Neurofibromatosis 2

Definition

Neurofibromatosis 2 (NF2) is a genetic disorder that causes tumors to form on the nerves of thebrain and spine (the central nervous system).

See also neurofibromatosis 1 (NF1).

Alternative Names

NF2; Bilateral Acoustic Neurofibromatosis (BAN); Central Bilateral Acoustic NF

Causes, incidence, and risk factors

NF2 is inherited, and the main risk factor is having a family history of NF2. Inheritance is autosomal dominant. This means that ifone parent has NF2, any child of that parent has a 50-50 chance of having it.

Symptoms

  • Hearing loss
  • Ringing and noises in the ears
  • Balance problems
  • Cataracts at a young age
  • Changes in vision
  • Headaches
  • Facial weakness
  • Coffee-colored marks on the skin

Signs and tests

Signs include:

  • Brain and spinal tumors
  • Acoustic (related to hearing) tumors
  • Skin tumors

Tests include:

  • Physical examination
  • Medical history
  • MRI
  • Genetic testing

Treatment

Most patients require surgery to remove tumors. Tumors also can be treated with radiation.

Support Groups

For information and support, see www.nf.org

Central nervous system
Central nervous system
Review Date: 11/21/2005
Reviewed By: Christos Ballas, M.D., Attending Psychiatrist, Hospital of the University of Pennsylvania, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
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