Neurocutaneous Melanosis in Association with Dandy-Walker Complex with Extensive Intracerebral and Spinal Cord Involvement.
10.3340/jkns.2014.56.1.61
- Author:
Kyoung Su SUNG
1
;
Young Jin SONG
Author Information
1. Department of Neurosurgery, College of Medicine, Dong-A University, Busan, Korea. ns2000@donga.ac.kr
- Publication Type:Case Report
- Keywords:
Melanoma;
Neurocutaneous syndrome;
Dandy-Walker syndrome
- MeSH:
Adolescent;
Central Nervous System;
Dandy-Walker Syndrome*;
Dilatation;
Fourth Ventricle;
Humans;
Hydrocephalus;
Intracranial Hemorrhages;
Male;
Melanocytes;
Melanoma;
Melanosis*;
Neurocutaneous Syndromes;
Nevus, Pigmented;
Spinal Cord*
- From:Journal of Korean Neurosurgical Society
2014;56(1):61-65
- CountryRepublic of Korea
- Language:English
-
Abstract:
Neurocutaneous melanosis (NCM) is a rare congenital syndrome consisting of benign or malignant melanotic tumors of the central nervous system with large or numerous cutaneous melanocytic nevi. The Dandy-Walker complex (DWC) is characterized by an enlarged posterior fossa with high insertion of the tentorium, hypoplasia or aplasia of the cerebellar vermis, and cystic dilatation of the fourth ventricle. These each two conditions are rare, but NCM associated with DWC is even more rare. Most patients of NCM with DWC present neurological symptoms early in life such as intracranial hemorrhage, hydrocephalus, and malignant transformation of the melanocytes. We report a 14-year-old male patient who was finally diagnosed as NCM in association with DWC with extensive intracerebral and spinal cord involvement.
