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伴有抗接触蛋白相关蛋白2(Caspr2)自身抗体的莫旺综合征早于胸腺瘤复发30个月。

Caspr2 autoantibody-associated Morvan syndrome predating thymoma relapse by 30 months.

作者信息

Masrori Pegah, Vaesen Bentein Hannah, Raskin Jo, Montagna Massimiliano, De Picker Livia, De Volder Ilse, Van Schil Paul E, Janssens Annelies, Mercelis Rudy

机构信息

Department of Neurology, Antwerp University Hospital, Edegem, Belgium.

Master of Medicine Student, KU Leuven, Leuven, Belgium.

出版信息

Lung Cancer. 2021 Mar;153:117-119. doi: 10.1016/j.lungcan.2021.01.012. Epub 2021 Jan 13.

DOI:10.1016/j.lungcan.2021.01.012
PMID:33485137
Abstract

Morvan's syndrome (MoS) is a rare autoimmune disorder characterized by central nervous system involvement, autonomic dysfunction and peripheral nerve hyperexcitability. MoS is believed to be caused by autoantibodies targeting contactin-associated protein 2 (Caspr2), a subunit of the neuronal voltage-gated potassium channel (VGKC) complex, usually in association with thymoma, less commonly with other malignancies. This case highlights an exceptional case of severe sleep disturbances and behavioural changes due to MoS, in a patient who would present with and be treated successfully for a second relapse of thymoma 30 months later. Originally he suffered from ocular myasthenia, another autoimmune disorder, which led to diagnosis of his original thymoma and first relapse.

摘要

莫旺综合征(MoS)是一种罕见的自身免疫性疾病,其特征为中枢神经系统受累、自主神经功能障碍和周围神经兴奋性增高。MoS被认为是由靶向接触蛋白相关蛋白2(Caspr2)的自身抗体引起的,Caspr2是神经元电压门控钾通道(VGKC)复合物的一个亚基,通常与胸腺瘤有关,较少与其他恶性肿瘤有关。本病例突出了一例因MoS导致严重睡眠障碍和行为改变的特殊病例,该患者在30个月后出现胸腺瘤第二次复发并成功接受治疗。最初,他患有另一种自身免疫性疾病——眼肌型重症肌无力,这导致了他最初胸腺瘤的诊断和首次复发。

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Caspr2 autoantibody-associated Morvan syndrome predating thymoma relapse by 30 months.伴有抗接触蛋白相关蛋白2(Caspr2)自身抗体的莫旺综合征早于胸腺瘤复发30个月。
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