MCID: CNT025
MIFTS: 43

Central Pontine Myelinolysis malady

Category: Neuronal diseases

Aliases & Classifications for Central Pontine Myelinolysis

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Aliases & Descriptions for Central Pontine Myelinolysis:

Name: Central Pontine Myelinolysis 11 46 47 13
Myelinolysis, Central Pontine 37 66
Osmotic Demyelination Syndrome 11
 
Myelinolysis Central Pontine 48
Cpm 46

Classifications:



External Ids:

Disease Ontology11 DOID:636
ICD1028 G37.2
SNOMED-CT60 6807001
MeSH37 D017590
NCIt43 C84623

Summaries for Central Pontine Myelinolysis

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NIH Rare Diseases:46 Central pontine myelinolysis (cpm) is a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly.  the part of the brain called the pons is especially sensitive and too much sodium can damage nerve fibers.  initial signs and symptoms appear within two to three days and include a depressed level of awareness, difficulty speaking, and difficulty swallowing.  additional symptoms often occur in the next one to two weeks, including impaired thinking, weakness or paralysis in the arms and legs, stiffness, impaired sensation, and difficulty with coordination.  severe cpm can lead to coma, “locked-in” syndrome, and death. treatment is focused on safely restoring sodium levels and relieving symptoms. many affected people improve over weeks to months; however, some have permanent disability or develop new symptoms later on including behavioral or intellectual impairment or movement disorders like parkinsonism or tremor. last updated: 7/21/2011

MalaCards based summary: Central Pontine Myelinolysis, also known as myelinolysis, central pontine, is related to gestational diabetes and kernicterus, and has symptoms including acute paralysis, dysphagia and dysarthria. An important gene associated with Central Pontine Myelinolysis is AQP1 (Aquaporin 1 (Colton Blood Group)), and among its related pathways are Bile secretion and Vasopressin-regulated water reabsorption. Affiliated tissues include pons, brain and liver, and related mouse phenotypes are vision/eye and renal/urinary system.

Disease Ontology:11 A demyelination disease that is characterized by severe damage to the myelin sheath of the pons' nerve cells and has symptom acute paralysis, has symptom dysphagia, and has symptom dysarthria.

NINDS:47 Central pontine myelinolysis (CPM) is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency (hyponatremia). The rapid rise in sodium concentration is accompanied by the movement of small molecules and pulls water from brain cells. Through a mechanism that is only partly understood, the shift in water and brain molecules leads to the destruction of myelin, a substance that surrounds and protects nerve fibers. Nerve cells (neurons) can also be damaged. Certain areas of the brain are particularly susceptible to myelinolysis, especially the part of the brain stem called the

Wikipedia:69 Central pontine myelinolysis (CPM), also known as Osmotic demyelination syndrome or Central pontine... more...

Related Diseases for Central Pontine Myelinolysis

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Diseases related to Central Pontine Myelinolysis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 101)
idRelated DiseaseScoreTop Affiliating Genes
1gestational diabetes30.2AQP1, AVP
2kernicterus29.8AQP4, MBP
3liposarcoma11.1
4dentine erosion10.9
5atypical lipomatous tumor10.9
6extrapontine myelinolysis10.9
7encephalopathy10.5
8anorexia nervosa10.4
9chandler syndrome10.3AVP, MBP
10adenosquamous breast carcinoma10.3AVP, MBP
11cogan-reese syndrome10.3AQP4, MBP
12ovarian papillary cystadenoma10.3AQP4, MBP
13lymphoma10.2
14dystonia10.2
15ataxia10.2
16bile duct cystadenoma10.2AQP4, MBP
17subpulmonary stenosis10.2AQP1, AQP4
18dystrophinopathies10.2AQP1, AQP4
19neurilemmoma10.2AQP4, MBP
20pulmonary tuberculosis10.2AQP1, AVP
21carotid artery disease10.2AQP4, AVP
22chondromalacia10.2AVP, MBP
23cerebellar ataxia10.2
24hepatitis10.2
25cerebritis10.2
26myoclonus10.2
27noninfectious dermatoses of eyelid10.2AQP4, MBP
28niemann-pick disease, type b10.1MAG, MBP
29biphasic synovial sarcoma10.1MAG, MBP
30lymphoepithelioma-like carcinoma10.1AQP4, MBP
3146 xy gonadal dysgenesis10.1AQP1, AQP4
32mohr-tranebjaerg syndrome10.1MAG, MBP
33alcohol dependence10.1
34wilson disease10.1
35b-cell lymphomas10.1
36diffuse large b-cell lymphoma10.1
37leukemia10.1
38hypophosphatemia10.1
39locked-in syndrome10.1
40quadriplegia10.1
41sjogren's syndrome10.1
42wernicke encephalopathy10.1
43pancreatitis10.1
44hypoglycemia10.1
45tremor10.1
46congenital syphilis10.0AQP1, AQP4
47cervical mullerian papilloma10.0AQP1, AQP4
48cardiac granular cell neoplasm10.0AVP, MAG
49chronic pain10.0
50atrophic rhinitis9.9AQP1, AQP4

