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Neurofibromatosis 2
NF2; Bilateral acoustic neurofibromatosis; Central bilateral acoustic NF
Neurofibromatosis 2 (NF2) is a disorder in which tumors form on the nerves of the brain and spine (the central nervous system). It is passed down (inherited) in families.
Although it has a similar name to neurofibromatosis type 1 , it is a different and separate condition.
Neurofibromatosis type 1
Neurofibromatosis-1 (NF1) is an inherited disorder in which nerve tissue tumors (neurofibromas) form in the: Bottom layer of skin (subcutaneous tissu...
Causes
NF2 can be passed down through families in an autosomal dominant pattern. This means that if 1 parent has NF2, any child of that parent has a 50% chance of inheriting the condition.
Autosomal dominant
Autosomal dominant is one of several ways that a trait or disorder can be passed down (inherited) through families. In an autosomal dominant disease,...
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 (café-au-lait)
- Headaches
- Hearing loss
- Ringing and noises in the ears
- Weakness of the face
Exams and Tests
Signs of NF2 include:
- Brain and spinal tumors
- Hearing-related (acoustic) tumors
- Skin tumors
Tests include:
- Genetic testing
- Medical history
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MRI
MRI
A magnetic resonance imaging (MRI) scan is an imaging test that uses powerful magnets and radio waves to create pictures of the body. It does not us...
ImageRead Article Now Book Mark Article - Physical examination
Treatment
Acoustic tumors can be observed, or treated with surgery or radiation .
Radiation
Radiation therapy uses high-powered x-rays, particles, or radioactive seeds to kill cancer cells.
People with this disorder may benefit from genetic counseling.
People with NF2 should be regularly evaluated with these tests:
- MRI of the brain and spinal cord
- Hearing and speech evaluation
- Eye exam
Support Groups
A support group can be helpful for emotional support and for giving and receiving practical advice. For information and support, visit www.ctf.org .
References
Evans DG. Neurofibromatosis 2. GeneReviews . Seattle, WA; University of Washington; 2011:8. www.ncbi.nlm.nih.gov/books/NBK1201/ . Accessed: October 24, 2015.
Sahin M. Neurocutaneous syndromes. In: Kliegman RM, Stanton BF, St Geme III JW, Schor NF, eds. Nelson Textbook of Pediatrics . 20th ed. Philadelphia, PA: Elsevier; 2016:chap 596.
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Central nervous system - illustration
The central nervous system is comprised of the brain and spinal cord. The peripheral nervous system includes all peripheral nerves.
Central nervous system
illustration
Review Date: 10/27/2015
Reviewed By: Chad Haldeman-Englert, MD, FACMG, Fullerton Genetics Center, Asheville, 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.
