Elsevier

Pediatric Neurology

Volume 2, Issue 6, November–December 1986, Pages 380-384
Pediatric Neurology

Case report
Spinal cord involvement in encephalocraniocutaneous lipomatosis

https://doi.org/10.1016/0887-8994(86)90084-6Get rights and content

Abstract

Encephalocraniocutaneous lipomatosis is a rare hamartomatosis involving the craniofacial region and the central nervous system. The most prominent clinical features are large areas of scalp alopecia, soft subcutaneous craniofacial masses, lipomas, connective tissue nevi of the eyelids and surrounding areas, pterygium-like choriostoma of the ocular conjunctiva, mental retardation, motor deficit, and seizures. Of the eight patients reported previously, three had spinal cord evaluations and two had evidence of lipomatosis. We report the third patient with this association, review the literature of encephalocraniocutaneous lipomatosis, and stress the importance of spinal cord evaluation during the newborn period.

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