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眼肌型重症肌无力:争议与新进展。

Ocular myasthenia gravis: controversies and updates.

机构信息

Department of Neuro-ophthalmology, Moorfields Eye Hospital, 162 City Road, London, EC1V 2PD, UK,

出版信息

Curr Neurol Neurosci Rep. 2014 Jan;14(1):421. doi: 10.1007/s11910-013-0421-9.

Abstract

The majority of patients with myasthenia gravis (MG) initially present with ocular symptoms. An unresolved question is whether there are clinical features at onset to guide clinicians to predict an individual patient's conversion risk from ocular MG (OMG) to generalized disease, or "secondary generalized MG" (SGMG), that is, a prognostic model. In light of the emerging theory that early corticosteroids may have a risk-modifying effect, the factors associated with secondary SGMG previously reported should be revisited. Studies showing potential risk-modifying effects of corticosteroids are useful, though flawed, owing to the heterogeneous retrospective studies and methods of reporting. Updates on other potential immunosuppressive agents are also discussed. Thymectomy in OMG has been recently reported in a few studies to be useful. MG associated with antibodies to muscle-specific kinase, usually associated with severe generalized MG, can cause a pure OMG syndrome. Recent serological developments in seronegative patients have also revealed antibodies to clustered anti-acetylcholine receptor and lipoprotein receptor-related protein-4.

摘要

大多数重症肌无力 (MG) 患者最初表现为眼部症状。一个悬而未决的问题是,在发病时是否存在临床特征可以指导临床医生预测个别患者从眼肌型 MG (OMG) 向全身性疾病(即预后模型)转变的风险。鉴于早期使用皮质类固醇可能具有风险修饰作用的新理论,应该重新审视先前报道的与继发性 SGMG 相关的因素。尽管由于回顾性研究和报告方法存在差异,显示皮质类固醇具有潜在风险修饰作用的研究存在缺陷,但它们仍很有用。还讨论了其他潜在免疫抑制剂的最新进展。眼肌型 MG 的胸腺切除术最近在一些研究中被报道是有用的。通常与严重全身性 MG 相关的肌肉特异性激酶抗体相关的 MG 可引起单纯眼肌型综合征。最近在血清阴性患者中的血清学发展也揭示了对聚集型乙酰胆碱受体和脂蛋白受体相关蛋白-4 的抗体。

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