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Reversible cerebral vasoconstriction syndrome

Reversible Cerebral Vasoconstriction Syndrome (RCVS) or benign CNS arteriopathy is one of a group of conditions which make up the reversible cerebral vasoconstriction syndromes, not to be confused with posterior reversible encephalopathy syndrome (PRES), although there is overlap both in the presumed underlying mechanisms and predisposing factors. 

It was first described by Calabrese et al in 1993, and named  “benign angiopathy of the central nervous system”. A number of terms have been used to describe this entity or subsets. These include:

Epidemiology

Typically it affects young women (20 - 50 years of age). 

Clinical presentation

Typically presents with a thunderclap headache, often occipital but also potentially diffuse. There may be a history of recurrent thunderclap headaches over days or weeks. Headache may be associated with photophobia, nausea and vomiting. Focal neurological deficits can occur, secondary to ischaemia.

Associated / predisposing conditions
  • pregnancy and puerperium
    • early puerperium / late pregnancy
    • eclampsia / preeclampsia / delayed postpartum eclampsia
  • exposure to drugs and blood products
    • phenylpropanolanine
    • pseudoephedrine
    • ergotamine
    • tartrate
    • methylsergine
    • bromocryptine
    • lisuride
    • SSRI
    • sumatriptan
    • isometheptine
    • 'recreational drugs'
      • cocaine
      • ecstasy
      • marijuana
      • amphetamines
    • tacrolimus
    • cyclophosphamide
    • erythropoetin
    • IV Ig
    • red blood cell transfusion
  • miscellaneous
  • idiopathic

Diagnosis

No validated criteria as such are available, however the following guidelines are reasonable:

  • Angiography (CTA / MRA / DSA) demonstrated multifocal narrowings in the circle of willis branches or arteries forming circle of willis. Post stenotic segments show dilatation giving classical beaded appearence / sausage shaped arteries.
  • No CT evidence of subarachnoid haemorrhage (although small cortical subarachnoid haemorrhages are seen in 22% of cases 2)
  • Normal (near normal) CSF
  • Appropriate clinical history (thunderclap headache)

 Confirmation of the diagnosis rests on:

  • Eventual resolution of angiographic findings (within 12 weeks)
  • Autopsy / biopsy absence of perivascular inflammation

Treatment and prognosis

Prognosis and complications

Spontaneous resolution usually occurs, with improvement in angiographic findings within 3 months. The natural history of the condition has not however been well characterised. In one review of 16 patients:

  • 71% had complete long term resolution with no neurological deficits
  • 31% minor cognitive impairment
  • 1 patient had a relapse
  • 54% stroke (ischaemic) (case 1)
  • 14% haemorrhagic stroke

Treatment and prognosis

Although no randomised controlled trials are available, treatment with calcium channel blockers seems to be efficacious and are thought to be a reasonable first line therapy. Short course glucocorticoid treatment has also been advocated.

Immunosuppresion is not required (c.f. primary cerebral angiitis). 

Differential diagnosis

The differential on clinical presentation is essentially that of a thunderclap headache, with the main concern being subarachnoid haemorrhage

When vascular imaging has been obtained, then the differential narrows to conditions that can cause intracranial arterial beading, namely:

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