Reversible cerebral vasoconstriction syndrome with concomitant recurrent subarachnoid hemorrhage and coronary vasospastic angina: A manifestation of systemic vasoconstriction syndrome.

BACKGROUND: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by thunderclap headache and reversible segmental cerebral arterial constriction. Although neurological complications are well recognized, concurrent extracerebral vasoconstriction remains under appreciated. CASE DESCRIPTION: A 53-year-old woman presented with sudden chest tightness immediately followed by a thunderclap headache. Initial brain computed tomography and magnetic resonance imaging showed no hemorrhage or infarction; however, magnetic resonance angiography demonstrated multifocal cerebral arterial narrowing, consistent with RCVS. During hospitalization, she developed recurrent nonaneurysmal subarachnoid hemorrhages (SAHs) on days 2 and 4. No bleeding source was identified on serial angiography. Recurrent episodes of chest tightness prompted coronary angiography with acetylcholine provocation, which revealed severe coronary vasospasm, establishing the diagnosis of vasospastic angina (VSA). Treatment with benidipine and nicorandil was initiated thereafter, and no recurrence of either cardiac or neurological symptoms was observed, with complete resolution of cerebral vasoconstriction on follow-up imaging. CONCLUSION: This case highlights a rare but clinically important association between RCVS, recurrent nonaneurysmal SAH, and coronary VSA confirmed by pharmacologic provocation. Although each condition is potentially reversible, their coexistence supports the concept of systemic vasoconstriction syndrome and underscores the need for vigilance regarding extracranial vascular involvement in patients with RCVS. Early recognition and targeted vasodilator therapy may be essential to prevent serious neurological and cardiovascular complications.