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A corticosteroid-responsive aneurysmal lenticulostriate vasculopathy

Primary angiitis of the CNS (PACNS) was initially described as granulomatous angiitis in 1952.1 Though it has been successfully treated with a combination of corticosteroids and cyclophosphamide,2 definitive diagnosis is difficult and clinicians are often reluctant to initiate toxic immunosuppressants without a clear diagnosis.

Isolated involvement of the lenticulostriate arteries is not reported in PACNS. Furthermore, aneurysm formation is rare in PACNS although more common in systemic vasculitides.3 We describe a woman with a symptomatic lenticulostriate vasculopathy presumed to be PACNS. The disease progressed until the institution of immunosuppressive therapy and regressed once treated.

### First presentation.

A 43-year-old woman presented in September 1999 with a left hemiparesis. She was normotensive with normal general examination. She had been healthy with no constitutional, rheumatologic, or neurologic symptoms. There were no stroke risk factors.

CT demonstrated a right internal capsule lacunar infarction and several more infarcts bilaterally within the lenticulostriate territories. Angiography demonstrated subtle irregularities of the lenticulostriate arteries. …