Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
September 2016
Next Case of the Month coming October 4 …
Reversible Cerebral Vasoconstriction Syndrome
- Diagnosis/Clinical Presentation:
- Reversible cerebral vasoconstriction syndrome (RCVS) is a clinical and radiologic syndrome whose primary features include the hyperacute onset of severe headache and segmental vasoconstriction of cerebral arteries--which can either be spontaneous or related to an exogenous trigger--that resolves by 3 months.
- The syndrome typically follows a benign course; however, reversible cerebral vasoconstriction syndrome may result in permanent disability or death in a minority of patients, secondary to complications such as ischemic stroke or intracranial hemorrhage.
- Key Diagnostic and Imaging Notes:
- The diagnostic evaluation of a patient with reversible cerebral vasoconstriction syndrome integrates clinical, laboratory, and radiologic findings.
- Imaging plays an important role by confirming the presence of cerebral vasoconstriction; monitoring potential complications such as ischemic stroke; and suggesting alternative diagnoses, including CNS vasculitis and aneurysmal subarachnoid hemorrhage.
- Noninvasive vascular imaging, including transcranial Doppler sonography and MR angiography, has played an increasingly important role in this regard, though conventional angiography remains the criterion standard for the evaluation of cerebral artery vasoconstriction.
- Newer imaging techniques, including high-resolution vessel wall imaging, may help in the future to better discriminate reversible cerebral vasoconstriction syndrome from primary angiitis of the CNS--an important clinical distinction.
- Differential Diagnosis:
- CNS Vasculitis
- Aneurysmal Subarachnoid Hemorrhage
- Treatment:
- Treatment is often supportive once the diagnosis is made, although vasoactive medications should be discontinued, and vasodilators such as calcium channel blockers can be considered in severe cases.
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