MCID: NRC006
MIFTS: 26

Neurocutaneous Melanosis malady

Rare diseases, Neuronal diseases categories

Summaries for Neurocutaneous Melanosis

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Sources:
44NIH Rare Diseases, 66Wikipedia, 34MalaCards
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NIH Rare Diseases:44 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 melanoma and dandy-walker complex. An important gene associated with Neurocutaneous Melanosis is NRAS (neuroblastoma RAS viral (v-ras) oncogene homolog). Affiliated tissues include skin, spinal cord and brain.

Wikipedia:66 Neurocutaneous melanosis is a congenital disorder characterized by the presence of congenital... more...

Aliases & Classifications for Neurocutaneous Melanosis

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44NIH Rare Diseases, 63UMLS
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Classifications:

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


Aliases & Descriptions:

neurocutaneous melanosis 44 63
neurocutaneous melanosis syndrome 44
melanosis, neurocutaneous 44


Related Diseases for Neurocutaneous Melanosis

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18GeneCards, 19GeneDecks
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Graphical network of the top 20 diseases related to Neurocutaneous Melanosis:



Diseases related to neurocutaneous melanosis

Symptoms for Neurocutaneous Melanosis

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Drugs & Therapeutics for Neurocutaneous Melanosis

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Neurocutaneous Melanosis

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Anatomical Context for Neurocutaneous Melanosis

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34MalaCards
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MalaCards organs/tissues related to Neurocutaneous Melanosis:

34
Skin, Spinal cord, Brain, Amygdala, Pons, Lung, Endothelial, Temporal lobe

Animal Models for Neurocutaneous Melanosis or affiliated genes

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Publications for Neurocutaneous Melanosis

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53PubMed
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Articles related to Neurocutaneous Melanosis:

