EMed

RCVS (Reversible Cerebral Vasoconstriction Syndrome)

Background

  • Recurrent thunderclap headache, ±seizures, ±strokes
  • Transient, reversible dysregulation of cerebral arterial tone with multifocal areas of cerebral vasoconstriction and dilation
  • Middle age
  • F > M

Clinical

Symptoms

  • Recurring thunderclap headache
  • ± seizures
  • ± strokes:
    • Infarcts 10%
    • Subcortical SAH (25%)
  • DDx SAH in recurring thunderclap

Precipitating

  • Sex
  • Vigorous exercise
  • Pregnancy
  • Drugs – e.g. cocaine, MDMA, cannabis
  • Vasoactive – SSRIs, nasal decongestants
  • Immunosuppressive agents or blood products
  • Hypercalcaemia

Differential Dx

  RCVS Primary CNS Vasculitis
CT Often normal initially Often normal initially
MRI Often normal initially Mostly abnormal initially
CSF Often normal, some ↑WBCs ↑WBCs, ↑protein
Onset Abrupt Insidious

Investigations

  • CT often normal
  • MRI often normal (early)
  • CTA may be normal (early)
  • Vasoconstriction/dilation progresses distal to proximal
  • Clasical CTA "sausages on a string" may appear late
CTA in RCVS

Management

  • Analgesia
  • Remove precipitans (e.g. cocaine/SSRIs)
  • Ca2+ channel blockers
  • Nimodipine 60mg orally every 6 hours or 1.2 mg/kg/hour IV
  • Triptans are contra-indicated

Prognosis

  • Good
  • CTA vasospasm normalises over time
  • Most make a complete recovery without recurrence

Content by Dr Íomhar O' Sullivan. Last review DrÍOS 25/09/24.