MCID: NRC006
MIFTS: 22

Neurocutaneous Melanosis malady

Neuronal category

Summaries for Neurocutaneous Melanosis

Sources:
43NIH Rare Diseases, 64Wikipedia, 33MalaCards
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NIH Rare Diseases:43 Neurocutaneous melanosis (ncm) is a rare, non-inherited condition of the central nervous system. it is characterized by melanocytic nevi in both the skin and the brain. two-thirds of people with ncm have giant congenital melanocytic nevi, and the remaining one-third have numerous lesions but no giant lesions. most patients present with neurological features early in life, which can be secondary to intracranial hemorrhages (bleeding in the brain), impairment of cerebrospinal fluid circulation (fluid around the brain and spinal cord), and/or malignant transformation of the melanocytes.the prognosis of patients with symptomatic neurocutaneous melanosis is extremely poor, even in the absence of malignancy. chemotherapy has been ineffective in the few patients in whom it has been tried. last updated: 4/18/2011

MalaCards: Neurocutaneous Melanosis, also known as neurocutaneous melanosis syndrome, is related to dandy-walker complex and giant congenital nevus. Affiliated tissues include brain, spinal cord and lung.

Wikipedia:64 Neurocutaneous melanosis is a congenital disorder characterized by the presence of congenital... more...

Aliases & Classifications for Neurocutaneous Melanosis

Sources:
43NIH Rare Diseases, 61UMLS
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Neuronal


Aliases & Descriptions:

neurocutaneous melanosis 43 61
neurocutaneous melanosis syndrome 43
melanosis, neurocutaneous 43


Related Diseases for Neurocutaneous Melanosis

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17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Neurocutaneous Melanosis:



Diseases related to neurocutaneous melanosis

Clinical Features for Neurocutaneous Melanosis

Drugs & Therapeutics for Neurocutaneous Melanosis

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5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Neurocutaneous Melanosis

Anatomical Context for Neurocutaneous Melanosis

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33MalaCards
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MalaCards organs/tissues related to Neurocutaneous Melanosis:

33
Brain, Spinal cord, Lung, Skin, Endothelial, Temporal lobe, Amygdala, Pons

Animal Models for Neurocutaneous Melanosis or affiliated genes

Sources:
28inGenious Targeting Laboratory
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Publications for Neurocutaneous Melanosis

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51PubMed
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Articles related to Neurocutaneous Melanosis:

