A 63-year-old man with severe, untreated rheumatoid arthritis and pleuritis developed an unusual neurological syndrome similar to Gerstman syndrome, followed by dementia and blindness, six weeks before his death. An autopsy showed extensive necrotizing vasculitis, resembling polyarteritis nodosa, involving the brain and resulting in numerous infarcts. The disease was most severe in the posterior portion of the cerebral hemispheres. Other organs were only slightly involved. Severe amyloidosis of cerebral arterioles and senile plaques were noted in the areas of brain with most severe vasculitis. Review of literature indicated only six cases of cerebral vasculitis in rheumatoid disease, five of which were treated with corticosteroids.