Graphical network of the top 20 diseases related to Central Pontine Myelinolysis:



Diseases related to central pontine myelinolysis

Symptoms for Central Pontine Myelinolysis

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Symptoms:

 11
  • acute paralysis
  • dysphagia
  • dysarthria

Drugs & Therapeutics for Central Pontine Myelinolysis

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Interventional clinical trials:

Search ClinicalTrials, NIH Clinical Center for Central Pontine Myelinolysis


Cochrane evidence based reviews: myelinolysis, central pontine

Genetic Tests for Central Pontine Myelinolysis

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Anatomical Context for Central Pontine Myelinolysis

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MalaCards organs/tissues related to Central Pontine Myelinolysis:

34
Pons, Brain, Liver, B cells, Pituitary, Endothelial, Lung

Animal Models for Central Pontine Myelinolysis or affiliated genes

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MGI Mouse Phenotypes related to Central Pontine Myelinolysis:

39
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053918.3AQP1, AQP4, BCL2, MBP
2MP:00053678.1AQP1, AQP4, AVP, BCL2
3MP:00053778.1AQP1, AQP4, BCL2, MAG, MBP
4MP:00053977.9AQP1, AQP4, BCL2, MAG, MBP
5MP:00036317.2AQP1, AQP4, AVP, BCL2, MAG, MBP
6MP:00053767.1AQP1, AQP4, AVP, BCL2, MAG, MBP
7MP:00053866.8AQP1, AQP4, AVP, BCL2, MAG, MBP

Publications for Central Pontine Myelinolysis

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Articles related to Central Pontine Myelinolysis:

(show top 50)    (show all 371)
idTitleAuthorsYear
1
Asymptomatic central pontine myelinolysis without hyponatriemia in diffuse large B cell lymphoma. (27488303)
2016
2
Central pontine myelinolysis: a rare presentation secondary to hyperglycaemia. (25917480)
2015
3
Urinary incontinence a first presentation of central pontine myelinolysis: a case report. (26209689)
2015
4
Locked-in Syndrome due to Central Pontine Myelinolysis: Case Report. (25379502)
2014
5
Central pontine myelinolysis in the hyperosmolar hyperglycaemic state. (22922267)
2013
6
Central pontine myelinolysis associated with Wilson disease in a 7-year-old child. (23704419)
2013
7
Central pontine myelinolysis in a chronic alcoholic: a clinical and brain magnetic resonance imaging follow-up. (24069831)
2013
8
Central pontine myelinolysis. (23760391)
2013
9
Recovery of locked-in syndrome in central pontine myelinolysis. (23826473)
2013
10
Rare association of central pontine myelinolysis with infantile tremor syndrome. (22412274)
2012
11
Corticospinal tract and pontocerebellar fiber of central pontine myelinolysis. (23342326)
2012
12
Central pontine myelinolysis secondary to hypokalaemic nephrogenic diabetes insipidus. (19940203)
2010
13
Central pontine myelinolysis due to rapid correction of hyponatremia induced by excessive water intake. (21063343)
2008
14
Central pontine myelinolysis in a patient with acute lymphoblastic leukemia after hematopoietic stem cell transplantation: a case report. (18437020)
2008
15
Central pontine myelinolysis. (17151584)
2006
16
Hyponatraemia and central pontine myelinolysis after elective colonoscopy. (15804252)
2005
17
Central pontine myelinolysis in chronic alcoholism demonstrated by magnetic resonance imaging and spectroscopy. (15073445)
2004
18
Benign type of central pontine myelinolysis in alcoholism--clinical, neuroradiological and electrophysiological findings. (14504969)
2003
19
Efficacy of clozapine in a non-schizophrenic patient with psychogenic polydipsia and central pontine myelinolysis. (12404683)
2002
20
Improvement of central pontine myelinolysis as demonstrated by repeated magnetic resonance imaging in a patient without evidence of hyponatremia. (10544728)
1999
21
Severe hyponatraemia secondary to beer potomania complicated by central pontine myelinolysis. (10622059)
1998
22
Central pontine myelinolysis in a previously healthy 4-year-old child with acute rotavirus gastroenteritis. (9113955)
1997
23
Extra and central pontine myelinolysis in a child with adrenal insufficiency. (9367298)
1997
24
Central pontine myelinolysis with complete recovery. (8136581)
1994
25
Central pontine myelinolysis secondary to frequent and rapid shifts in plasma glucose in a diabetic haemodialysis patient. (8396751)
1993
26
Movement disorders as sequelae of central pontine myelinolysis: report of three cases. (1584236)
1992
27
Magnetic resonance imaging in central pontine myelinolysis. (2277006)
1990
28
Central pontine myelinolysis following correction of hyponatremia: MRI diagnosis. (2358009)
1990
29
Central pontine myelinolysis after liver transplantation. (2648187)
1989
30
Cerebellar ataxia with recovery related to central pontine myelinolysis. (3171622)
1988
31
Distribution of demyelinating lesions in pontine and extrapontine myelinolysis--three autopsy cases including one case devoid of central pontine myelinolysis. (3962604)
1986
32
Central pontine myelinolysis and abnormalities in serum sodium. (3815936)
1986
33
Central pontine myelinolysis. A case report with typical neuropathological findings. (3716763)
1986
34
Neurological complications associated with parenteral treatment: central pontine myelinolysis and Wernicke's encephalopathy. (3081224)
1986
35
Changing concepts in treatment of severe symptomatic hyponatremia. Rapid correction and possible relation to central pontine myelinolysis. (4014266)
1985
36
Central pontine myelinolysis in a patient with adrenal insufficiency. (4019226)
1985
37
Central pontine myelinolysis with widespread extrapontine lesions: a report of two cases. (6568941)
1984
38
Evolution of central pontine myelinolysis on CT. (6421119)
1984
39
Central pontine myelinolysis: correlation between CT and electrophysiologic data. (6410789)
1983
40
Central pontine myelinolysis: an immunofluorescent study. (7051753)
1982
41
Central pontine myelinolysis. (7329883)
1981
42
Demyelination of the lateral geniculate nucleus in central pontine myelinolysis. (7235634)
1981
43
Association of central pontine myelinolysis and Marchiafave-Bignami disease. (569789)
1978
44
Central pontine myelinolysis and Rosenthal fibers of the brainstem. Association with emaciation and prolonged intravenous hyperalimentation. (4117115)
1972
45
Central pontine myelinolysis in childhood. (5110124)
1971
46
Subacute necrotizing encephalomyelopathy. Its relationship to central pontine myelinolysis. (5478948)
1970
47
Central pontine myelinolysis and cirrhosis of the liver. A report of four cases. (5912488)
1966
48
Central pontine myelinolysis. A pathogenetics hypothesis. (5890887)
1965
49
CENTRAL PONTINE MYELINOLYSIS. (14200786)
1964
50
CENTRAL PONTINE MYELINOLYSIS. (A CASE REPORT). (14191012)
1964

