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伴有单纯眼肌症状的重症肌无力患者的胸腺切除术

Thymectomy in myasthenia with pure ocular symptoms.

作者信息

Schumm F, Wiethölter H, Fateh-Moghadam A, Dichgans J

出版信息

J Neurol Neurosurg Psychiatry. 1985 Apr;48(4):332-7. doi: 10.1136/jnnp.48.4.332.

Abstract

Eighteen patients with exclusively ocular symptoms of myasthenia were thymectomised. Suspected thymoma, resistance to pyridostigmine therapy or relapse following immunosuppressive therapy were taken as indications for surgery. The mean preoperative observation period before operation was 40 months, and after operation was 26 months. There was no operative or postoperative morbidity or mortality. Histological thymic abnormalities were found in all patients (in one case, thymoma; in four, persistent thymus; in 13, thymic hyperplasia). The histological abnormalities were identical to those found in generalised myasthenia. This included the distribution of T-cell subtypes as identified by use of monoclonal antibodies. The severity of ocular symptoms was rated using a score developed for this purpose. The score progressively declined after surgery to an average of 70% of its initial amount in 80% of patients. Full remission occurred in three cases. No patient developed generalized myasthenia. Antibody titres against acetylcholine receptors if elevated preoperatively also dropped following surgery, with one exception. Clear criteria for the expected therapeutic success of thymectomy could not be identified. Based on our results, and on the assumed significance of the thymus gland for pathogenesis, thymectomy should be considered in patients with pure ocular symptoms.

摘要

18例仅有眼部症状的重症肌无力患者接受了胸腺切除术。疑似胸腺瘤、对吡啶斯的明治疗耐药或免疫抑制治疗后复发被视为手术指征。术前平均观察期为40个月,术后为26个月。无手术或术后并发症或死亡情况。所有患者均发现组织学胸腺异常(1例为胸腺瘤;4例为持续胸腺;13例为胸腺增生)。组织学异常与全身型重症肌无力患者相同。这包括使用单克隆抗体鉴定的T细胞亚群分布。使用为此目的制定的评分系统对眼部症状的严重程度进行评分。术后80%的患者评分逐渐下降至初始值的平均70%。3例完全缓解。无患者发展为全身型重症肌无力。术前升高的抗乙酰胆碱受体抗体滴度术后也下降,但有1例除外。无法确定胸腺切除术预期治疗成功的明确标准。基于我们的结果以及胸腺对发病机制的假定重要性,对于仅有眼部症状的患者应考虑胸腺切除术。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0978/1028297/4ae65c2c6dc6/jnnpsyc00108-0039-a.jpg

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