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家族性婴儿型重症肌无力。三例报告及成年期随访

Familial infantile myasthenia gravis. Report of three cases with follow-up until adult life.

作者信息

Gieron M A, Korthals J K

出版信息

Arch Neurol. 1985 Feb;42(2):143-4. doi: 10.1001/archneur.1985.04060020053015.

Abstract

We describe three siblings with familial infantile myasthenia gravis (FIMG). Contrary to previous reports suggesting a benign course of FIMG beyond the infantile period, our two patients had severe respiratory exacerbations in early adult life. Thymectomy was performed in the two more affected siblings, with only brief beneficial effect in one and no improvement in the other. Steroid therapy, employed in two patients who underwent thymectomy, resulted in no long-term improvement. The third patient has not required therapy. Recognition of FIMG has important therapeutic implications, because this disease shows rather poor response to thymectomy and steroid therapy.

摘要

我们描述了三名患有家族性婴儿型重症肌无力(FIMG)的兄弟姐妹。与之前关于FIMG在婴儿期之后病程良性的报道相反,我们的两名患者在成年早期出现了严重的呼吸加重情况。对病情较重的两名兄弟姐妹进行了胸腺切除术,其中一人仅获得短暂的有益效果,另一人则无改善。对两名接受胸腺切除术的患者采用了类固醇疗法,但未取得长期改善效果。第三名患者无需治疗。认识FIMG具有重要的治疗意义,因为这种疾病对胸腺切除术和类固醇疗法的反应相当不佳。

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