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.
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:
- benign angiopathy of the CNS
- drug-induced arteritis
- migrainous vasospasm
- Call-Fleming syndrome
- postpartum angiopathy
Typically it affects young women (20-50 years of age).
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 or convexity subarachnoid haemorrhage.
CSF studies are normal or near normal.
- pregnancy and puerperium
- early puerperium / late pregnancy
- eclampsia / pre-eclampsia / delayed postpartum eclampsia
- exposure to drugs and blood products
- 'recreational drugs'
- IV Ig
- red blood cell transfusion
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.
- there is a suggestion of centripetal propagation of the vasoconstriction if the MRA is obtained at onset, compared with post remission of thunder clap headache 5
- no CT evidence of subarachnoid haemorrhage (although small convexity 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).
When vascular imaging has been obtained, then the differential narrows to conditions that can cause intracranial arterial beading, namely:
- 1. Calabrese LH, Dodick DW, Schwedt TJ et-al. Narrative review: reversible cerebral vasoconstriction syndromes. Ann. Intern. Med. 2007;146 (1): 34-44. Ann. Intern. Med. (full text) - Pubmed citation
- 2. Ducros A, Boukobza M, Porcher R et-al. The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain. 2007;130 (Pt): 3091-101. doi:10.1093/brain/awm256 - Pubmed citation
- 3. Marder CP, Donohue MM, Weinstein JR et-al. Multimodal imaging of reversible cerebral vasoconstriction syndrome: a series of 6 cases. AJNR Am J Neuroradiol. 2012;33 (7): 1403-10. AJNR Am J Neuroradiol (abstract) - doi:10.3174/ajnr.A2964 - Pubmed citation
- 4. Tan LH, Flower O. Reversible cerebral vasoconstriction syndrome: an important cause of acute severe headache. Emerg Med Int. 09;2012: 303152. Emerg Med Int (full text) - doi:10.1155/2012/303152 - Free text at pubmed - Pubmed citation
- 5. Shimoda M, Oda S, Hirayama A et-al. Centripetal Propagation of Vasoconstriction at the Time of Headache Resolution in Patients with Reversible Cerebral Vasoconstriction Syndrome. AJNR Am J Neuroradiol. 2016;37 (9): 1594-8. doi:10.3174/ajnr.A4768 - Pubmed citation