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July 20, 2021; 97 (3) Research Article

Reversible Cerebral Vasoconstriction Syndrome

Symptoms, Incidence, and Resource Utilization in a Population-Based US Cohort

View ORCID ProfileJessica Magid-Bernstein, Setareh S. Omran, Neal S. Parikh, Alexander E. Merkler, Babak Navi, Hooman Kamel
First published May 28, 2021, DOI: https://doi.org/10.1212/WNL.0000000000012223
Jessica Magid-Bernstein
From the Clinical and Translational Neuroscience Unit, Feil Family Brain and Mind Research Institute and Department of Neurology (J.M.-B., S.S.O., N.S.P., A.E.M., B.N., H.K.), Weill Cornell Medicine, New York, NY; and Department of Neurology (S.S.O.), University of Colorado School of Medicine, Aurora.
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  • ORCID record for Jessica Magid-Bernstein
Setareh S. Omran
From the Clinical and Translational Neuroscience Unit, Feil Family Brain and Mind Research Institute and Department of Neurology (J.M.-B., S.S.O., N.S.P., A.E.M., B.N., H.K.), Weill Cornell Medicine, New York, NY; and Department of Neurology (S.S.O.), University of Colorado School of Medicine, Aurora.
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Neal S. Parikh
From the Clinical and Translational Neuroscience Unit, Feil Family Brain and Mind Research Institute and Department of Neurology (J.M.-B., S.S.O., N.S.P., A.E.M., B.N., H.K.), Weill Cornell Medicine, New York, NY; and Department of Neurology (S.S.O.), University of Colorado School of Medicine, Aurora.
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Alexander E. Merkler
From the Clinical and Translational Neuroscience Unit, Feil Family Brain and Mind Research Institute and Department of Neurology (J.M.-B., S.S.O., N.S.P., A.E.M., B.N., H.K.), Weill Cornell Medicine, New York, NY; and Department of Neurology (S.S.O.), University of Colorado School of Medicine, Aurora.
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Babak Navi
From the Clinical and Translational Neuroscience Unit, Feil Family Brain and Mind Research Institute and Department of Neurology (J.M.-B., S.S.O., N.S.P., A.E.M., B.N., H.K.), Weill Cornell Medicine, New York, NY; and Department of Neurology (S.S.O.), University of Colorado School of Medicine, Aurora.
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Hooman Kamel
From the Clinical and Translational Neuroscience Unit, Feil Family Brain and Mind Research Institute and Department of Neurology (J.M.-B., S.S.O., N.S.P., A.E.M., B.N., H.K.), Weill Cornell Medicine, New York, NY; and Department of Neurology (S.S.O.), University of Colorado School of Medicine, Aurora.
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Reversible Cerebral Vasoconstriction Syndrome
Symptoms, Incidence, and Resource Utilization in a Population-Based US Cohort
Jessica Magid-Bernstein, Setareh S. Omran, Neal S. Parikh, Alexander E. Merkler, Babak Navi, Hooman Kamel
Neurology Jul 2021, 97 (3) e248-e253; DOI: 10.1212/WNL.0000000000012223

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Abstract

Objective To estimate the incidence of hospitalization for reversible cerebral vasoconstriction syndrome (RCVS), we identified RCVS-related hospital admissions across 11 US states in 2016.

Methods We tested the validity of ICD-10 code I67.841 in 79 patients with hospital admissions for RCVS or other cerebrovascular diseases at 1 academic and 1 community hospital. After determining that this code had a sensitivity of 100% (95% confidence interval [CI], 82%–100%) and a specificity of 90% (95% CI, 79%–96%), we applied it to administrative data from the Healthcare Cost and Utilization Project on all hospital admissions across 11 states. Age- and sex-standardized RCVS incidence was calculated using census data. Descriptive statistics were used to analyze associated diagnoses.

Results Across 5,067,250 hospital admissions in our administrative data, we identified 222 patients with a discharge diagnosis of RCVS in 2016. The estimated annual age- and sex-standardized incidence of RCVS hospitalization was 2.7 (95% CI, 2.4–3.1) cases per million adults. Many patients had concomitant neurologic diagnoses, including subarachnoid hemorrhage (37%), ischemic stroke (16%), and intracerebral hemorrhage (10%). In the 90 days before the index admission, 97 patients had an emergency department (ED) visit and 34 patients a hospital admission, most commonly for neurologic, psychiatric, and pregnancy-related diagnoses. Following discharge from the RCVS hospital admission, 58 patients had an ED visit and 31 had a hospital admission, most commonly for neurologic diagnoses.

Conclusions Using population-wide data, we estimated the age- and sex-standardized incidence of hospitalization for RCVS in US adults as approximately 3 per million per year.

Glossary

CI=
confidence interval;
ED=
emergency department;
ICD-10=
International Classification of Diseases–10;
IQR=
interquartile range;
PRES=
posterior reversible encephalopathy syndrome;
RCVS=
reversible cerebral vasoconstriction syndrome;
SAH=
subarachnoid hemorrhage

Footnotes

  • Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • CME Course: NPub.org/cmelist

  • Podcast: NPub.org/nq4cfl

  • Received September 28, 2020.
  • Accepted in final form April 14, 2021.
  • © 2021 American Academy of Neurology
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