CPM
MCID: CNT025
MIFTS: 41

Central Pontine Myelinolysis (CPM) malady

Categories: Rare diseases, Neuronal diseases

Aliases & Classifications for Central Pontine Myelinolysis

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Aliases & Descriptions for Central Pontine Myelinolysis:

Name: Central Pontine Myelinolysis 11 48 49 13
Myelinolysis, Central Pontine 39 68
Osmotic Demyelination Syndrome 11
 
Myelinolysis Central Pontine 50
Cpm 48

Classifications:



External Ids:

Disease Ontology11 DOID:636
ICD1030 G37.2
SNOMED-CT62 6807001
MeSH39 D017590
NCIt45 C84623

Summaries for Central Pontine Myelinolysis

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NIH Rare Diseases:48 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 extrapontine myelinolysis and atypical lipomatous tumor, and has symptoms including acute paralysis, dysphagia and dysarthria. An important gene associated with Central Pontine Myelinolysis is MBP (Myelin Basic Protein), and among its related pathways are Bile secretion and MECP2 and Associated Rett Syndrome. Affiliated tissues include pons, brain and liver, and related mouse phenotypes are behavior/neurological and hearing/vestibular/ear.

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:49 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 pons.  Some individuals will also have damage in other areas of the brain, which is called extrapontine myelinolysis (EPM). Experts estimate that 10 percent of those with CPM will also have areas of EPM.

Wikipedia:71 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 97)
idRelated DiseaseScoreTop Affiliating Genes
1extrapontine myelinolysis11.5
2atypical lipomatous tumor11.0
3encephalopathy10.3
4anorexia nervosa10.2
5lymphoma10.1
6dystonia10.1
7ataxia10.1
8cerebellar liponeurocytoma10.1BCL2, MBP
9crest syndrome10.1AQP4, MBP
10trichomalacia10.1AQP4, MBP
11gastric dilatation10.1AQP4, MBP
12clear cell adenoma10.1AQP4, MBP
13ehlers-danlos syndrome, kyphoscoliotic form10.1AQP1, AQP4
14superior vena cava syndrome10.0AQP1, AQP4
15cerebellar ataxia10.0
16hepatitis10.0
17leukemia10.0
18hypophosphatemia10.0
19cerebritis10.0
20myoclonus10.0
21tremor10.0
22urea cycle disorder10.0AQP4, MBP
23lung adenoid cystic carcinoma10.0AQP1, BCL2
24leiomyoma10.0AQP1, AQP4
25schwannoma of twelfth cranial nerve10.0AQP4, MBP
26rickets due to defect in vitamin d 25-hydroxylation10.0MAG, MBP
27urethral gland abscess10.0AQP1, AQP4
28nerve fibre bundle defect10.0AQP4, MBP
29uterine corpus cancer10.0AQP1, AQP4
30lice infestation10.0MAG, MBP
31basal cell carcinoma 410.0AQP1, AQP4
32mohr-tranebjaerg syndrome10.0MAG, MBP
33korsakoff's amnesic syndrome10.0MAG, MBP
34alcohol dependence10.0
35wilson disease10.0
36b-cell lymphomas10.0
37diffuse large b-cell lymphoma10.0
38locked-in syndrome10.0
39quadriplegia10.0
40sjogren's syndrome10.0
41wernicke encephalopathy10.0
42pancreatitis10.0
43hypoglycemia10.0
44chondrocalcinosis10.0AQP1, BCL2
45histiocytoid hemangioma9.9AQP1, AQP4
46ovarian disease9.9AQP1, AQP4, MBP
47niemann-pick disease9.9AQP1, AQP4, MBP
48white piedra9.9MAG, MBP
49neonatal jaundice9.8AQP4, MBP
50megakaryocytic leukemia9.8BCL2, MAG, MBP

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



Diseases related to central pontine myelinolysis

Symptoms & Phenotypes for Central Pontine Myelinolysis

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Symptoms:

