摘要
目的回顾性分析自发性下跳性眼震(spontaneous downbeat nystagmus,sDBN)患者的病因分布、临床特征以及眼震特点。方法收集2013年至2018年期间,在首都医科大学附属北京同仁医院神经内科就诊的sDBN患者,回顾性分析患者的临床特征、颅脑MRI、眼震视图等辅助检查以及治疗转归。结果共收集sDBN患者12例,年龄22~70岁,平均年龄(45±18.8岁),12例(100%)患者均主诉头晕和/或眩晕,8例(66.7%)有振动幻视。12例患者中,颅-颈交界部压迫性病变占5例(41.7%),包括后颅窝囊肿2例、Arnold-Chiari畸形3例(其中1例患者合并小细胞肺癌及副肿瘤综合征),抗癫痫药物中毒2例,自身免疫性脑炎和脊髓小脑性共济失调各1例,特发性3例。眼震视图检查可见所有患者均有凝视诱发性眼震、扫视性跟踪以及前庭眼动反射抑制不能,8例(66.7%)存在视动性眼震异常,9例(75%)Romberg征阳性,8例(66.7%)步态异常;向上凝视时眼震减轻、慢相速度(slow phase velocity,SPV)最小,向下和侧方凝视时眼震增强、SPV增大,而仰卧悬头位时SPV最大。结论累及前庭小脑的各种病变可导致sDBN,多数患者主诉头晕和/或眩晕、振动幻视。特征性的眼震视图检查参数有助于定位诊断,避免漏诊。
Objective To retrospectively analyze the etiology distribution,clinical features,and the characteristics of spontaneous downbeat nystagmus(sDBN).Methods Patients with sDBN were included from 2013 to 2018 from Department of Neurology,Beijing Tongren Hospital,Capital Medical University.Patients with positional DBN,non-persistent DBN or nystag with torsional component were excluded.The etiology,clinical features,brain MRI,videonystagmorgaphy(VNG),laboratory tests,treatments and outcomes of these cases were collected and analyzed.Results 12 cases with sDBN were collected.The average age of the patients was 45±18.8(22-70)years old,100%(12/12)of the patients had dizziness and/or vertigo,66.7%(8/12)of the patients had oscillopsia.41.7%(5/12)was caused by the compressive lesion at the craniocervical junction,of which 2 cases had a cyst at the posterior cranium,3 cases with Arnold-Chiari malformation(one case with Arnold-Chiari malformation also had small cell lung cancer and paraneoplastic syndrome).2 cases were caused by antiepileptic drug intoxication,autoimmune encephalitis and spinal cerebellar ataxia attributed to one case respectively,3 cases were idiopathic sDBN.All the patients had gaze evoked nystagmus,saccadic smooth pursuit and impaired vestibular ocular reflex suppression on VNG test.Abnormal optokinetic nystagmus,positive Romberg sign and gait disturbance were found in 66.7%(8/12),75%(9/12)and 66.7%(8/12)of all the patients respectively.VNG test indicated that the SPV of DBN was increased when the eyes gaze downward and laterally,which got maximum at the head hanging position.Conclusions Various diseases involving the vestibular cerebellum can cause sDBN,and most patients complain of dizziness and/or vertigo,oscillopsia.The specific VNG abnormalities would help topical differential diagnosis.
作者
崔世磊
孙晓静
孔秀云
刘雪菲
杨彦
江汉秋
王佳伟
CUI Shilei;SUN Xiaojing;KONG Xiuyun;LIU Xuefei;YANG Yan;JIANG Hanqiu;WANG Jiawei(不详;Department of Neurology,Beijing Tongren Hospital,Capital Medical University,Beijing 100730,China)
出处
《中国神经免疫学和神经病学杂志》
CAS
北大核心
2021年第2期120-124,130,共6页
Chinese Journal of Neuroimmunology and Neurology
基金
国家自然科学基金(81771313)
北京市自然科学基金(7192040)
“首都临床特色应有研究”专项(Z171100001017039)
首都卫生发展科研专项(首发2020-2-2056)
首都医科大学附属同仁医院“重点医学发展计划”专项(trzdyxzy201704)。
关键词
下跳性眼震
小脑
眼震视图
downbeat nystagmus
cerebellar
videonystagmorgaphy
