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Topic: Neurofibromatosis Type 2


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In the News (Wed 23 Dec 09)

  
  AANS.org | Education and Meetings | AANS Scientific Journals | Neurosurgical Focus
Neurofibromatosis type 1 is present in approximately 100,000 people in the United States and so is the most common single-gene disease.
Neurofibromatosis type 1 is characterized by multiple cutaneous and subcutaneous neurofibromas, ranging from a few (five--six required for diagnosis) to an uncountable number (Fig.
Multiple microneuromas, averaging 2 to 3 mm in diameter, were left in situ in the ninth, 10th, and 11th cranial nerves, and a l-cm neuroma was left in situ in the fifth cranial nerve.
www.aans.org /education/journal/neurosurgical/mar98/4-3-1.asp   (19462 words)

  
 Neurofibromatosis 2 - Genetics Home Reference
Neurofibromatosis 2 is a disorder in which noncancerous tumors called schwannomas develop in cells that form the protective covering for nerves in the inner ear.
Mutations in the NF2 gene cause neurofibromatosis 2.
Neurofibromatosis 2 is considered to have an autosomal dominant pattern of inheritance because affected people may have affected relatives in successive generations (such as parents and children).
ghr.nlm.nih.gov /condition=neurofibromatosis2   (754 words)

  
 Rare Pediatric Disease Database
Neurofibromatosis type 2 (NF2) is a genetic disorder that is characterized by the presence of tumors that grow on both sides of the 8th cranial nerve which is responsible for hearing.
This condition was once called “bilateral acoustic neurofibromatosis or central neurofibromatosis.” The 8th cranial nerve is named the vestibulocochlear nerve for its two parts, the vestibular and cochlear parts of the inner ear where sound is transmitted to the brain for hearing.
The treatment of NF 2 is usually surgical removal of the enlarged tumors on the 8th auditory nerve.
www.madisonsfoundation.org /content/3/1/display.asp?did=276   (619 words)

  
 The involvement of calpain-independent proteolysis of the tumor suppressor NF2 (merlin) in schwannomas and meningiomas ...   (Site not responding. Last check: 2007-10-09)
Neurofibromatosis type 2 (NF2) protein, also known as merlin or schwannomin, is a tumor suppressor, and NF2 is mutated in most schwannomas and meningiomas.
The neurofibromatosis type 2 gene is inactivated in schwannomas.
Neurofibromatosis type 2 (NF2) gene is somatically mutated in mesothelioma but not in lung cancer.
www.nature.com /doifinder/10.1038/nm0898-915   (965 words)

  
 Neurofibromatosis
Neurofibromatosis, type 2, (NF-2) is a rare inherited disorder characterized by the development of benign tumors on both auditory nerves (acoustic neuromas).
NF-2 is an autosomal dominant genetic trait, meaning it affects both genders equally and that each child of an affected parent has a 50% chance of inheriting the gene.
We are learning more about the function of the NF2 gene through studies of families with neurofibromatosis type 2 and through work in model organisms, particularly mice.
www.ncbi.nlm.nih.gov /books/bv.fcgi?call=bv.View..ShowSection&rid=gnd.section.210   (212 words)

  
 Paediatric presentation of type 2 neurofibromatosis -- Evans et al. 81 (6): 496 -- Archives of Disease in Childhood
Neurofibromatosis type 2 (NF2) was first described in 1820 by the Scottish surgeon JH Wishart.
Type of mutation in the neurofibromatosis type 2 gene (NF2) frequently determines severity of disease.
Differential diagnosis of type 2 neurofibromatosis: molecular discrimination of NF2 and sporadic vestibular schwannomas.
adc.bmjjournals.com /cgi/content/full/81/6/496   (2538 words)

