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Reversible cerebral vasoconstriction syndrome

Reversible cerebral vasoconstriction syndrome (RCVS), also know as 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. 

Terminology

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:

Epidemiology

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

Clinical presentation

A thunderclap headache is the usual primary symptom, 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.

CSF studies are normal or near normal. 

Associated/predisposing conditions
  • pregnancy and puerperium
    • early puerperium / late pregnancy
    • eclampsia / pre-eclampsia / delayed postpartum eclampsia
  • exposure to drugs and blood products
    • phenylpropanolamine
    • pseudoephedrine
    • ergotamine
    • tartrate
    • methysergide
    • bromocryptine
    • lisuride
    • SSRI
    • sumatriptan
    • isometheptene
    • 'recreational drugs'
      • cocaine
      • ecstasy
      • marijuana
      • amphetamines
    • tacrolimus
    • cyclophosphamide
    • erythropoietin
    • IV Ig
    • red blood cell transfusion
  • miscellaneous
  • idiopathic

Radiographic features

No validated criteria have been defined for diagnosis confirmation; however the following guidelines are reasonable when applied to the clinical context:

  • angiography (CTA/MRA/DSA) demonstrated multifocal narrowings in the circle of Willis and its branches; post-stenotic segments show dilatation giving classical beaded appearance/sausage-shaped arteries
  • no CT evidence of subarachnoid haemorrhage (although small cortical subarachnoid haemorrhages are seen in ~ 20% of cases 2)
  • confirmation of the diagnosis rests on eventual resolution of angiographic findings within 12 weeks

Treatment and prognosis

Spontaneous resolution usually occurs, with improvement in angiographic findings within three 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
  • One patient had a relapse
  • 54% stroke (ischaemic) (see case 1)
  • 14% haemorrhagic stroke

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 therapy has also been advocated.

Immunosuppression is not required (cf. 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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