Harvard Mass General Brigham Neurology Residency.
Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA.
Curr Opin Ophthalmol. 2020 Nov;31(6):462-468. doi: 10.1097/ICU.0000000000000703.
Neuromyelitis optica spectrum disorder is an autoimmune disease that causes optic neuritis and transverse myelitis. Attacks can cause severe neurological damage leading to blindness and paralysis. Understanding of the immunopathogenesis of this disease has led to major breakthroughs in diagnosis and treatment. In the past 18 months, three successful phase 3 clinical trials have been published using targeted approaches to preventing relapses.
Updates in epidemiology, imaging, quality of life and treatment for acute relapse and prevention have been published in the past 18 months. Epidemiology studies are distinguishing patients based on their antigen specificity for aquaporin-4 and myelin oligodendrocyte glycoprotein, which are increasingly recognized as separate immunological conditions. Imaging by MRI and optical coherence tomography continue to be developed as tools to distinguish neuromyelitis optica spectrum disorders (NMOSD) from other diseases. This is especially relevant as the recent clinical trials showed differences in response between aquaporin-4 seropositive and seronegative patients. The three drugs that were tested for prevention of NMOSD relapses were eculizumab, inebilizumab, and satralizumab. All of the trials were worldwide, placebo-controlled, double-masked studies that demonstrated a clear benefit with each approach.
Recent research in NMOSD has resulted in improved diagnosis and approved treatments.
视神经脊髓炎谱系疾病是一种自身免疫性疾病，可导致视神经炎和横贯性脊髓炎。发作可导致严重的神经损伤，导致失明和瘫痪。对这种疾病的免疫发病机制的理解导致了在诊断和治疗方面的重大突破。在过去的 18 个月中，已经发表了三项使用靶向方法预防复发的成功的 3 期临床试验。
在过去的 18 个月中，发表了关于急性复发和预防的流行病学、影像学、生活质量和治疗的更新。流行病学研究根据水通道蛋白 4 和髓鞘少突胶质细胞糖蛋白的抗原特异性对患者进行区分，这两种蛋白越来越被认为是两种不同的免疫状态。MRI 和光学相干断层扫描的影像学检查继续作为区分视神经脊髓炎谱系疾病（NMOSD）和其他疾病的工具得到发展。这一点尤为重要，因为最近的临床试验表明，在对水通道蛋白 4 阳性和阴性患者的反应方面存在差异。用于预防 NMOSD 复发的三种药物是依库珠单抗、伊奈利珠单抗和萨替鲁单抗。所有试验均为全球性、安慰剂对照、双盲研究，均表明每种方法均有明显获益。
NMOSD 的最新研究导致了改进的诊断和已批准的治疗方法。
