MCID: CNT025
MIFTS: 42

Central Pontine Myelinolysis malady

Categories: Rare diseases, Neuronal diseases

Aliases & Classifications for Central Pontine Myelinolysis

About this section

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

About this section
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 gestational diabetes and kernicterus, 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 Glial Cell Differentiation. Affiliated tissues include pons, brain and liver, and related mouse phenotypes are renal/urinary system 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

Wikipedia:71 Central pontine myelinolysis (CPM), also known as osmotic demyelination syndrome or central pontine... more...

Related Diseases for Central Pontine Myelinolysis

About this section

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

(show top 50)    (show all 95)
idRelated DiseaseScoreTop Affiliating Genes
1gestational diabetes29.9AQP1, AVP
2kernicterus29.6AQP4, MBP
3extrapontine myelinolysis11.5
4atypical lipomatous tumor10.9
5encephalopathy10.4
6anorexia nervosa10.2
7adenosquamous breast carcinoma10.2AVP, MBP
8cogan-reese syndrome10.2AQP4, MBP
9ovarian papillary cystadenoma10.2AQP4, MBP
10bile duct cystadenoma10.2AQP4, MBP
11subpulmonary stenosis10.2AQP1, AQP4
12dystrophinopathies10.2AQP1, AQP4
13neurilemmoma10.1AQP4, MBP
14pulmonary tuberculosis10.1AQP1, AVP
15carotid artery disease10.1AQP4, AVP
16chondromalacia10.1AVP, MBP
17noninfectious dermatoses of eyelid10.1AQP4, MBP
18lymphoma10.1
19dystonia10.1
20ataxia10.1
21niemann-pick disease, type b10.1MAG, MBP
22biphasic synovial sarcoma10.1MAG, MBP
23lymphoepithelioma-like carcinoma10.1AQP4, MBP
2446 xy gonadal dysgenesis10.1AQP1, AQP4
25mohr-tranebjaerg syndrome10.1MAG, MBP
26cerebellar ataxia10.0
27hepatitis10.0
28leukemia10.0
29hypophosphatemia10.0
30cerebritis10.0
31myoclonus10.0
32tremor10.0
33congenital syphilis10.0AQP1, AQP4
34cervical mullerian papilloma10.0AQP1, AQP4
35cardiac granular cell neoplasm10.0AVP, MAG
36alcohol dependence10.0
37wilson disease10.0
38b-cell lymphomas10.0
39diffuse large b-cell lymphoma10.0
40locked-in syndrome10.0
41quadriplegia10.0
42sjogren's syndrome10.0
43wernicke encephalopathy10.0
44pancreatitis10.0
45hypoglycemia10.0
46atrophic rhinitis9.9AQP1, AQP4
47secondary lacrimal atrophy9.9MAG, MBP
48gemistocytic astrocytoma9.9AVP, MBP
49lens disease9.9AQP1, AQP4, MBP
50newborn respiratory distress syndrome9.9AQP1, AQP4, 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

About this section

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:00053678.9AQP1, AQP4, AVP, BCL2
2MP:00053778.5AQP1, AQP4, BCL2, MAG, MBP
3MP:00053978.5AQP1, AQP4, BCL2, MAG, MBP
4MP:00053918.3AQP1, AQP4, BCL2, MBP
5MP:00053868.0AQP1, AQP4, AVP, BCL2, MAG, MBP
6MP:00053767.9AQP1, AQP4, AVP, BCL2, MAG, MBP
7MP:00036317.9AQP1, AQP4, AVP, BCL2, MAG, MBP

Drugs & Therapeutics for Central Pontine Myelinolysis

About this section

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

About this section

Anatomical Context for Central Pontine Myelinolysis

About this section

MalaCards organs/tissues related to Central Pontine Myelinolysis:

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

Publications for Central Pontine Myelinolysis

About this section

Articles related to Central Pontine Myelinolysis:

