Reversible Cerebral Vasoconstriction Syndrome (RCVS)

Created 17/03/2022, last revision 07/11/2023

  • Reversible Cerebral Vasoconstriction Syndrome (RCVS) is characterized by severe headache (thunderclap headache – TCH) 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 the ages 20-50
    • patients with RCVS usually have a history of migraine
  • may last from several weeks to months, with varying degrees of severity; attacks may recur
  • serious complications, such as a stroke, may be associated with RCVS if not promptly diagnosed and treated
  • the exact cause of RCVS is unknown; it is associated with 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 (TCH)
    • short duration (hours) and usually recurring over days or weeks
    • rapid and abrupt onset may mimic SAH
  • other symptoms:
    • photosensitivity/photophobia
    • nausea, vomiting
    • confusion
    • changes in vision
  • complications of RCVS:
    • TIA/ischemic stroke (usually after 3-4 days of persistent headache) with focal deficits
    • epileptic seizures
    • ICH
    • SAH

Diagnostic evaluation

Brain imaging (CT/MRI)

  • usually normal findings at the onset of symptoms; neuroimaging must exclude SAH
  • maximum of parenchymal changes (T2/FLAIR) may be seen within 2-3 weeks as a complication of RCVS  [Chen, 2018]
    • vasogenic or cytotoxic edema (hyperintense lesions)
    • nontraumatic, non-aneurysmal convexial SAH (sulcal hyperintensities on FLAIR)
    • lobar hemorrhage
    • border zone (watershed) infarcts  Border zone infarcts (BZI)
  • vessel wall imaging (T1C+ black blood)
    • RCVS shows no or mild contrast enhancement of the affected arterial wall (DDx of vasculitis where a prominent enhancement is present)
    • intracranial atherosclerotic plaques may also show some degree of focal enhancement   (Mandell, 2016)
  • some MRI lesions may be reversible

Vascular imaging (CTA, MRA, DSA)

  • multifocal segmental stenosis of large to medium-sized cerebral arteries   Reversible cerebral vasoconstriction syndrome (Call-Flemming syndrome) on DSA
    • intracranial ICA is usually not involved (DDx of PACNS)
  • variable and fluctuating findings
  • often resolves completely within 8-12 weeks


Laboratory test

  • CSF is normal
  • exclude hypercalcemia
  • consider testing for the presence of drugs
  • order blood samples to rule out vasculitis → more here
  • consider brain and meningeal biopsy if atypical course raises suspicion of PACNS

Differential diagnosis


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Reversible Cerebral Vasoconstriction Syndrome (RCVS)