(show top 50)    (show all 118)
idTitleAuthorsYear
1
Neurocutaneous melanosis. (23392328)
2013
2
Letters to the editor: Neurocutaneous melanosis. (23870039)
2013
3
Rapidly progressive and fatal neurocutaneous melanosis presenting as recurrent headache. (23679165)
2013
4
Neuropathology of neurocutaneous melanosis: histological foci of melanotic neurones and glia may be undetectable on MRI. (22294043)
2012
5
Neurocutaneous melanosis associated with Dandy-Walker complex and an intracranial cavernous angioma. (22134415)
2012
6
Rapid response to therapy of neurocutaneous melanosis with leptomeningeal melanoma. (19722276)
2010
7
Teaching NeuroImages: neurocutaneous melanosis. (20458064)
2010
8
Neurocutaneous melanosis and the Dandy-Walker complex: an uncommon but not so insignificant association. (19711088)
2009
9
Symptomatic neurocutaneous melanosis and Dandy-Walker malformation in an adult. (19398571)
2009
10
The vascular endothelial cell is a new origin of melanin pigment on cynomolgus macaque with neurocutaneous melanosis. (19067973)
2008
11
Spinal neurocutaneous melanosis without cutaneous nevi. (18827686)
2008
12
Neurocutaneous melanosis in an adult patient with diffuse leptomeningeal melanosis and a rapidly deteriorating course: case report and review of the literature. (18407407)
2008
13
SCALP syndrome: sebaceous nevus syndrome, CNS malformations, aplasia cutis congenita, limbal dermoid, and pigmented nevus (giant congenital melanocytic nevus) with neurocutaneous melanosis: a distinct syndromic entity. (18234391)
2008
14
Neurocutaneous melanosis with acute disseminated encephalomyelitis. (17103186)
2007
15
Neurocutaneous melanosis in a newborn with giant congenital melanocytic nevus. (17727949)
2007
16
Progressive brainstem compression in an infant with neurocutaneous melanosis and Dandy-Walker complex following ventriculoperitoneal shunt placement for hydrocephalus. Case report. (18154021)
2007
17
Coexisting intracranial meningeal melanocytoma, dermoid tumor, and Dandy-Walker cyst in a patient with neurocutaneous melanosis. Case report. (16572661)
2006
18
Neurocutaneous melanosis and congenital melanocytic nevus in the head. (15942284)
2005
19
Neurocutaneous melanosis associated with Hirschsprung's disease in a male neonate. (16080919)
2005
20
Neurocutaneous melanosis: clinical presentations. (16192883)
2005
21
Childhood malignant blue nevus of the ear associated with two intracranial melanocytic tumors-metastases or neurocutaneous melanosis? (15493000)
2004
22
An unusual case of neurocutaneous melanosis. (15504713)
2004
23
Diffuse leptomeningeal hyperintensity on fluid-attenuated inversion recovery MR images in neurocutaneous melanosis. (14729544)
2004
24
Neurocutaneous melanosis associated with Dandy-Walker malformation and a meningohydroencephalocele. Case report. (15287463)
2004
25
Neurocutaneous melanosis. (15295923)
2004
26
Neurocutaneous melanosis with transposition of the great arteries and renal agenesis. (12869156)
2003
27
Neurocutaneous melanosis associated with malignant leptomeningeal melanoma in an adult: clinical significance of 5-S-cysteinyldopa in the cerebrospinal fluid---case report. (14723271)
2003
28
Neurocutaneous melanosis in association with encephalocraniocutaneous lipomatosis. (12140461)
2002
29
Neurocutaneous melanosis: a case of primary intracranial melanoma with metastasis. (11292138)
2001
30
Neurocutaneous melanosis and psychosis: a case report. (11285085)
2001
31
Neurocutaneous melanosis presenting with intracranial amelanotic melanoma. (10219411)
1999
32
Congenital neurocutaneous melanosis. (9347228)
1997
33
Multiple nodular lesions seen in a patient with neurocutaneous melanosis. (8990708)
1996
34
Neurocutaneous melanosis with Dandy-Walker malformation complicated by primary spinal leptomeningeal melanoma. (8877612)
1996
35
Primary temporal melanoma without diffuse leptomeningeal involvement: a variant of neurocutaneous melanosis. (7809226)
1995
36
MR of parenchymal neurocutaneous melanosis. (7793388)
1995
37
Neurocutaneous melanosis. Case report and a brief review. (8079124)
1994
38
Neurocutaneous melanosis. (8305762)
1994
39
Neurocutaneous melanosis: definition and review of the literature. (1869648)
1991
40
Neurocutaneous melanosis and temporal lobe tumor in a child: MR study. (1882745)
1991
41
Contrast-enhanced MR imaging of neurocutaneous melanosis. (1902050)
1991
42
Neurocutaneous melanosis, neurofibromatosis and spinal meningioma: an unusual association. (2113761)
1990
43
Neurocutaneous melanosis associated with intraspinal lipoma. (2677828)
1989
44
Intraspinal melanotic arachnoid cyst and lipoma in neurocutaneous melanosis. (2922652)
1989
45
Neurocutaneous melanosis with interstitial lung involvement--a rare association. (3169871)
1988
46
Neurocutaneous melanosis and psychosis. (3358470)
1988
47
Rhabdomyosarcoma arising in a congenital giant nevus associated with neurocutaneous melanosis in a neonate. (3430311)
1987
48
The coincidence of neurocutaneous melanosis and encephalofacial angiomatosis. (3815935)
1986
49
Congenital neurocutaneous melanosis with metastatic orbital malignant melanoma. (3808623)
1986
50
Neurocutaneous melanosis. Report of a case and review of the literature. (835391)
1977

Variations for Neurocutaneous Melanosis

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Expression for genes affiliated with Neurocutaneous Melanosis

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Neurocutaneous Melanosis

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Pathways for genes affiliated with Neurocutaneous Melanosis

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Compounds for genes affiliated with Neurocutaneous Melanosis

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GO Terms for genes affiliated with Neurocutaneous Melanosis

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Products for genes affiliated with Neurocutaneous Melanosis

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Sources for Neurocutaneous Melanosis

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet