Queen Square MS Centre, UCL Queen Square Institute of Neurology, Faculty of Brain Sciences, University College London, London, UK.
Department of Ophthalmology, Tel Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Mult Scler. 2022 Apr;28(4):676-679. doi: 10.1177/13524585221074947.
Aquaporin-4 antibody (AQP4-Ab) Neuromyelitis Optica Spectrum Disorder (NMOSD) is a rare neuroinflammatory syndrome presenting predominantly with optic neuritis and transverse myelitis. We report a case of radiologically isolated longitudinally extensive optic neuritis in an asymptomatic 12-year-old female with positive serum AQP4-Ab, with resolution of imaging changes after immune therapy. By contrast to patients with radiologically isolated syndrome, of which some will never convert to multiple sclerosis, the pathogenicity of AQP4-Ab in the context of sub-clinical disease, supported treatment in our patient. Given the severe morbidity in AQP4-Ab NMOSD, prognostic biomarkers for disease severity are required to guide optimal therapy for patients.
水通道蛋白 4 抗体(AQP4-Ab)相关视神经脊髓炎谱系疾病(NMOSD)是一种罕见的神经炎症综合征,主要表现为视神经炎和横贯性脊髓炎。我们报告了一例 12 岁无症状女性患者的影像学孤立性长节段视神经炎,其血清 AQP4-Ab 阳性,经免疫治疗后影像学改变得到缓解。与影像学孤立综合征患者不同,其中一些患者永远不会发展为多发性硬化症,在亚临床疾病的背景下,AQP4-Ab 的致病性支持了对我们患者的治疗。鉴于 AQP4-Ab NMOSD 的严重发病率,需要疾病严重程度的预后生物标志物来指导患者的最佳治疗。