急性播散性脑脊髓炎临床特征及复发因素分析

Clinical Features and Risk factors for Recurrence in Children with Acute Disseminated Encephalomyelitis

目的分析急性播散性脑脊髓炎(acute disseminated encephalomyelitis,ADEM)患儿临床特征及实验室资料,分析本病预后相关因素,探讨临床特征与复发相关关系,以指导临床治疗和预后评估。方法 34位ADEM患儿入组,根据临床单次发作和多次发作分为单相型组(n=27)和多相型组(n=7),分析两组临床、实验室检查、影像学资料,两组间进行比较及统计学处理,分析与多相型发作相关的危险因素。结果本病临床症状非特异性,以发热、运动障碍、头痛头晕、意识障碍居多,临床发生率分别占58.8%、55.9%、41.2%和29.4%。其它常见临床症状包括惊厥、视神经累及症状、自主神经症状等。头颅核磁共振(magnetic resonance imaging,MRI)及实验室检查结果显示双侧病灶、脑电图(electroencephalogram,EEG)异常、皮质病灶三项指标异常率最高,分别为76.5%、64.7%和58.8%。多相型组复发时5例(71.4%)有视神经累及症状。复发相关因素分析提示,发病时间(发病距皮质激素大剂量治疗时间)、自主神经症状、视觉诱发电位(visual evoked potential,VEP)异常与再发相关,比值比(odds ratio,OR)分别为10.03,20.00,31.25,与单相组比较,差异有统计学意义(P<0.05)。结论 ADEM患儿的临床特征与急性病毒性脑炎无异,以发热、运动障碍、头痛头晕、意识障碍为主。临床主要依赖头颅MRI改变,结合脑病症状建立诊断。自主神经症状、VEP异常在多相型组多见,提示可能与复发有关,是否与视神经脊髓炎抑或儿童多发性硬化相关,有待随访后下结论。

Objective To review clinical features and laboratory dates of children with acute disseminated encephalomyelitis（ADEM）,and analyze the risk factors of recurrence of ADEM.To explore the significance of clinical treatment and prognosis assessment of the disease.Methods Thirty-four cases with acute disseminated encephalomyelitis were recruited in the study,they were divided into two groups（single-phase group and multiphase group）based on their clinical manifestation.Analyzed the dinical examinalion,laboratory examination and imaging date of two growps.Statistic study was done between the two groups,and risk factors of recurrence of the disease were analyzed.Results Clinical symptoms of ADEM were not particular,fever,dyskinesia,headache,dizziness,and unconsciousness were in the majority.The incidence of these unspecific symptoms were 58.8%,55.9%,41.2%,and 29.4%,respectively.Neurologic symptoms including seizures,damage of optic nerve,and autonomic symptoms were also commonly observed.MRI and laboratory test results showed that bilateral lesions of the brain,EEG abnormal,the cortical lesions of the brain were the three most prominent indicators of the disease,the incidence of these abnormality were 76.5%,64.7%,and 58.8%,respectively.5 cases of multiphase group had had optic nerve involvement（71.4%）.Analysis of risk factors of recurrence of the disease showed that there were significant differences in delayed high-dose corticosteroids therapy,autonomic symptoms,abnormal visual evoked potential（VEP）between multiphase group and single-phase group.Odds ratios（OR）of these data were 10.03,20.00,31.25,compared with single phase group（P〈0.05）.Conclusion Clinical features of acute disseminated encephalomyelitis in children were not different from those of viral encephalitis.The diagnosis was established basically relying on brain magnetic resonance,combined with symptoms of encephalopathy.Autonomic symptoms,VEP abnormalities were frequently found in multiphase group,further study should be done to clarify the relationship between recurrence of ADEM and multiple sclerosis or neuromyelitis optica in children.