MCID: CNT025
MIFTS: 38

Central Pontine Myelinolysis

Categories: Rare diseases, Neuronal diseases

Aliases & Classifications for Central Pontine Myelinolysis

MalaCards integrated aliases for Central Pontine Myelinolysis:

Name: Central Pontine Myelinolysis 12 50 51 14
Myelinolysis, Central Pontine 42 69
Osmotic Demyelination Syndrome 12
Myelinolysis Central Pontine 52
Cpm 50

Classifications:



External Ids:

Disease Ontology 12 DOID:636
ICD10 33 G37.2
MeSH 42 D017590
NCIt 47 C84623
SNOMED-CT 64 6807001
UMLS 69 C0206083

Summaries for Central Pontine Myelinolysis

NINDS : 51 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. The initial symptoms of myelinolysis, which begin to appear 2 to 3 days after hyponatremia is corrected, include a depressed level of awareness, difficulty speaking (dysarthria or mutism), and difficulty swallowing (dysphagia).  Additional symptoms often arise over the next 1-2 weeks, including impaired thinking, weakness or paralysis in the arms and legs, stiffness, impaired sensation, and difficulty with coordination.  At its most severe, myelinolysis can lead to coma, “locked-in” syndrome (which is the complete paralysis of all of the voluntary muscles in the body except for those that control the eyes), and death.  Although many affected people improve over weeks to months, some have permanent disability. Some also develop new symptoms later, including behavioral or intellectual impairment or movement disorders like parkinsonism or tremor. Anyone, including adults and children, who undergoes a rapid rise in serum sodium is at risk for myelinolysis. Some individuals who are particularly vulnerable are those with chronic alcoholism and those who have had a liver transplant. Myelinolysis has occurred in individuals undergoing renal dialysis, burn victims, people with HIV-AIDS, people over-using water loss pills (diuretics), and women with eating disorders such as anorexia or bulimia.  The risk for CPM is greater if the serum (blood) sodium was low for at least 2 days before correction. 

MalaCards based summary : Central Pontine Myelinolysis, also known as myelinolysis, central pontine, is related to extrapontine myelinolysis and encephalopathy. An important gene associated with Central Pontine Myelinolysis is MBP (Myelin Basic Protein), and among its related pathways/superpathways are Bile secretion and Spinal Cord Injury. Affiliated tissues include pons, brain and liver, and related phenotypes are behavior/neurological and hearing/vestibular/ear

Disease Ontology : 12 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.

NIH Rare Diseases : 50 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

Wikipedia : 72 Central pontine myelinolysis (CPM) is a neurological disorder caused by severe damage of the myelin... more...

Related Diseases for Central Pontine Myelinolysis

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

(show top 50) (show all 95)
id Related Disease Score Top Affiliating Genes
1 extrapontine myelinolysis 11.5
2 encephalopathy 10.3
3 anorexia nervosa 10.2
4 cogan-reese syndrome 10.1 AQP4 MBP
5 hepatitis 10.1
6 lymphoma 10.1
7 dystonia 10.1
8 ataxia 10.1
9 tricho onychic dysplasia 10.1 AQP4 MBP
10 mixed cell adenoma 10.1 AQP4 MBP
11 tracheal stenosis 10.1 AQP4 MBP
12 ulcerative colitis 10.1 AQP4 MBP
13 cellular schwannoma 10.1 AQP4 MBP
14 efemp2-related cutis laxa 10.1 AQP1 AQP4
15 microcephaly, cerebellar hypoplasia, and cardiac conduction defect syndrome 10.1 AQP1 AQP4
16 sudden sensorineural hearing loss 10.1 AQP1 AQP4
17 laryngeal adenoid cystic carcinoma 10.1 AQP1 BCL2
18 cerebellar ataxia 10.0
19 leukemia 10.0
20 hypophosphatemia 10.0
21 cerebritis 10.0
22 pancreatitis 10.0
23 myoclonus 10.0
24 tremor 10.0
25 chondrocalcinosis 10.0 AQP1 BCL2
26 alcohol dependence 10.0
27 wilson disease 10.0
28 b-cell lymphomas 10.0
29 diffuse large b-cell lymphoma 10.0
30 locked-in syndrome 10.0
31 quadriplegia 10.0
32 sjogren's syndrome 10.0
33 wernicke encephalopathy 10.0
34 hyperglycemia 10.0
35 hypokalemia 10.0
36 hypoglycemia 10.0
37 cervix squamous papilloma 9.9 AQP1 AQP4
38 interstitial keratitis 9.9 AQP1 AQP4
39 niemann-pick disease, type b 9.9 MAG MBP
40 palmoplantar keratoderma, bothnian type 9.9 AQP1 AQP4
41 epithelioid cell synovial sarcoma 9.9 MAG MBP
42 hypoxia 9.8 MAG MBP
43 mohr-tranebjaerg syndrome 9.8 MAG MBP
44 calciphylaxis 9.8 AQP1 AQP4
45 schizophrenia 9.8
46 myocardial infarction 9.8
47 malaria 9.8
48 lung cancer 9.8
49 hodgkin lymphoma 9.8
50 celiac disease 9.8

