What is the treatment for Reversible Cerebral Vasoconstriction Syndrome (RCVS)?

Management of Reversible Cerebral Vasoconstriction Syndrome (RCVS)

Calcium channel blockers are the first-line treatment for Reversible Cerebral Vasoconstriction Syndrome (RCVS), with nimodipine or verapamil being the most effective options for symptom relief and prevention of complications. 1, 2

Diagnosis and Clinical Features

• RCVS is characterized by recurrent severe thunderclap headaches with diffuse segmental narrowing of cerebral arteries that resolves within 3 months 1
• Common triggers include serotonergic agents, sympathomimetic drugs, marijuana, SSRIs, triptans, and decongestants 3
• Diagnosis requires high clinical suspicion and neuroimaging showing characteristic "string of beads" appearance of cerebral arteries 2
• Differential diagnoses include aneurysmal subarachnoid hemorrhage, cervical artery dissection, and primary angiitis of the central nervous system 1

Acute Management

• Immediate discontinuation of any potential triggering medications or substances 4, 1
• Oral calcium channel blockers are the mainstay of treatment:
  • Nimodipine (30-60 mg every 4-6 hours) has been reported to reduce headache intensity 1, 2
  • Verapamil is an effective alternative when nimodipine is not available 4, 5
• For severe cases with neurological deterioration, intra-arterial calcium channel blockers (verapamil) may be administered during cerebral angiography 4, 5
• Avoid glucocorticoids as they have been reported as independent predictors of worse outcomes 1
• Bed rest and analgesics for symptomatic relief of headaches 1

Monitoring and Follow-up

• Serial transcranial Doppler ultrasonography can be used to monitor cerebral blood flow velocities 2
• Patients with mean flow velocity of middle cerebral artery >120 cm/s have higher risk of ischemic complications 2
• Follow-up neuroimaging (MRA or CTA) at 12 weeks to confirm resolution of vasoconstriction 1, 5
• Monitor for potential complications including posterior reversible encephalopathy syndrome (PRES), ischemic stroke, and intracerebral hemorrhage 1, 2

Special Considerations

• For patients with psychiatric comorbidities, careful medication management is required:
  • Avoid restarting serotonergic medications (SSRIs, SNRIs) during acute phase 6
  • Consider non-serotonergic alternatives for psychiatric conditions 6
• In cases with hypertension, blood pressure control is important but avoid excessive lowering that might compromise cerebral perfusion 1
• For pregnant or postpartum patients with RCVS, close monitoring is essential as they may have more severe disease course 1, 2

Prognosis

• Most patients (90-95%) have favorable outcomes with complete resolution of symptoms and radiological abnormalities within 3 months 1, 2
• Approximately 5-10% of patients may have permanent neurological deficits 1
• Rare cases may be fatal, particularly those with severe hemorrhagic complications 1