Benign angiopathy of the central nervous system presenting with intracerebral hemorrhage

Primary central nervous system vasculitis has traditionally been described as an aggressive condition, with significant morbidity and mortality. A subgroup of patients has been identified who have a similar clinical presentation, but with a benign course. This syndrome of BACNS is successfully treated with low-dose steroids and calcium-channel blockers. Histologic confirmation, when performed, is normal.

Intracerebral hemorrhage is a rare presenting finding in the setting of BACNS. We present 2 patients with acute onset of headache and neurologic impairment secondary to an ICH.

Cerebral angiography showed characteristic findings of diffuse vasculitis. Both patients were subjected to biopsy and both failed to reveal evidence of vasculitis.

This report is the first to document the normal histologic features of BACNS in the setting of an ICH. Although these angiographic changes are similar to vasculitis, these processes can be differentiated on clinical grounds, which is of vital importance as the treatment and clinical course of BACNS is more benign. Furthermore, the presence of an ICH in the setting of vasoconstriction seen on angiography may represent a novel feature in some patients with BACNS and is not necessarily a harbinger of the more malignant PCNSV.