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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