6例抗神经节苷脂抗体阳性变异型吉兰巴雷谱系病患儿临床分析

Clinical analysis of anti-ganglioside antibody-positive variant Guillain-Barrésyndrome spectrum diseases

目的探讨抗神经节苷脂抗体阳性的变异型吉兰巴雷谱系病患儿的临床特征及预后情况。方法2022年8月—2023年7月河南省人民医院诊治变异型吉兰巴雷谱系病患儿6例,分析患儿临床资料和预后情况,包括临床症状和体征、脑脊液生化检查、白细胞介素(interleukin,IL)(IL-1β、-5、-6、-8、-17)、抗神经节苷脂抗体、神经电生理和头颅MRI检查结果及治疗转归情况等。结果6例患儿中Miller-Fisher综合征3例,Bickerstaff’s脑干脑炎2例,面瘫合并瞳孔散大1例。临床表现为视力下降4例,眼球活动障碍2例,合并眼睑下垂2例,球麻痹2例,肢体无力和腱反射减弱2例,感觉过敏1例,痛性眼麻痹1例,面瘫合并瞳孔散大1例。2例脑脊液呈蛋白-细胞分离现象;肺炎支原体抗体阳性1例,EB病毒抗体阳性1例,单纯疱疹病毒Ⅰ抗体阳性2例,巨细胞病毒抗体阳性3例,风疹病毒抗体阳性3例;5例IL-1β、-5、-6、-8、-17不同程度升高;单一抗神经节苷脂抗体阳性3例,2种抗神经节苷脂抗体阳性2例,6种抗神经节苷脂抗体阳性1例。5例行神经电生理检查,其中3例面神经传导波幅低平、潜伏时间延长;1例上肢运动神经传导波幅低平,传导速度减慢;1例下肢H波消失。6例患儿头颅MRI检查均未见特异性改变。6例患儿糖皮质激素治疗均有效,随访1年,GBS残疾量表评分≤2分,预后均良好,其中2例分别于病程第1、2个月后复发,再次应用糖皮质激素治疗后症状缓解;合并癫痫1例,联合应用抗癫痫药物(左乙拉西坦片+丙戊酸镁缓释片)规律口服3年余,目前癫痫无临床发作。结论变异型吉兰巴雷谱系病呈多样性、重叠性特点;抗神经节苷脂抗体阳性种类越多,其临床症状越严重;部分病例有复发,总体预后良好。

Objective To investigate the clinical characteristics and prognosis of children with anti-ganglioside antibody-positive variant Guillain-Barrésyndrome(GBS)spectrum disease.Methods The clinical data and prognosis of 6children with variant GBS spectrum disease in Henan Provincial People's Hospital from August 2022 to July 2023 were analyzed,including clinical symptoms,signs,cerebrospinal fluid biochemical examination,interleukin(IL)-1β,IL-5,IL-6,IL-8,IL-17,anti-ganglioside antibodies,neuroelectrophyiological examination,head MRI scan and treatment outcomes.Results In 6 patients,there were 3 patients with Miller-Fisher syndrome,2 with Bickerstaff's brainstem encephalitis,and 1 with facial palsy complicated with mydriasis.The clinical manifestations included visual acuity loss in4 patients,eye movement disturbance in 2,eyelid ptosis in 2,bulbar paralysis in 2,limb weakness and weakened tendon reflex in 2,hyperesthesia in 1,painful ophthalmoplegia in 1,and facial paralysis with dilated pupil in 1.Cerebrospinal fluid detected protein-cell separation in 2 patients.Mycoplasma pneumoniae antibody was positive in 1 patient,Epstein-Barr virus antibody was positive in 1,herpes simplex virus Ⅰ antibody was positive in 2,cytomegalovirus antibody was positive in 3 and rubella vires antibody was positive in 3.IL-1β,-5,-6,-8 and-17 increased in different degrees in 5 patients.Single anti-ganglioside antibody was positive in 3 patients,two anti-ganglioside antibodies were positive in 2,and six anti-ganglioside antibodies were positive in 1.Neuroelectrophysiological examination was performed in 5 patients,among whom 3 patients had flattened amplitude and prolonged latency of facial nerve conduction,1 patient had low amplitude and slowed conduction velocity of motor nerve conduction in the upper limb,and 1 had disappeared H wave in the lower limb.Head MRI showed no specific changes in 6 patients.Glucocorticoid treatment was effective.The follow-up survey was conducted for 1 year,showing GBS disability sacle score≥2,and a good prognosis.One patient relapsed in 1 month after onset and one patient relapsed in 2 months after onset,who were relieved after readministration of glucocorticoid.One patient was complicated with epilepsy,and no clinical seizure of epilepsy was found at present after oral administration of levetiracetam tablets and magnesium valproate sustained-release tablets for more than 3 years.Conclusions The variant GBS spectrum disease is diverse and overlapping.The more the types of positive anti-ganglioside antibodies,the severer the clinical symptoms.Some of the patients develop recurrences and the overall prognosis is good.