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目的探讨视神经脊髓炎谱系疾病(NMOSD)的临床特点。方法回顾性分析2例以最后区综合征(不明原因的呃逆、恶心和呕吐)为首发症状NMOSD患者的临床表现和辅助检查特点,结合国内外文献,复习和总结NMOSD的临床特点和诊治方法。结果 2例患者均有脑干、第四脑室周围、颈髓受累。1例累及视神经。血清水通道蛋白4(AQP4)抗体均(+),予大剂量甲泼尼龙冲击治疗后症状明显改善。结论最后区综合征是NMOSD的特征性表现,早期识别最后区综合征对疾病诊断和治疗尤为重要。
Objective To investigate the clinical features of optic neuromyelitis spectrum disease (NMOSD). Methods The clinical manifestations and laboratory features of two patients with first-onset syndrome (unexplained hiccups, nausea and vomiting) were retrospectively reviewed. The clinical features and diagnosis and treatment of patients with NMOSD were reviewed and summarized based on the literature both at home and abroad. Results 2 patients had brain stem, the fourth ventricle around, cervical involvement. One case involved the optic nerve. Serum aquaporin 4 (AQP4) antibody (+), to a large dose of methylprednisolone treatment significantly improved symptoms. Conclusion The final zone syndrome is the characteristic manifestation of the NMOSD. Early identification of the final zone syndrome is particularly important for the diagnosis and treatment of the disease.