Miller-Fisher综合征和Bickerstaff脑干脑炎的临床特点及鉴别诊断

Clinical features and differential diagnosis of Miller-Fisher syndrome and Bickerstaff's brainstem encephalitis

目的探讨Miller-Fisher综合征(MFS)和Bickerstaff脑干脑炎(BBE)的临床特点及鉴别诊断。方法回顾性分析12例MFS和15例BBE患者的临床资料。结果MFS组表现为视物成双12例,瞳孔对光反射消失3例,周围性面瘫7例,饮水呛咳1例,四肢无力6例,感觉障碍4例,共济失调10例;BBE组表现为视物成双11例,中枢性面瘫4例,耳鸣4例,饮水呛咳6例,四肢无力10例,感觉障碍2例,共济失调10例,嗜睡或昏迷5例,病理征9例。脑脊液检查MFS组和BBE组各有10例表现为蛋白升高;MFS组脑脊液白细胞升高2例,BBE组6例。MFS组3例、BBE组1例肌电图出现神经源性损害。BBE组有6例CT或MRI检查异常,主要部位为脑干、丘脑等。两组患者治疗后预后良好。结论MFS和BBE有相似特点,临床表现可以相互重叠,提示这两种疾病密切相关并形成一个连续的疾病谱;主要鉴别点是BBE患者可以有意识障碍、病理征和影像学异常。

Objective To study the clinical characteristics and differential diagnosis of Miller-Fisher syndrome （MFS） and Bickerstaff＇s brainstem encephalitis （BBE）. Methods The clinical data of 12 patients with diagnosis of MFS and 15 patients with diagnosis of BBE were analyzed retrospectively. Results In MFS group, the patients showed diplopia （12 cases ）, loss of pupillary light reflex （3 cases ）, peripheral facial paralysis （7 cases ）, hydroposia bucking （1 case）, weakness of limbs （6 cases） , sensory disability （4 cases） and ataxia （lO cases）. In BBE group, the patients presented with diplopia （ 11 cases ） , central facial palsy （ 4 cases ）, tinnitus （ 4 cases ） , hydroposia bucking （6 cases）, weakness of limbs （10 cases）, sensory disability （2 cases）, ataxia （10 cases）, somnolence or coma （5 cases） and positive Babinski＇s sign （9 cases）. CSF examination showed elevation of protein in 10 patients from BBE group and 10 patients from MSF group, however, leucocytosis was found in 6 cases from BBE group and 2 cases from MSF group. There were 4 patients of MFS and 1 patient of BBE with abnormal results of eleetromyogram （EMG） examination. 6 patients in BBE group had abnormal results of CT or MRI examinations, and the lesions mainly located in brainstem, thalamus and so on. Both groups had good prognosis after therapies. Conclusions MFS and BBE have similar and overlapped clinical characteristics. This indicates that these two diseases are closely related and may form a continuous spectrum. The main identifications of BBE are conscious disturbance, positive reflex of Babinski＇s sign and abnormal imaging results in some patients.