 11
  • acute paralysis
  • dysphagia
  • dysarthria

MGI Mouse Phenotypes related to Central Pontine Myelinolysis according to GeneCards Suite gene sharing:

41
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053868.4AQP1, AQP4, BCL2, MAG, MBP
2MP:00053778.4AQP1, AQP4, BCL2, MAG, MBP
3MP:00053918.4AQP1, AQP4, BCL2, MBP
4MP:00053978.4AQP1, AQP4, BCL2, MAG, MBP
5MP:00053768.3AQP1, AQP4, BCL2, MAG, MBP
6MP:00036318.3AQP1, AQP4, BCL2, MAG, MBP

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:

36
Pons, Brain, Liver, B cells, Pituitary, Endothelial, Lung

Publications for Central Pontine Myelinolysis

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Articles related to Central Pontine Myelinolysis:

(show top 50)    (show all 375)
idTitleAuthorsYear
1
Clinical Evolution of Central Pontine Myelinolysis in a Patient with Alcohol Withdrawal: A Blurred Clinical Horizon. (27610136)
2016
2
Poster 282 Functional Gains after Diagnosis of Central Pontine Myelinolysis due to Hypophosphatemia in a Leukemia Patient on an Acute Inpatient Rehabilitation Unit: A Case Report. (27673041)
2016
3
IMAGES IN CLINICAL MEDICINE. Central Pontine Myelinolysis. (26886548)
2016
4
Central pontine myelinolysis during pregnancy: Pathogenesis, diagnosis and management. (27922286)
2016
5
Striking Central Pontine Myelinolysis in a Patient With Alcohol Dependence Syndrome Without Hyponatremia. (26720620)
2016
6
Hypertensive brainstem encephalopathy mimicking central pontine myelinolysis: a potential pitfall. (26935223)
2016
7
Central pontine myelinolysis caused by hypernatremia. (27772775)
2016
8
Psychiatric symptoms and limb tremors associated with central pontine myelinolysis: A case of alcoholism without hyponatremia. (27882183)
2016
9
Asymptomatic central pontine myelinolysis without hyponatriemia in diffuse large B cell lymphoma. (27488303)
2016
10
Central pontine myelinolysis in Sjogren's syndrome with hypokalemia. (26929036)
2016
11
Central pontine myelinolysis secondary to hyperglycaemia. (27407176)
2016
12
Osmotic myelinolysis: Does extrapontine myelinolysis precede central pontine myelinolysis? Report of two cases and review of literature. (25969642)
2015
13
Urinary incontinence a first presentation of central pontine myelinolysis: a case report. (26209689)
2015
14
The owl and the monkey - The varied faces of pons in central pontine myelinolysis. (26588651)
2015
15
Diffuse large B-cell lymphoma presenting with central pontine myelinolysis: a case report. (26044457)
2015
16
Central pontine myelinolysis-induced mania: A case study. (25732324)
2015
17
A Case of Central Pontine Myelinolysis Caused by Hypophosphatemia Secondary to Refeeding Syndrome. (26557081)
2015
18
A Rare Case of Central Pontine Myelinolysis in Overcorrection of Hyponatremia with Total Parenteral Nutrition in Pregnancy. (26798530)
2015
19
Central Pontine Myelinolysis with Minimal Hyponatremia in the Setting of AIDS. (26605097)
2015
20
Central pontine myelinolysis presenting as isolated sixth nerve palsy in third trimester of pregnancy. (25745319)
2015
21
Central pontine myelinolysis in a case of alcohol dependence syndrome. (27212829)
2015
22
Central pontine myelinolysis: a rare presentation secondary to hyperglycaemia. (25917480)
2015
23
Central pontine myelinolysis. (25395281)
2014
24
Plasma exchange successfully treats central pontine myelinolysis after acute hypernatremia from intravenous sodium bicarbonate therapy. (24708786)
2014
25
Central pontine myelinolysis: electrolytes and beyond. (24682140)
2014
26
Locked-in Syndrome due to Central Pontine Myelinolysis: Case Report. (25379502)
2014
27
