Reversible Cerebral Vasoconstriction Syndrome

Aka: Reversible Cerebral Vasoconstriction Syndrome, RCVS, Postpartum Cerebral Angiopathy, CNS Pseudovasculitis, Migraine Angiopathy, Call-Fleming Syndrome

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II. Pathophysiology

  1. Idiopathic, medium-sized cerebral artery vasospasm

III. Epidemiology

  1. Most commonly occurs in women in their 5th decade of life (40-50 years old)

IV. Risk Factors

  1. Pregnancy or Postpartum
  2. Medications
    1. Triptans (e.g. Sumatriptan)
    2. Selective Serotonin Reuptake Inhibitors
    3. Hypertension
    4. Decongestants
    5. Sympathomimetics (e.g. Cocaine, Methamphetamine)

V. Symptoms

  1. Thunderclap Headache
    1. Recurrent, sudden onset Headaches over a 1-3 week period
    2. Nausea or Vomiting
    3. Photophobia
    4. Confusion
    5. Blurred Vision
  2. Associated Neurologic Effects (may not be transient if significant Hemorrhage or ischemia)
    1. Seizure
    2. Cerebrovascular Accident
    3. Subarachnoid Hemorrhage

VI. Differential Diagnosis

  1. See Thunderclap Headache
  2. Pregnancy Induced Hypertension
  3. Posterior Reversible Encephalopathy Syndrome (PRES)
    1. Slower onset of Headache (contrast with Thunderclap Headache in RCVS)

VII. Labs

  1. Lumbar Puncture
    1. Exclude Subarachnoid Hemorrhage (esp. if presentation >6 hours after onset of Thunderclap Headache)

VIII. Imaging

  1. CT Head
    1. Exclude Intracranial Hemorrhage (lower test sensivity after 6 hours of Thunderclap Headache)
  2. CT Angiogram (or MR Brain/MRA Brain and Neck)
    1. May be diagnostic for RCVS

IX. Management

  1. Discontinue causative factors (e.g. Sympathomimetics)
  2. Manage related conditions
    1. Manage Uncontrolled Hypertension (or Pregnancy Induced Hypertension)
  3. Headache prophylaxis
    1. Calcium Channel Blockers
  4. Headache abortive care
    1. Oral Magnesium may be effective

X. Prognosis

  1. Episodes decrease over time with treatment and with distance from precipitating factors
  2. However, minor Disability may persist in up to 29% of patients

XI. References

  1. Pensa and Roth in Herbert (2020) EM:Rap 20(12): 4-5
  2. Sattar (2010) Expert Rev Cardiovasc Ther 8(10): 1417-21. [PubMed]
    1. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3020907/

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