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

Workup and Treatment of Reversible Cerebral Vasoconstriction Syndrome (RCVS)

Calcium channel blockers, particularly nimodipine, are the first-line treatment for RCVS, with glucocorticoids being contraindicated as they have been reported to worsen outcomes. 1

Diagnostic Workup

Clinical Presentation

• Recurrent thunderclap headaches (severe, sudden-onset headaches reaching maximum intensity within 1 minute)
• Headaches typically recur daily for approximately 2 weeks 2
• May be accompanied by:
  • Neurological deficits
  • Blood pressure surges (in about one-third of patients) 2
  • Nausea/vomiting
  • Photophobia/phonophobia

Risk Factors to Identify

• Vasoactive substances (present in over 50% of cases) 1:
  • Recreational drugs (especially cannabis)
  • SSRIs (selective serotonin reuptake inhibitors)
  • Nasal decongestants
  • Triptans
  • Sympathomimetic drugs 3
• Postpartum state
• Physical triggers:
  • Sexual activity
  • Exertion
  • Coughing
  • Showering/bathing 4

Imaging Studies

1. First-line imaging:

   • MRI with MR angiography (MRA) and MR venography (MRV) 2
   • CT angiography (CTA) if MRI unavailable

2. Gold standard (but not routinely recommended):

   • Cerebral catheter digital subtraction angiography (DSA) 4

3. Transcranial Doppler:

   • Patients with mean flow velocity >120 cm/s in middle cerebral artery have higher risk of ischemic complications 2

Laboratory Tests

• Complete blood count
• Inflammatory markers (ESR, CRP) to help differentiate from vasculitis 3
• Toxicology screen (to identify potential triggers) 3
• Lumbar puncture (if subarachnoid hemorrhage suspected) 2

Treatment Approach

Immediate Management

1. Remove precipitating factors:

   • Discontinue all vasoactive substances 1
   • Identify and treat any underlying conditions

2. First-line pharmacological therapy:

   • Calcium channel blockers:
     • Nimodipine (60 mg every 4-6 hours) 1, 4
     • Verapamil (alternative option)
   • Continue for 4-12 weeks with gradual tapering

3. Pain management:

   • Analgesics for headache control
   • Avoid vasoconstrictive medications

What to Avoid

• Glucocorticoids: Independent predictor of worse outcomes, should be avoided 1
• Triptans and other vasoconstrictors: Can worsen vasoconstriction
• Unnecessary invasive procedures: Catheter angiography should not be implemented routinely 2

Management of Complications

• Subarachnoid hemorrhage: Supportive care, pain control
• Ischemic stroke: Standard stroke management protocols
• Posterior reversible encephalopathy syndrome (PRES): Blood pressure control, removal of precipitating factors

Severe/Refractory Cases

• Consider intra-arterial therapies in deteriorating cases 1
• Intensive care monitoring for patients with neurological deterioration

Monitoring and Follow-up

• Clinical monitoring for resolution of headaches (typically within 1-3 weeks)
• Follow-up imaging at 12 weeks to confirm resolution of vasoconstriction 1
• Monitor for rare cases of relapse (reported in a small proportion of patients) 2

Prognosis

• Majority of patients (90-95%) have favorable outcomes with complete resolution 1
• Approximately 5-10% may have permanent neurological deficits
• Death is rare but possible in severe cases 1
• Complications occur in different timeframes:
  • Early complications (first week): Cortical subarachnoid hemorrhage (22%), intracerebral hemorrhage (6%), seizures (3%), PRES (9%)
  • Later complications (second week): TIAs (16%), cerebral infarction (4%) 5

Special Considerations

• Women tend to have higher rates of stroke and cortical subarachnoid hemorrhage 5
• RCVS can be confused with primary angiitis of the central nervous system (PACNS) or aneurysmal subarachnoid hemorrhage, so careful differentiation is essential 1, 6

Remember that early recognition and appropriate management, including calcium channel blockers and removal of triggers, are crucial for improving outcomes in patients with RCVS.