Reversible Cerebral Vasoconstriction Syndrome (RCVS)

Background

• Reversible cerebral vasoconstriction syndrome (RCVS) is a clinical and radiologic condition typically characterized by a combination of thunderclap headache and reversible vasoconstriction.
  • Thunderclap headache manifests as a hyperacute onset of severe headache with or without other neurologic signs and symptoms.
  • Reversible vasoconstriction is demonstrated as segmental vasoconstriction of cerebral arteries on imaging that resolves within 3 months.
• RCVS is not a single disease entity but a common presentation of multiple disorders characterized by reversible vasoconstriction of the cerebral vasculature.
• RCVS commonly affects patients aged 20-50 years (mean 42-45 years).
• 25%-60% of cases are secondary to an identifiable trigger.
• Potential triggers of RCVS include vasoactive medications and recreational drugs, pregnancy and postpartum states, infusion of blood products, sexual activity, and Valsalva maneuvers.
• Most patients present with thunderclap headache but up to 20% of patients may present with a seizure.

Evaluation

• Consider the diagnosis in a patient with recurrent thunderclap headache or with cryptogenic stroke.
• Confirmation of the diagnosis requires neuroimaging showing multifocal segmental vasoconstriction of medium and large vessels in cerebral arteries, with resolution of vasoconstriction confirmed within 3 months.
  • The diagnosis requires the exclusion of alternative diagnoses (especially subarachnoid hemorrhage and primary angiitis of the central nervous system [PACNS]).
  • Other important diagnoses to consider include migraine headache, posterior reversible encephalopathy syndrome (PRES), and cortical vein thrombosis.
• Initial testing should include a noncontrast computed tomography (CT) of the head and, if that is negative, perform a lumbar puncture.
• Vasoconstriction is usually identified with noninvasive neuroimaging such as CT angiography, magnetic resonance angiography (MRA), or transcranial Doppler sonography.
• Catheter angiography (digital subtraction angiography) is the imaging criterion standard for evaluation of cerebral vasculature but is usually not needed and not used due to the invasive nature of the procedure.
• Obtain urine to test for catecholamines to exclude pheochromocytoma and perform a toxicology screen to identify possible triggers.
• Routine blood tests are typically normal.

Management

• There are no randomized trials to guide treatment of patients with RCVS; treatment is guided by observational data and expert opinion.
• Rest, avoidance of headache triggers, and observation may be sufficient in patients who show no signs of clinical progression and no brain lesion.
• Start symptomatic treatment early in patients with consistent clinical and imaging findings and also in probable cases where initial imaging is normal.
  • Identify and eliminate any precipitating or aggravating factors.
  • Provide analgesics for headache, such as acetaminophen and simple opioids.
  • Manage the blood pressure control according to guidelines for patients with acute stroke.
• Calcium channel blockers (nimodipine, verapamil) may relieve symptoms but may not prevent complications such as hemorrhage or ischemic stroke.
• Endovascular therapy may be indicated in patients with severe refractory cerebral vasoconstriction and clinical deterioration.