Erschienen in:
01.02.2012 | Case Report
Neurocutaneous melanosis associated with Dandy–Walker complex and an intracranial cavernous angioma
verfasst von:
Kyung Hwan Kim, Sang-Bong Chung, Doo-Sik Kong, Ho-Jun Seol, Hyung Jin Shin
Erschienen in:
Child's Nervous System
|
Ausgabe 2/2012
Einloggen, um Zugang zu erhalten
Excerpt
Neurocutaneous melanosis (NCM) is a rare, non-familial phacomatosis that is characterized by large and/or numerous congenital melanocytic nevi and abnormal proliferation of the melanin-containing cells in the central nervous system (CNS) [
9‐
11,
20]. Since Di Rocco et al. reported a 10% incidence of NCM and an association with cystic malformations of the posterior fossae [
9], approximately 8% to 10% of patients with NCM have been found to harbor an associated Dandy–Walker malformation [
6,
12,
13]. Dandy–Walker malformation is noted for vermian hypoplasia, cerebellar dysgenesis, cystic dilatation of the posterior fossa, and an enlarged fourth ventricle [
3,
9,
10]. Intracranial cavernous angioma (CA) represents 1.7% to 18% of all vascular malformations in children, and the most frequent presentations in symptomatic CA are seizures, followed by focal neurologic deficits, hemorrhage, and headache [
14]. …
