CPM
MCID: CNT025
MIFTS: 49

Central Pontine Myelinolysis (CPM)

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Central Pontine Myelinolysis

MalaCards integrated aliases for Central Pontine Myelinolysis:

Name: Central Pontine Myelinolysis 12 53 54 15
Myelinolysis, Central Pontine 44 73
Osmotic Demyelination Syndrome 12
Myelinolysis Central Pontine 55
Cpm 53

Classifications:



External Ids:

Disease Ontology 12 DOID:636
ICD10 33 G37.2
MeSH 44 D017590
NCIt 50 C84623
SNOMED-CT 68 6807001
UMLS 73 C0206083

Summaries for Central Pontine Myelinolysis

NINDS : 54 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 neuromyelitis optica and optic nerve disease, and has symptoms including dysarthria, dysphagia and acute paralysis. An important gene associated with Central Pontine Myelinolysis is MBP (Myelin Basic Protein), and among its related pathways/superpathways are Neuroscience and Aquaporin-mediated transport. Affiliated tissues include pons, liver and brain, 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 : 53 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.

Wikipedia : 76 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 131)
# Related Disease Score Top Affiliating Genes
1 neuromyelitis optica 30.1 AQP1 AQP4 MBP
2 optic nerve disease 29.9 AQP4 MBP
3 diabetes insipidus, nephrogenic, autosomal 29.9 AQP1 AQP4 AVP
4 extrapontine myelinolysis 11.7
5 cleft palate, isolated 11.3
6 well-differentiated liposarcoma 11.1
7 encephalopathy 10.6
8 anorexia nervosa 10.5
9 lymphoma 10.3
10 movement disease 10.3
11 dystonia 10.3
12 aceruloplasminemia 10.3
13 hepatitis 10.3
14 hypophosphatemia 10.3
15 myoclonus 10.3
16 alcohol dependence 10.2
17 sjogren syndrome 10.2
18 wilson disease 10.2
19 diabetes mellitus, ketosis-prone 10.2
20 b-cell lymphomas 10.2
21 diffuse large b-cell lymphoma 10.2
22 leukemia 10.2
23 locked-in syndrome 10.2
24 quadriplegia 10.2
25 hyperglycemia 10.2
26 hypokalemia 10.2
27 pancreatitis 10.2
28 hypoglycemia 10.2
29 tremor 10.2
30 myelitis 10.1 AQP4 MBP
31 neuroblastoma 10.1
32 colorectal adenoma 10.1
33 nervous system disease 10.1 AQP4 MBP
34 oculomotor nerve paralysis 10.1 AVP MBP
35 alexia 10.1 AQP4 MBP
36 third cranial nerve disease 10.1 AVP MBP
37 cranial nerve disease 10.1 AQP4 MBP
38 balo concentric sclerosis 10.1 AQP4 MBP
39 transverse myelitis 10.1 AQP4 MBP
40 internuclear ophthalmoplegia 10.1 AQP4 MBP
41 acute disseminated encephalomyelitis 10.1 AQP4 MBP
42 subependymoma 10.1 AQP1 AQP4
43 traumatic brain injury 10.1 AQP4 MBP
44 motion sickness 10.1 AQP1 AVP
45 impaired renal function disease 10.1 AQP1 AVP
46 autoimmune optic neuritis 10.1 AQP4 MBP
47 dysferlinopathy 10.1 AQP1 AQP4
48 anxiety 10.1
49 neutropenia 10.1
50 granulocytopenia 10.1

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



Diseases related to Central Pontine Myelinolysis

Symptoms & Phenotypes for Central Pontine Myelinolysis

Symptoms:

12
  • dysarthria
  • dysphagia
  • acute paralysis

MGI Mouse Phenotypes related to Central Pontine Myelinolysis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.88 AQP1 AQP4 AVP BCL2 MAG MBP
2 hearing/vestibular/ear MP:0005377 9.77 AQP1 AQP4 BCL2 MAG MBP
3 homeostasis/metabolism MP:0005376 9.73 AQP1 AQP4 AVP BCL2 MAG MBP
4 hematopoietic system MP:0005397 9.72 AQP1 AQP4 BCL2 MAG MBP
5 nervous system MP:0003631 9.63 AQP1 AQP4 AVP BCL2 MAG MBP
6 renal/urinary system MP:0005367 9.26 AQP1 AQP4 AVP BCL2
7 vision/eye MP:0005391 8.92 AQP1 AQP4 BCL2 MBP