(show top 50)    (show all 119)
idTitleAuthorsYear
1
Letters to the editor: Neurocutaneous melanosis. (23870039)
2013
2
Rapidly progressive and fatal neurocutaneous melanosis presenting as recurrent headache. (23679165)
2013
3
A Giant Congenital Melanocytic Nevus Associated with Neurocutaneous Melanosis. (24173435)
2013
4
Intramedullary and retroperitoneal melanocytic tumor associated with congenital blue nevus and nevus flammeus: an uncommon combination of neurocutaneous melanosis and phacomatosis pigmentovascularis--case report. (24077274)
2013
5
Multiple congenital melanocytic nevi and neurocutaneous melanosis are caused by postzygotic mutations in codon 61 of NRAS. (23392294)
2013
6
Neuropathology of neurocutaneous melanosis: histological foci of melanotic neurones and glia may be undetectable on MRI. (22294043)
2012
7
Neurocutaneous melanosis associated with Dandy-Walker complex and an intracranial cavernous angioma. (22134415)
2012
8
Stable neurocutaneous melanosis in a ten-year-old boy with controlled partial-complex seizures. A case report. (24028916)
2012
9
Fatal leptomeningeal melanoma in neurocutaneous melanosis. (21906139)
2012
10
Dermal melanocytosis of the scalp associated to intracranial melanoma: malignant blue nevus, neurocutaneous melanosis, or neurocristic cutaneous hamartoma? (22123266)
2012
11
Leptomeningeal melanocytosis in an adult male without large congenital nevi: a rare and atypical case of neurocutaneous melanosis. (21726502)
2011
12
Neurocutaneous melanosis: assessment on F-18 FDG and [11C]-methionine PET/CT and MRI. (21892043)
2011
13
Rapid response to therapy of neurocutaneous melanosis with leptomeningeal melanoma. (19722276)
2010
14
Symptomatic neurocutaneous melanosis and Dandy-Walker malformation in an adult. (19398571)
2009
15
Neurocutaneous melanosis: a fatal disease in early childhood. (19349541)
2009
16
The pathologic features of neurocutaneous melanosis in a cynomolgus macaque. (19276048)
2009
17
Neurocutaneous melanosis in an adult patient with diffuse leptomeningeal melanosis and a rapidly deteriorating course: case report and review of the literature. (18407407)
2008
18
Neurocutaneous melanosis in association with Dandy-Walker malformation: case report and literature review. (18477004)
2008
19
Neurocutaneous melanosis in a newborn with giant congenital melanocytic nevus. (17727949)
2007
20
Progressive brainstem compression in an infant with neurocutaneous melanosis and Dandy-Walker complex following ventriculoperitoneal shunt placement for hydrocephalus. Case report. (18154021)
2007
21
Parenchymal neurocutaneous melanosis in association with intraventricular dermoid and Dandy-Walker variant: a case report. (16799276)
2006
22
Neurocutaneous melanosis associated with Dandy-Walker malformation. (16489502)
2006
23
Neurocutaneous melanosis associated with Hirschsprung's disease in a male neonate. (16080919)
2005
24
Neurocutaneous melanosis: clinical presentations. (16192883)
2005
25
Neurocutaneous melanosis: an atypical presentation. (16335879)
2005
26
Neurocutaneous melanosis presenting with hydrocephalus. Case report and review of the literature. (16206742)
2005
27
Childhood malignant blue nevus of the ear associated with two intracranial melanocytic tumors-metastases or neurocutaneous melanosis? (15493000)
2004
28
Diffuse leptomeningeal hyperintensity on fluid-attenuated inversion recovery MR images in neurocutaneous melanosis. (14729544)
2004
29
Fundus features of a case of neurocutaneous melanosis. (15621879)
2004
30
Neurocutaneous melanosis. (14576958)
2004
31
Neurocutaneous melanosis with transposition of the great arteries and renal agenesis. (12869156)
2003
32
Neurocutaneous melanosis in association with the Dandy-Walker complex, complicated by melanoma: report of a case and literature review. (12047644)
2002
33
Neurocutaneous melanosis and psychosis: a case report. (11285085)
2001
34
Giant congenital melanocytic nevi: the significance of neurocutaneous melanosis in neurologically asymptomatic children. (11252085)
2001
35
Age-related changes of the MR appearance of CNS involvement in neurocutaneous melanosis complex. (11149097)
2000
36
Neurocutaneous melanosis with hydrocephalus, intraspinal arachnoid collections and syringomyelia: case report and literature review. (10789914)
2000
37
MR imaging of symptomatic neurocutaneous melanosis in children. (8995166)
1997
38
Neurocutaneous melanosis. Case report and a brief review. (8079124)
1994
39
Neurocutaneous melanosis presenting as an intradural mass of the cervical canal: magnetic resonance features and the presence of melanin as a clue to diagnosis: case report. (7808620)
1994
40
Neurocutaneous melanosis and temporal lobe tumor in a child: MR study. (1882745)
1991
41
Neurocutaneous melanosis, neurofibromatosis and spinal meningioma: an unusual association. (2113761)
1990
42
Intraspinal melanotic arachnoid cyst and lipoma in neurocutaneous melanosis. (2922652)
1989
43
Neurocutaneous melanosis and psychosis. (3358470)
1988
44
Fatal pneumococcal infection in neurocutaneous melanosis. (3198896)
1988
45
Rhabdomyosarcoma arising in a congenital giant nevus associated with neurocutaneous melanosis in a neonate. (3430311)
1987
46
Neurocutaneous melanosis associated with Dandy-Walker syndrome. (3665294)
1987
47
Neurocutaneous melanosis with malignant melanoma of the leptomeninges. (21164250)
1985
48
Neurocutaneous melanosis with hydrocephalus and syringomyelia. Case report. (3964849)
1985
49
Neurocutaneous melanosis with extensive intracerebral and spinal cord involvement. Report of two cases. (6470790)
1984
50
Computed tomographic findings of primary malignant leptomeningeal melanoma in neurocutaneous melanosis. (115293)
1979

Genetic Variations for Neurocutaneous Melanosis

Expression for genes affiliated with Neurocutaneous Melanosis

Sources:
15Gene Expression Omnibus DataSets
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Search GEO for disease gene expression data for Neurocutaneous Melanosis.

Pathways for genes affiliated with Neurocutaneous Melanosis

Compounds for genes affiliated with Neurocutaneous Melanosis

GO Terms for genes affiliated with Neurocutaneous Melanosis

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Sources for Neurocutaneous Melanosis

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet