Moon Yeji, Park Kyung-Ah, Han Jinu, Hwang Jeong-Min, Kim Seong-Joon, Han Sueng-Han, Lee Byung Joo, Kang Min Chae, Goh Yong Hyu, Lim Byung Chan, Yang Hee Kyung, Jung Jae Ho
Department of Ophthalmology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea.
Department of Ophthalmology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.
Neurol Sci. 2024 Mar;45(3):1173-1183. doi: 10.1007/s10072-023-07125-9. Epub 2023 Oct 19.
To investigate the rate of development of symptomatic central nervous system (CNS) demyelinating attacks or recurrent optic neuritis (ON) after the first episode of ON and its risk factors for Korean pediatric patients.
This multicenter retrospective cohort study included the patients under 18 years of age (n=132) diagnosed with ON without previous or simultaneous CNS demyelinating diseases. We obtained the clinical data including the results of neuro-ophthalmological examinations, magnetic resonance images (MRIs), antibody assays, and laboratory tests. We investigated the chronological course of demyelinating disease with respect to the occurrence of neurological symptoms and/or signs, and calculated the 5-year cumulative probability of CNS demyelinating disease or ON recurrence. RESULTS: During the follow-up period (63.1±46.7 months), 18 patients had experienced other CNS demyelinating attacks, and the 5-year cumulative probability was 14.0±3.6%. Involvement of the extraorbital optic nerve or optic chiasm and asymptomatic lesions on the brain or spinal MRI at initial presentation were significant predictors for CNS demyelinating attack after the first ON. The 5-year cumulative probability of CNS demyelinating attack was 44.4 ± 24.8% in the AQP4-IgG group, 26.2±11.4% in the MOG-IgG group, and 8.7±5.9% in the double-negative group (P=0.416). Thirty-two patients had experienced a recurrence of ON, and the 5-year cumulative probability was 24.6±4.0%. In the AQP4-IgG group, the 5-year cumulative probability was 83.3±15.2%, which was significantly higher than in the other groups (P<0.001).
A careful and multidisciplinary approach including brain/spinal imaging and antibody assay can help predict further demyelinating attacks in pediatric ON patients.
探讨韩国儿科患者首次发作视神经炎（ON）后出现有症状的中枢神经系统（CNS）脱髓鞘发作或复发性视神经炎的发生率及其危险因素。
这项多中心回顾性队列研究纳入了18岁以下被诊断为ON且既往无或同时无CNS脱髓鞘疾病的患者（n = 132）。我们获取了包括神经眼科检查结果、磁共振成像（MRI）、抗体检测和实验室检查结果在内的临床数据。我们根据神经症状和/或体征的出现情况研究了脱髓鞘疾病的时间进程，并计算了CNS脱髓鞘疾病或ON复发的5年累积概率。结果：在随访期（63.1±46.7个月）内，18例患者经历了其他CNS脱髓鞘发作，5年累积概率为14.0±3.6%。首次发作时眶外视神经或视交叉受累以及脑或脊髓MRI上的无症状病变是首次ON发作后CNS脱髓鞘发作的重要预测因素。水通道蛋白4-IgG（AQP4-IgG）组CNS脱髓鞘发作的5年累积概率为44.4±24.8%，髓鞘少突胶质细胞糖蛋白-IgG（MOG-IgG）组为26.2±11.4%，双阴性组为8.7±5.9%（P = 0.416）。32例患者经历了ON复发，5年累积概率为24.6±4.0%。在AQP4-IgG组中，5年累积概率为83.3±15.2%，显著高于其他组（P<0.001）。
包括脑/脊髓成像和抗体检测在内的仔细的多学科方法有助于预测儿科ON患者的进一步脱髓鞘发作。
