Zhu Man-Min, Xu Zu-Cai, Yu Chang-Yin, Huang Hao
Department of Neurology Affiliated Hospital of Zunyi Medical University Zunyi China.
Ibrain. 2024 Sep 13;11(2):268-273. doi: 10.1002/ibra.12175. eCollection 2025 Summer.
Neuromyelitis optica spectrum disorder (NMOSD) is a group of autoimmune disorders characterized by inflammatory involvement of the optic nerve, spinal cord, and central nervous system. NMOSD is often associated with other autoimmune disorders, including Sjogren's syndrome (SS). While NMOSD typically occurs at a peak in young or older individuals, the coexistence of NMOSD and SS in a youngster is rare. Here, we presented a case of a 14-year-old girl with NMOSD and SS who responded well to immunosuppressive therapy but experienced a severe relapse after discontinuation of therapy. We described the clinical course of a case over 8 years, underscoring the importance of long-term treatment for NMOSD and SS. This case, along with the review of relevant literature, will raise awareness of this type of disease and facilitate early diagnosis and treatment to avoid serious sequelae.
视神经脊髓炎谱系障碍(NMOSD)是一组自身免疫性疾病,其特征是视神经、脊髓和中枢神经系统发生炎症性病变。NMOSD常与其他自身免疫性疾病相关,包括干燥综合征(SS)。虽然NMOSD通常在年轻人或老年人中达到发病高峰,但NMOSD与SS在青少年中同时存在的情况很少见。在此,我们报告了一例14岁患有NMOSD和SS的女孩,她对免疫抑制治疗反应良好,但在治疗中断后经历了严重复发。我们描述了该病例8年多的临床病程,强调了NMOSD和SS长期治疗的重要性。该病例以及相关文献的回顾将提高对这类疾病的认识,并促进早期诊断和治疗以避免严重后遗症。