NEURO-BEHCETS DISEASE

We are reporting a case of Behcet's disease without vasculitis but wit h acute neutrophilic inflammation which involved the brain and other o rgans. The patient exhibited waxing and waning neurological deficits w hich were unresponsive to treatment. The neuroradiologic findings simu lated those of multiple sclerosis. The neuropathological examination r evealed an acute, focal, though disseminated encephalitis involving th e frontal lobe, internal capsule, basal ganglia, cerebellum, and brain stem. The acute inflammation consisted of a neutrophilic and eosinophi lic infiltration of the perivascular spaces and parenchyma without evi dence of vasculitis, fibrinoid necrosis, or thrombosis. Cultures and s pecial stains for microbial organisms were negative. Ultrastructural e xamination revealed no viral structures or other microorganisms. In si tu hybridization for Epstein-Barr virus (EBV), herpes simplex virus ty pe I (HSV-I), and cytomegalovirus (CMV) was negative. In this case, th e lack of inflammation in the vessel walls points out that the necroti zing lesions in neuro-Behcet's disease need not be the result of vascu litis and superimposed thrombosis, but may occur as a result of primar y, acute neutrophilic inflammation.