Benign cerebral angiopathy; postpartum cerebral angiopathy: characteristics and treatment

Benign cerebral angiopathy and postpartum cerebral angiopathy are reversible cerebral arterial vasoconstriction syndromes. Presentation includes recurrent severe headaches, altered consciousness, and focal neurologic deficits; ischemic and/or hemorrhagic strokes can occur. No standard management has been established, but most authors agree that 1) acute-phase treatment includes cessation of vasoconstrictors, treatment of associated conditions, vasospasm treatment (calcium channel antagonists), and corticosteroids; 2) other measures include headache relief, blood pressure control, and stroke, cerebral edema, and seizure treatment; 3) definitive diagnosis requires conventional angiography and exclusion of alternative diagnosis; 4) a second arterial examination after 4 to 6 weeks is mandatory to confirm reversibility of vasoconstriction; 5) brain biopsy is indicated to rule out cerebral vasculitis in severe cases with clinical deterioration under steroid treatment or atypical findings; 6) immunosuppression should be reserved for patients with brain-leptomeningeal biopsy-proven vasculitis or used while waiting for a brain biopsy result; and 7) long-term measures include secondary stroke prevention and treatment of complications.