Encephalocraniocutaneous Lipomatosis: Complete Neuroradiologic Evaluation and Follow-up of Two Cases

is a rare neurocutaneous syndrome characterized by uni-lateral scalp, facial, and ocular lesions and ipsilateral ce-rebral malformations. To define the neuroimaging features of this disorder we studied two patients affected by ECCL and compared our data with those reported in the litera-ture. Sonographic, CT, and MR imaging examinations showed quite specific CNS findings that are highly sugges-tive of the diagnosis of ECCL. To our knowledge this is the first report of a complete neuroradiologic evaluation and follow-up of this disorder. Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome first described in 1970 by Haberland and Perou (1); it is character-ized by unilateral lipomas of the cranium, face, and neck, ipsilateral lipodermoids of the eye, and ipsi