Hundreds of US Adults With Reversible Cerebral Vasoconstriction Syndrome Hospitalized Every Year

The incidence of reversible cerebral vasoconstriction syndrome (RCVS) cases leading to hospitalizations is approximately 3 per million adults every year, and the incidence is more than 3 times higher in women than men, according to study results published in Neurology.

The diagnostic criteria for RCVS in patients with severe acute headaches with or without additional neurologic signs include cerebral artery vasoconstriction on angiography, a normal or near-normal cerebrospinal fluid profile, no evidence of cerebral aneurysm, and reversibility of angiographic findings within 12 weeks.

Using administrative claims data from 11 states in the US in 2016, the age- and sex-standardized incidence rate of hospitalization for RCVS and health care utilization were determined.

Data from NewYork-Presbyterian/Weill Cornell Medical Center and UC Health Memorial Hospital Central in Colorado were used to validate RCVS diagnoses using ICD-10 code I67.841. Applying this code to more than 5 million hospital admissions from the Healthcare Cost and Utilization Project on all emergency department visits and hospital admissions led to the identification of 222 patients with a hospital discharge diagnosis of RCVS during 2016.

Most patients with a diagnosis of RCVS were from Massachusetts (56 patients, 25.2%) and New York (55 patients, 24.8%). The majority of patients (77.5%) were women, and their mean age was 46.9 years.

The estimated overall age- and sex-standardized incidence of RCVS cases leading to hospital admission was 2.7 (95% CI, 2.4-3.1) cases per million per year. The incidence in women was more than 3-fold higher than in men, with 4.1 (95% CI, 3.5-4.8) cases per million vs 1.2 (95% CI, 0.9-1.6) cases per million, respectively.

Concomitant neurologic diagnoses during the index hospital admission for RCVS were common and included subarachnoid hemorrhage (82 patients, 36.9%), ischemic stroke (36 patients, 16.2%), intracerebral hemorrhage (23 patients, 10.4%), seizures (22 patients, 9.9%), and posterior reversible encephalopathy syndrome (17 patients, 7.7%).

More than a third of patients (38.7%) presented to the emergency department at least once in the 90 days prior to the index RCVS diagnosis, and about a quarter (24.7%) presented for a neurologic diagnosis. In the 90 days prior to the index RCVS diagnosis, 50 patients (22.5%) had at least one hospitalization, including 16 patients (7.2%) hospitalized with a primary neurologic diagnosis. Other common admissions were related to pregnancy or psychiatric diagnoses.

Following discharge from the index hospital admission for RCVS, 70 patients (31.5%) presented to the emergency department at least once, including 33 patients (14.8%) who presented for a neurologic diagnosis. At least 1 hospital admission with a neurologic primary discharge diagnosis was documented for 31 patients (14.0%).

The study had several limitations, including potential misclassification of diagnoses due to the use of administrative claims data, lack of data on neuroimaging or laboratory tests or long-term data on preceding and follow-up events, and potential selection bias from the inclusion of the particular states.

“In a large, heterogeneous sample of patients in the U.S., we found that the age- and  sex-standardized incidence of RCVS leading to hospitalization in U.S. adults is approximately 3 per million per year. We found a substantial rate of neurovascular complications in patients with RCVS, emphasizing the importance of timely diagnosis of RCVS,” concluded the researchers.

Disclosure: Some study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors’ disclosures. 


Magid-Bernstein J, Omran SS, Parikh NS, Merkler AE, Navi B, Kamel H. RCVS: symptoms, incidence, and resource utilization in a population-based US cohort. Neurology. Published online May 28, 2021. doi:10.1212/WNL.0000000000012223