摘要
目的探讨儿童急性播散性脑脊髓炎(ADEM)临床特点和预后。方法回顾性分析66例ADEM患儿的临床资料。应用Kurtzke扩展残疾状况量表(EDSS)评估入院时神经功能损伤情况,应用mRS评估最后一次随访时神经功能恢复情况,并分组比较其临床资料。结果66例ADEM患儿中42例(63.6%)有前驱感染史,5例(7.6%)发病于疫苗接种后。所有患儿均有脑病表现,其中有意识障碍50例(75.8%)、认知障碍9例(13.6%)、精神症状6例(9.1%)、认知障碍合并精神症状1例(1.5%)。其他临床症状包括发热46例(69.7%),锥体束征33例(50.0%),惊厥32例(48.5%),头痛27例(40.1%),共济失调26例(39.4%),言语障碍14例(21.2%),颅神经受累11例(16.7%)(面神经受累7例,展神经受累1例,视神经受累2例,面神经合并视神经1例),锥体外系症状9例(13.6%),感觉异常7例(10.6%)。MRI病灶以皮质下白质(84.8%)、深部核团(36.4%)、脑干(16.7%)及小脑(15.2%)多见,脊髓受累(7.6%)较少。不同神经功能损伤组临床资料差异无统计学意义(均P>0.05)。56例给予大剂量甲泼尼龙治疗,10例给予地塞米松治疗;两种糖皮质激素治疗方案患者最后一次随访mRS评分差异无统计学意义(t=1.47,P=0.15)。康复组、好转组及恢复不佳组年龄(F=4.47,P=0.02)、入院EDSS评分(F=38.62,P<0.01)及惊厥(χ^2=9.64,P=0.01)、言语障碍(χ^2=17.64,P<0.01)及认知障碍(χ^2=24.15,P<0.01)比率差异有统计学意义。结论儿童ADEM多有前驱感染史或疫苗接种史。临床表现多样,以脑病最为常见。MRI对诊断具有重要意义。多数患儿经糖皮质激素治疗后预后良好,预后可能与发病年龄、入院时EDSS、发病时有无皮质受累有关。
Objective To investigate the clinical features and prognosis of acute disseminated encephalomyelitis(ADEM)in children.Methods The clinical data of 66 ADEM children were retrospectively.The Kurtzke extended disability status scale(EDSS)was used to evaluate the neurological impairment at admission,and the mRS was used to evaluate the neurological recovery at the last follow-up.The clinical data were grouped and compared.Results Among the 66 ADEM children,42 cases(63.6%)had prodromal infection,and 5 cases(7.6%)onset time was after vaccination.All the children presented with encephalopathy,including 50 cases of conscious impairment(75.8%),9 cases of cognitive impairment(13.6%),6 cases of mental symptoms(9.1%),and 1 case of cognitive impairment combined with psychotic symptoms(1.5%).Other clinical symptoms include fever(46 cases,69.7%),pyramidal tract damage(33 cases,50.0%),convulsion(32 cases,48.5%),headache(27 cases,40.1%),ataxia(26 cases,39.4%),speech disorders(14 cases,21.2%),cranial nerve damage(11 cases,16.7%)(facial nerve involvement in 7 cases,nerve involvement in 1 case,optic nerve involvement in 2 cases,facial nerve combined optic 1 case),extrapyramidal symptoms(9 cases,13.6%),abnormal sensation(7 cases,10.6%).MRI lesions were mainly found in subcortical white matter(84.8%),deep nucleus mass(36.4%),brainstem(16.7%)and cerebellum(15.2%),while spinal cord was less involved(7.6%).There was no statistically significant difference in clinical data between different groups with neurological impairment(all P>0.05).Fifty-six patients were treated with high-dose methylprednisolone and 10 with dexamethasone.At the last follow-up,there was no statistically significant difference in mRS scores(t=1.47,P=0.15).There were significant differences in age(F=4.47,P=0.02),EDSS score(F=38.62,P<0.01),and the rates of comorption with convulsion(χ^2=9.64,P=0.01),speech impairment(χ^2=17.64,P<0.01),cognitive impairment(χ^2=24.15,P<0.01)in the rehabilitation group,improvement group and poor recovery group.Conclusions ADEM in children usually have a history of prodrome infection or vaccination,ADEM have various clinical manifestations and encephalopathy is the most common.MRI is very important for diagnosis.Most children with ADEM have a good prognosis after hormone therapy,and the prognosis may be related to the onset age,the EDSS at admission,and the present or absence of cortical involvement at the time of onset.
作者
曾向东
胡文广
刘平
邓佳
徐洋
ZENG Xiang-dong;HU Wen-guang;LIU Ping(Department of Pediatric Neurology,Chengdu Women's&Children's Central Hospital,Chengdu 610091,China)
出处
《临床神经病学杂志》
CAS
2020年第1期35-39,共5页
Journal of Clinical Neurology
关键词
急性播散性脑脊髓炎
临床特点
预后
acute disseminated encephalomyelitis
clinical features
prognosis
