Concurrent Extracerebral Vasoconstriction in Patients with Reversible Cerebral Vasoconstriction Syndrome: A Cross-Sectional Study

Background: Reversible cerebral vasoconstriction syndrome (RCVS) is an uncommon and often underrecognized neurovascular disorder. We aimed to investigate the clinical presentations associated with extracerebral vasoconstriction in patients diagnosed with RCVS. Methods: In this cross-sectional study, we analyzed data from a single-center cohort of patients with RCVS in Korea. Extracerebral vasoconstriction in individuals diagnosed with RCVS was defined by the following criteria: (1) the presence of sudden and severe pain in extracerebral regions (primarily the chest or abdomen) coinciding with the onset of RCVS, (2) resolution of the pain following the administration of vasodilators, and (3) confirmation of vasoconstriction through imaging studies or, at a minimum, the exclusion of other potential causes associated with the pain. Results: Among the 80 eligible patients (median age, 53 years; female sex, 82.5%), 8 patients (10%) experienced extracerebral vasoconstriction. Regarding pain location, four patients reported chest pain, two reported abdominal pain, and two reported pains in both the chest and abdomen. When comparing visit route, the patients were associated with emergency department (odds ratio [OR]: 6; 95% confidence interval [CI]: 1.1–33; reference: outpatient) and inpatient consultation (OR: 25; 95% CI: 1.1–560) compared to those without extracerebral vasoconstriction. Patients with extracerebral vasoconstriction had no prior history of precipitating conditions or medication use before the onset of RCVS. The treatment response to vasodilators was excellent in all patients, and none reported neurovascular or extracerebral complications during the bout of RCVS. Conclusions: A co-occurrence of extracerebral vasoconstriction was not exceptionally uncommon among patients with RCVS. Our findings suggest that extracerebral vasoconstriction may be underrecognized in individuals with RCVS.