Primary optic neuropathy in Behçet's syndrome. (July 2019)

• Record Type: Journal Article
• Authors: Yang, Qinglin; Sun, Lin; Wang, Qian; Wang, Jiawei; Meng, Chao; Chang, Qinglin; Shi, Xuehui; Cui, Shilei; Liu, Lei; Lai, Chuntao
• Is Part Of: Multiple sclerosis. Volume 25:Number 8(2019)
• Page Start: 1132
• Page End: 1140
• Publication Date: 2019-07
• Subjects: Optic neuropathy -- optic neuritis -- Behçet's syndrome -- International Criteria for Behçet's Disease -- clinical feature -- magnetic resonance imaging -- Sunflower-like sign -- prognosis; Central nervous system -- Diseases -- Periodicals; Myelin sheath -- Diseases -- Periodicals; Inflammation -- Periodicals; Multiple sclerosis -- Periodicals; Central Nervous System Diseases -- Periodicals; Demyelinating Diseases -- Periodicals; Inflammation -- Periodicals; Multiple Sclerosis -- Periodicals; Système nerveux central -- Maladies -- Périodiques; Gaine de myéline -- Maladies -- Périodiques; Inflammation (Pathologie) -- Périodiques; Sclérose en plaques -- Périodiques; Electronic journals; 616.834005
• DOI: 10.1177/1352458518786058
• Languages: English
• ISSNs: 1352-4585

Abstract

Background: Primary optic neuropathy in Behçet's syndrome (PONBS) is limited to a few case reports. Objective: To investigate the clinical features, magnetic resonance imaging (MRI) changes, and visual prognosis of PONBS. Methods: Sixty-one patients who presented with first onset of optic neuritis and fulfilled the International Criteria for Behçet's Disease (ICBD) were evaluated. Results: 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–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). Conclusion: 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.