Multiple Cerebral Aneurysms Associated With Neurofibromatosis Type 1

J Stroke Cerebrovasc Dis. 2019 Jul;28(7):e83-e91. doi: 10.1016/j.jstrokecerebrovasdis.2019.04.019. Epub 2019 May 9.

Abstract

Neurofibromatosis type 1 (NF1) is a rare disease with an incidence of approximately one in every 3000 births. NF1 is mainly recognized as a tumor suppressor. Vasculopathy in NF1 is well described in the literature, but the association between NF1 and cerebral aneurysm has not been determined. We report a case of a 67-year-old female with NF1 accompanied by 8 cerebral aneurysms. Two of the patient's unruptured aneurysms, the large distal anterior cerebral artery (ACA) aneurysm and anterior communicating artery aneurysm, were initially treated with microsurgical clipping. The peripheral ACA aneurysm gradually increased in size and ruptured after 5 years. Coil embolization was performed for the ruptured aneurysm. Four of the 5 remaining unruptured aneurysms were treated surgically. The patient is currently well, without neurological deficit, and coil embolization is scheduled for the last remaining aneurysm. NF1 is a probable risk factor for multiple cerebral aneurysms due to vessel wall vulnerability. Therapeutic indications for patients with NF1 who show multiple cerebral aneurysms include strict follow-up and aggressive treatment to avoid subarachnoid hemorrhage.

Keywords: Multiple cerebral aneurysms; neurofibromatosis type 1; subarachnoid hemorrhage; therapeutic indication.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Aneurysm, Ruptured / diagnostic imaging
  • Aneurysm, Ruptured / etiology*
  • Aneurysm, Ruptured / therapy
  • Cerebral Angiography / methods
  • Computed Tomography Angiography
  • Embolization, Therapeutic
  • Endovascular Procedures
  • Female
  • Humans
  • Intracranial Aneurysm / diagnostic imaging
  • Intracranial Aneurysm / etiology*
  • Intracranial Aneurysm / therapy
  • Neurofibromatosis 1 / complications*
  • Neurofibromatosis 1 / diagnosis
  • Neurosurgical Procedures
  • Risk Factors
  • Treatment Outcome