We report on the clinical symptomatology, diagnostic difficulty and inadequate possibilities for treatment of 6 patients suffering from primary agiitis of intracranial vessels. They died at age 36 to 64 years. The duration of the disease varied between 6 weeks and 11 1/2 years and was diagnosed, autoptically in all cases, as giant cell angiitis (2 cases) and, trombangiitis obliterans (4 cases). A degenerative cerebrovascular process had already been assumed clinically in four of the cases, and intracranial veins were also involved in three (very distinctly in one). The vascular disease was generalised or multilocular in four observations. Although autoimmunological processes were probably involved, the etiopathogenesis is still, in the final analysis, more or less unknown.