Intracranial aneurysms in Ehlers-Danlos syndrome type IV in early childhood

Pediatr Neurol. 2001 Oct;25(4):336-9. doi: 10.1016/s0887-8994(01)00315-0.


Ehlers-Danlos syndrome type IV is of special interest to neurologists because of the risk of cerebrovascular complications. We describe a 5-year-old female with Ehlers-Danlos syndrome type IV, demonstrating multiple intracranial aneurysms and right middle cerebral artery stenosis. The diagnosis of Ehlers-Danlos syndrome type IV was confirmed by electron microscopic examination of a skin biopsy. To our knowledge, this is the youngest reported patient with intracranial aneurysms associated with the Ehlers-Danlos syndrome type IV. Ehlers-Danlos syndrome type IV should be considered in the differential diagnosis of cerebrovascular disorder and stroke in early childhood.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Age of Onset
  • Biopsy
  • Cerebrovascular Disorders / diagnosis
  • Child, Preschool
  • Constriction, Pathologic
  • Diagnosis, Differential
  • Ehlers-Danlos Syndrome / complications
  • Ehlers-Danlos Syndrome / diagnosis*
  • Ehlers-Danlos Syndrome / pathology
  • Female
  • Humans
  • Intracranial Aneurysm / etiology*
  • Intracranial Aneurysm / pathology
  • Magnetic Resonance Angiography
  • Middle Cerebral Artery / pathology*
  • Skin / pathology*