  
 Neurofibromatosis   (Site not responding. Last check: 2007-10-09)
NF1 Gene and Neurofibromatosis Type 1 - by Sonja A. Rasmussen and J.M. Friedman.
Neurofibromatosis, Type I - from University of Maryland.
Neurofibromatosis Type 1 in Past Populations: Detection and Impact of the Disorder - by Elizabeth Finnis, Department of Anthropology, McMaster University.
www.genomelink.org /neurofibromatosis   (1262 words)

  
 eMedicine - Neurofibromatosis, Type 2 : Article by Beth A Pletcher, MD   (Site not responding. Last check: 2007-10-09)
Background: Central neurofibromatosis or neurofibromatosis type 2 (NF2) is a multisystem genetic disorder associated with bilateral vestibular schwannomas, spinal cord schwannomas, meningiomas, gliomas, and juvenile cataracts with a paucity of cutaneous features, which are seen more consistently in neurofibromatosis type 1 (NF1).
Meningioma to the left of midline in a patient with neurofibromatosis type 2
Aguiar PH, Tatagiba M, Samii M: The comparison between the growth fraction of bilateral vestibular schwannomas in neurofibromatosis 2 (NF2) and unilateral vestibular schwannomas using the monoclonal antibody MIB 1.
www.emedicine.com /neuro/topic496.htm   (4021 words)

  
 Neurofibromatosis (Types 1 & 2)   (Site not responding. Last check: 2007-10-09)
The most common of the neurocutaneous syndromes (phakomatoses), consisting of Neurofibromatosis Type 1 (1 in 3,000) and Neurofibromatosis Type 2 (1 in 50,000).
Type 2 (NF 2) is also known as bilateral acoustic neurofibromatosis
The NF 2 gene mapped on chromosome 22.
www.5mcc.com /Assets/SUMMARY/TP1064.html   (136 words)

  
 ACR Learning File Web
Axial T2- weighted images (Images 2 and 3; 2900/90/1) reveal an extra-axial right-sided parafalcine mass (Image 3, scan 3) which was poorly seen on the contrasted T1-weighted image (not shown).
The presence of bilateral acoustic schwannomas establishes the diagnosis of neurofibromatosis-2, the central form of neurofibromatosis.
One fourth of intracranial meningiomas in the pediatric population occur in patients with neurofibromatosis.
www.learningfile.com /learning_file/viewcase.php?section=nu&case_num=422   (568 words)

  
 Untitled
Neurofibromatosis type 2 protein co-localizes with elements of the cytoskeleton.
Neurofibromatosis 2 antisense oligodeoxynucleotides induce reversible inhibition of schwannomin synthesis and cell adhesion in STS26T and T98G cells.
A missense mutation in the neurofibromatosis 2 gene occurs in patients with mild and severe phenotypes.
ethesis.helsinki.fi /julkaisut/laa/haart/vk/sainio/lahteet.html   (5201 words)

  
 Neurofibromatosis Type 2 (NF-2)
Neurofibromatosis Type 2 (NF-2) is a rare genetic disorder that is primarily characterized by benign (noncancerous) tumors of the nerves that transmit sound impulses from the inner ears to the brain (bilateral acoustic neuromas vestibular schwannomas).
The term "Neurofibromatosis" is sometimes also used to describe a second, distinct form of NF known as Neurofibromatosis Type I (NF-1).
More common than NF-2, NF-1 is primarily characterized by the development of multiple noncancerous (benign) tumors of nerves and skin (neurofibromas) and areas of abnormally decreased or increased coloration (hypo- or hyperpigmentation) of the skin, such as pale tan or light brown discolorations (cafe-au-lait spots) on the skin of the trunk or other regions.
www.webmd.com /hw/ear_disorders/nord792.asp   (663 words)

  
 British Columbia Neurofibromatosis Foundation -   (Site not responding. Last check: 2007-10-09)
Neurofibromatosis 2 (NF2) is a genetic disorder in which tumors called schwannomas are found on nerves in the inner ear.
Neurofibromatosis type 2 is far less common than neurofibromatosis1 affecting about 1 in 40,000 births.
One of the tumor types associated with NF2 (the meningiomas) is known to frequently be responsive to female hormones in its non-genetic form.
www.bcnf.bc.ca /nf2.html   (1679 words)

  
 Acoustic Neurofibromatosis - Discussion Message Board Forum   (Site not responding. Last check: 2007-10-09)
Molecular genetics of neurofibromatosis 2 and related tumors.of medical sciences in the field of neurofibromatosis from the National.committee for NIH Consensus Conference on Acoustic Neuroma.
Neurofibromatosis type 1NF1 has a.imaging method of choice in children with neurofibromatosis based on its high.
Neurofibromatosis type 2 Definitions via.Code translations and terms via UMLS.Neurofibromatosis type 2 specific web sites.Send Neurofibromatosis type 2 to medical search.It was formerly called bilateral acoustic neurofibromatosis or central.Scientists have classified the disorders as neurofibromatosis type 1 NF1 and.4600 8009426825.Fax 3019180009.
www.webula.net /boards?b=web_Acoustic_Neurofibromatosis_1109988320   (595 words)

