成年发病的急性播散性脑脊髓炎并吉兰-巴雷综合征6例分析  

Co-existence of acute disseminated encephalomyelitis and GBS in adults:six cases report and analysis

作　　者：陈林林 韩晓琛 安利[1] 白文浩 王晓艳 姚生 CHEN Linlin;HAN Xiaochen;AN Li;BAI Wenhao;WANG Xiaoyan;YAO Sheng(不详;Department of Neurology,Sixth Medical Center of Chinese PLA General Hospital,Beijing 100048,China)

机构地区：[1]解放军总医院第六医学中心神经内科,100048

出　　处：《中国神经免疫学和神经病学杂志》2021年第2期110-114,119,共6页Chinese Journal of Neuroimmunology and Neurology

摘　　要：目的探讨成年发病的急性播散性脑脊髓炎(acute disseminated encephalomyelitis,ADEM)并吉兰-巴雷综合征(Guillain-Barrésyndrome,GBS)的临床表现、脑脊液、神经电生理、影像学特点及预后。方法收集6例患者的临床资料,对其临床症候、脑脊液结果、神经电生理、头和脊髓影像改变特点及预后进行回顾性分析。结果(1)6例患者中ADEM并GBS 4例,急性播散性脊髓炎(acute disseminated myelitis,ADM)并GBS 2例;脑累及4例、脊髓累及6例、周围神经累及6例(脑神经4例,脊神经6例)。(2)发病年龄40~67岁;急性起病2例,亚急性起病4例;前驱感染史4例,发热1例,无明确前驱史1例。首发症状分别为头晕(2例)、复视(1例)、双下肢无力(2例)及腹痛(1例)。后期主要表现为肢体无力和尿便障碍(6例)、四肢深反射减弱及消失(2例)、双下肢跟、膝腱反射减弱及消失(4例)及锥体束损害(6例)。(3)脑脊液:颅内压增高1例(230 mmH 2O),余5例正常;蛋白均不同程度增高(470~1310 mg/L);脑脊液有核细胞数增高5例〔(10~130)×106/L〕,红细胞增高4例〔(8~220)×106/L〕;髓鞘碱性蛋白(MBP)增高4例,24 h鞘内IgG合成率增高5例;脑脊液寡克隆区带、抗神经节苷脂(GM)抗体谱及脑脊液病毒筛查均阴性。(4)肌电图:双侧上肢运动、感觉神经传导速度减慢3例,波幅降低2例;双侧下肢运动、感觉神经传导速度减慢6例,波幅降低5例;F波检查5例(F波出现率降低4例,潜伏期延长5例);视觉诱发电位异常2例。(5)头MRI发现病灶4例(多发小病灶3例,单发小病灶1例),脊髓MRI均发现病灶(多发局灶性4例、节段性2例)。(6)治疗及预后:静脉注射免疫球蛋白(IVIg)治疗1例,糖皮质激素治疗1例,二者联合治疗4例,6例均预后良好。结论本组患者ADEM并GBS可累及脑、脊髓、神经根和周围神经,临床表型变异性明显;肢体无力及尿便障碍多见,深反射减低或消失及锥体束损害是主要的体征。脑脊液未�Objective To investigate the clinical,electrophysiological,cerebrospinal fluid(CSF),imaging features and outcomes of co-existence of acute disseminated encephalomyelitis(ADEM)and Guillain-BarréSyndrome(GBS)in adults.Methods We diagnosed and retrospectively analyzed 6 adults with ADEM and GBS.We collected detailed clinical data,cerebrospinal fluid(CSF),head and spinal cord images,and peripheral electrophysiological studies.Results We identified 4 adults with ADEM and GBS,and 2 with acute disseminated myelitis(ADM)and GBS.All the patients had central never system(CNS)and peripheral nervous system(PNS)involvements,including cerebral(n=4),spinal cord(n=6),cranial nerve(n=4)and spinal nerve(n=6).The age of onset ranged from 40 to 67 years old.There were 3 males and 3 females.The onset manifestations were consistent with acute onset(n=2)and subacute onset(n=4);4 patients with preceding infection,one patient with fever,and one patient with uncertain pre-infection.Onset symptoms were dizziness(n=2),diplopia(n=1),weakness of lower limbs(n=2)and abdominal pain(n=1).Muscle weakness(n=6),urination and defecation dysfunction(n=6),pyramidal tract damage(n=6)and absent tendon reflexes(n=2)were seen in the later stage.CSF tests showed elevated intracranial pressure(up to 230 mmH 2 O)in 1/6 and normal in 5/6,increased CSF protein(470 mg/L-1310 mg/L)in 6/6,increased nucleated cells[(10-130)×106/L]in 4/6,increased erythrocyte[(8-220)×106/L]in 4/6,increased myelin basic protein(MBP)in 4/6,increased indices of 24 hours intrathecal immunoglobulin synthesis in 5/6,negative oligoclonal bands,GM antibodies and cerebrospinal fluid virus screening in 6/6.Electrophysiology tests showed decreased motor and sensory nerve conduction velocity[bilateral upper limb(n=3),and bilateral lower limb(n=6)],decreased amplitude[bilateral upper limb(n=2),and bilateral lower limb(n=5)],prolonged F wave latency in 5/5 and abnormal F wave rate in 4/5.Visual evoked potential(VEP)examinations was abnormal in 2/6.Head MRI was abnormal in 4 patients[multiple

关 键 词：ADEM GBS 临床特点 磁共振波谱学 电生理 预后 

分 类 号：R744.53[医药卫生—神经病学与精神病学]