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40岁以上人群的重症肌无力

Myasthenia gravis in individuals over 40.

作者信息

Aarli Johan A, Romi Frederik, Skeie Geir Olve, Gilhus Nils Erik

机构信息

Department of Neurology, University of Bergen, Bergen, Norway.

出版信息

Ann N Y Acad Sci. 2003 Sep;998:424-31. doi: 10.1196/annals.1254.055.

Abstract

Myasthenia gravis (MG) in individuals over 40 years of age comprises three groups: early-onset MG with prolonged disease duration, late-onset MG with thymoma, and late-onset of nonthymomatous MG. The clinical features do not differ between the three groups, except that early-onset patients with prolonged disease duration usually have a less severe disease. More than 60% of our MG patients are now more than 50 years of age, often with disease onset after age 40. Although 2 out of the 3 patients in Erb's original description had onset of myasthenic symptoms after age 40, this was apparently infrequent in 1879, when the disease was first identified. Onset of MG after age 40 is now common. For example, in our material, 88/184 (47.8%) had onset of MG after age 40. Eighteen (20.5%) had a thymoma. The female:male ratio in the early-onset group was 2.4:1, whereas it was 1:1.1 among those with onset after age 40. There was no human leukocyte antigen association for MG with thymoma. Antibodies to the acetylcholine receptor were detected in 88% of sera from nonthymomatous MG and in 100% of those with late-onset MG with thymoma. Antibodies to titin were found in sera from 85.7% of MG patients with thymoma (all age groups) and in 58% of nonthymomatous MG with late onset and acetylcholine receptor antibodies. Late-onset, nonthymomatous MG comprises two subgroups, one corresponding to delayed early onset and one immunologically similar to that seen in patients with MG and thymoma.

摘要

40岁以上的重症肌无力(MG)患者可分为三组:疾病持续时间长的早发型MG、伴有胸腺瘤的晚发型MG和非胸腺瘤性晚发型MG。除了疾病持续时间长的早发型患者病情通常较轻外,三组的临床特征并无差异。我们现在的MG患者中超过60%年龄超过50岁,发病往往在40岁以后。虽然在埃尔布最初描述的3例患者中有2例肌无力症状在40岁以后出现,但在1879年该病首次被发现时,这显然并不常见。MG在40岁以后发病现在很常见。例如,在我们的资料中,184例中有88例(47.8%)MG发病于40岁以后。18例(20.5%)有胸腺瘤。早发型组的女性与男性比例为2.4:1,而40岁以后发病的患者中该比例为1:1.1。MG伴胸腺瘤与人类白细胞抗原无关联。在非胸腺瘤性MG患者的88%血清中以及伴有胸腺瘤的晚发型MG患者的100%血清中检测到乙酰胆碱受体抗体。在所有年龄组的MG伴胸腺瘤患者的85.7%血清中以及58%伴有乙酰胆碱受体抗体的晚发型非胸腺瘤性MG患者血清中发现了抗肌联蛋白抗体。晚发型非胸腺瘤性MG包括两个亚组,一个对应于延迟的早发型,另一个在免疫方面与MG伴胸腺瘤患者所见相似。

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