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Clinical and neuroradiological signs in adults with type 1 neurofibromatosis.
MedLine Citation:
PMID:  23246213     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
INTRODUCTION: Type 1 neurofibromatosis is the most common neurocutaneous syndrome. Most published case series study the paediatric population. MATERIAL AND METHODS: Cross-sectional study of cases of type 1 neurofibromatosis from neurology departments that were recorded in a database. We analysed the different clinical variables providing the diagnosis as well as demographic and neuroradiological variables. RESULTS: We found a total of 31 patients with type 1 neurofibromatosis. The mean age was 28.9 years and 58.4% were women. Subjects with unidentified bright objects (UBOs) were younger than those without them (22.45±8.22 years vs. 32.5±10.64; P=.011). In contrast, subjects with neurofibromas were older than those without them (30.56±10.68 years vs. 18.25±4.34; P=.032). No sex differences were found in the presentation of clinical or radiological variables. Seven patients (22.6%) had tumours; 3 were optic pathway gliomas (1 bilateral), 3 were plexiform neurofibromas, and 1 was a pilocytic astrocytoma in the brainstem. CONCLUSIONS: Patients with type 1 neurofibromatosis presented both peripheral neurofibromas and tumorous lesions of the central nervous system. Subjects with neurofibromas were older than those who did not present them, while subjects with UBOs were younger than those without such lesions.
Authors:
P E Jiménez Caballero; F López Espuela; J C Portilla Cuenca; R M Romero Sevilla; J A Fermín Marrero; I Casado Naranjo
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-12-13
Journal Detail:
Title:  Neurologia (Barcelona, Spain)     Volume:  -     ISSN:  1578-1968     ISO Abbreviation:  Neurologia     Publication Date:  2012 Dec 
Date Detail:
Created Date:  2012-12-18     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9005460     Medline TA:  Neurologia     Country:  -    
Other Details:
Languages:  ENG; SPA     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.
Affiliation:
Sección de Neurología, Hospital San Pedro de Alcántara, Cáceres, España. Electronic address: pjimenez1010j@yahoo.es.
Vernacular Title:
Manifestaciones clínicas y neurorradiológicas en los adultos con neurofibromatosis tipo 1.
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