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Neurofibromatosis 2

NF2; Bilateral acoustic neurofibromatosis; Central bilateral acoustic NF

 

Neurofibromatosis 2 (NF2) is a disorder in which tumors form on the nerves of the brain and spine (the central nervous system). It is passed down (inherited) in families.

Although it has a similar name to neurofibromatosis type 1, it is a different and separate condition.

Causes

 

NF2 can be passed down through families in an autosomal dominant pattern. This means that if 1 parent has NF2, any child of that parent has a 50% chance of inheriting the condition.

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 (café-au-lait)
  • Headaches
  • Hearing loss
  • Ringing and noises in the ears
  • Weakness of the face

 

Exams and Tests

 

Signs of NF2 include:

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

Tests include:

  • Genetic testing
  • Medical history
  • MRI
  • Physical examination

 

Treatment

 

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

People with this disorder may benefit from genetic counseling.

People with NF2 should be regularly evaluated with these tests:

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

 

Support Groups

 

A support group can be helpful for emotional support and for giving and receiving practical advice. For information and support, visit www.ctf.org.

 

 

References

Evans DG. Neurofibromatosis 2. GeneReviews. Seattle, WA; University of Washington; 2011:8. www.ncbi.nlm.nih.gov/books/NBK1201/. Accessed: October 24, 2015.

Sahin M. Neurocutaneous syndromes. In: Kliegman RM, Stanton BF, St Geme III JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 596.

 
  • Central nervous system

    Central nervous system - illustration

    The central nervous system is comprised of the brain and spinal cord. The peripheral nervous system includes all peripheral nerves.

    Central nervous system

    illustration

    • Central nervous system

      Central nervous system - illustration

      The central nervous system is comprised of the brain and spinal cord. The peripheral nervous system includes all peripheral nerves.

      Central nervous system

      illustration

    A Closer Look

     

       

      Review Date: 10/27/2015

      Reviewed By: Chad Haldeman-Englert, MD, FACMG, Fullerton Genetics Center, Asheville, NC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

      The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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