Neurological manifestations of Behçet's disease

Abstract

Behçet's disease is a vasculitis of unknown origin characterised as a triad consisting of recurrent aphthous oral and genital ulcerations and uveitis. Articular, vascular and neurological manifestations may occur. Neurological manifestations are observed in 5 to 20% of patients, particularly in males during the third decade and may be classified in: - Vascular involvement: cerebral venous thrombosis with intracranial hypertension and/or cerebral arteries thrombosis or aneurysms. With anticoagulation, prognosis of cerebral venous thrombosis is usually good. Prognosis of arterial manifestations is unknown due to the rarity of cases. - Central nervous system involvement: meningitis, meningoencephalitis eventually with psychic disturbance. Medullar and periphereal involvement are rare. Prognosis is generally severe due to a remitting/relapsing course and functional disability. Delay in diagnosis is still too long, in part due to the rarity of the disease and in part due to inaugural neurological form. In such cases, extraneurological inflammatory manifestations are of great clinical help. Extensive clinical examination is necessary, searching for cutaneous, articular, ocular and rarely digestive or renal manifestations. Neurological manifestations should be regarded as an emergency requiring urgent therapy. As for other vasculitides, corticosteroids in association with immunosuppressive agents (mostly cyclophosphamide or imuran) are used. In our experience prognosis is correlated with the localisation of the vasculitis and with therapeutic observance. Among treated patients relapses dropped from 71 to 21% and sequelae observed in 44% of cases, severe in 20% of cases.