title: Clinical and neuroradiological signs in adults with type 1 neurofibromatosis.
abstract: BACKGROUND
  Type 1 neurofibromatosis is the most common neurocutaneous syndrome. Most published case series study the paediatric population.
  
  
  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.affiliation: Sección de Neurología, Hospital San Pedro de Alcántara, Cáceres, España. pjimenez1010j@yahoo.es
authors.forename: P E
authors.initials: PE
authors.lastname: Jiménez Caballero
authors.forename: F
authors.initials: F
authors.lastname: López Espuela
authors.forename: J C
authors.initials: JC
authors.lastname: Portilla Cuenca
authors.forename: R M
authors.initials: RM
authors.lastname: Romero Sevilla
authors.forename: J A
authors.initials: JA
authors.lastname: Fermín Marrero
authors.forename: I
authors.initials: I
authors.lastname: Casado Naranjo
mesh_terms: D000328:Adult; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000000981:diagnostic imaging; D009485:Neuroradiography; D055815:Young Adult