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.
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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.
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Property / Canonical URI: https://id.who.int/icd/entity/403221860 / rank
 
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CID11:ID_403221860
Property / CURIE: CID11:ID_403221860 / rank
 
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dki-india-ID_403221860
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Concluído
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Property / Knowledge Architect: https://pauloleads.com.br/cases-publicos/ / rank
 
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15 August 2026
Timestamp+2026-08-15T00:00:00Z
Timezone+00:00
CalendarGregorian
Precision1 day
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Property / Collection date: 15 August 2026 / rank
 
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Latest 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.
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    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.

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      CID11:ID_403221860
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      dki-india-ID_403221860
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      Concluído
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      15 August 2026
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