Reversible cerebral vasoconstriction syndrome: rare or underrecognized in children?

Dev Med Child Neurol. 2013 Apr;55(4):385-9. doi: 10.1111/j.1469-8749.2012.04433.x. Epub 2012 Oct 15.


Reversible cerebral vasoconstriction syndrome (RCVS) is a clinicoradiological diagnosis comprising 'thunderclap' headaches and reversible segmental vasoconstriction of cerebral arteries, occasionally complicated by ischaemic or haemorrhagic stroke. We report a case of RCVS in a 13-year-old male with severe thunderclap headaches and no focal neurological signs. Brain imaging showed multiple posterior circulation infarcts; cerebral computed tomography, magnetic resonance imaging, and catheter angiography showed multifocal irregularity and narrowing, but in different arterial segments. Laboratory studies did not support a diagnosis of vasculitis. Symptoms resolved over 3 weeks; magnetic resonance angiography 3 months later was normal and remained so after 2 years. We highlight the typical clinical features of RCVS in this case and suggest that the diagnosis should be considered in children with thunderclap headaches or stroke syndromes where headache is a prominent feature, especially if cerebrovascular imaging studies appear to be evolving or discrepant.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Catheterization
  • Cerebral Angiography
  • Cerebral Arteries / diagnostic imaging
  • Cerebral Arteries / pathology*
  • Cerebral Arteries / physiopathology*
  • Cerebral Infarction / complications*
  • Cerebral Infarction / diagnosis*
  • Cerebral Infarction / diagnostic imaging
  • Cerebral Infarction / pathology
  • Cerebral Infarction / physiopathology
  • Diagnosis, Differential
  • Headache / drug therapy
  • Headache / etiology*
  • Headache / therapy
  • Humans
  • Magnetic Resonance Angiography
  • Male
  • Stroke / complications
  • Stroke / diagnosis
  • Syndrome
  • Tomography, X-Ray Computed
  • Vasoconstriction
  • Vasospasm, Intracranial / complications*
  • Vasospasm, Intracranial / diagnosis*
  • Vasospasm, Intracranial / diagnostic imaging
  • Vasospasm, Intracranial / pathology
  • Vasospasm, Intracranial / physiopathology