Article Text

Download PDFPDF
Neurofibromatosis type 1 with subarachnoid haemorrhage from the left vertebral arteriovenous fistula: case presentation and literature review
  1. Toshinori Nishizawa1,2,
  2. Takahiro Tsuchiya3,
  3. Yoshihiro Terasawa2,4 and
  4. Yasuhiro Osugi2,5
  1. 1Department of General Internal Medicine, St Luke's International University, Chuo-ku, Japan
  2. 2Department of General Internal Medicine, Toyota Regional Medical Center, Toyota, Aichi, Japan
  3. 3Department of Neurosurgery, Tokyo University Hospital, Bunkyo-ku, Tokyo, Japan
  4. 4Department of General Internal Medicine, Kuchinotsu Hospital, Minamishimabara, Nagasaki, Japan
  5. 5Department of Community Based Medicine, Fujita Health University, Toyoake, Aichi, Japan
  1. Correspondence to Dr Toshinori Nishizawa; nishizawa.toshinori{at}


We present the case of a 47-year-old woman with neurofibromatosis type 1 (NF1) with subarachnoid haemorrhage (SAH) from the left vertebral arteriovenous fistula, along with a review of previous cases. Our patient had a family history of NF1 and presented to the emergency department with a sudden-onset severe headache and neck pain. CT scan showed SAH. CT angiography revealed a left vertebral arteriovenous fistula and an epidural haematoma. She underwent direct surgery and was discharged without neurologic deficits. To our knowledge, this is the first case of SAH caused by perimedullary drainage of a vertebral arteriovenous fistula associated with NF1. In a literature search, we identified 40 cases of vertebral arteriovenous fistula associated with NF1. The majority of vertebral arteriovenous fistulas occurred on the left side and in women. Patients with vertebral arteriovenous fistula typically experience neck pain, radiculopathy, radiculomyelopathy and bruits.

  • dermatology
  • general practice / family medicine
  • coma and raised intracranial pressure
  • headache (including migraines)

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.


  • Contributors TN took care of the patient and wrote the report. TN and TT reviewed 40 cases of vertebral arteriovenous fistula associated with neurofibromatosis type 1 in a literature search. YT and YO read and approved the final version of the report.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.