Postpartum angiopathy and other cerebral vasoconstriction syndromes

The phenomenon of reversible cerebral arterial segmental vasoconstriction has been associated with several conditions, including pregnancy and puerperium (“postpartum angiopathy”), thunderclap headache, and use of vasoconstrictive medications. Patients with cerebral vasoconstriction typically present with sudden, severe, and recurrent (“thunderclap”) headaches and can develop strokes. Cerebral vasoconstriction syndromes are under-recognized, are poorly characterized, and are frequently misdiagnosed as primary cerebral vasculitis. This article presents an illustrative case report and reviews the historical aspects, clinical and imaging characteristics, etiology, differential diagnosis, management, and prognosis of cerebral vasoconstriction syndromes.