Extracranial vertebral artery involvement in neurofibromatosis type I. Report of four cases and literature review.
Neurofibromatosis type 1 (NF-1) is one of the most common inherited diseases and as an autosomal dominant genetic disorder results from NF-1 gene mutation with 100% penetration and wide phenotypic variability. The disease can involve a wide variety of tissues derived from all three embryonic layers. NF-1 vasculopathy has been described primarily in peripheral arteries, but arteries supplying the CNS may also be involved. Of those, extracranial vertebral involvement is the commonest and most important. A series of four patients with NF-1 and vascular disease of the vertebral artery is described with a review of the pathophysiology, vascular phenotypes, their management and the pertinent literature.
Pereira
V M
VM
Service de Neuroradiologie Diagnostique et Thérapeutique, Hôpital de Bicetre, Le Kremlin-Bicetre, Paris, France - Department of Neuroradiology, University of Sao Paulo, Brasil - vitormpbr@hotmail.com.
Geiprasert
S
S
Krings
T
T
Caldas
J G M P
JG
Toulgoat
F
F
Ozanne
A
A
Mercier
P
P
Lasjaunias
P L
PL