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



Diseases related to Central Pontine Myelinolysis

Symptoms & Phenotypes for Central Pontine Myelinolysis

MGI Mouse Phenotypes related to Central Pontine Myelinolysis:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.77 MAG MBP AQP1 AQP4 BCL2
2 hearing/vestibular/ear MP:0005377 9.72 MAG MBP AQP1 AQP4 BCL2
3 hematopoietic system MP:0005397 9.65 MAG MBP AQP1 AQP4 BCL2
4 homeostasis/metabolism MP:0005376 9.55 BCL2 MAG MBP AQP1 AQP4
5 nervous system MP:0003631 9.35 MAG MBP AQP1 AQP4 BCL2
6 vision/eye MP:0005391 8.92 MBP AQP1 AQP4 BCL2

Drugs & Therapeutics for Central Pontine Myelinolysis

Search Clinical Trials , NIH Clinical Center for Central Pontine Myelinolysis

Cochrane evidence based reviews: myelinolysis, central pontine

Genetic Tests for Central Pontine Myelinolysis

Anatomical Context for Central Pontine Myelinolysis

MalaCards organs/tissues related to Central Pontine Myelinolysis:

39
Pons, Brain, Liver, Eye, B Cells, Endothelial, Pituitary

Publications for Central Pontine Myelinolysis

Articles related to Central Pontine Myelinolysis:

