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Optic neuropathy in Behçet's syndrome
1Department of Clinical Neurosciences, Royal Free Hospital, University College London, Pond Street, London, NW3 2QG, UK, d.kidd@ucl.ac.uk.
Journal of Neurology
|September 27, 2013
Summary
Behçet's syndrome can cause inflammatory optic neuropathy, affecting vision. Most cases improve with steroid treatment, with visual acuity at its lowest point predicting the final outcome.
Area of Science:
- Ophthalmology
- Neurology
- Rheumatology
Background:
- Behçet's syndrome is a multisystem inflammatory disorder.
- Optic neuropathy is a rare but serious ocular manifestation.
Purpose of the Study:
- To describe the clinical characteristics of primary optic neuropathy in Behçet's syndrome.
- To review treatment responses and outcomes in published cases.
Main Methods:
- Case series of four patients with Behçet's syndrome and optic neuropathy.
- Comprehensive literature review of all reported cases.
- Analysis of clinical features, investigations, and treatment responses.
Main Results:
- A total of 20 cases were identified, presenting as unilateral or bilateral inflammatory optic neuropathy.
- Optic disc swelling occurred in 45% of cases; pain in 20%.
- Most cases responded to steroids, with or without immunosuppression; nadir visual acuity determined outcome.
Conclusions:
- Primary optic neuropathy in Behçet's syndrome is treatable with immunosuppressive therapy.
- Early treatment is crucial for preserving visual function.
- Nadir visual acuity is a key prognostic indicator for visual recovery.
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