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Reversible Cerebral Vasoconstriction Syndrome
Aka: Reversible Cerebral Vasoconstriction Syndrome, RCVS, Postpartum Cerebral Angiopathy, CNS Pseudovasculitis, Migraine Angiopathy, Call-Fleming Syndrome
- Pathophysiology
- Idiopathic, medium-sized cerebral artery vasospasm
- Epidemiology
- Most commonly occurs in women in their 5th decade of life (40-50 years old)
- Risk Factors
- Pregnancy or Postpartum
- Medications
- Triptans (e.g. Sumatriptan)
- Selective Serotonin Reuptake Inhibitors
- Hypertension
- Decongestants
- Sympathomimetics (e.g. Cocaine, Methamphetamine)
- Symptoms
- Thunderclap Headache
- Recurrent, sudden onset Headaches over a 1-3 week period
- Nausea or Vomiting
- Photophobia
- Confusion
- Blurred Vision
- Associated Neurologic Effects (may not be transient if significant Hemorrhage or ischemia)
- Seizure
- Cerebrovascular Accident
- Subarachnoid Hemorrhage
- Differential Diagnosis
- See Thunderclap Headache
- Pregnancy Induced Hypertension
- Posterior Reversible Encephalopathy Syndrome (PRES)
- Slower onset of Headache (contrast with Thunderclap Headache in RCVS)
- Labs
- Lumbar Puncture
- Exclude Subarachnoid Hemorrhage (esp. if presentation >6 hours after onset of Thunderclap Headache)
- Imaging
- CT Head
- Exclude Intracranial Hemorrhage (lower test sensivity after 6 hours of Thunderclap Headache)
- CT Angiogram (or MR Brain/MRA Brain and Neck)
- May be diagnostic for RCVS
- Management
- Discontinue causative factors (e.g. Sympathomimetics)
- Manage related conditions
- Manage Uncontrolled Hypertension (or Pregnancy Induced Hypertension)
- Headache prophylaxis
- Calcium Channel Blockers
- Headache abortive care
- Oral Magnesium may be effective
- Prognosis
- Episodes decrease over time with treatment and with distance from precipitating factors
- However, minor Disability may persist in up to 29% of patients
- References
- Pensa and Roth in Herbert (2020) EM:Rap 20(12): 4-5
- Sattar (2010) Expert Rev Cardiovasc Ther 8(10): 1417-21. [PubMed]
- https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3020907/