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Behçet disease (BD) is an idiopathic, chronic, relapsing, multisystem, vascular-inflammatory disease of unknown origin. The nervous system is involved in 5% to 15% of affected individuals. Despite its rarity and low profile, the various CNS manifestations and patient demographics often lead neuro-Behçet disease (NBD) to be included in the differential diagnosis of patients with cerebral sinus thrombosis and of young people admitted with clinical presentations suggestive of MS and related syndromes (e.g., cerebral vasculitic disorders) or of stroke.
Al-Araji and Kidd, authors of several previous studies of NBD, review the available evidence on prevalence, clinical findings, prognosis, and treatment. Using previously reported series and genera…