(show top 50) (show all 385)
id Title Authors Year
1
Central pontine myelinolysis in a type 1 diabetes patient with chronic hepatitis. ( 28583270 )
2017
2
Acute Psychosis as Main Manifestation of Central Pontine Myelinolysis. ( 28392953 )
2017
3
Atypical presentation of central pontine myelinolysis in hyperglycemia. ( 28924482 )
2017
4
Central pontine myelinolysis and poorly controlled diabetes: MRI's hints for pathogenesis. ( 28914383 )
2017
5
Does Accidental Overcorrection of Symptomatic Hyponatremia in Chronic Heart Failure Require Specific Therapeutic Adjustments for Preventing Central Pontine Myelinolysis? ( 28270885 )
2017
6
Central pontine myelinolysis associated with hypokalemia in a diabetic patient with sepsis. ( 28488657 )
2017
7
Central Pontine Myelinolysis Induced by Alcohol Withdrawal: A Case Report. ( 28289647 )
2017
8
Wilson's disease presenting as central pontine myelinolysis. ( 28785820 )
2017
9
Central Pontine Myelinolysis and Localized Fluorodeoxyglucose Uptake Seen on (18)F-FDG PET/CT. ( 28217021 )
2017
10
Acute necrotic pancreatitis: A rare and not always fatal cause of central pontine myelinolysis. ( 28757177 )
2017
11
Psychiatric symptoms and limb tremors associated with central pontine myelinolysis: A case of alcoholism without hyponatremia. ( 27882183 )
2016
12
Asymptomatic central pontine myelinolysis without hyponatriemia in diffuse large B cell lymphoma. ( 27488303 )
2016
13
IMAGES IN CLINICAL MEDICINE. Central Pontine Myelinolysis. ( 26886548 )
2016
14
Striking Central Pontine Myelinolysis in a Patient With Alcohol Dependence Syndrome Without Hyponatremia. ( 26720620 )
2016
15
Hypertensive brainstem encephalopathy mimicking central pontine myelinolysis: a potential pitfall. ( 26935223 )
2016
16
Clinical Evolution of Central Pontine Myelinolysis in a Patient with Alcohol Withdrawal: A Blurred Clinical Horizon. ( 27610136 )
2016
17
Central pontine myelinolysis caused by hypernatremia. ( 27772775 )
2016
18
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
19
Central pontine myelinolysis during pregnancy: Pathogenesis, diagnosis and management. ( 27922286 )
2016
20
Central pontine myelinolysis secondary to hyperglycaemia. ( 27407176 )
2016
21
Central pontine myelinolysis in Sjogren's syndrome with hypokalemia. ( 26929036 )
2016
22
A Rare Case of Central Pontine Myelinolysis in Overcorrection of Hyponatremia with Total Parenteral Nutrition in Pregnancy. ( 26798530 )
2015
23
A Case of Central Pontine Myelinolysis Caused by Hypophosphatemia Secondary to Refeeding Syndrome. ( 26557081 )
2015
24
Central pontine myelinolysis presenting as isolated sixth nerve palsy in third trimester of pregnancy. ( 25745319 )
2015
25
Diffuse large B-cell lymphoma presenting with central pontine myelinolysis: a case report. ( 26044457 )
2015
26
Central Pontine Myelinolysis with Minimal Hyponatremia in the Setting of AIDS. ( 26605097 )
2015
27
Central pontine myelinolysis: a rare presentation secondary to hyperglycaemia. ( 25917480 )
2015
28
Osmotic myelinolysis: Does extrapontine myelinolysis precede central pontine myelinolysis? Report of two cases and review of literature. ( 25969642 )
2015
29
The owl and the monkey - The varied faces of pons in central pontine myelinolysis. ( 26588651 )
2015
30
Urinary incontinence a first presentation of central pontine myelinolysis: a case report. ( 26209689 )
2015
31
Central pontine myelinolysis in a case of alcohol dependence syndrome. ( 27212829 )
2015
32
Central pontine myelinolysis-induced mania: A case study. ( 25732324 )
2015
33
Plasma exchange successfully treats central pontine myelinolysis after acute hypernatremia from intravenous sodium bicarbonate therapy. ( 24708786 )
2014
34
Central pontine myelinolysis: electrolytes and beyond. ( 24682140 )
2014
35
Central pontine myelinolysis without electrolyte disorder, alcoholism or denutrition. ( 24906708 )
2014
36
Osmotic demyelination syndrome: central pontine myelinolysis and extrapontine myelinolysis. ( 24745890 )
2014
37
Central pontine myelinolysis following pediatric living donor liver transplantation: a case report and review of literature. ( 24725019 )
2014
38
Central pontine myelinolysis. ( 25395281 )
2014
39
Locked-in Syndrome due to Central Pontine Myelinolysis: Case Report. ( 25379502 )
2014
40
Wernicke's encephalopathy and central pontine myelinolysis in hyperemesis gravidarum. ( 23546346 )
2013
41
Central pontine myelinolysis in advanced HIV infection with tuberculosis and multicentric Castleman's disease. ( 23970776 )
2013
42
Central pontine myelinolysis with meticulous correction of hyponatraemia in chronic alcoholics. ( 23813514 )
2013
43
Central pontine myelinolysis. ( 23608854 )
2013
44
Central pontine myelinolysis in the hyperosmolar hyperglycaemic state. ( 22922267 )
2013
45
Central pontine myelinolysis in a patient with non-Hodgkin lymphoma. ( 23384152 )
2013
46
Central pontine myelinolysis in a patient with persistent mild hypernatremia following cadaver donor liver transplantation. ( 23904947 )
2013
47
Central pontine myelinolysis following acute hypoglycemia. ( 23948485 )
2013
48
Recovery of locked-in syndrome in central pontine myelinolysis. ( 23826473 )
2013
49
Central Pontine Myelinolysis Presenting With Tremor in a Child With Celiac Disease. ( 23390116 )
2013
50
Central pontine myelinolysis associated with Wilson disease in a 7-year-old child. ( 23704419 )
2013

Variations for Central Pontine Myelinolysis

Expression for Central Pontine Myelinolysis

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

Pathways for Central Pontine Myelinolysis

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

id Super pathways Score Top Affiliating Genes
1 11.11 AQP1 AQP4
2 11 AQP1 AQP4 MAG MBP
3 10.95 MAG MBP
4 9.86 MAG MBP

GO Terms for Central Pontine Myelinolysis

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

id Name GO ID Score Top Affiliating Genes
1 myelin sheath GO:0043209 9.13 BCL2 MAG MBP
2 compact myelin GO:0043218 8.32 MAG

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

id Name GO ID Score Top Affiliating Genes
1 negative regulation of neuron apoptotic process GO:0043524 9.4 BCL2 MAG
2 cellular response to hypoxia GO:0071456 9.37 AQP1 BCL2
3 response to toxic substance GO:0009636 9.32 BCL2 MBP
4 cellular response to mechanical stimulus GO:0071260 9.26 AQP1 MAG
5 renal water homeostasis GO:0003091 9.16 AQP1 AQP4
6 water transport GO:0006833 8.96 AQP1 AQP4
7 multicellular organismal water homeostasis GO:0050891 8.62 AQP1 AQP4

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

id Name GO ID Score Top Affiliating Genes
1 protease binding GO:0002020 9.16 BCL2 MBP
2 water channel activity GO:0015250 8.96 AQP1 AQP4
3 water transmembrane transporter activity GO:0005372 8.62 AQP1 AQP4

Sources for Central Pontine Myelinolysis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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