A Case of Neurocutaneous Giant Melanosis Associated with Dandy-Walker Syndrome.

Hong,  Su Young; Ban,  Ji Eun; Rhu,  Jae Ho; Choi,  Sun Seob; Hwang,  Kyu Geun

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Case Report

Journal of the Korean Child Neurology Society 2001;9(1):171-177.
Published online May 30, 2001.

A Case of Neurocutaneous Giant Melanosis Associated with Dandy-Walker Syndrome.

Su Young Hong, Ji Eun Ban, Jae Ho Rhu, Sun Seob Choi, Kyu Geun Hwang

¹Department of Pediatrics, College of Medicine, Dong-A University, Pusan, Korea.
²Department of Diagnostic Radiology, College of Medicine, Dong-A University, Pusan, Korea.

Abstract

Neurocutaneous melanosis is a rare congenital phacomatosis, characterized by the presence of multiple pigmented skin nevi at birth. Meningeal melanosis tending to become malignant and seizure. Dandy-Walker syndrome is a developmental disorder of brain characterized by cystic dilatation of the fourth ventricle and agenesis or hypoplasia of the cerebellar vermis. We studied a case of Dandy-Walker syndrome with neurocutaneous giant melanosis in a 3 years old boy.

Key Words: Neurocutaneous giant melanosis, Dandy-Walker syndrome

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