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Neurofibromatosis Type 2

Authors:
  • Cynthia M. Johnson, MA
Publication Type:

Condition

Neurofibromatosis Type 2

(NF2)

Definition

Neurofibromatosis is an inherited problem that causes tumors to form in the nervous system. They form in the nerves or the tissue around them. Neurofibromatosis has three types: neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis. The type a person has depends on the genes that are affected.

This article focuses on NF2. These tumors tend to form in the central nervous system. The eighth cranial nerve is the one most affected. This can lead to problems with hearing and balance.

Central Nervous System.

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Causes

NF2 is caused by a problem with a gene that makes proteins to help control nerve growth. The gene is not able to make these proteins and tumors form.

In most people, the gene issue passes from a parent to their child. The gene change can also happen in a person who does not have family members with NF2.

Risk Factors

This risk is highest in people with other family who have NF2.

Symptoms

The problems a person has depends on the tumor size and where it is. Smaller ones may not cause symptoms.

A person with NF2 may have:

  • Hearing loss or ringing in the ears (tinnitus)
  • Poor balance
  • Headaches
  • Pain or numbness in the face
  • Problems seeing, such as double or blurry eyesight
  • Weakness or tingling in the arms or legs
  • Bumps under the skin or small flesh colored skin flaps
  • Dark, rough, hairy patches over raised skin

Diagnosis

The doctor will ask about symptoms and health history. The doctor may ask if any family members have NF2. A physical exam will be done. It may take a few years before NF2 is diagnosed.

These tests may be done:

  • Blood tests to look for signs of the gene problem
  • An MRI scan to look for tumors
  • Electrodiagnostic testing to check the electrical activity of the muscles and nerves
  • A brainstem auditory evoked response (BAER) test to measure brainwave activity

Treatment

The goals of treatment are to:
  • Manage any problems the tumors cause
  • Remove any tumors causing problems
There is no cure for NF2. People who do not have symptoms may not need to be treated. The tumors grow slowly and rarely turn into cancer. A doctor who treats NF2 can watch for any tumor changes.

People who do have symptoms may need:

  • Radiation and medicines to shrink tumors
  • Surgery to remove tumors or repair related problems, such as hearing loss or cataracts

Prevention

NF2 often cannot be prevented. People who have a family history of NF2 may want to see a genetic counselor before having children.

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RESOURCES:

CANADIAN RESOURCES:

CANADIAN RESOURCES:

References

  • About NF2. Children's Tumor Foundation website. Available at: https://www.ctf.org/understanding-nf/nf2.
  • Neurofibromatosis type 2. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/neurofibromatosis-type-2.
  • Neurofibromatosis. National Institute of Neurological Disorders and Stroke website. Available at: https://www.ninds.nih.gov/health-information/disorders/neurofibromatosis.
  • Tamura, R. Current understanding of neurofibromatosis 1, 2, and Schwannomatosis. International Journal of Molecular Sciences, 2021; 22(11): 5850.

Contributors

  • James P. Cornell, MD
Last Updated:
2022-08-01

(C) Copyright 2022 EBSCO Information Services

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.