伴有极后区综合征的AQP4-IgG阴性视神经脊髓炎谱系疾病合并干燥综合征1例

AQP4-IgG-Negative Neuromyelitis Optica Spectrum Disorder Complicated with Sjögren’s Syndrome Presenting with Area Postrema Syndrome: A Case Report

摘要: 视神经脊髓炎谱系疾病是一种免疫介导的中枢神经系统炎症性脱髓鞘疾病。典型的临床表现包括视神经炎和纵向广泛的横贯性脊髓炎。极后区综合征在视神经脊髓炎谱系疾病的临床症状中较为少见，多表现为原因不明的恶心、呕吐，临床上容易误诊。伴有极后区综合征的AQP4-IgG阴性的视神经谱系疾病合并干燥综合征罕见报道，现将我院诊治的1例病例报告如下。

Abstract: Optic Neuromyelitis Spectrum Disease (ONSD) is an immune-mediated inflammatory demyelinating disorder of the central nervous system. The typical clinical manifestations include optic neuritis and longitudinally extensive transverse myelitis. Posterior pole syndrome is relatively rare among the clinical symptoms of ONSD, often presenting as unexplained nausea and vomiting, which can be easily misdiagnosed. Rare reports have documented AQP4-IgG-negative optic neuropathy spectrum disease complicated by Sjögren’s syndrome with posterior pole syndrome. Here, we report a case diagnosed and treated in our hospital.