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眼肌型与全身型重症肌无力:与乙酰胆碱受体抗体滴度相关的连续谱。

Ocular versus generalized myasthenia gravis: a continuum associated with acetylcholine receptor antibody titers.

机构信息

Copenhagen Neuromuscular Center, section 8077, Department of Neurology, Rigshospitalet, University of Copenhagen, Inge Lehmanns vej 7-9, DK, 2100 Copenhagen, Denmark.

Copenhagen Neuromuscular Center, section 8077, Department of Neurology, Rigshospitalet, University of Copenhagen, Inge Lehmanns vej 7-9, DK, 2100 Copenhagen, Denmark.

出版信息

Neuromuscul Disord. 2024 Oct;43:39-43. doi: 10.1016/j.nmd.2024.07.002. Epub 2024 Jul 10.

Abstract

The aim of this study was to evaluate clinical and serological differences between the ocular myasthenia gravis (oMG) and generalized MG (gMG). This study is a retrospective chart review, in which data was collected from patients fulfilling 2 of 3 diagnostic MG criteria (positive antibodies, evidence of neuromuscular transmission defect on neurophysiological examination, positive effect of pyridostigmine treatment). 350 patients were included and data concerning demographics and MG medical history were collected. Patients with oMG accounted for 15.7 % of the included patients. The two subgroups differed significantly in oMG having a later age at onset, lower AChR antibody-titers, longer doctor-to-diagnosis delay and less intensive MG treatment. Additionally, patients with oMG were faster at reaching a well-controlled disease state. Thymus pathology, number of antibody-positive (95.9 % of gMG and 94.5 % of oMG), sex, number of other autoimmune diseases and delay before drug stability did not differ between oMG and gMG. In conclusion, oMG is presumably a milder form of gMG characterized by lower AChR antibody-titers, a milder phenotype, and a quicker response to a less aggressive treatment. But otherwise, oMG and gMG show very similar characteristics, including the same frequency of positive AChR antibodies, which seems new compared to previous reports.

摘要

本研究旨在评估眼肌型重症肌无力(oMG)和全身型重症肌无力(gMG)的临床和血清学差异。这是一项回顾性图表研究,其中的数据来自符合以下 3 项诊断重症肌无力标准中的 2 项的患者(阳性抗体、神经生理学检查证实存在神经肌肉传递缺陷、吡啶斯的明治疗有阳性效果)。共纳入 350 例患者,收集了有关人口统计学和重症肌无力病史的数据。oMG 患者占纳入患者的 15.7%。这两个亚组在 oMG 中存在显著差异,即发病年龄较晚、乙酰胆碱受体抗体滴度较低、从发病到确诊的时间间隔较长以及重症肌无力治疗强度较低。此外,oMG 患者更快达到病情控制良好的状态。胸腺瘤病理、抗体阳性患者数量(gMG 为 95.9%,oMG 为 94.5%)、性别、其他自身免疫性疾病数量以及达到药物稳定前的时间间隔在 oMG 和 gMG 之间无差异。总之,oMG 可能是 gMG 的一种较轻的形式,其特征为乙酰胆碱受体抗体滴度较低、表型较轻,且对治疗的反应更快,治疗的侵袭性也较低。但除此之外,oMG 和 gMG 表现出非常相似的特征,包括相同频率的阳性乙酰胆碱受体抗体,这似乎与以前的报告不同。

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