背景:髓磷脂少突胶质细胞糖蛋白抗体相关疾病(MOGAD)包括各种年龄依赖性临床表型,可能是单相的,多相,或慢性。视神经炎(ON)是一种常见的表现,常与其他MOGAD表型结合出现,特别是在年幼的孩子。尽管视网膜神经纤维层(RNFL)有永久性结构损伤,儿童经常经历完全的视觉恢复。
目的:独立于ON病史分析MOGAD的进展及对患儿视觉系统的影响。
方法:这项回顾性研究包括符合特定标准的儿童:髓鞘少突胶质细胞糖蛋白(MOG)免疫球蛋白G(IgG)血清阳性,MOGAD的急性表现,和书面的一般同意。主要观察指标为整体乳头周围视网膜神经纤维层(pRNFL)厚度,以及近距离和远距离视力,使用描述性统计分析。
结果:我们确定了10例患者,首次事件的中位年龄为7.7岁:7例患者表现为急性播散性脑脊髓炎(ADEM)(ON5/7,ADEM仅1/7,横贯性脊髓炎(TM)1/7),2具有隔离ON,1例患有视神经脊髓炎谱系障碍(NMOSD)样表型的ON患者。在ON患者中,5/8受到双边影响,3例最初诊断为单侧ON,但随后累及对方眼。没有先前ON的患者均未显示视力下降,如果评估,pRNFL的减少。
结论:我们队列中大多数儿童MOGAD-ON患者表现为急性眼膜缺损和视盘水肿。所有患者视力完全恢复,独立于复发或初始视力丧失的数量。在没有进一步复发的情况下,pRNFL厚度降低了几个月,并在12个月后稳定在降低的水平。MOGAD可能对视觉系统没有亚临床/“沉默”影响,因为没有ON的患者视力和pRNFL没有受到影响。
BACKGROUND: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) comprises various age-dependent clinical phenotypes and may be monophasic, multiphasic, or chronic. Optic neuritis (ON) is a common manifestation and frequently appears in combination with other MOGAD phenotypes, particularly in young children. Despite permanent structural damage to the retinal nerve fiber layer (RNFL), children often experience complete visual recovery.
OBJECTIVE: To analyze the progression and impact of MOGAD on the visual system of pediatric patients independently of the history of ON.
METHODS: This retrospective study included children who met specific criteria: myelin oligodendrocyte glycoprotein (MOG) immunoglobulin G (IgG) seropositivity, acute presentation of MOGAD, and written general consent. Main outcome measures were global peripapillary retinal nerve fiber layer (pRNFL) thickness, and near and distance visual acuity, analyzed using descriptive statistics.
RESULTS: We identified 10 patients with median age of 7.7 years at first event: 7 patients manifested with acute disseminated encephalomyelitis (ADEM) (with ON 5/7, ADEM only 1/7, with transverse myelitis (TM) 1/7), 2 with isolated ON, and 1 patient with neuromyelitis optica spectrum disorder (NMOSD)-like phenotype with ON. Among ON patients, 5/8 were affected bilaterally, with 3 initially diagnosed with unilateral ON but experiencing subsequent involvement of the fellow eye. None of the patients without previous ON showed a deterioration of visual acuity and, if evaluated, a reduction of the pRNFL.
CONCLUSIONS: Most pediatric MOGAD-ON patients in our cohort presented with acute vison loss and optic disc edema. All patients achieved complete visual recovery, independent of number of relapses or initial visual loss. The pRNFL thickness decreased for several months and stabilized at reduced levels after 12 months in the absence of further relapses. MOGAD may not have subclinical/\'silent\' effects on the visual system, as visual acuity and pRNFL were not affected in patients without ON.