NEURO-BEHCETS DISEASE

We are reporting a case of Behcet's disease without vasculitis, but wi th acute neutrophilic inflammation which involved the brain and other organs. The patient exhibited waxing and waning neurological deficits which were unresponsive to treatment, The neuroradiologic findings sim ulated those of multiple sclerosis. The neuropathological examination revealed an acute, focal, though disseminated encephalitis involving t he frontal lobe, internal capsule, basal ganglia, cerebellum, and brai n stem. The acute inflammation consisted of a neutrophilic and eosinop hilic infiltration of the perivascular spaces and parenchyma without e vidence of vasculitis, fibrinoid necrosis, or thrombosis. Cultures and special stains for microbial organisms were negative. Ultrastructural examination revealed no viral structures or other microorganisms. In situ hybridization for Epstein-Barr virus (EBV), herpes simplex virus type I (HSVI), and cytomegalovirus (CMV) was negative, In this case, t he lack of inflammation in the vessel walls points out that the necrot izing lesions in neuro-Behcet's disease need not be the result of vasc ulitis and superimposed thrombosis, but may occur as a result of prima ry, acute neutrophilic inflammation.