  
 © The Centre for Genetics Education   (Site not responding. Last check: 2007-10-09)
The two types of neurofibromatosis are called type 1 (NF1) and type 2 (NF2).
Neurofibromatosis type 2 (NF2) is much less common than NF1 with an estimated birth frequency of 1 in 33-40,000.
In some cases, the schwannomas develop on only one side (unilateral) and other nerves may be affected by different types of tumours that impact on the control of swallowing, speech, eye movements and facial sensations.
www.genetics.com.au /factsheet/41.htm   (1093 words)

  
 Occurrence and characterization of peripheral nerve involvement in neurofibromatosis type 2 -- Sperfeld et al. 125 (5): ...
Occurrence and characterization of peripheral nerve involvement in neurofibromatosis type 2 -- Sperfeld et al.
Occurrence and characterization of peripheral nerve involvement in neurofibromatosis type 2
Neurofibromatosis type 2 (NF2) is a rare autosomal dominant
brain.oxfordjournals.org /cgi/content/abstract/125/5/996   (368 words)

  
 Neurofibromatosis - CancerIndex
Neurofibromatosis 2 (NF2) is much rarer, this is characterised by multiple tumours on the nerves of the brain and spine, and can affect hearing.
International Neurofibromatosis Association The Association aims to promote awareness of neurofibromatosis, encourage collaborations among scientists and NF organisations throughout the world.
Neurofibromatosis, type 2 and the NF2 gene (National Center for Biotechnology Information) an overview of neurofibromatosis and the NF2 oncogene with links to other gene databases.
www.cancerindex.org /clinks6c.htm   (323 words)

  
 Journal of Neuroscience Nursing: Neurofibromatosis type 2--living with the complications: a case study.@ HighBeam ...
Neurofibromatosis type 2--living with the complications: a case study.
More than nine different types of neurofibromatosis have been identified, but other than types 1 and 2, they are quite rare.
All types of neurofibromatosis persist for a lifetime, so nurses from different backgrounds may encounter such a...
highbeam.com /library/doc0.asp?docid=1G1:135815915&refid=ink_tptd_mag   (221 words)

  
 Focal amyotrophy in neurofibromatosis 2 -- Trivedi et al. 69 (2): 257 -- Journal of Neurology, Neurosurgery, and ...
Neurofibromatosis type 2 (NF2) is an autosomal dominant disorder characterised by bilateral vestibular schwannomas and other
Neurofibromatosis type 2 (NF2), formerly known as bilateral acoustic or central neurofibromatosis, is established as a distinct
Neurofibromatosis type 2 in an adolescent boy with a polyneuropathy and a mutation in the NF2 gene.
jnnp.bmjjournals.com /cgi/content/full/69/2/257   (2370 words)

  
 Abstracts: Neurofibromatosis type 2 protein merlin is highly expressed in human fibroblasts and linked to the plasma ...
Abstracts: Neurofibromatosis type 2 protein merlin is highly expressed in human fibroblasts and linked to the plasma membrane
Neurofibromatosis type 2 (NF2) is an autosomal dominant disorder characterized by bilateral vestibular schwannomas and meningiomas.
In summary, merlin is concentrated in or linked to the plasma membrane and the localization in the spreading membranes may indicate a cell-substrate adhesion function.
hum-molgen.org /documents/abstracts/0045.html   (336 words)

  
 Ear, Nose & Throat Journal: Rapid growth of acoustic neuromas after stereotactic radiotherapy in type 2 ...
We describe a rare complication of stereotactic radiotherapy for large acoustic neuromas in a patient with type 2 neurofibromatosis.
The patient was diagnosed with type 2 neurofibromatosis, and she underwent stereotactic radiotherapy for treatment of the two acoustic neuromas; the jugular foramen tumors were not irradiated.
Stereotactic radiosurgery in the management of acoustic neuromas associated with neurofibromatosis type 2.
www.findarticles.com /p/articles/mi_m0BUM/is_12_81/ai_95954201   (1355 words)

