儿童急性播散性脑脊髓炎与临床孤立综合征的临床及影像学比较研究

目的探讨急性播散性脑脊髓炎(acute disseminated encephalomyelitis,ADEM)与临床孤立综合征(clini-cally isolated syndrome,CIS)的鉴别诊断要点。资料与方法搜集39例患儿首发中枢神经系统脱髓鞘病变时的临床及影像学资料。其中CIS 18例,包括女13例,男5例;ADEM共21例,其中女8例,男13例。平均随访时间4.2年,最短随访时间为2年。由一名儿科神经医师对CIS及ADEM的临床表现进行了归类总结。由一名资深神经影像学医师对患者ADEM及CIS头颅MRI表现进行分析,内容包括病灶的位置、大小、形态。对计数资料采用Fisher精确检验,对计量资料进行非参数Mann-Whitney U检验。分别应用几种CIS的诊断标准对患儿进行评价,比较分析每种标准诊断的特异性、敏感性、阳性预测值及阴性预测值。结果 39例儿童ADEM与CIS患者中,ADEM发病年龄小,平均约(6.14±3.41)岁;CIS平均发病年龄(10.05±2.87)岁,两者之间差异有统计学意义(P=0.000);ADEM临床表现以发热和运动障碍为主,分别占76%和71%,高于CIS的33%和22%(P=0.011,0.004)。而CIS中视力障碍及脑脊液寡克隆带阳性出现率分别占50%和61%,与ADEM的0%和24%比较,差异具有统计学意义(P=0.000,0.025)。随访中儿童ADEM的改良的Rankin量表评分不如CIS。影像学特征中,与CIS比较,中央白质病变及脑萎缩在ADEM中表现更为明显,分别占100%和38%(P=0.001,0.023);ADEM病灶分布相对对称。CIS的视神经及脊髓病变出现率分别为22%、17%,ADEM未出现此类病变(P=0.037,P=0.089)。"黑洞"在CIS中具有显著特异性,与ADEM比较差异具有统计学意义(P=0.037)。Callen诊断MS的标准敏感性最高,为87%。KIDMUS(2个均满足)MS诊断标准及Callen的鉴别MS与ADEM标准特异性均较高,分别为92%和90%。KID-MUS敏感性最差,为33%。Barkhof的成人MS标准在儿童中敏感性及特异性均较低,分别为22%和57%。结论临床及影像学特征的结合,可以帮助早期诊断与鉴别ADEM与CIS。在诊断CIS时应该用Callen诊断MS标准为主,而鉴别ADEM和CIS时可以用Callen的鉴别标准。

The Comparative Study of Clinical and MRI Features in Pediatric ADEM and CIS

Objective To investigate the differential diagnostic features of acute disseminated encephalomyelitis and clinically isolated syndrome in children.Materials and Methods This study included 39 children who had a first demyelinating event since 1998.Their clinical and MRI data were collected and reviewed.The patients were followed up to the mean of 4.2 years.Eighteen patients had a definitive diagnosis of MS,of them,13 patients were female and 5 were male.Twenty one patients had ADEM and remained relapse free after at least 2 years of follow up,of them,8 patients were female and 13 were male.The clinical manifestations of CIS and ADEM were analyzed by a pediatric neurologist.An experienced neuroradiologist reviewed the MRI images of CIS and ADEM.Information on the location,size and pattern of the lesions were gathered.Location of the lesion included subcortical,central,and periventricular white matter,cortex,deep gray matter,brain stem,and cerebellum.The data were statistically analyzed by using Fisher’s test or Mann Whitney test.Several diagnostic criteria were tested for the sensitivity and specificity.Results In 39 cases,ADEM occurred more frequently in children less than 10 years,and males were predominant,which were significantly different from CIS.Movement disorders and Fever had a higher incidence in ADEM(P=0.011,0.004).However,visual impairment and presence of oligoclonal CSF bands were more often observed in children who developed MS(P=0.000,0.025).Modified Rankin Scale of ADEM was lower than that of CIS according to the results of follow up.As to MRI features,the incidence of central white matte lesion and brain atrophy was significantly higher in ADEM than that in CIS(P=0.001,0.023).Compared with CIS,ADEM lesions were more symmetric.In CIS,optic neuritis and myelitis had a high incidence(22%,17%).Black holes were more characteristic of CIS(P=0.037).The Callen diagnostic MS criteria were the most sensitive(87%) for the diagnosis of CIS.The KIDMUS criteria and Callen MS ADEM criteria had higher specificity(92 % and 90%).The KIDMUS criteria showed relatively lower sensitivity(33%).The Barkhof criteria had a lower sensitivity of 22% and a lower specificity of 57% in the diagnosis of pediatric CIS.Conclusion Some clinical and imaging features may be helpful in the diagnosis of CIS and distinguishing ADEM from CIS.Callen diagnostic MS criteria is suitable for the diagnosis of CIS,while Callen MS ADEM criteria show advantage on the differentiation of ADEM and MS.