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    Neurofibromatosis 2

    NF2; Bilateral acoustic neurofibromatosis; Central bilateral acoustic NF

    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)

    Causes

    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

    Exams 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.

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    • Central nervous system

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      • Central nervous system

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      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.

      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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