LAUSR

Behçet’s disease (BD) is a chronic inflammatory disorder with higher prevalence along the ancient silk road (from Japan to the Mediterranean region), whereas it is rare in North America. BD is often associated with HLA-B51 and has a wide range of clinical manifestations (involving the eyes, oral mucosa, skin, and nervous system). Oral ulcers are the most common and usually the first manifestation of the disease; anogenital ulcers are present in 75% of patients, whereas other skin complications (erythema nodosum, papulopustular, and acneiform lesions) are present in 60% of patients. Neurological involvement, often referred to as neuro-Behçet disease (NBD), is reported in approximately 10% of patients with BD, showing a male predominance (male-tofemale ratio = 3:1) and can affect both the central and peripheral nervous systems. Involvement of the central nervous system can be classified into parenchymal (hemispheric, brainstem, spinal cord, meningoencephalitis) and nonparenchymal (cerebral venous thrombosis, stroke, aneurysms). Proposed diagnostic criteria for NBD include (1) satisfying the International Study Group BD criteria, (2) the presence of a compatible neurological syndrome along with characteristic abnormalities in neuroimaging and/or cerebrospinal fluid, and (3) no better explanation for the symptoms. Headache, cognitive-behavioral changes, pseudobulbar speech, brainstem problems, and motor and sensory alterations are commonly seen in NB.