Abstract
Purpose :
Behcet's disease (BD) is an inflammatory disease characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis and skin manifestations. Neuro-ophthalmological manifestations of BD are rare, and usually associated with neurological involvement.The aim of our study is to report the spectrum of neuro-ophthalmological manifestations of BD and to evaluate the prognosis these patients.
Methods :
Medical records of patients with a diagnostic of neuro-behcet, seen in a single tertiary care center between 1987and 2015 were retrospectively reviewed. Patients with neuro-ophthalmological manifestations of the disease were included in the study. The diagnosis of neuro-behcet was based on neuro-imaging and lumbar puncture results. All patients underwent a detailed ophthalmological examination and were followed in the internal medicine and ophthalmology departments.
Results :
Out of the 217 patients diagnosed with neuro-behcet disease, 29 presented with neuro-ophthalmological manifestations.The male/female ratio was 1.2. Mean age at diagnosis was 25.3 years. Neuro-ophthalmological manifestations included papillitis in 8 patients (27.5%), optic disc swelling in 21 patients (72.4%), retrobulbar optic neuritis in 3 patients (10.3%), optic disc atrophy in 9 patients (31%) and cranial nerve palsy in 8 patients (27.5%). Among patients with cranial nerve palsy, 6 patients had a sixth nerve palsy and 2 patients had a third nerve palsy. All patients were treated with corticosteroids and immunosuppressive drugs were administered in 23 patients (79%). At initial examination, 23 patients (79%) had a decreased visual acuity, 23 patients (79%) had visual field defects. After treatment, VA was normalized or stabilized in 14 patients (60.8%), and visual field was normalized or stabilized in 18 patients (78.2%). Diplopia was improved in 5 patients (62,5%).
Conclusions :
A large spectrum of neuro-ophthalmological manifestations have been described in BD. Some of them result from increased intracranial pressure (papilledema or diplopia), while others result directly from a local inflammatory process (optic neuritis and cranial nerve involvement). Although these manifestation are rare, they are potentially severe and disabling. Recognition of these manifestations is crucial for establishing an early diagnosis, especially in cases where they appear as the first presentation of the disease. Prompt treatment is the main prognostic factor for the visual outcome.
This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.