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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

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

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

Most patients need surgery to remove tumors. Tumors also can be treated with radiation.

Support Groups

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

See also: Drugs associated with Brain Tumor

Review Date: 10/25/2007
Reviewed By: Chad Haldeman-Englert, M.D., Division of Human Genetics, Children's Hospital of Philadelphia, Philadelphia, PA. Review provided by VeriMed Healthcare Network.