Department of Neurology, UT Southwestern Medical Center, Dallas, TX, USA.
Department of Pathology, Children's Medical Center, Dallas, TX, USA.
Mult Scler. 2022 Jan;28(1):160-163. doi: 10.1177/13524585211037582. Epub 2021 Aug 23.
Neuromyelitis optica spectrum disorder is an inflammatory condition of the central nervous system typically manifesting as myelitis, optic neuritis, and/or area postrema syndrome. Here, we present a pediatric patient who developed symptoms consistent with area postrema syndrome with positive anti-aquaporin-4 (AQP4) antibodies who was also found to have an ovarian teratoma. Pathological specimens revealed the presence of aquaporin-4. This was felt to be the antigenic trigger that led to the patient's condition. She suffered no further clinical attacks and seroconverted to negative AQP4 status upon teratoma removal. This case varies from others, in that the paraneoplastic presentation occurred in a pediatric patient and in that the patient has not required maintenance immunotherapy after teratoma removal.
视神经脊髓炎谱系疾病是一种中枢神经系统炎症性疾病，通常表现为脊髓炎、视神经炎和/或视上核综合征。在此，我们介绍了一位儿科患者，其出现了与视上核综合征一致的症状，且抗水通道蛋白-4（AQP4）抗体阳性，同时还发现患有卵巢畸胎瘤。病理标本显示存在水通道蛋白-4。这被认为是导致患者发病的抗原触发因素。她没有再出现临床发作，并且在畸胎瘤切除后转为抗 AQP4 抗体阴性。与其他病例不同的是，本例副肿瘤表现发生在儿科患者中，且患者在畸胎瘤切除后无需维持免疫治疗。
