Vasculitis of the central nervous system (CNS) is classified as primary angiitis or as vasculitis secondary to a variety of diseases. A wide spectrum of clinical features may occur. A definite diagnosis is hampered by the difficulty in obtaining tissue for histology. Consequently, a diagnosis is frequently made on the basis of clinical presentation, brain magnetic resonance imaging, and cerebral angiography without pathologic confirmation. Recent experience shows that there are multiple other conditions that can mimic CNS vasculitis, many of which have different therapies. Most patients with CNS vasculitis should be treated aggressively with a combination of immunosuppressive medications. The prognosis is greatly improved with early recognition and therapy.
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