儿童多发性硬化生活能力、神经心理和眼科预后长期随访观察

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。

The follow-up study on the prognosis of the physical,psychological and visual features of pediatric multiple sclerosis

ObjectiveTo analyze the prognosis of pediatric multiple sclerosis (MS) by summarizing their physical, visual and psychological features. Methods MS patients diagnosed by McDonald over 3 years in Beijing Children′s Hospital, Capital Medical University were retrospectively recruited and systematically followed up. Follow-up examinations included physical examination, activities of daily living assessment, the Kurtzke Expanded Disability Status Scale (EDSS), neuropsychological examination, and systematic eye examination, including corrected visual acuity , optical coherence tomography (OCT) to measure the retinal nerve fiber layer (RNFL) thickness , pattern visual evoked potential (P-VEP) , etc. ResultsA total of 23 cases of pediatric MS were included, 10 males and 13 females. Age of onset ranged from 2.3 to 13.8 years (average 7.7 years). 20 out of 23 cases were RRMS, 2 cases were PRMS, and 1case was SPMS. Twenty-three cases all had recurrent relapses for 1 to 10 times. The follow-up duration ranged from 3.6 to 14 years (average 7.7 years). In ADL assessment, one case was moderate dependent and another case was entirely dependent, both of them were PRMS. Sixteen cases completed EDSS score assessment, 11 of which were 0, 4 were lower than 2.5, and one PRMS was 4.0. Fifteen cases completed neuropsychological examination, two cases′ Wechsler intelligence tests were below the average level and other 2 at borderline, one case′s social adaptation ability was at borderline, 6 cases had attention deficit, 4 cases had mild anxiety that all aged from 8 to 13 years. Thirty-two eyes of 16 cases completed eye examination. The visual acuity of 30 eyes ranged from 0.5 to 1.5, and the fields of view were all normal, while the other 2 cases only had light sensation. The RNFL thickness of optic neuritis group was thinned in each quadrant than non-optic neuritis group in OCT, the difference was statistically significant (P <0.001). The P100 amplitude and P100 latency differences between the two groups were statistically significant (P <0.05). ConclusionThe prognosis of relapsing-remitting multiple sclerosis is better than other types. Most of RRMS present optic atrophy without visual disability, while other types often present legacy of physical and visual disability. OCT examination can be used to quantitatively determine the extent of the optic nerve involvement. Pediatric multiple sclerosis can present a variety of neuropsychological problems including cognitive impairment, anxiety, attention deficit and etc.