  
 Expression of the Neurofibromatosis Type 2 Gene in Human Tissues -- den Bakker et al. 47 (11): 1471 -- Journal of ...
The neurofibromatosis Type 2 tumor suppressor gene is implicated
Claudio JO, Marineau C, Rouleau GA (1994) The mouse homologue of the neurofibromatosis type 2 gene is highly conserved.
Huynh DP, Pulst SM (1996) Neurofibromatosis 2 antisense oligodeoxynucleotides induce reversible inhibition of schwannomin synthesis and cell adhesion in STS26T and T98G cells.
www.jhc.org /cgi/content/full/47/11/1471   (4425 words)

  
 Neurofibromatosis Type 2 - MedPix™ Medical Image Database and Teaching Files   (Site not responding. Last check: 2007-10-09)
Neurofibromatosis 2 (NF2) is an autosomal dominant disorder characterized by a high incidence of bilateral vestibular Schwannomas (neurilemmoma, aka 'acoustic neuroma') (96%) as well as Schwannomas of other cranial and peripheral nerves, and other benign intracranial tumors including meningiomas, ependymomas, spinal 'neurofibromas', and gliomas.
NF 2 should be suspected in any patient with a family history or anyone younger than age 30 with an vestibular Schwannoma.
Neurofibromatosis 2 (NF2) tumor suppressor schwannomin and its interacting protein HRS regulate STAT signaling.
rad.usuhs.mil /medpix/medpix.html?mode=single&comebackto=mode=geo_browse&recnum=4438   (569 words)

  
 Neurofibromatosis 2
Neurofibromatosis 2 (previously called bilateral acoustic neurofibromatosis or central neurofibromatosis and abbreviated as NF2, NFII or BAN) affects about 1 in 40,000 people without regard to sex or race.
Neurofibromatosis 1 (Recklinghausen disease) and neurofibromatosis 2 (bilateral acoustic neurofibromatosis).
NF2 in children Nunes F, MacCollin M. Neurofibromatosis 2 in the pediatric population.
neurosurgery.mgh.harvard.edu /NFclinic/NF2.htm   (2345 words)

  
 Neurofibromatosis 2
Neurofibromatosis 2 (NF2) is characterized by bilateral vestibular schwannomas with associated symptoms of tinnitus, hearing loss, and balance dysfunction.
Aguiar PH, Tatagiba M, Samii M, Dankoweit-Timpe E, Ostertag H (1995) The comparison between the growth fraction of bilateral vestibular schwannomas in neurofibromatosis 2 (NF2) and unilateral vestibular schwannomas using the monoclonal antibody MIB 1.
Kluwe L, Pulst SM, Koppen J, Mautner VF (1995) A 163-bp deletion in the neurofibromatosis 2 (NF2) gene associated with variant phenotypes [corrected].
www.geneclinics.org /profiles/nf2/details.html   (5449 words)

  
 Publications   (Site not responding. Last check: 2007-10-09)
Pre-symptomatic Diagnosis for Neurofibromatosis Type 2 with Chromosome 22 Markers.
Neurofibromatosis Type 2: A new Mechanism of Tumour Suppressor.
Confirmation of the SCA 2 Locus as an Alternative Locus for Dominantly Inherited Spinocerebellar Ataxias and Refinement of the Candidate Region.
www.medicine.mcgill.ca /neurogen/papers.html   (2467 words)

  
 Neurofibromatosis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS)
Other or variant types of the neurofibromatoses may exist, but are not yet identified.
Previously, NF1 was known as peripheral neurofibromatosis (or von Recklinghausen's neurofibromatosis) because some of the symptoms--skin spots and tumors--seemed to be limited to the outer nerves, or peripheral nervous system, of the affected person.
It was formerly called bilateral acoustic neurofibromatosis or central neurofibromatosis because the tumors, which cause progressive hearing loss, were thought to grow primarily on the auditory nerve, a branch of the eighth cranial nerve responsible for hearing.
www.ninds.nih.gov /disorders/neurofibromatosis/detail_neurofibromatosis.htm   (1632 words)

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