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

Treatment for Reversible Cerebral Vasoconstriction Syndrome (RCVS)

Calcium channel blockers, particularly nimodipine, are the first-line treatment for RCVS, while corticosteroids should be avoided as they have been associated with worse outcomes. 1

Diagnosis and Clinical Presentation

RCVS is characterized by:

• Recurrent thunderclap headaches (severe, sudden-onset headaches)
• Reversible segmental narrowing of cerebral arteries
• Resolution of vasoconstriction within 3 months

Key diagnostic features:

• Multiple thunderclap headaches recurring over approximately 1 week (94% of patients) 2
• Vasoconstriction visible on cerebral angiography ("string and beads" appearance)
• Normal or near-normal cerebrospinal fluid analysis (to differentiate from aneurysmal subarachnoid hemorrhage)

Treatment Algorithm

First-line Management:

1. Eliminate precipitating factors:

   • Discontinue vasoactive substances (cannabis, selective serotonin reuptake inhibitors, nasal decongestants) 2
   • Identify and manage other triggers (pregnancy/postpartum state, exertion, sexual activity)

2. Calcium channel blockers:

   • Nimodipine: 60 mg orally every 4-6 hours for 4-12 weeks 1, 2
   • Verapamil: Alternative option if nimodipine is not available

3. Supportive care:

   • Bed rest
   • Analgesics for headache management
   • Blood pressure control if hypertensive

Important Treatment Considerations:

• DO NOT use corticosteroids: They are independently associated with worse outcomes in RCVS and should be avoided 1, 3
• Monitor for complications: One-third to half of cases develop hemorrhagic or ischemic brain lesions 1
• Timing of complications: Hemorrhagic complications typically occur early (first week), while ischemic events occur later (second week) 2

For Severe or Deteriorating Cases:

• Consider intra-arterial therapy for severe, refractory cases 4
• Neurointerventional techniques should be reserved for severe deteriorating cases 1
• Intensive care monitoring for patients with neurological deficits or complications

Monitoring and Follow-up

• Transcranial Doppler: Patients with mean flow velocity of middle cerebral artery >120 cm/s have higher risk of ischemic complications 4
• Follow-up neuroimaging (MRA or CTA) at 3 months to confirm resolution of vasoconstriction
• Most patients recover without sequelae, though approximately 5-10% may have permanent neurological deficits 1

Differential Diagnosis

RCVS must be differentiated from:

• Aneurysmal subarachnoid hemorrhage
• Primary angiitis of the central nervous system (PACNS)
• Cervical artery dissection
• Posterior reversible encephalopathy syndrome (PRES), which often co-occurs with RCVS

Prognosis

• Generally favorable with appropriate management
• Approximately 5-10% of patients may have permanent neurological deficits 1
• Relapse occurs in a small proportion of patients (approximately 5%) 3
• Mortality is rare but has been reported

The management of RCVS focuses on supportive care, removal of triggers, and calcium channel blockers, with careful monitoring for complications that may occur at different time points during the course of the syndrome.