Encephalocraniocutaneous lipomatosis: Complete neuroradiologic evaluation and follow-up of two cases

Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndro me characterized by unilateral scalp, facial, and ocular lesions and ipsila teral cerebral malformations. To define the neuroimaging features of this d isorder we studied two patients affected by ECCL and compared our data with those reported in the literature. Sonographic, CT, and MR imaging examinat ions showed quite specific CNS findings that are highly suggestive of the d iagnosis of ECCL, To our knowledge this is the first report of a complete n euroradiologic evaluation and follow-up of this disorder.