Neurocutaneous melanocytosis (Q93770): Difference between revisions
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Melanocitose neurocutânea (NCM) é um transtorno neurológico congênito raro caracterizado por agregados anormais de nevos melanocíticos no sistema nervoso central (melanocitose leptomeníngea) associada a nevos melanocíticos congênitos grandes ou gigantes (CMN; ver este termo). A NCM pode ser assintomática ou apresentar comprometimento neurológico variavelmente grave e progressivo, às vezes resultando em morte. A prevalência é estimada em 1/50.000-1/200.000. A incidência de NCM sintomática parece ser de aproximadamente um terço a metade destes casos. | |||
| description / en | description / en | ||
Neurocutaneous melanocytosis (NCM) is a rare congenital neurological disorder characterised 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. | |||
Revision as of 08:52, 16 August 2026
Neurocutaneous melanocytosis (NCM) is a rare congenital neurological disorder characterised 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.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | ID_403221860 |
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| English | Neurocutaneous melanocytosis |
Neurocutaneous melanocytosis (NCM) is a rare congenital neurological disorder characterised 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. |
