Neuro-Behçet's disease showing severe atrophy of the cerebrum

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Abstract
A 38-year-old female died 6 years after the onset of what was, clinically and histopathologically, consistent with Neuro-Behçet's disease. Pathologically the cerebrum showed severe atrophy. The main changes were observed in the grey and white matter, the diencephalon and the basal ganglia by light microscopy. All these changes originated in softenings around blood vessels, especially small vessels or capillaries. These foci fused together to form large regions of softening. Glial or mesenchymal reactions were minimal. In the white matter there was slight perivascular-infiltration, mainly consisting of lymphocytes. In view of these findings, it is suggested that these changes were caused by an allergic vasculitis. The present case of Neuro-Behçet's disease is the first one showing general atrophy of the cerebrum. It is very important in relation to demyelinating encephalitis.