This is a retrospective single centre study from Paris, France, between the periods of 1986 to 2015. Twenty-nine out of 217 (13.3%) neuro-Behcet’s disease (NBD) patients presented with neuro-ophthalmic manifestations (55% of men and mean age of 28 years). Sixteen out of 29 patients (55%) had non-parenchymal (NPM) – related signs, and 13/29 (45%) had parenchymal (PM) manifestations. NPM signs included papilloedema due to cerebral venous thrombosis (CVT) in all 16 patients; 6/16 also had sixth nerve palsy. PM signs included papillitis in 7/13 patients, retrobulbar optic neuritis in 4/13 and 2/13 had third nerve palsy. Of the patients, 93.1% had visual symptoms of decreased vision, visual field defects and / or diplopia at initial presentation. All patients received corticosteroids and 79% had immunosuppressive agents – Azathioprine (12 patients), Mycofenolate Mofetil (four patients), Interferon-alpha in three patients, Cyclophosphamide in four patients and Infliximab in one patient. Patients who developed CVT were treated with anticoagulant and Acetazolamide; 66.7% of patients improved or stabilised after treatment and worsened in 33.3%. The mean LogMAR+/-SD visual acuity improved from 0.4+/-0.3 at diagnosis to 0.2+/-0.3 post therapy; 10.3% were legally blind at diagnosis and down to 3.45% after treatment. The authors recommended a multidisciplinary approach including ophthalmologists, neurologists and medical internists for the management of these patients. Early prompt treatment is the key to improve visual outcome of this group of patients.
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