Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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January 21, 2016
Reversible Cerebral Vasoconstriction Syndrome (RCVS)
- Background:
- First described by Calabrese et al in 1993 in patients presenting with severe headache and with or without seizures or neurologic deficit
- The proposed mechanism is a disturbance in the control of cerebrovascular tone, either idiopathic (primary) or secondary (due to vasoactive substances, postpartum period, hypercalcemia, exercise, sexual activity, etc).
- Clinical Presentation:
- Usually occurs in young women (20–50 years of age)
- Sudden onset of severe “thunderclap” headache with or without photophobia, nausea, vomiting, focal neurologic symptoms, or seizures
- May be complicated by localized convexity subarachnoid hemorrhage, ischemic or hemorrhagic stroke, or posterior reversible encephalopathy syndrome (PRES).
- Key Diagnostic Features:
- CT/MR may be normal but can show hemorrhage or infarction
- Angiography (CTA/MRA/DSA) shows multifocal stenosis with poststenotic dilatation (“string of beads” or “sausage string” appearance) in circle of Willis vessels or branches. This appearance typically resolves spontaneously within 1–3 months.
- Differential Diagnosis:
- Aneurysmal subarachnoid hemorrhage
- Cerebral vasculitis (primary angiitis)
- Arterial dissection
- Intracranial venous thrombosis
- PRES
- Vascular malformations
- Treatment:
- Discontinuation of vasoactive drugs
- Calcium channel blockers and glucocorticoids are reasonable first-line therapies.
- Spontaneous resolution usually occurs, with improvement of angiographic findings within 3 months.
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