Primary optic neuropathy in Behçet’s syndrome

Published on 2018-07-09T12:00:00Z (GMT) by
<div>Background:<p>Primary optic neuropathy in Behçet’s syndrome (PONBS) is limited to a few case reports.</p>Objective:<p>To investigate the clinical features, magnetic resonance imaging (MRI) changes, and visual prognosis of PONBS.</p>Methods:<p>Sixty-one patients who presented with first onset of optic neuritis and fulfilled the International Criteria for Behçet’s Disease (ICBD) were evaluated.</p>Results:<p>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%; <i>p</i> = 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).</p>Conclusion:<p>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.</p></div>

Cite this collection

Yang, Qinglin; Sun, Lin; Wang, Qian; Wang, Jiawei; Meng, Chao; Chang, Qinglin; Shi, Xuehui; Cui, Shilei; Liu, Lei; Lai, Chuntao (2018): Primary optic neuropathy in Behçet’s syndrome. SAGE Journals. Collection.