首次发作表现为脑干症状的视神经脊髓炎谱系疾病临床和神经影像学特征

Clinical and neuroimaging characters of patients with neuromyelitis optica spectrum disorders initiated with brainstem symptoms

目的探讨首次发作表现为脑干症状的视神经脊髓炎谱系疾病（NMOSD）临床和神经影像学特征。方法回顾性分析5例首次发作表现为脑干症状的NMOSD患者的临床资料。结果 5例患者均为女性,发病年龄20~39岁,平均病程17.8个月。4例患者表现为顽固性恶心、呕吐,其中1例伴有呃逆。周围性面神经麻痹、面部瘙痒、眼球运动障碍患者各2例。味觉减退、三叉神经麻痹各1例。3例患者AQP4抗体阳性。脑MRI显示病灶位于延髓背侧、桥脑背侧、中脑导水管周围,呈长T1、长T2信号,FLAIR、DWI上呈稍高信号,病灶无强化。结论 NMOSD首次发作可以表现为脑干症状,其中最常见的是顽固性恶心、呕吐。病灶通常位于脑干室管膜周围。

Objective To investigate the clinical and neuroimaging characters of patients with neuromyelitis optica spectrum disorders（ NMOSD） initiated with brainstem symptoms. Methods Five cases of NMOSD initiated with brainstem symptoms were retrospectively analyzed.Results All the patients were female. Onset age ranged from 20 to 39 years old,and the mean duration was 17. 8 months. Four patients presented with intractable nausea and vomiting,one of them accompanied with hiccup. Pruritus of the face and oculomotor dysfunction occurred in 2 patients. Hypogeusia,facial palsy and trigeminal nerve palsy occurred in 1 patient. Aquaporin 4（ AQP4） specific antibodies were positive in 3 patients. Brain MRI revealed periaqueductal lesions in dorsal of the medulla oblongata and pons,with hypointese on T1 WI,hyperintense lesions on T2 WI,slight hyperintense on fluid attenuated inversion recovery（ FLAIR） imaging and diffusion weighted imaging（ DWI）. Conclusion NMOSD may initiate with different brainstem symptoms,and the most common are intractable nausea and vomiting. The lesions usually locates in periaqueductal.