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Neurofibromatosis 2


Definition Alternative Names Causes, incidence, and risk factors Symptoms Signs and tests Treatment Support Groups References

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 pattern. 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
  • Headaches
  • Hearing loss
  • Ringing and noises in the ears
  • Weakness of the face

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.

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.

Physician Reference

International Classification of Diseases, 9th Revision (ICD9)
237.72
Review Date: 11/14/2011
Reviewed By: Chad Haldeman-Englert, MD, Wake Forest School of Medicine, Department of Pediatrics, Section on Medical Genetics, Winston-Salem, NC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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