%0 Journal Article
%T Primary optic neuropathy in Beh£¿et¡¯s syndrome
%A Chao Meng
%A Chuntao Lai
%A Jiawei Wang
%A Lei Liu
%A Lin Sun
%A Qian Wang
%A Qinglin Chang
%A Qinglin Yang
%A Shilei Cui
%A Xuehui Shi
%J Multiple Sclerosis Journal
%@ 1477-0970
%D 2019
%R 10.1177/1352458518786058
%X Primary optic neuropathy in Beh£¿et¡¯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 Beh£¿et¡¯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%; p£¿=£¿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¨CMeier 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¨C0.54). 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
%K Optic neuropathy
%K optic neuritis
%K Beh£¿et¡¯s syndrome
%K International Criteria for Beh£¿et¡¯s Disease
%K clinical feature
%K magnetic resonance imaging
%K Sunflower-like sign
%K prognosis
%U https://journals.sagepub.com/doi/full/10.1177/1352458518786058