 |
Back to Health Library Print This Page  Email to a Friend  |
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, incidence, and risk factors
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
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.
Reviewed By: Luc Jasmin, MD, PhD, Departments of Anatomy and Neurological Surgery, University of California, San Francisco, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
