A man of 32-years-old suffering recurrent oral and genital ulceration showed signs of involvement of the central nervous system for three years before death. These included spastic quadriplegia, pseudbulbar plasy, akinesia and mutism. Brain CT showed remarkable ventricular dilatation. Pathologically, the main changes were observed in the brain stem and the white matter. All these changes originated in softenings and perivascular cuffing. These foci fused together to form large regions of softenings. In the middle cerebellar peduncle, these were small abscesses in the hemorrhagic lesion. In view of these findings, it is suggested that ventricular dilatation was caused by softenings in the white matter.