Abstract
Purpose Behcet's disease (BD) is an inflammatory disease characterized by recurrent oral aphthous ulcers (major criteria), genital ulcers, uveitis and skin manifestations (minor criteria). Neuro-ophthalmological manifestations (NOM) are rare. The aim of our study is to report the spectrum of NOM of BD, and evaluate their prognosis. Methods Medical records of patients with neuro-Behcet, seen in a single tertiary center between 1987 and 2015 were retrospectively reviewed. Patients with NOM were included in the study. The diagnosis of neuro-behcet disease was based on neuro-imaging and lumbar puncture. All patients were evaluated both in the internal medicine and ophthalmology departments. Results Out of the 217 patients diagnosed with neuro-BD, 29 presented with NOM. The M/F ratio was 1.2. Mean age at diagnosis was 25.3 years. Clinical NOM included papillitis (27.5%), papilledema (51.7%), retrobulbar optic neuritis (10.3%), optic disc atrophy (31%) and cranial nerve palsy (27.5%). All patients were treated with corticosteroids and immunosuppressive drugs were required in 23 patients. Initially, 79% of patients had a decreased visual acuity, and 79% had visual field defects. After treatment, vision improved or stabilized in 66.7% and worsened in 33.3%. The mean ± SD LogMAR visual acuity improved from 0.4 ± 0.3 at diagnosis to 0.2 ± 0.3 after therapy. 10.3 and 3.4% patients were respectively legally blind at diagnosis and at the end of follow-up. Conclusions Although NOM of BD are rare, they are potentially severe and disabling. Recognition of NOM is crucial for establishing an early diagnosis. Prompt treatment is the main prognostic factor for the visual outcome for these patients.