Drugs & Therapeutics for Central Pontine Myelinolysis

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Optic Nerve Sheath Diameter As A Predictor Of Early Tacrolimus Neurotoxicity In Living Donor Liver Transplantation Recruiting NCT03799770

Search 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:

41
Pons, Liver, Brain, Eye, B Cells, Lung, Pituitary

Publications for Central Pontine Myelinolysis

Articles related to Central Pontine Myelinolysis:

(show top 50) (show all 396)
# Title Authors Year
1
Simultaneous acute Marchiafava-Bignami disease and central pontine myelinolysis: A case report of a challenging diagnosis. ( 29465574 )
2018
2
Latent systemic lupus erythematosus presenting with hypokalaemic quadriparesis and central pontine myelinolysis. ( 29429118 )
2018
3
Central Pontine Myelinolysis in Pediatric Diabetic Ketoacidosis. ( 29973999 )
2018
4
Desmopressin-Induced Severe Hyponatremia with Central Pontine Myelinolysis: A Case Report. ( 29696555 )
2018
5
A case of chronic asymptomatic central pontine myelinolysis with histological evidence of remyelination. ( 29177819 )
2018
6
Central Pontine Myelinolysis After Living-Donor Liver Transplant: A Report of 2 Cases. ( 29137593 )
2017
7
Central pontine myelinolysis associated with hypokalemia in a diabetic patient with sepsis. ( 28488657 )
2017
8
Wilson's disease presenting as central pontine myelinolysis. ( 28785820 )
2017
9
Acute necrotic pancreatitis: A rare and not always fatal cause of central pontine myelinolysis. ( 28757177 )
2017
10
Subacute central pontine myelinolysis secondary to hyperglycaemia. ( 29169807 )
2017
11
Central pontine myelinolysis in a type 1 diabetes patient with chronic hepatitis. ( 28583270 )
2017
12
Central Pontine Myelinolysis and Localized Fluorodeoxyglucose Uptake Seen on (18)F-FDG PET/CT. ( 28217021 )
2017
13
Atypical presentation of central pontine myelinolysis in hyperglycemia. ( 28924482 )
2017
14
Abrus Precatorius Poisoning and Central Pontine Myelinolysis. ( 29675076 )
2017
15
Acute Psychosis as Main Manifestation of Central Pontine Myelinolysis. ( 28392953 )
2017
16
Central pontine myelinolysis and poorly controlled diabetes: MRI's hints for pathogenesis. ( 28914383 )
2017
17
Central Pontine Myelinolysis Induced by Alcohol Withdrawal: A Case Report. ( 28289647 )
2017
18
Does Accidental Overcorrection of Symptomatic Hyponatremia in Chronic Heart Failure Require Specific Therapeutic Adjustments for Preventing Central Pontine Myelinolysis? ( 28270885 )
2017
19
Asymptomatic central pontine myelinolysis without hyponatriemia in diffuse large B cell lymphoma. ( 27488303 )
2016
20
IMAGES IN CLINICAL MEDICINE. Central Pontine Myelinolysis. ( 26886548 )
2016
21
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
22
Central pontine myelinolysis in Sjogren's syndrome with hypokalemia. ( 26929036 )
2016
23
Central pontine myelinolysis caused by hypernatremia. ( 27772775 )
2016
24
Central pontine myelinolysis during pregnancy: Pathogenesis, diagnosis and management. ( 27922286 )
2016
25
Is Central Pontine Myelinolysis Reversible? ( 29095576 )
2016
26
Psychiatric symptoms and limb tremors associated with central pontine myelinolysis: A case of alcoholism without hyponatremia. ( 27882183 )
2016
27
Striking Central Pontine Myelinolysis in a Patient With Alcohol Dependence Syndrome Without Hyponatremia. ( 26720620 )
2016
28
Central pontine myelinolysis secondary to hyperglycaemia. ( 27407176 )
2016
29
Hypertensive brainstem encephalopathy mimicking central pontine myelinolysis: a potential pitfall. ( 26935223 )
2016
30
Clinical Evolution of Central Pontine Myelinolysis in a Patient with Alcohol Withdrawal: A Blurred Clinical Horizon. ( 27610136 )
2016
31
Central pontine myelinolysis presenting as isolated sixth nerve palsy in third trimester of pregnancy. ( 25745319 )
2015
32
A Case of Central Pontine Myelinolysis Caused by Hypophosphatemia Secondary to Refeeding Syndrome. ( 26557081 )
2015
33
Central pontine myelinolysis in a case of alcohol dependence syndrome. ( 27212829 )
2015
34
Central pontine myelinolysis: a rare presentation secondary to hyperglycaemia. ( 25917480 )
2015
35
Central pontine myelinolysis-induced mania: A case study. ( 25732324 )
2015
36
The owl and the monkey - The varied faces of pons in central pontine myelinolysis. ( 26588651 )
2015
37
Diffuse large B-cell lymphoma presenting with central pontine myelinolysis: a case report. ( 26044457 )
2015
38
A Rare Case of Central Pontine Myelinolysis in Overcorrection of Hyponatremia with Total Parenteral Nutrition in Pregnancy. ( 26798530 )
2015
39
Osmotic myelinolysis: Does extrapontine myelinolysis precede central pontine myelinolysis? Report of two cases and review of literature. ( 25969642 )
2015
40
Urinary incontinence a first presentation of central pontine myelinolysis: a case report. ( 26209689 )
2015
41
Central Pontine Myelinolysis with Minimal Hyponatremia in the Setting of AIDS. ( 26605097 )
2015
42
Locked-in Syndrome due to Central Pontine Myelinolysis: Case Report. ( 25379502 )
2014
43
Central pontine myelinolysis without electrolyte disorder, alcoholism or denutrition. ( 24906708 )
2014
44
Central pontine myelinolysis following pediatric living donor liver transplantation: a case report and review of literature. ( 24725019 )
2014
45
Plasma exchange successfully treats central pontine myelinolysis after acute hypernatremia from intravenous sodium bicarbonate therapy. ( 24708786 )
2014
46
Osmotic demyelination syndrome: central pontine myelinolysis and extrapontine myelinolysis. ( 24745890 )
2014
47
Central pontine myelinolysis: electrolytes and beyond. ( 24682140 )
2014
48
Central pontine myelinolysis. ( 25395281 )
2014
49
Central pontine myelinolysis following acute hypoglycemia. ( 23948485 )
2013
50
Central pontine myelinolysis. ( 23760391 )
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

