Paraneoplastic Neuromyelitis Optica Spectrum Disorder Associated with Atypical Thymic Carcinoid: A Case Report.

IF 1.1 4区医学 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS

Annals of Thoracic and Cardiovascular Surgery Pub Date : 2022-10-20 Epub Date: 2021-04-27 DOI:10.5761/atcs.cr.20-00354

Suguru Mitsui, Yugo Tanaka, Kenji Kimura, Naoe Jimbo, Norio Chihara, Yoshimasa Maniwa

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引用次数: 2

Abstract

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune inflammatory disease, occasionally accompanied by malignant tumors. Immunosuppressive therapy is the mainstay treatment for idiopathic NMOSD; no guidelines have been published for paraneoplastic NMOSD because it is rarely reported in the literature. We report a rare case of a 67-year-old man with paraneoplastic NMOSD associated with thymic carcinoid whose cells expressed aquaporin-4 antibody. After surgical resection, the patient's symptoms improved, and serum aquaporin-4 autoantibody turned negative. We believe that radiographic examination for mediastinal tumors in patients with NMOSD is necessary because thymic epithelial tumors could have a role in the pathogenesis of paraneoplastic NMOSD. After mediastinal tumor has been detected, they should be surgically resected to improve neurological symptoms.

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非典型胸腺类癌伴视谱神经脊髓炎1例。

视神经脊髓炎谱系障碍(NMOSD)是一种自身免疫性炎症性疾病，偶尔伴有恶性肿瘤。免疫抑制治疗是特发性NMOSD的主要治疗方法;由于文献中很少报道，目前还没有关于副肿瘤性NMOSD的指南。我们报告一例罕见的67岁男性副肿瘤NMOSD合并胸腺类癌，其细胞表达水通道蛋白-4抗体。手术切除后，患者症状好转，血清水通道蛋白-4自身抗体转为阴性。我们认为胸腺上皮肿瘤可能在副肿瘤性NMOSD的发病机制中起作用，因此有必要对NMOSD患者的纵隔肿瘤进行影像学检查。纵隔肿瘤发现后，应手术切除以改善神经系统症状。

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