Osmotic demyelination syndrome: central pontine myelinolysis and extrapontine myelinolysis. (24745890)
2014
28
Central pontine myelinolysis following pediatric living donor liver transplantation: a case report and review of literature. (24725019)
2014
29
Central pontine myelinolysis without electrolyte disorder, alcoholism or denutrition. (24906708)
2014
30
Central pontine myelinolysis associated with Wilson disease in a 7-year-old child. (23704419)
2013
31
Wernicke's encephalopathy and central pontine myelinolysis in hyperemesis gravidarum. (23546346)
2013
32
Central Pontine Myelinolysis Presenting With Tremor in a Child With Celiac Disease. (23390116)
2013
33
Central pontine myelinolysis in advanced HIV infection with tuberculosis and multicentric Castleman's disease. (23970776)
2013
34
Central pontine myelinolysis in the hyperosmolar hyperglycaemic state. (22922267)
2013
35
Can central pontine myelinolysis be prevented through non-rapid serum sodium correction? (23065440)
2013
36
Central pontine myelinolysis. (23760391)
2013
37
Central pontine myelinolysis with meticulous correction of hyponatraemia in chronic alcoholics. (23813514)
2013
38
Central pontine myelinolysis. (23608854)
2013
39
Central pontine myelinolysis in a patient with persistent mild hypernatremia following cadaver donor liver transplantation. (23904947)
2013
40
Evidence of aquaporin involvement in human central pontine myelinolysis. (24252214)
2013
41
Central pontine myelinolysis in a chronic alcoholic patient with hyperglycemic hyperosmotic state. (24030096)
2013
42
Central pontine myelinolysis following acute hypoglycemia. (23948485)
2013
43
Recovery of locked-in syndrome in central pontine myelinolysis. (23826473)
2013
44
Central pontine myelinolysis in a chronic alcoholic: a clinical and brain magnetic resonance imaging follow-up. (24069831)
2013
45
Brainstem somatosensory and auditory evoked responses in central pontine myelinolysis. (23975561)
2013
46
Central pontine myelinolysis in a patient with non-Hodgkin lymphoma. (23384152)
2013
47
Central pontine myelinolysis: a case report and clinical-pathological review. (22023701)
2012
48
Asymptomatic central pontine myelinolysis: a case report. (23185169)
2012
49
Pontine hyperperfusion during recovery from central pontine myelinolysis. (22466847)
2012
50
Rare association of central pontine myelinolysis with infantile tremor syndrome. (22412274)
2012

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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Pathways related to Central Pontine Myelinolysis according to GeneCards Suite gene sharing:

idSuper pathwaysScoreTop Affiliating Genes
19.3AQP1, AQP4
29.2MAG, MBP
39.2MAG, MBP
48.1AQP1, AQP4, MAG, MBP

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:00432098.7BCL2, MAG, MBP

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

(show all 7)
idNameGO IDScoreTop Affiliating Genes
1cellular response to hypoxiaGO:007145610.1AQP1, BCL2
2multicellular organismal water homeostasisGO:00508919.9AQP1, AQP4
3renal water homeostasisGO:00030919.9AQP1, AQP4
4response to toxic substanceGO:00096369.8BCL2, MBP
5cellular response to mechanical stimulusGO:00712609.7AQP1, MAG
6negative regulation of neuron apoptotic processGO:00435249.7BCL2, MAG
7water transportGO:00068339.3AQP1, AQP4

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

idNameGO IDScoreTop Affiliating Genes
1protease bindingGO:00020209.8BCL2, MBP
2water channel activityGO:00152509.5AQP1, AQP4
3water transmembrane transporter activityGO:00053729.3AQP1, AQP4

Sources for Central Pontine Myelinolysis

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet