Granulomatous Angiitis of the Nervous System

Primary Angiitis of the Central Nervous System at Conventional Angiography

“…The histopathologic diagnosis of primary angiitis of the CNS used at our institution required the fulfillment of three criteria (2,9). First, there must be a transmural inflammation of small-or medium-sized blood vessels of the meninges and/or cortex of the brain.…”

“…Brain biopsy provides a definitive diagnosis, but it is invasive and findings may be negative in some patients with primary angiitis of the CNS because of sampling error (5,9). Given these limitations of brain biopsy, some authors have recommended presumptive treatment of primary angiitis of the CNS on the basis of clinical history and typical arteriographic findings (7,10).…”

Multifocal Hemorrhagic Vasculopathy: Possibly a Manifestation of Central Nervous System Vasculitis

“…5,6 Most patients with untreated PACNS have a progressive course that leads to death within 1 year of symptom onset. 7,8 Diagnosis is rarely established by a single test. Rather, it is typically based on angiographic findings of beading produced by alternating stenosis and dilation and histopathologic features of vasculitis on brain/leptomeningeal biopsy.…”

Isolated Angiitis of the Central Nervous System: Report of a Patient with an Unusually Prolonged Course

“…IAC is a rare disorder, with only 35 cases diagnosed ante mortem until 1997 [3]. With protean clinical manifestations, the course of the untreated disease is rapidly progressive, with death occurring within 9-12 months [4].…”