Related Experiment Videos
Primary optic neuropathy in Behçet's syndrome.
Qinglin Yang1, Lin Sun1, Qian Wang1
1Department of Neurology, Beijing Tongren Hospital, Capital Medical University, Beijing, China.
Multiple Sclerosis (Houndmills, Basingstoke, England)
|July 10, 2018
Summary
Primary optic neuropathy in Behçet's syndrome (PONBS) often affects females and may present with a unique MRI "sunflower-like" sign. Males face a significantly higher risk of severe visual loss from this condition.
Area of Science:
- Ophthalmology
- Neurology
- Rheumatology
Background:
- Primary optic neuropathy in Behçet's syndrome (PONBS) is rare, with limited case reports.
- Behçet's syndrome is a multisystem inflammatory disease.
Purpose of the Study:
- To characterize the clinical presentation, MRI findings, and visual outcomes of PONBS.
- To identify risk factors for visual loss in PONBS.
Main Methods:
- Retrospective evaluation of 61 patients with first-onset optic neuritis meeting International Criteria for Behçet's Disease (ICBD).
- Analysis of clinical features, MRI findings (optic nerve signal and perineural enhancement), and visual prognosis.
- Kaplan-Meier survival analysis to estimate visual loss risk.
Main Results:
- Female predominance (1.7:1 ratio) and isolated optic neuropathy were common.
- Perineural enhancement around the orbital optic nerve (68.7%) and a sunflower-like MRI sign (71.7%) were frequent findings.
- Males had a significantly higher cumulative risk of severe visual loss (62.5% vs. 14.7% in females at 24 months).
Conclusions:
- PONBS often presents as isolated optic neuropathy, particularly in females, with a potential distinctive MRI sunflower sign.
- Short-term recurrence of PONBS is uncommon.
- Males are at a substantially higher risk for severe visual impairment in PONBS.