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血清阴性全身型重症肌无力:胸腺病变发生率低。

Seronegative generalized myasthenia gravis: low frequency of thymic pathology.

作者信息

Verma P K, Oger J J

机构信息

Division of Neurology, University of British Columbia, Vancouver, Canada.

出版信息

Neurology. 1992 Mar;42(3 Pt 1):586-9. doi: 10.1212/wnl.42.3.586.

Abstract

We have reviewed thymus histology, preoperative serum acetylcholine receptor antibody status, and clinical features of all 50 patients who underwent thymectomy for generalized myasthenia gravis in the University of British Columbia-affiliated hospitals over the last 8 years. Seven patients had thymoma, 25 had hyperplasia, and 18 had a normal thymus. The seven thymoma patients all had severe limb involvement and all had circulating antibodies against the acetylcholine receptor. Patients without circulating antibodies had only minimal limb involvement and half of them had a normal thymus. Only two of the 32 (6%) patients with abnormal thymus were antibody-negative; this contrasts with six of the 18 patients (33%) with normal thymus. We conclude that seronegative patients with generalized myasthenia gravis constitute a distinct subgroup which may not be immunologically homogeneous and could be subgrouped with normal or abnormal thymic histology.

摘要

我们回顾了过去8年在英属哥伦比亚大学附属医院接受胸腺切除术治疗全身型重症肌无力的50例患者的胸腺组织学、术前血清乙酰胆碱受体抗体状态及临床特征。7例患者患有胸腺瘤,25例有胸腺增生,18例胸腺正常。7例胸腺瘤患者均有严重的肢体受累,且均有抗乙酰胆碱受体循环抗体。无循环抗体的患者仅有轻微的肢体受累,其中一半胸腺正常。32例胸腺异常的患者中只有2例(6%)抗体阴性;相比之下,18例胸腺正常的患者中有6例(33%)抗体阴性。我们得出结论,全身型重症肌无力血清阴性患者构成一个独特的亚组,其免疫状态可能不均一,可根据胸腺组织学正常或异常进行亚组划分。

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