Primary Angiitis of the Central Nervous System in Adults and Children

Primary angiitis of the central nervous system (PACNS) is a rare disease, although it is increasingly recognized both in adults and children. Little is known about pathogenesis, but efforts at classification into subtypes are being made, and the distinction of PACNS from reversible cerebral vasoconstriction syndrome has been a major advance. The prognosis for improvement, or at least stabilization, of neurologic function is good with prompt and aggressive treatment, but the diagnosis continues to be challenging. Refinement of treatment strategies is needed. Multicenter collaboration may be crucial to make additional progress via randomized trials.

Key points

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The prognosis of both adult and childhood primary angiitis of the central nervous systems has improved with prompt and aggressive treatment.
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Classification into subtypes remains a work in progress, primarily because of the lack of knowledge about pathogenesis.
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Cyclophosphamide and glucocorticoids remain the usual therapeutic approach, but less toxic regimens are appropriate for some patients and are a major goal for future research.