Adamantinomatous craniopharyngioma (H & E image)

Cerebral vasculitis presenting with dementia

Vasculitis rarely affects the CNS, as opposed to other organ systems and the peripheral nervous system, and the primary presentation of vasculitis as dementia is extremely rare. Dementia usually occurs late in the course of the disease and is preceded by a history of stroke attributable either to the vasculitis directly, to abnormal coagulation, or to cardiac embolism. Primary angiitis of the CNS, an isolated granulomatous angiitis of the nervous system, is the archetypical vasculitic cause of dementia and the most difficult to diagnose because of its lack of associated extracranial abnormalities.