Preferred Label : Neurocutaneous melanocytosis;
ICD-11 definition : Neurocutaneous melanocytosis (NCM) is a rare congenital neurological disorder characterized
by abnormal aggregations of naevomelanocytes within the central nervous system (leptomeningeal
melanocytosis) associated with large or giant congenital melanocytic naevi (CMN; see
this term). NCM can be asymptomatic or present as variably severe and progressive
neurological impairment, sometimes resulting in death. Prevalence is estimated at
1/50,000-1/200,000. The incidence of symptomatic NCM appears to be approximately a
third to a half of these.;
Origin ID : 403221860;
Automatic exact mappings (from CISMeF team)
Neurocutaneous melanocytosis (NCM) is a rare congenital neurological disorder characterized
by abnormal aggregations of naevomelanocytes within the central nervous system (leptomeningeal
melanocytosis) associated with large or giant congenital melanocytic naevi (CMN; see
this term). NCM can be asymptomatic or present as variably severe and progressive
neurological impairment, sometimes resulting in death. Prevalence is estimated at
1/50,000-1/200,000. The incidence of symptomatic NCM appears to be approximately a
third to a half of these.
