Neurofibromatosis 2 (NF2) is a genetic disorder in which tumors form on the nerves of the brain and spine (the central nervous system).
Although it has a similar name to neurofibromatosis type 1, it is a different and separate condition.
Causes of Neurofibromatosis 2
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% chance of inheriting the condition.
The main risk factor is having a family history of the condition.
Neurofibromatosis 2 Symptoms
Symptoms of NF2 include:
- Balance problems
- Cataracts at a young age
- Changes in vision
- Coffee-colored marks on the skin (café-au-lait)
- Hearing loss
- Ringing and noises in the ears
- Weakness of the face
Tests and Exams
- Brain and spinal tumors
- Hearing-related (acoustic) tumors
- Skin tumors
- Genetic testing
- Medical history
- Physical examination
Treatment of Neurofibromatosis 2
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
For information and support, visit www.ctf.org.
Ferner RE. Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective. Lancet Neurol. 2007;6:340-351.
Sahin M. Neurocutaneous syndromes. In: Kliegman RM, Stanton BF, St. Geme III JW, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 589.
|Review Date: 10/29/2013
Reviewed By: Chad Haldeman-Englert, MD, FACMG, 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, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.