以自发垂直性眼球震颤为主要体征的脑干小脑病变(附2例报告及文献复习)  

Brainstem and Cerebellar Disorders Presenting Spontaneous Vertical Nystagmus (2 Case Report and Literature Review)

作　　者：吴菁[1] 陈伟[1] 孙旭红[1] 崔国红[1] 张绮婷 唐学梅[1] 舒良[1] 方洁[1] 严为宏[1] 刘建仁[1] WU Jing;CHEN Wei;SUN Xu-hong;CUI Guo-hong;ZHANG Qi-ting;TANG Xue-mei;SHU Liang;FANG Jie;YAN Wei-hong;LIU Jian-ren(Department of Neurology,Shanghai Ninth People's Hospital Affiliated to Shanghai Jiaotong University School of Medicine,Shanghai 200011,China;Department of Neurology,Tongren Hospital,Shanghai Jiaotong University School of Medicine,Shanghai 200336,China)

机构地区：[1]上海交通大学医学院附属第九人民医院神经内科,上海200011 [2]上海交通大学医学院附属同仁医院神经内科,上海200336

出　　处：《中国临床神经科学》2018年第6期636-644,共9页Chinese Journal of Clinical Neurosciences

基　　金：上海交通大学医学院(理学)基金课题(编号:14XJ10063);上海市科委医学引导类科技支撑项目(编号:17411964000)

摘　　要：目的通过对自发性垂直性眼球震颤病例分析及复习相关文献,加强临床医生对该体征的认识。方法回顾性分析2例表现为自发性垂直性眼球震颤患者的临床及影像学资料,结合相关文献复习进行探讨。结果病例1表现为突发呃逆、呕吐、吞咽困难,查体见垂直上跳性眼球震颤(UBN),头颅MRI示延髓背侧病灶,血清AQP-4抗体阳性,诊断为视神经脊髓炎谱系疾病(极后区综合征)。病例2表现为发作性头晕伴视物晃动,查体见垂直下跳性眼球震颤(DBN),头颅MRI证实为Arnold-Chiari畸形Ⅰ型。分析产生垂直性眼球震颤的病因为:病变分别累及延髓背侧旁正中区以及双侧小脑绒球,导致垂直眼球活动中枢通路受损。结论垂直性眼球震颤提示脑干-小脑垂直眼球活动中枢通路受损,UBN和DBN具有不同的病因与定位意义。Aim To enhance the clinicians'understanding of vertical nystagmus by reviewing the clinical cases of spontaneous vertical nystagmus and the relevant literature.Methods The clinical and neuroimaging data of two patients with spontaneous vertical nystagmus were retrospectively analyzed and concluded with relevant literature.Results One patient who was diagnosed with neuromyelitis optica spectrum disorders (area postrema syndrome)manifested upbeat nystagmus (UBN),hiccup and dysphagia,the cranial MRI showed lesion in dorsal medulla and the serum AQP4antibody was positive. The other patient presented with downbeat nystagmus (DBN)and paroxysmal vertigo,cranial MRI proved to be Arnold-Chiari I malformation.The pathophysiology of UBN in case 1and DBN in case 2was the impairment of vertical ocular movement pathway in paramedian region of dorsal medulla oblongata and bilateral flocculus respectively.Conclusion Vertical nystagmus indicates impaired cerebello-brainstem vertical ocular movement pathway,and UBN and DBN have different causes and significance of localization.

关 键 词：垂直上跳性眼球震颤 垂直下跳性眼球震颤 垂直性前庭眼反射 自发性 

分 类 号：R742[医药卫生—神经病学与精神病学]