Department of Neurology, Beijing Tongren Hospital, Capital Medical University, Beijing, China.
Department of Radiology, Beijing Tongren Hospital, Capital Medical University, Beijing, China.
Mult Scler. 2019 Jul;25(8):1132-1140. doi: 10.1177/1352458518786058. Epub 2018 Jul 9.
Primary optic neuropathy in Behçet's syndrome (PONBS) is limited to a few case reports.
To investigate the clinical features, magnetic resonance imaging (MRI) changes, and visual prognosis of PONBS.
Sixty-one patients who presented with first onset of optic neuritis and fulfilled the International Criteria for Behçet's Disease (ICBD) were evaluated.
The female-to-male ratio was 1.7:1. No patient had other central nervous system (CNS) disease. In 67 eyes with optic nerve abnormalities on MRI scan, perineural enhancement around the orbital optic nerve (46 eyes, 68.7%) was significantly more frequent than was increased signal in the optic nerve itself (31 eyes, 46.3%; = 0.000), typically with sunflower-like appearance on coronal view (33 eyes, 71.7%). Two patients (3.6%) relapsed during follow-up (median 12 months). Kaplan-Meier survival analysis estimated the cumulative risk of severe visual loss (⩽0.1) at 24 months was 14.7% in females versus 62.5% in males (hazard ratio (HR), 0.16; 95% confidence interval, 0.05-0.54).
PONBS frequently presents with isolated optic neuropathy in females. The sunflower-like sign might be a distinctive MRI feature. Short-term recurrence is very rare. Males have a higher cumulative risk of severe visual loss.
Behçet 综合征（BS）中的原发性视神经病变（PONBS）仅限于少数病例报告。
探讨 PONBS 的临床特征、磁共振成像（MRI）改变和视力预后。
对 61 例首次出现视神经炎且符合 Behçet 病国际诊断标准（ICBD）的患者进行评估。
男女比例为 1.7:1。无患者有其他中枢神经系统（CNS）疾病。在 67 例 MRI 扫描视神经异常的眼中，眶内视神经周围神经鞘强化（46 眼，68.7%）明显比视神经本身信号增强（31 眼，46.3%）更常见（=0.000），冠状位典型表现呈向日葵样外观（33 眼，71.7%）。2 例（3.6%）在随访中复发（中位时间 12 个月）。Kaplan-Meier 生存分析估计女性 24 个月严重视力丧失（⩽0.1）的累积风险为 14.7%，而男性为 62.5%（风险比（HR），0.16；95%置信区间，0.05-0.54）。
PONBS 常以女性孤立性视神经病变为首发表现。向日葵样征可能是其独特的 MRI 特征。短期复发非常罕见。男性严重视力丧失的累积风险较高。
