摘要
目的探讨Bickerstaff脑干脑炎(BBE)和Miller Fisher综合征(MFS)的临床特征,研究两者的症状学特点及疾病分类学关系。方法对湘雅二医院2003-01-2013-12符合BBE(21例)和MFS(67例)诊断标准的患者的临床资料进行回顾性分析。结果 BBE和MFS患者均存在以上呼吸道感染为主的前驱感染症状〔10例(48%)、36例(54%)〕,并具有眼外肌麻痹和共济失调的共同症状〔21例(100%)、67例(100%)〕,头痛〔8例(42%)、20例(30%)〕、眼内肌麻痹〔8例(38%)、17例(25%)〕、延髓麻痹〔8例(50%)、32例(48%)〕、面瘫〔6例(35%)、20例(30%)〕、浅表感觉障碍〔4例(25%)、28例(42%)〕等症状在两者中均常见且发生率相近;两者均有脑脊液蛋白-细胞分离现象〔5例(25%)、23例(38%)〕和头部影像学检查〔10例(59%)、2例(4%)〕异常。两者主要的不同点在于BBE患者的中枢神经系统受累表现如意识障碍〔16例(76%)〕、腱反射亢进〔11例(52%)〕、Babinski征〔6例(32%)〕、头部MRI脑干部位异常信号〔10例(59%)〕、脑电图异常〔7例(78%)〕较MFS患者〔0例、0例、8例(12%)、2例(4%)、0例〕常见。两组患者预后均较好,随访期间(≥1年)均未见复发病例,MFS患者59例(88%)症状完全消失,BBE患者10例(48%)症状完全消失,3例(14%)明显改善,6例BBE死亡。结论BBE与MFS临床特点相似且存在交叉重叠,两者可能形成同一连续性自身免疫性疾病谱。
Objective The aims of our study were to clarify the clinical profiles,outcomes and nosological relationship of Bickerstaff brainstem encephalitis（BBE）and Miller Fisher syndrome（MFS）by studying their clinical features and laboratory characteristics.Methods We reviewed medical records of 21 BBE and 67 MFS patients diagnosed by strict criteria at the second Xiangya Hospital in Changsha Hauan,China between 2003 and 2013.We collected data on clinical profiles and outcomes of BBE and MFS.Results In our study,we found that BBE and MFS shared many similarities：upper respiratory infection was the most frequent preceding symptom（48%vs.54%）,and ophthalmoplegia,ataxia were the common clinical features.There was no significant difference between the two groups in the proportion of patients presenting with headach（42%vs.30%）,internal ophthalmoplegia（38%vs.25%）,bulbar palsy（50%vs.48%）,facial palsy（35%vs.30%）,surperficial sense impairment（25% vs.42%）.CSF albuminocytological dissociation（25% vs.38%）and abnormal MRI on T2-weighted images（59% vs.4%）were prentent in both BBE and MFS.Compared with MFS,our BBE patients had more evidence of CNS involvement because almost all had impaired consciousness（76%）,hyperreflexia（52%）,Babinski sign（32%）and abnormal EEG findings（78%）and high-intensity abnormalities on MRI on T2-weighted images of the brainstem（59%）.Both outcomes generally are good,by 1year after disease onset 48% of the 21 BBE patients and 88% of the 67 MFS patients show complete remission with no residual symptoms.In addition,6BBE patients died.Conclusions Because of the similarities in the clinical presentations of BBE and MFS,we could make conclusive that BBE and MFS are not two distinct conditions,but form a single autoimmune continuous spectrum.
出处
《中国神经免疫学和神经病学杂志》
CAS
2015年第2期77-81,共5页
Chinese Journal of Neuroimmunology and Neurology
