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RCVS2 Score for Reversible Cerebral Vasoconstriction Syndrome

Distinguishes reversible cerebral vasoconstriction syndrome from other intracranial arteriopathies.

RCVS2 Score for Reversible Cerebral Vasoconstriction Syndrome
Thunderclap headache pattern (single or recurrent)
Intracranial internal carotid artery involvement
Vasoconstrictive trigger identified (e.g. vasoactive substance, postpartum)
Sex
Subarachnoid hemorrhage present
Result0 points

Score <=2 argues against RCVS (high sensitivity/specificity for exclusion in the derivation cohort).

RCVS2 score
0

When to use

  • Patients with suspected RCVS presenting with recurrent thunderclap headache and/or a multifocal segmental intracranial arteriopathy, to help distinguish RCVS from mimics such as primary CNS vasculitis.
  • Intended for a first presentation of new abnormal intracranial vascular imaging in patients aged 18-55 years; the score has not been tested outside this age range.

Formula

RCVS2 = Recurrent/single thunderclap headache (+5) + intracranial ICA involvement (-2) + vasoconstrictive trigger (+3) + female sex (+1) + subarachnoid hemorrhage (+1). Range -2 to +10.

Pearls and pitfalls

  • A score <=2 had ~100% specificity and ~85% sensitivity for excluding RCVS in the derivation/validation cohorts; a score >=5 had ~99% specificity and ~90% sensitivity for RCVS.

References

  1. Rocha EA, Topcuoglu MA, Silva GS, Singhal AB. RCVS2 score and diagnostic approach for reversible cerebral vasoconstriction syndrome. Neurology. 2019;92(7):e639-e647.