NEURO-BEHCET DISEASE WITH DEMYELINATION AND GLIOSIS OF THE FRONTAL WHITE-MATTER

A rare case of neuro-Behcet disease with diffuse demyelination and gli osis of the frontal white matter is reported clinico-pathologically. T he disease began with genital ulcer and recurrent oral aphthosis when the patient was 42 years of age. There was erythema, moderate fever, C SF (cerebrospinal fluid)-pleocytosis and elevated CSF-globulin. He was diagnosed as having neuro-Behcet disease and treated with prednisolon e. He gradually became euphoric, disinhibited, indifferent and demente d. His cranial CT showed diffuse low density areas in the bilateral fr ontal white matter. He became bedridden, akinetic mute and died from r espiratory dysfunction 3 1/2 years after onset. The following neuropad iological findings were observed: 1) Moderate demyelination and gliosi s was present mainly in the frontal and parietal white matter. 2) Ther e were many micro-spongious necrotic foci in the gray and white matter s of the cerebrum, basal ganglia, thalamus, midbrain and pons, some of which were accompanied by gliosis. 3) From 1/2 to 1/3 of all micro-ne crotic foci in the frontal white matter were old and accompanied by gl iosis. The white matter containing numerous micro-necrotic foci had my elin pallor and gliosis. 4) There was neither micro-necrosis nor glios is in the occipital lobe. The pathogenetic correlation of white matter lesions with primary and secondary circulatory disturbances is discus sed.