Jenkins Thomas M, Toosy Ahmed T
Department of Neurology, Royal Hallamshire Hospital, Sheffield, UK.
Department of Brain Repair and Rehabilitation, UCL Institute of Neurology, University College London, London, UK.
Eye Brain. 2010 Jun 17;2:83-94. doi: 10.2147/eb.s8386. eCollection 2010.
Acute optic neuritis (ON) has various etiologies. The most common presentation is inflammatory, demyelinating, idiopathic, or "typical" ON, which may be associated with multiple sclerosis. This must be differentiated from "atypical" causes of ON, which differ in their clinical presentation, natural history, management, and prognosis. Clinical "red flags" for an atypical cause of ON include absent or persistent pain, exudates and hemorrhages on fundoscopy, very severe, bilateral, or progressive visual loss, and failure to recover. In typical ON, steroids shorten the duration of the attack, but do not influence visual outcome. This is in contrast to atypical ON associated with conditions such as sarcoidosis and neuromyelitis optica, which require aggressive immunosuppression and sometimes plasma exchange. The visual prognosis of typical ON is generally good. The prognosis in atypical ON is more variable. New developments aimed at designing better treatments for patients who fail to recover are discussed, focusing on recent research elucidating mechanisms of damage and recovery in ON. Future therapeutic directions may include enhancing repair processes, such as remyelination or adaptive neuroplasticity, or alternative methods of immunomodulation. Pilot studies investigating the safety and proof-of-principle of stem cell treatment are currently underway.
急性视神经炎(ON)有多种病因。最常见的表现是炎症性、脱髓鞘性、特发性或“典型”ON,其可能与多发性硬化症相关。这必须与ON的“非典型”病因相鉴别,后者在临床表现、自然病程、治疗和预后方面有所不同。ON非典型病因的临床“警示信号”包括无疼痛或持续性疼痛、眼底镜检查发现渗出物和出血、非常严重、双侧或进行性视力丧失以及视力未恢复。在典型ON中,类固醇可缩短发作持续时间,但不影响视力预后。这与结节病和视神经脊髓炎等疾病相关的非典型ON形成对比,后者需要积极的免疫抑制治疗,有时还需要进行血浆置换。典型ON的视力预后通常良好。非典型ON的预后则更具变数。本文讨论了针对视力未恢复患者设计更好治疗方法的新进展,重点关注近期阐明ON损伤和恢复机制的研究。未来的治疗方向可能包括增强修复过程,如髓鞘再生或适应性神经可塑性,或采用其他免疫调节方法。目前正在进行研究干细胞治疗安全性和原理验证的试点研究。