children include decreased growth velocity [22,23], steroid-induced diabetes, hyperlipidemia, central obesity, immunocompromised state, and pathological bone fractures [24]. Treatment with azathioprine requires white blood cell monitoring, and there is a potential association with teratogenic effects, particularly lymphoma [25][26][27][28].…”

Section: Discussionmentioning

confidence: 99%