Variations for Central Pontine Myelinolysis

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Expression for genes affiliated with Central Pontine Myelinolysis

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Search GEO for disease gene expression data for Central Pontine Myelinolysis.

Pathways for genes affiliated with Central Pontine Myelinolysis

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GO Terms for genes affiliated with Central Pontine Myelinolysis

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Cellular components related to Central Pontine Myelinolysis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1myelin sheathGO:00432098.6BCL2, MAG, MBP

Biological processes related to Central Pontine Myelinolysis according to GeneCards Suite gene sharing:

(show all 14)
idNameGO IDScoreTop Affiliating Genes
1glycerol transportGO:001579310.2AQP1, AQP4
2cellular water homeostasisGO:000999210.2AQP1, AQP4
3cellular response to mechanical stimulusGO:00712609.9AQP1, MAG
4negative regulation of axonogenesisGO:00507719.8MAG, MBP
5water transportGO:00068339.6AQP1, AQP4, AVP
6negative regulation of cysteine-type endopeptidase activity involved in apoptotic processGO:00431549.6AQP1, AVP
7multicellular organismal water homeostasisGO:00508919.6AQP1, AQP4, AVP
8renal water homeostasisGO:00030919.4AQP1, AQP4, AVP
9cellular response to hypoxiaGO:00714569.4AQP1, BCL2
10positive regulation of peptidyl-serine phosphorylationGO:00331389.3AVP, BCL2
11response to toxic substanceGO:00096369.2BCL2, MBP
12positive regulation of cell growthGO:00303079.0AVP, BCL2
13response to nicotineGO:00350948.8AVP, BCL2
14negative regulation of apoptotic processGO:00430668.7AQP1, AVP, BCL2

Molecular functions related to Central Pontine Myelinolysis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1water transmembrane transporter activityGO:00053729.9AQP1, AQP4
2water channel activityGO:00152509.9AQP1, AQP4
3glycerol channel activityGO:00152549.9AQP1, AQP4
4protease bindingGO:00020209.2BCL2, MBP

Sources for Central Pontine Myelinolysis

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
25GTR
26HGMD
27HMDB
28ICD10
29ICD10 via Orphanet
30ICD9CM
31IUPHAR
32KEGG
35MedGen
37MeSH
38MESH via Orphanet
39MGI
42NCI
43NCIt
44NDF-RT
47NINDS
48Novoseek
50OMIM
51OMIM via Orphanet
55PubMed
56QIAGEN
61SNOMED-CT via Orphanet
65Tumor Gene Family of Databases
66UMLS
67UMLS via Orphanet