Department of Neurology, Urasoe General Hospital.
Present Address: Department of Neurology, Musashino Hospital.
Rinsho Shinkeigaku. 2024 Aug 27;64(8):564-571. doi: 10.5692/clinicalneurol.cn-001943. Epub 2024 Jul 27.
Neuromyelitis optica spectrum disorders (NMOSD) is one of autoimmune inflammatory diseases and is characterized by area postrema syndrome, brainstem syndrome, optic neuritis, and/or myelitis. Typical myelitis is longitudinally extended transverse myelitis (LETM) which extends over three vertebral bodies. Several previous case reports have suggested association between cancer and NMOSD. A 50-year-old woman had breast cancer and underwent mastectomy and, 10 months later, she had developed acutely progressive dysbasia. Spine MRI showed LETM in 13 vertebrae length and blood test revealed positive anti-aquaporin 4 (anti-AQP4) antibody based on enzyme-linked immunosorbent assay with index of over 40. She was treated by intravenous methylprednisolone, plasma exchange, and intravenous immunoglobulin, followed by oral prednisolone. The condition had mostly recovered after the treatment. A small population of NMOSD has the aspect of paraneoplastic neurological syndrome. The age of onset in patients with cancer-associated NMOSD tends to be higher than that in individuals with NMOSD due to any causes of NMOSD.
视神经脊髓炎谱系疾病（NMOSD）是一种自身免疫性炎症性疾病，其特征为后区综合征、脑干综合征、视神经炎和/或脊髓炎。典型的脊髓炎为长节段横贯性脊髓炎（LETM），超过三个椎体。一些先前的病例报告表明癌症与 NMOSD 之间存在关联。一名 50 岁女性患有乳腺癌并接受了乳房切除术，10 个月后，她出现了进行性运动障碍。脊柱 MRI 显示 13 个椎体长度的 LETM，血液检查显示基于酶联免疫吸附试验的抗水通道蛋白 4（抗 AQP4）抗体阳性，指数超过 40。她接受了静脉注射甲基泼尼松龙、血浆置换和静脉注射免疫球蛋白治疗，随后口服泼尼松龙。治疗后病情基本恢复。一小部分 NMOSD 具有副肿瘤性神经系统综合征的特征。与任何原因导致的 NMOSD 相比，NMOSD 伴癌患者的发病年龄往往更高。
