Neurofibromatosis: A Common Neurocutaneous Disorder

Neurofibromatosis (NF) type 1 (NF-l) is one of the most common of the neurocutaneous conditions, whereas NF type 2 (NF-2) accounts for an extremely small percentage of the total cases of NF. Indeed, most physicians will probably encounter at least one or two patients with NF-1 during the course of t...

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Published inMayo Clinic proceedings Vol. 73; no. 11; pp. 1071 - 1076
Main Author Karnes, Pamela S.
Format Journal Article
LanguageEnglish
Published Rochester, MN Elsevier Inc 01.11.1998
Mayo Medical Ventures
Elsevier Limited
Subjects
Biological and medical sciences
Chromosomes, Human, Pair 17 - genetics
Chromosomes, Human, Pair 22 - genetics
Genetic Testing
Humans
Medical sciences
Neoplasms - genetics
Neurofibromatoses - complications
Neurofibromatoses - diagnosis
Neurofibromatoses - genetics
Neurofibromatoses - therapy
Neurofibromatosis 1
Neurofibromatosis 2
Neurology
Tumors of the nervous system. Phacomatoses
NF
MRI
Human
Neurofibromatosis II
Skin disease
Nervous system diseases
Review
Malignant tumor
Neurofibromatosis Recklinghausen
Genetic disease
Gene
Central nervous system disease
Deletion
Evolution
Benign neoplasm
Mutation
Neurofibroma
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Summary:Neurofibromatosis (NF) type 1 (NF-l) is one of the most common of the neurocutaneous conditions, whereas NF type 2 (NF-2) accounts for an extremely small percentage of the total cases of NF. Indeed, most physicians will probably encounter at least one or two patients with NF-1 during the course of their practice. The manifestations can be varied and subtle; thus, the condition can sometimes be difficult to recognize. Nonetheless, the diagnosis of NF-1 is often clinically possible by the time the person is 10 years old. In this article, the diagnostic criteria for the most common types of NF are discussed, the common and some of the serious manifestations of both NF-1 and NF-2 are described, and suggestions for follow-up care are offered. Of importance, physicians must recognize that, although NF-1 and NF-2 share a common name, they are due to mutations in two different genes. Cure is not yet possible; thus, treatment is primarily symptomatic. A multidisciplinary treatment team is often helpful, particularly for patients with complicated problems.
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ISSN:0025-6196
1942-5546
DOI:10.4065/73.11.1071