Reference Index - Disease & Conditions

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Central nervous system
Central nervous system


Neurofibromatosis 2

Definition:

Neurofibromatosis 2 (NF2) is a genetic disorder in which tumors form on the nerves of the brain and spine (the central nervous system).

See also: Neurofibromatosis 1 (NF1)



Alternative Names:

NF2; Bilateral acoustic neurofibromatosis; Central bilateral acoustic NF



Causes, incidence, and risk factors:

NF2 is passed down through families in an autosomal dominant fashion. This means that if one parent has NF2, any child of that parent has a 50-50 chance of having it.

The main risk factor is having a family history of the condition.



Symptoms:

Symptoms of NF2 include:

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


Signs and tests:

Signs include:

  • Brain and spinal tumors
  • Hearing-related (acoustic) tumors
  • Skin tumors

Tests include:

  • Genetic testing
  • Medical history
  • MRI
  • Physical examination


Treatment:

Acoustic neuromas can be observed, or treated with surgery or radiation .

Patients may benefit from genetic counseling.

Every year, patients with NF2 should be evaluated with:

  • MRI of the brain and spinal cord
  • Hearing and speech evaluation
  • Eye exam


Support Groups:

For information and support, visit www.nf.org .



Expectations (prognosis):



Complications:



Calling your health care provider:



Prevention:



References:

Ferner RE. Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective. Lancet Neurol. 2007;6:340-351.

Haslam RHA. Neurocutaneous syndromes. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 596.




Review Date: 10/14/2009
Reviewed By: Luc Jasmin, MD, PhD, Departments of Anatomy and Neurological Surgery, University of California, San Francisco, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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