(show top 50)    (show all 375)
idTitleAuthorsYear
1
Asymptomatic central pontine myelinolysis without hyponatriemia in diffuse large B cell lymphoma. (27488303)
2016
2
IMAGES IN CLINICAL MEDICINE. Central Pontine Myelinolysis. (26886548)
2016
3
Central pontine myelinolysis secondary to hyperglycaemia. (27407176)
2016
4
Central pontine myelinolysis in Sjogren's syndrome with hypokalemia. (26929036)
2016
5
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
6
Striking Central Pontine Myelinolysis in a Patient With Alcohol Dependence Syndrome Without Hyponatremia. (26720620)
2016
7
Central pontine myelinolysis during pregnancy: Pathogenesis, diagnosis and management. (27922286)
2016
8
Central pontine myelinolysis caused by hypernatremia. (27772775)
2016
9
Psychiatric symptoms and limb tremors associated with central pontine myelinolysis: A case of alcoholism without hyponatremia. (27882183)
2016
10
Clinical Evolution of Central Pontine Myelinolysis in a Patient with Alcohol Withdrawal: A Blurred Clinical Horizon. (27610136)
2016
11
Hypertensive brainstem encephalopathy mimicking central pontine myelinolysis: a potential pitfall. (26935223)
2016
12
Central pontine myelinolysis: a rare presentation secondary to hyperglycaemia. (25917480)
2015
13
Urinary incontinence a first presentation of central pontine myelinolysis: a case report. (26209689)
2015
14
Osmotic myelinolysis: Does extrapontine myelinolysis precede central pontine myelinolysis? Report of two cases and review of literature. (25969642)
2015
15
A Rare Case of Central Pontine Myelinolysis in Overcorrection of Hyponatremia with Total Parenteral Nutrition in Pregnancy. (26798530)
2015
16
Central pontine myelinolysis in a case of alcohol dependence syndrome. (27212829)
2015
17
Diffuse large B-cell lymphoma presenting with central pontine myelinolysis: a case report. (26044457)
2015
18
Central pontine myelinolysis presenting as isolated sixth nerve palsy in third trimester of pregnancy. (25745319)
2015
19
A Case of Central Pontine Myelinolysis Caused by Hypophosphatemia Secondary to Refeeding Syndrome. (26557081)
2015
20
Central pontine myelinolysis-induced mania: A case study. (25732324)
2015
21
Central Pontine Myelinolysis with Minimal Hyponatremia in the Setting of AIDS. (26605097)
2015
22
The owl and the monkey - The varied faces of pons in central pontine myelinolysis. (26588651)
2015
23
Locked-in Syndrome due to Central Pontine Myelinolysis: Case Report. (25379502)
2014
24
Central pontine myelinolysis following pediatric living donor liver transplantation: a case report and review of literature. (24725019)
2014
25
Central pontine myelinolysis. (25395281)
2014
26
Central pontine myelinolysis without electrolyte disorder, alcoholism or denutrition. (24906708)
2014
27
Osmotic demyelination syndrome: central pontine myelinolysis and extrapontine myelinolysis. (24745890)
2014
28
Plasma exchange successfully treats central pontine myelinolysis after acute hypernatremia from intravenous sodium bicarbonate therapy. (24708786)
2014
29
Central pontine myelinolysis: electrolytes and beyond. (24682140)
2014
30
Central pontine myelinolysis in the hyperosmolar hyperglycaemic state. (22922267)
2013
31
Central pontine myelinolysis associated with Wilson disease in a 7-year-old child. (23704419)
2013
32
Central pontine myelinolysis in a chronic alcoholic: a clinical and brain magnetic resonance imaging follow-up. (24069831)
2013
33
Central pontine myelinolysis. (23760391)
2013
34
Recovery of locked-in syndrome in central pontine myelinolysis. (23826473)
2013
35
Central Pontine Myelinolysis Presenting With Tremor in a Child With Celiac Disease. (23390116)
2013
36
Central pontine myelinolysis following acute hypoglycemia. (23948485)
2013
37
Brainstem somatosensory and auditory evoked responses in central pontine myelinolysis. (23975561)
2013
38
Central pontine myelinolysis in a patient with non-Hodgkin lymphoma. (23384152)
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 with meticulous correction of hyponatraemia in chronic alcoholics. (23813514)
2013
43
Can central pontine myelinolysis be prevented through non-rapid serum sodium correction? (23065440)
2013
44
Central pontine myelinolysis. (23608854)
2013
45
Wernicke's encephalopathy and central pontine myelinolysis in hyperemesis gravidarum. (23546346)
2013
46
Central pontine myelinolysis in advanced HIV infection with tuberculosis and multicentric Castleman's disease. (23970776)
2013
47
Rare association of central pontine myelinolysis with infantile tremor syndrome. (22412274)
2012
48
Corticospinal tract and pontocerebellar fiber of central pontine myelinolysis. (23342326)
2012
49
Asymptomatic central pontine myelinolysis: a case report. (23185169)
2012
50
Central pontine myelinolysis associated with pancreatic diabetes. (21732062)
2012

Variations for Central Pontine Myelinolysis

About this section

Expression for genes affiliated with Central Pontine Myelinolysis

About this section
Search GEO for disease gene expression data for Central Pontine Myelinolysis.

Pathways for genes affiliated with Central Pontine Myelinolysis

About this section

GO Terms for genes affiliated with Central Pontine Myelinolysis

About this section

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
1cellular water homeostasisGO:000999210.2AQP1, AQP4
2glycerol transportGO:001579310.2AQP1, AQP4
3negative regulation of cysteine-type endopeptidase activity involved in apoptotic processGO:004315410.1AQP1, AVP
4cellular response to mechanical stimulusGO:007126010.1AQP1, MAG
5negative regulation of axonogenesisGO:005077110.1MAG, MBP
6cellular response to hypoxiaGO:00714569.9AQP1, BCL2
7positive regulation of cell growthGO:00303079.7AVP, BCL2
8positive regulation of peptidyl-serine phosphorylationGO:00331389.7AVP, BCL2
9multicellular organismal water homeostasisGO:00508919.7AQP1, AQP4, AVP
10response to nicotineGO:00350949.7AVP, BCL2
11response to toxic substanceGO:00096369.6BCL2, MBP
12renal water homeostasisGO:00030919.5AQP1, AQP4, AVP
13negative regulation of apoptotic processGO:00430669.3AQP1, AVP, BCL2
14water transportGO:00068339.0AQP1, AQP4, AVP

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

idNameGO IDScoreTop Affiliating Genes
1glycerol channel activityGO:001525410.0AQP1, AQP4
2water channel activityGO:00152509.6AQP1, AQP4
3protease bindingGO:00020209.6BCL2, MBP
4water transmembrane transporter activityGO:00053729.5AQP1, AQP4

Sources for Central Pontine Myelinolysis

About this section
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