儿童Bickerstaff脑干脑炎19例临床特征分析  

Clinical features of 19 children with Bickerstaff brainstem encephalitis

作　　者：卓秀伟[1] 丁昌红[1] 李久伟[1] 张炜华[1] 杨欣英[1] 邓劼[1] 田小娟 韩彤立[1] 方方[1] Zhuo Xiuwei;Ding Changhong;Li Jiuwei;Zhang Weihua;Yang Xinying;Deng Jie;Tian Xiaojuan;Han Tongli;Fang Fang(Department of Neurology,Beijing Children′s Hospital,Capital Medical University,Beijing 100045,China)

机构地区：[1]首都医科大学附属北京儿童医院神经内科,100045

出　　处：《中华儿科杂志》2019年第5期363-367,共5页Chinese Journal of Pediatrics

摘　　要：目的总结儿童Bickerstaff脑干脑炎(BBE)的临床特点。方法对北京儿童医院神经内科2015年10月至2018年1月收治的19例BBE患儿(男11例、女8例)的临床资料进行回顾性病例分析,总结其临床特点、治疗方法及预后。结果19例BBE患儿起病年龄1岁8月龄至12岁11月龄。18例有前驱感染史,以上呼吸道感染(9例)最常见,其次为单纯发热(5例)。神经系统首发症状以嗜睡或意识障碍(8例)最常见,其次为肢体无力(5例)。单纯BBE 6例,BBE叠加Guillain-Barré综合征(GBS)13例。除意识改变、共济失调及眼肌麻痹三联征之外,常见的神经系统伴随症状还包括抽搐发作(5例)、复视(3例)、眼球震颤(7例)、面肌无力(7例)、球麻痹(13例)及自主神经症状(9例)。16例患儿腱反射减弱或消失,8例巴氏征阳性。10例患儿合并低钠血症,其中4例为重度低钠血症,10例存在脑脊液蛋白细胞分离。19例患儿均送检血清神经节苷脂抗体,GM1抗体和GQ1b抗体阳性各2例,1例GM1抗体阳性同时合并GD1b抗体阳性。14例患儿行肌电图检查,8例提示神经源性损害,均为BBE叠加GBS型。16例患儿完成视频脑电图监测,8例监测到背景活动减慢,其中2例监测到发作间期局灶性痫样放电。18例患儿完成头颅磁共振成像(MRI)检查,3例提示颅内T2液体衰减反转恢复序列(T2-FLAIR)高信号,累及脑干、基底节、丘脑、小脑、胼胝体或皮层;11例完成脊髓MRI平扫,1例提示颈胸段脊髓斑片状异常信号,4例脊髓增强MRI均提示部分神经根强化。19例患儿均给予1~2个疗程丙种免疫球蛋白冲击治疗,2例予血浆置换,15例给予激素治疗。随访时间为3~20个月,2例失访,12例于病程3个月内、3例于6个月内症状消失。1例随诊1年8个月仍有轻度肌力下降和共济失调,1例随诊2年3个月仍遗留自主神经症状,这2例均为BBE叠加GBS。结论儿童BBE与成人相似,常见叠加GBS类型,可合并中枢神经系统脱髓鞘疾Objective To summarize the clinical features of Bickerstaff brainstem encephalitis (BBE) in children.Methods In this retrospective study,data of 19 patients with BBE (11 males and 8 females) were collected from Department of Neurology,Beijing Children′s Hospital from October 2015 to January 2018.The clinical features,treatment and prognosis were analyzed.Results The onset age of BBE ranged from 1 year and 8 months to 12 years and 11 months.There were 18 cases with preceding infection.The most common infection was upper respiratory tract infection (9 cases),followed by simple fever (5 cases).The most common initial neurological symptoms were lethargy or disturbance of consciousness (8 cases),followed by limb weakness (5 cases).There were 6 cases of simple BBE and 13 cases of BBE overlapping Guillain-Barré syndrome (GBS).Besides the characteristic triad of altered mental status,ataxia,and ophthalmoplegia,there were other symptoms including convulsion (5 cases),diplopia (3 cases),nystagmus (7 cases),facial muscular weakness (7 cases),bulbar palsy (13 cases) and autonomic nerve symptoms (9 cases).Hypo or areflexia was seen in 16 cases.Positive Babinski′s signs were seen in 8 cases.Hyponatremia was present in 10 cases in whom 4 showed severe hyponatremia.Albumin-cytological dissociation of cerebrospinal fluid was seen in 10 cases.The autoimmune antibodies were examined in all 19 patients.Anti-ganglioside antibodies including anti-GM1 IgG antibody was positive in 2 patients and one of whom was also found with positive anti-GD1b IgG antibody.Anti-GQ1b IgG antibody was present in 2 patients.Electromyography was performed in 14 cases and 8 cases,who were all BBE overlapping GBS,showed neurological damage.A total of 16 cases were monitored by video electroencephalography and 8 cases showed slow waves of background.In addition to,interictal focal discharge was detected in 2 cases.T2 fluid-attenuated inversion recovery (FLAIR) sequence abnormal signals were detected in 3 of 18 cases performed brain magnetic resonance im

关 键 词：脑干 脑炎 格林-巴利综合征 MILLER-FISHER综合征 抗GQ1b抗体 

分 类 号：R742.9[医药卫生—神经病学与精神病学]