Reversible Cerebral Vasoconstriction Syndrome (RCVS)

Created 17/03/2022, last revision 10/05/2022

  • Reversible Cerebral Vasoconstriction Syndrome (RCVS) is characterized by severe headaches (thunderclap headache) and spontaneous development of reversible cerebral vasoconstriction
  • it affects all age groups, more often present in women (F:M ratio is 2.4:1) between 20-50 years of age
    • a history of migraine is often found in patients with RCVS
  • can last for several weeks to months to varying degrees; attacks may recur
  • serious complications, such as a stroke, can be associated with RCVS if not promptly diagnosed and treated
  • the exact cause of RCVS is unknown; it is related to certain conditions (secondary RCVS)
  • Call-Fleming syndrome
  • acute benign cerebral angiopathy
  • CNS pseudovasculitis
  • cerebral vasculopathy
  • drug-induced cerebral arteritis
  • isolated benign cerebral vasculitis
  • migraine angiitis
  • postpartum cerebral angiopathy
  • thunderclap headache associated with vasospasms
  • drugs
    • SSRIs
    • cannabinoids, ecstasy, cocaine, amphetamine
    • nasal decongestants or triptans  [Kato, 2016]
    • immunosuppressive drugs (tacrolimus, cyclophosphamide, IVIG
    • birth control pills
    • ergotamine
    • sumatriptan
    • bromocriptine
    • red blood cell transfusion, erythropoietin
  • alcohol (especially binge drinking)
  • uncontrolled hypertension
  • endocrine disorders
  • certain tumors
  • hypercalcemia
  • migraine
  • pheochromocytoma
  • bronchial carcinoid
  • head trauma
  • pregnancy and puerperium
    • also known as postpartum cerebral angiopathy (postpartum angiitis) (Calado, 2006)
    • it usually develops a few days to weeks after an otherwise uncomplicated delivery

Clinical presentation

  • headaches
    • a common symptom, fluctuating, often dramatic, referred to as a “thunderclap” headache (rapid and abrupt onset may mimic SAH)
    • short duration (hours) and usually recurring over days or weeks
  • other symptoms:
    • photosensitivity/photophobia
    • nausea, vomiting
    • confusion
    • changes in vision
  • complications of RCVS:
    • TIA/ischemic stroke (usually after 3-4 days of lasting headaches) with focal deficits
    • epileptic seizures
    • ICH
    • SAH

Diagnostic evaluation

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Differential diagnosis


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