关键词: Acute disseminated encephalomyelitis Blindness Demyelination Optic neuropathy Papillitis

Mesh : Humans Optic Neuritis / immunology blood Child Female Male Myelin-Oligodendrocyte Glycoprotein / immunology Aquaporin 4 / immunology Child, Preschool Visual Acuity / physiology Prospective Studies Adolescent Autoantibodies / blood Follow-Up Studies Infant

来  源:   DOI:10.1016/j.ejpn.2024.01.003

Abstract:
OBJECTIVE: The objective of this study was to look at the clinical outcomes, and to determine the proportion of children with visual recovery after the first demyelinating event of optic neuritis (ON).
METHODS: In this observational study, children with the first clinical event of optic neuritis at an age less than 18 years were evaluated. High-contrast visual acuity, colour vision, Expanded Disability Status Scale (EDSS), Anti-MOG and AQP-4 antibodies were assessed.
RESULTS: Of the 55 screened, 45 children (77 eyes), median age-98 months, 30 (67%) bilateral were enrolled. Fifty of 77 eyes (67%) had Snellen visual acuity less than 6/60. Twelve children (27%) were MOG seropositive and 3 had AQP-4 positivity. At median follow up of 35 months, 10 (22%) children had one or more relapses. At follow up, the median (IQR) visual acuity improved from nadir of 2.1 (1-2.7) logMAR to 0 (0-0.18) logMAR and 64/77 eyes (83%) had visual recovery. The diagnosis at last follow up was isolated ON in 39/45 (86.6%), relapsing ON (5, 11%), AQP-4 positive NMOSD (3, 7%), MOG antibody associated demyelination (12, 27%), dual seronegative ON (30,67%) and Multiple sclerosis (1, 2%).
CONCLUSIONS: Most children with first demyelinating event as ON have a monophasic illness. Despite severe acute-phase visual loss, most eyes with ON will recover good visual functions. The risk of AQP-4 disease and multiple sclerosis is low in this group.
摘要:
目的:本研究的目的是观察临床结果,并确定视神经炎(ON)首次脱髓鞘事件后视力恢复的儿童比例。
方法:在这项观察性研究中,我们对年龄小于18岁时首次发生视神经炎临床事件的儿童进行了评估.高对比度视力,色觉,扩展的残疾状态量表(EDSS),评估抗MOG和AQP-4抗体。
结果:在55个筛选中,45名儿童(77眼)中位年龄-98个月,30例(67%)双边入组。77只眼中的50只(67%)具有小于6/60的Snellen视敏度。12名儿童(27%)MOG血清阳性,3名AQP-4阳性。在中位随访35个月时,10名(22%)儿童有一次或多次复发。在跟进时,中位(IQR)视力从最低点2.1(1-2.7)logMAR提高至0(0-0.18)logMAR,64/77眼(83%)视力恢复.末次随访诊断为39/45(86.6%),复发(5,11%),AQP-4阳性NMOSD(3,7%),MOG抗体相关脱髓鞘(12,27%),双血清阴性ON(30,67%)和多发性硬化症(1,2%)。
结论:大多数首次脱髓鞘事件为ON的儿童患有单相疾病。尽管严重的急性期视力丧失,大多数开的眼睛会恢复良好的视觉功能。这一组患AQP-4疾病和多发性硬化的风险较低。
公众号