Takeuchi Yosuke, Masuda Teruaki, Otsuka Mami, Takeda Atsunobu, Kimura Noriyuki
Department of Neurology, Faculty of Medicine, Oita University, Yufu, JPN.
Department of Ophthalmology, Oita University, Yufu, JPN.
Cureus. 2025 May 22;17(5):e84602. doi: 10.7759/cureus.84602. eCollection 2025 May.
Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disease of the central nervous system (CNS) that can result in severe neurological sequelae. Aquaporin-4, a cause of complement-mediated inflammation in NMOSD, is expressed on alveolar epithelial cells and in the CNS, and organizing pneumonia (OP) is recognized as an extra-CNS symptom in NMOSD. We herein present a case of NMOSD following OP. The patient initially presented with area postrema syndrome, and the coexistence of OP made the diagnosis of NMOSD challenging. A review of previously reported cases of NMOSD with OP suggests that careful attention to area postrema syndrome as an onset symptom is needed for the early diagnosis and treatment of NMOSD in male and older patients with OP.
视神经脊髓炎谱系障碍（NMOSD）是一种中枢神经系统（CNS）炎症性疾病，可导致严重的神经后遗症。水通道蛋白4是NMOSD中补体介导炎症的一个病因，在肺泡上皮细胞和中枢神经系统中表达，而机化性肺炎（OP）被认为是NMOSD的一种中枢神经系统外症状。我们在此报告一例OP后发生NMOSD的病例。该患者最初表现为最后区综合征，OP的并存使得NMOSD的诊断具有挑战性。对先前报道的NMOSD合并OP病例的回顾表明，对于患有OP的男性和老年患者，早期诊断和治疗NMOSD需要密切关注以最后区综合征作为起病症状。
