Management of the patient and family with neurofibromatosis 2: a consensus conference statement

@article{Evans2005ManagementOT,
  title={Management of the patient and family with neurofibromatosis 2: a consensus conference statement},
  author={Dgr Evans and Medine İrem Başer and Brian F. O'Reilly and Jeremy G. Rowe and Michael J. Gleeson and Shakeel R. Saeed and Andrew Thomas King and S. M. Huson and Richard S. C. Kerr and Nick W M Thomas and Rachel E A Irving and Robert J Macfarlane and Rosalie E. Ferner and Ross McLeod and Daniel Moffat and Richard Thomas Ramsden},
  journal={British Journal of Neurosurgery},
  year={2005},
  volume={19},
  pages={12 - 5}
}
A consensus conference on neurofibromatosis 2 (NF2) was held in 2002 at the request of the United Kingdom (UK) Neurofibromatosis Association, with particular emphasis on vestibular schwannoma (VS) surgery. NF2 patients should be managed at specialty treatment centres, whose staff has extensive experience with the disease. All NF2 patients and their families should have access to genetic testing because presymptomatic diagnosis improves the clinical management of the disease. Some clinical… 
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[Neurofibromatosis type 2].
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TLDR
Clinicians should suspect NF2 in children presenting with meningioma, schwannoma, and skin features, such as neurofibromas/schwannomas, but fewer than 6 café au lait patches, who thus fall short of a diagnosis of neurof fibromatosis type 1.
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TLDR
There is considerable variability in growth rates of VSs in patients with NF2, but they tend to be higher in patients who are younger at onset of signs or symptoms, while the number of non-VS cerebral or spinal tumors or different types of constitutional NF2 mutations is unknown.
Evaluation of clinical diagnostic criteria for neurofibromatosis 2
TLDR
None of the existing sets of criteria for neurofibromatosis 2 are adequate at initial assessment for diagnosing people who present without bilateral vestibular schwannomas as having NF2, particularly people with a negative family history of NF2.
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TLDR
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TLDR
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TLDR
Increased clinical awareness, better imaging techniques, and molecular diagnostics have made pediatric diagnosis of neurofibromatosis 2 feasible, but outcomes appear to be worse than in adult patients.
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TLDR
Details of patients who have been detected as having VS on a screening MRI scan without any known central nervous system pathology, signs, or symptoms are analysed.
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TLDR
Clinical, electrophysiological, and imaging data from four NF2 patients seen at a specialist neurofibromatosis clinic over a 4 year period are described in whom symptomatic focal amyotrophy preceded the diagnosis of NF2.
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TLDR
There are likely two rather than three subtypes of neurofibromatosis 2, and classification of patients to subtype may aid in counseling about long-term prognosis and in formulating individualized guidelines for medical surveillance.
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