1.-To review concepts about phakomatosis as syndromic diseases including neurological and extra-neurological features.
2.-To show radiological findings of neurologic affection of these syndroms
3.-To explain some of the findings from an embryological point of view.

 

Introduction
Neurofibromatosis type 1
Optic pathway gliomas
White matter abnormalities
Neurofibromas
Bone dysplasia
Vascular abnormalities
Neurofibromatosis type 2
Meninigiomas
Schwannomas
Tuberous sclerosis
Subependymal/Cerebral hamartomas
Astrocytoma
White matter lesions
Sturge-Weber Syndrome
Angioma
Choroidal angioma
Cranial asymmetry
Von Hippel-Lindau Disease
Cerebellar Hamngioblastoma
Papillary cystadenoma of the endolymphatic sac
Visceral manifestations

MRI is an excellent, non-invasive modality for detection, diagnosis and evolution of neurological processes of neurocutaneous syndromes.
This poster presents the vast spectrum of neuro-radiological features of phakomatosis and encloses them in a frame of syndromic diseases.