The rare case of a 20-year-old male with rapidly progressive primary angiitis of the CNS with a good outcome.

Rapidly progressive primary angiitis of the CNS (PACNS) is recognized as a clinical subtype of PACNS that is almost uniformly fatal. PACNS is usually characterized by subacute progression over several months. The most common symptoms are headaches and cognitive dysfunction, followed by focal neurologic deficits.(1) Compared with this more indolent presentation, patients with rapidly progressive PACNS are more likely to present in coma with quadriparesis and MR imaging notable for bilateral infarcts.(2) Various treatment combinations including high-dose corticosteroids, cyclophosphamide, azathioprine, methotrexate, rituximab, and plasma exchange have been described(1,3); however, no good outcomes have been reported to date. The largest case series of 11 patients had only 1 survivor who at 14 months had a modified Rankin Scale (mRS) of 5 (bedridden, requiring constant nursing care).(2) One other survivor of rapidly progressive PACNS had an mRS of 5 at the 3-month follow-up.(4) We describe the treatment and outcome of a patient with rapidly progressive PACNS who returned to his premorbid baseline with an mRS of 0.