Departments of Neurology, and Ophthalmology and Visual Sciences, University of Michigan, 1000 Wall Street, Ann Arbor, MI, 48105, USA.
Departments of Neurology and Ophthalmology, Programs in Neuroscience and Immunology, University of Colorado, Denver, CO, USA.
J Neurol. 2022 Jan;269(1):111-124. doi: 10.1007/s00415-020-10352-1. Epub 2021 Jan 3.
Optic neuritis (ON) is an inflammatory optic neuropathy that is often a harbinger of central nervous system (CNS) demyelinating disorders. ON is frequently misdiagnosed in the clinical arena, leading to either inappropriate management or diagnostic delays. As a result, patients may fail to achieve optimal recovery. The treatment response to corticosteroids and long term risk of multiple sclerosis was established in the first clinical trials conducted roughly 30 years ago. Spontaneous resolution was observed in the vast majority of patients and intravenous high-dose corticosteroids hastened recovery; half of the patients eventually developed multiple sclerosis. Over the ensuing decades, the number of inflammatory conditions associated with ON has significantly expanded exposing substantial variability in the prognosis, treatment, and management of ON patients. ON subtypes can frequently be distinguished by distinct clinical, serological, and radiological profiles allowing expedited and specialized treatment. Guided by an increased understanding of the immunopathology underlying optic nerve and associated CNS injuries, novel disease management strategies are emerging to minimize vision loss, improve long-term surveillance strategies, and minimize CNS injury and disability. Knowledge regarding the clinical signs and symptoms of different ON subtypes is essential to guide acute therapy, prognosticate recovery, accurately identify underlying CNS inflammatory disorders, and facilitate study design for the next generation of clinical and translational trials.
视神经炎(ON)是一种炎症性视神经病变,常是中枢神经系统(CNS)脱髓鞘疾病的先兆。ON 在临床领域经常被误诊,导致治疗不当或诊断延误。结果,患者可能无法实现最佳康复。大约 30 年前进行的首批临床试验确定了皮质类固醇的治疗反应和多发性硬化症的长期风险。大多数患者观察到自发缓解,静脉内大剂量皮质类固醇可加速恢复;一半的患者最终发展为多发性硬化症。在随后的几十年中,与 ON 相关的炎症性疾病数量显著增加,暴露出 ON 患者的预后、治疗和管理存在很大的差异。ON 亚型通常可以通过独特的临床、血清学和影像学特征来区分,从而可以进行加速和专门的治疗。随着对视神经和相关 CNS 损伤的免疫病理学的深入了解,新的疾病管理策略正在出现,以最大限度地减少视力丧失,改善长期监测策略,并最大限度地减少 CNS 损伤和残疾。了解不同 ON 亚型的临床症状和体征对于指导急性治疗、预测恢复、准确识别潜在的 CNS 炎症性疾病以及为下一代临床和转化试验的设计提供便利至关重要。