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 the findings from an embryological point of view.

Magnetic resonance, through its classical sequences and advanced methods, such as diffusion and spectroscopy, allows a clear and correct diagnosis of this infrequent group of diseases. Phakomatosis are a group of hereditary diseases of neuroectoderm, distinguished by cutaneous manifestation, due to that are also named neurocutaneous diseases. These are Neurofibromatosis, Tuberous sclerosis, Von Hippel-Lindau’s disease and the Sturge-Weber’s syndrome. Essential lesions are neurogenous tumor, tuber, hemangioblastoma and angiomatosis respectively.

We have underwent retrospectively through the cases of the last five years of our data base, reviewing radiological findings in MRI of neurological affection of neurocutaneous syndromes. Histologic examination of cutaneus lesions were performed in all cases with dermatological lesions.

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.

[email protected] [email protected]