Leger Benjamin, Gueguen Antoine, Lecler Augustin, Boudot de la Motte Marine, Lamirel Cédric, Bensa Caroline, Vignal Catherine, Marignier Romain, Papeix Caroline, Deschamps Romain
Department of Neurology, Hôpital Fondation Adolphe de Rothschild, Paris, France.
Department of Neuroradiology, Hôpital Fondation Adolphe de Rothschild, Paris, France.
Eur J Neurol. 2025 Feb;32(2):e70067. doi: 10.1111/ene.70067.
Patients diagnosed with optic neuritis (ON) who did not fulfil the diagnostic criteria for multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMO-SD), tested negative myelin oligodendrocyte glycoprotein immunoglobulin G and for which a systemic disease has been excluded are classified as having idiopathic ON (IDON).
This was a monocentric retrospective observational study. Inclusion criteria were as follows: patients with IDON, absence of an alternative diagnosis during the first 2 years, follow-up of at least 5 years.
Thirty-six patients were included. After a median follow-up of 9 years, a diagnosis of IDON was retained for 77.8% (n = 28) of patients, whereas 22.2% (n = 8) converted to an alternative diagnosis after a median of 6 years. Four patients converted to MS, two to clinically isolated syndrome and two to seronegative NMO-SD. Among the 28 patients who remained diagnosed with IDON, 42.9% (n = 12) experienced recurrent ON, occurring mostly (90%) within the first 5 years of the disease. Maintenance therapy was initiated in 10 of the 12 patients, among whom 6 patients had no recurrence under treatment. For the 28 patients who remained with IDON, the final best corrected visual acuity (BCVA) was variable. Respectively, 35.7% and 25.9% of patients had a BCVA inferior to 0.5 and 0.2, whereas 50% recovered a final BCVA of 10/10.
A significant proportion of the cohort converted to an alternative diagnosis after 2 years, encouraging an extended follow-up of IDON patients. Maintenance therapies were often effective in case of recurrent ON.
被诊断为视神经炎（ON）但不符合多发性硬化症（MS）、视神经脊髓炎谱系障碍（NMO-SD）的诊断标准，髓鞘少突胶质细胞糖蛋白免疫球蛋白G检测呈阴性且已排除全身性疾病的患者被归类为患有特发性视神经炎（IDON）。
这是一项单中心回顾性观察研究。纳入标准如下：IDON患者，在最初2年内无其他诊断，随访至少5年。
纳入36例患者。中位随访9年后，77.8%（n = 28）的患者仍被诊断为IDON，而22.2%（n = 8）的患者在中位6年后转为其他诊断。4例患者转为MS，2例转为临床孤立综合征，2例转为血清阴性NMO-SD。在仍被诊断为IDON的28例患者中，42.9%（n = 12）经历了复发性ON，大多（90%）发生在疾病的前5年内。12例患者中的10例开始了维持治疗，其中6例患者在治疗期间无复发。对于仍患有IDON的28例患者，最终最佳矫正视力（BCVA）各不相同。分别有35.7%和25.9%的患者BCVA低于0.5和0.2，而50%的患者最终BCVA恢复到10/10。
相当一部分队列患者在2年后转为其他诊断，这促使对IDON患者进行延长随访。复发性ON时维持治疗通常有效。
