Reversible Cerebral Vasoconstriction Syndrome (RCVS)

David Goldemund M.D.
Updated on 12/01/2024, published on 17/03/2022

  • Reversible Cerebral Vasoconstriction Syndrome (RCVS) is characterized by severe headache (thunderclap headache – TCH) and the spontaneous development of reversible cerebral vasoconstriction
  • it affects all age groups, but is more prevalent in women (F:M ratio is 2.4:1) between the ages of 20-50
    • patients with RCVS usually have a history of migraine headaches
  • may last from several weeks to months, with varying degrees of severity; attacks may occur repeatedly
  • if not promptly diagnosed and treated, RCVS can lead to serious complications, such as a stroke
  • the exact cause of RCVS is unknown; it has been 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
    • oral contraception
    • 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 typically 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 (typically occurring after 3-4 days of persistent headache) with associated focal deficits
    • epileptic seizures
    • intracerebral hemorrhage (ICH)
    • subarachnoid hemorrhage (SAH)

Diagnostic evaluation

Brain imaging (CT/MRI)

  • usually, normal findings at the onset of symptoms; neuroimaging must exclude SAH
  • a maximum of parenchymal changes (in 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 only mild contrast enhancement of the affected arterial wall (DDx of vasculitis where a prominent enhancement is typically present)
    • intracranial atherosclerotic plaques may also exhibit some degree of focal enhancement   (Mandell, 2016)
  • some parenchymal 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 typically not involved, which helps distinguish it from PACNS
  • findings on vascular imaging can be variable and fluctuating
  • RCVS often resolves completely within 8-12 week


Laboratory test

  • CSF is normal
  • exclude hypercalcemia
  • consider drugs test
  • order blood samples to rule out vasculitis → more here
  • brain and meningeal biopsy should be considered if an atypical course raises suspicion of PACNS

Differential diagnosis


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  • in some cases with mild symptoms, the condition resolves without treatment
  • calcium channel blockers (nimodipine, verapamil) induce arterial dilation – verapamil (ISOPTIN, LEKOPTIN, VERAPAMIL) 160-240 mg PO 1x daily, nimodipine (NIMOTOP 60 mg) 60mg PO 4-6x daily for 4-12 weeks
    • may relieve spasms and lead to pain relief, and may also serve as a preventive measure
    • may be administered orally (PO) or intravenously (IV)
  • symptomatic therapy:
    • analgetics for headache
    • blood pressure correction
    • maintenance of adequate fluid intake
  • corticosteroids are not proven to be effective and are not recommended
  • if a stroke is confirmed, standard acute stroke therapy and secondary prevention should be implemented

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Reversible Cerebral Vasoconstriction Syndrome (RCVS)