GO Terms for Central Pontine Myelinolysis

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

# Name GO ID Score Top Affiliating Genes
1 myelin sheath GO:0043209 9.13 BCL2 MAG MBP
2 compact myelin GO:0043218 8.62 MAG MBP

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

(show all 14)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of apoptotic process GO:0043066 9.69 AQP1 AVP BCL2
2 transmembrane transport GO:0055085 9.67 AQP1 AQP4 BCL2
3 membrane organization GO:0061024 9.54 AVP MBP
4 cellular response to hypoxia GO:0071456 9.52 AQP1 BCL2
5 response to toxic substance GO:0009636 9.48 BCL2 MBP
6 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.46 AVP BCL2
7 positive regulation of cell growth GO:0030307 9.43 AVP BCL2
8 cellular response to mechanical stimulus GO:0071260 9.4 AQP1 MAG
9 negative regulation of cysteine-type endopeptidase activity involved in apoptotic process GO:0043154 9.37 AQP1 AVP
10 renal water homeostasis GO:0003091 9.33 AQP1 AQP4 AVP
11 response to nicotine GO:0035094 9.32 AVP BCL2
12 axon regeneration GO:0031103 9.26 BCL2 MAG
13 water transport GO:0006833 9.13 AQP1 AQP4 AVP
14 multicellular organismal water homeostasis GO:0050891 8.8 AQP1 AQP4 AVP

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

# Name GO ID Score Top Affiliating Genes
1 protease binding GO:0002020 9.26 BCL2 MBP
2 water channel activity GO:0015250 9.16 AQP1 AQP4
3 water transmembrane transporter activity GO:0005372 8.96 AQP1 AQP4
4 channel activity GO:0015267 8.8 AQP1 AQP4 BCL2

Sources for Central Pontine Myelinolysis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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