Нейрошкірний меланоцитоз
ORPHA:2481· ICD-10 D22.4· Neurocutaneous melanocytosis
Визначення(English summary)
Neurocutaneous melanocytosis (NCM) is a rare congenital neurological disorder characterized by abnormal aggregations of nevomelanocytes within the central nervous system (leptomeningeal melanocytosis) associated with large or giant congenital melanocytic nevi (CMN; see this term). NCM can be asymptomatic or present as variably severe and progressive neurological impairment, sometimes resulting in death.
- Поширеність
- 1-9 / 100 000
- Успадкування
- Not applicable
- Вік початку
- Childhood