Annals of Thoracic and Cardiovascular Surgery
Online ISSN : 2186-1005
Print ISSN : 1341-1098
ISSN-L : 1341-1098

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Paraneoplastic Neuromyelitis Optica Spectrum Disorder Associated with Atypical Thymic Carcinoid: A Case Report
Suguru MitsuiYugo TanakaKenji KimuraNaoe JimboNorio ChiharaYoshimasa Maniwa
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JOURNAL OPEN ACCESS Advance online publication

Article ID: cr.20-00354

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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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