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目的通过对儿童多发性硬化(MS)系统随访,总结其躯体、心理预后及视力特点。方法回顾性收集首都医科大学附属北京儿童医院近10年来经McDonald诊断标准评估确诊的MS病历,对病程3年以上的MS病例进行系统的随访,随访内容包括日常生活活动能力评估(ADL),扩展的功能障碍量表评分(EDSS),神经心理评估(抑郁、焦虑、注意力、社会生活能力)和智力检测,眼科检查[最佳矫正视力、光学相干断层扫描(OCT)测量视神经纤维层(RNFL)厚度、图形视觉诱发电位(P-VEP)]等,对预后进行综合分析。结果 23例MS患儿进入分析。男10例,女13例,平均起病年龄7.7岁;复发缓解型20例,进展复发型2例,继发进展型1例。23例均复发,次数1～10次。平均随访7.7(3.6～14)年,均行ADL评分,完全依赖和中度依赖各1例,均为进展复发型;16例行EDSS评分,EDSS 0分11例,EDSS≤2.5分4例,EDSS 4.0分1例(为进展复发型);15例完成神经心理检查,韦氏智力测验2例低于平常,2例边界,1例社会适应能力临界,6例存在注意力缺陷,4例存在轻度焦虑情绪,均无抑郁情绪。16例患儿32眼完善眼科检查,30眼矫正视敏度0.5～1.5,视野无异常,余2眼仅存光感;合并视神经炎MS患儿眼底均提示视神经萎缩,OCT检查结果显示视神经眼在各个象限的RNFL厚度与无视神经炎眼相比明显变薄(P<0.001);P-VEP检查中视神经炎眼较无视神经炎眼P100潜伏期明显延长,振幅明显减低(P<0.05)结论儿童MS复发缓解型预后尚好,多合并视神经萎缩,但无视力严重受损;其他类型预后差,多遗留躯体残疾及视力残疾,OCT检查可对视神经受累程度定量测定。儿童MS可出现认知损害、焦虑和注意力缺陷等多种神经心理问题。
Objective To summarize the somatic, psychological prognosis and visual characteristics of children with multiple sclerosis (MS) through systematic follow-up. Methods We retrospectively collected the MS records of Beijing Children’s Hospital Affiliated to Capital Medical University who had been diagnosed by McDonald’s diagnostic criteria in the past 10 years and systematically followed up the MS cases with more than 3 years of disease duration. The follow-up activities included assessment of daily living activity (ADL) (EDSS), neuropsychological assessment (depression, anxiety, attention, social viability) and intelligence testing, ophthalmic examination [best corrected visual acuity, optical coherence tomography (OCT) measurement of optic nerve fiber layer ) Thickness, graphic visual evoked potential (P-VEP)], etc., the prognosis of a comprehensive analysis. Results 23 cases of MS children into the analysis. 10 males and 13 females, with an average age of onset of 7.7 years; 20 cases of relapsing remission, 2 cases of recurrent relapse and 1 case of secondary progress. 23 cases were recurrence, the number of 1 to 10 times. The patients were followed up for an average of 7.7 (3.6-14) years with ADL scores. One patient was completely dependent and moderately dependent, both of whom had progressive recurrent disease. EDSS score was 16 in 16 patients, EDSS was 0 in 11 patients, EDSS was 2.5 in 4 patients, EDSS 4.0 points in 1 cases (for the progress of relapse); 15 cases to complete neuropsychological tests, Weber’s Intelligence Test in 2 cases lower than usual, 2 cases of border, 1 case of social adaptability, 6 cases of attention deficit, 4 cases Mild anxiety, no depression. 32 eyes of 16 children underwent ophthalmologic examinations, 30 eyes corrected visual acuity of 0.5 to 1.5, no visual field abnormality, and only 2 eyes remained light perception. Optic nerve atrophy was observed in the fundus of children with MS, OCT showed that optic nerve eyes The thickness of RNFL in each quadrant was significantly thinner than that of non-optic neuritis (P <0.001). P-VEP showed significantly longer P100 latency and amplitude than that of non-optic neuritis (P <0.05). Conclusions Children MS recurrence and remission of the prognosis is good, combined with optic atrophy, but no serious impairment of visual acuity; other types of poor prognosis, multiple residual physical disability and visual impairment, OCT can quantitatively determine the degree of involvement of the optic nerve. Children with MS can appear cognitive impairment, anxiety and attention deficit and other neuropsychological problems.