Clinical and neuroradiological signs in adults with type 1 neurofibromatosis.
Neurologia
; 28(6): 361-5, 2013.
Article
in En, Es
| MEDLINE
| ID: mdl-23246213
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.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Neurofibromatosis 1
Type of study:
Diagnostic_studies
/
Observational_studies
/
Prevalence_studies
/
Risk_factors_studies
Limits:
Adult
/
Female
/
Humans
/
Male
Language:
En
/
Es
Journal:
Neurologia
Year:
2013
Document type:
Article