视神经脊髓炎谱系疾病患者脑干损害的临床和影像学特点

Clinical and MRI features of brainstem abnormalities in neuromyelitis optica spectrum disorder

目的分析视神经脊髓炎谱系疾病(NMOSD)脑干损害的临床特点和影像学特征。方法回顾性分析确诊的79例NMOSD患者,包括53例视神经脊髓炎和26例长节段横贯性脊髓炎脑干损害的临床特点,影像学特征以及与水通道蛋白4(AQP4)抗体的关系。结果其中17例(17/79,21.5%)经临床或影像学证实有脑干损害。脑干损害组(17例)和无脑干损害组(61例)在性别、病程、单一/复发病程、扩展残疾状态评分(EDSS)无统计学差异,脑干损害组发病年龄小于无脑干损害组(32.29岁和39.39岁,P<0.05)。17例脑干损害的患者,12例有脑干受累的相关症状,包括复视(4/12,33.3%)、恶心和呕吐(9/12,75%)、呃逆(4/12,33.3%)、饮水呛咳(2/12,16.7%)、眩晕(2/12,16.7%)。9例(9/12,75%)以脑干症状作为首发症状。受累部位在中脑(2/17,11.8%)、脑桥(6/17,35.3%)、延髓(12/17,70.6%)、颈髓延髓交界区(5/17,29.4%)。脑干损害组AQP4抗体阳性率82.4%(14/17),无脑干损害组AQP4抗体阳性率75.8%(47/62),两组无显著性差异。结论 NMOSD脑干损害比较常见,呃逆、呕吐可作为疾病的首发症状,延髓或延髓颈髓延续病灶是较为特异的表现,早期识别有助于诊断和治疗。

Objective To evaluate the clinical characteristics and the corresponding MRI findings of brainstem symptoms in patients with neuromyelitis optica spectrum disorder（ NMOSD）. Methods The study was a retrospective case series of 79 patients with NMOSD including 53 with neuromyelitis optica（ NMO）,26 with longitudinally extensive transverse myelitis（ LETM）. The clinical symptoms /signs of brainstem abnormalities were evaluated and corresponding brainstem lesions were viewed by MRI. Serum AQP4 antibody was tested by cell based immunofluorescence assay. Results Brainstem abnormalities were observed in 17 patients（ 17 /79,21. 5%）. There was no statistical difference in sex,duration,course of disease（ relapsing or monophasic） and EDSS scores among patients with and without brainstem lesions. Age at onset was significantly younger in patients with brainstem lesions than patients without that（ 32. 29 vs 39. 39,P〈 0. 05）. The most frequently observed signs were diplopia（ 33. 3%）,nausea and vomiting（ 75%）,hiccups（ 33. 3%）,bucking（ 16. 7%） and vertigo（ 16. 7%）. The brainstem events occurred as initial symptoms in 9 patients. Brainstem lesions were involved in midbrain（ 2 /17,11. 8%）,pons（ 2 /17,35. 3%）,medulla（ 12 /17,70. 6%）,the medulla lesions extending to upper cervical cord（ 5 /17,29. 4%）. The AQP4-antibody positivity do not differ regarding patients with or without brainstem abnormalities. Conclusion Brainstem symptoms /signs are common in patients of NMOSD. Vomiting and hiccups can be the first symptoms. The medulla lesions and the lesions extending to upper cervical cord were unique to NMOSD. The observations of brainstem lesions are helpful to the early diagnose and initiation of the treatment.