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Behçet's disease is a chronic multisystemic inflammatory disease that usually presents in the young adult. Central nervous lesions occur in 5 to 7% of patients and are the most severe manifestations of this disease.We retrospectively reviewed the MR images of patients with neurological manifestations of Behçet's disease evaluated in the Neuroradiology Department of Garcia de Orta Hospital and the MRI center of Caselas, Portugal, between 1994 and January 2000.There were 8 cases of Neuro- Behçet. Patients' ages ranged from 24 to 46 years (mean 36.25). There were 4 males and 4 females (male/female ratio = 1:1). In 3 cases (37.5%) there was brainstem involvement, the basal ganglia and thalamus were affected in 2 cases (25%) and the internal capsule and corona radiata in 2 cases (25%). In 3 cases (37.5%) there was telencephalic white matter involvement and in 1 case (12.5%) the spinal cord was involved.The topography of the lesions, the absence of a defined arterial territory distribution and the partial or total regression of lesions over time help to distinguish BD from other vasculitic processes and inflammatory/demyelinating diseases.