MCID: CNT025
MIFTS: 45

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 49 50 14
Myelinolysis, Central Pontine 41 69
Osmotic Demyelination Syndrome 12
Myelinolysis Central Pontine 51
Cpm 49

Classifications:



External Ids:

Disease Ontology 12 DOID:636
ICD10 32 G37.2
MeSH 41 D017590
NCIt 46 C84623
SNOMED-CT 64 6807001
UMLS 69 C0206083

Summaries for Central Pontine Myelinolysis

NINDS : 50 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 diabetes insipidus, nephrogenic, autosomal and extrapontine myelinolysis, 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/superpathways are Neuroscience and Aquaporin-mediated transport. Affiliated tissues include pons, brain and liver, and related phenotypes are behavior/neurological and hearing/vestibular/ear

NIH Rare Diseases : 49 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

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.

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 110)
# Related Disease Score Top Affiliating Genes
1 diabetes insipidus, nephrogenic, autosomal 29.5 AQP1 AQP4 AVP
2 extrapontine myelinolysis 11.6
3 encephalopathy 10.4
4 bulimia nervosa 2 10.3
5 ataxia and polyneuropathy, adult-onset 10.2
6 hepatitis 10.2
7 lymphoma 10.2
8 dystonia 10.2
9 balo concentric sclerosis 10.2 AQP4 MBP
10 oculomotor nerve paralysis 10.2 AVP MBP
11 third cranial nerve disease 10.2 AVP MBP
12 acute disseminated encephalomyelitis 10.2 AQP4 MBP
13 transverse myelitis 10.1 AQP4 MBP
14 dysferlinopathy 10.1 AQP1 AQP4
15 aceruloplasminemia 10.1
16 ataxia-oculomotor apraxia 3 10.1
17 leukemia 10.1
18 hypophosphatemia 10.1
19 cerebritis 10.1
20 pancreatitis 10.1
21 myoclonus 10.1
22 tremor 10.1
23 subependymoma 10.1 AQP1 AQP4
24 internuclear ophthalmoplegia 10.1 AQP4 MBP
25 intracranial hypertension, idiopathic 10.1 AQP1 AQP4
26 myelitis 10.1 AQP4 MBP
27 traumatic brain injury 10.1 AQP4 MBP
28 impaired renal function disease 10.1 AQP1 AVP
29 spinal cord disease 10.1 AQP4 MBP
30 motion sickness 10.1 AQP1 AVP
31 niemann-pick disease, type a 10.1 MAG MBP
32 alcohol dependence 10.0
33 sjogren syndrome 10.0
34 wilson disease 10.0
35 b-cell lymphomas 10.0
36 diffuse large b-cell lymphoma 10.0
37 locked-in syndrome 10.0
38 quadriplegia 10.0
39 wernicke encephalopathy 10.0
40 hyperglycemia 10.0
41 hypokalemia 10.0
42 hypoglycemia 10.0
43 intracranial hypertension 10.0 AQP1 AQP4
44 spindle cell synovial sarcoma 10.0 MAG MBP
45 hereditary neuropathies 10.0 MAG MBP
46 meniere disease 10.0 AQP1 AQP4
47 pelizaeus-merzbacher disease 10.0 MAG MBP
48 brain edema 9.9 AQP1 AQP4
49 relapsing-remitting multiple sclerosis 9.9 AQP4 MBP
50 dentatorubral-pallidoluysian atrophy 9.9

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
  • acute paralysis
  • dysphagia
  • dysarthria

MGI Mouse Phenotypes related to Central Pontine Myelinolysis:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.88 AQP4 AVP BCL2 MAG AQP1 MBP
2 hearing/vestibular/ear MP:0005377 9.77 AQP4 BCL2 MAG AQP1 MBP
3 homeostasis/metabolism MP:0005376 9.73 AQP4 AVP BCL2 MAG AQP1 MBP
4 hematopoietic system MP:0005397 9.72 MBP AQP4 BCL2 MAG AQP1
5 nervous system MP:0003631 9.63 AQP4 AVP BCL2 MAG AQP1 MBP
6 renal/urinary system MP:0005367 9.26 AQP4 AVP BCL2 AQP1
7 vision/eye MP:0005391 8.92 AQP1 MBP 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:

38
Pons, Brain, Liver, Eye, B Cells, Endothelial, Pituitary

Publications for Central Pontine Myelinolysis

Articles related to Central Pontine Myelinolysis:

(show top 50) (show all 390)
# Title Authors Year
1
Latent systemic lupus erythematosus presenting with hypokalaemic quadriparesis and central pontine myelinolysis. ( 29429118 )
2018
2
Subacute central pontine myelinolysis secondary to hyperglycaemia. ( 29169807 )
2017
3
A case of chronic asymptomatic central pontine myelinolysis with histological evidence of remyelination. ( 29177819 )
2017
4
Acute Psychosis as Main Manifestation of Central Pontine Myelinolysis. ( 28392953 )
2017
5
Central Pontine Myelinolysis After Living-Donor Liver Transplant: A Report of 2 Cases. ( 29137593 )
2017
6
Central Pontine Myelinolysis Induced by Alcohol Withdrawal: A Case Report. ( 28289647 )
2017
7
Central pontine myelinolysis and poorly controlled diabetes: MRI's hints for pathogenesis. ( 28914383 )
2017
8
Central pontine myelinolysis in a type 1 diabetes patient with chronic hepatitis. ( 28583270 )
2017
9
Central pontine myelinolysis associated with hypokalemia in a diabetic patient with sepsis. ( 28488657 )
2017
10
Does Accidental Overcorrection of Symptomatic Hyponatremia in Chronic Heart Failure Require Specific Therapeutic Adjustments for Preventing Central Pontine Myelinolysis? ( 28270885 )
2017
11
Central Pontine Myelinolysis and Localized Fluorodeoxyglucose Uptake Seen on (18)F-FDG PET/CT. ( 28217021 )
2017
12
Wilson's disease presenting as central pontine myelinolysis. ( 28785820 )
2017
13
Atypical presentation of central pontine myelinolysis in hyperglycemia. ( 28924482 )
2017
14
Acute necrotic pancreatitis: A rare and not always fatal cause of central pontine myelinolysis. ( 28757177 )
2017
15
Clinical Evolution of Central Pontine Myelinolysis in a Patient with Alcohol Withdrawal: A Blurred Clinical Horizon. ( 27610136 )
2016
16
IMAGES IN CLINICAL MEDICINE. Central Pontine Myelinolysis. ( 26886548 )
2016
17
Is Central Pontine Myelinolysis Reversible? ( 29095576 )
2016
18
Striking Central Pontine Myelinolysis in a Patient With Alcohol Dependence Syndrome Without Hyponatremia. ( 26720620 )
2016
19
Central pontine myelinolysis secondary to hyperglycaemia. ( 27407176 )
2016
20
Central pontine myelinolysis during pregnancy: Pathogenesis, diagnosis and management. ( 27922286 )
2016
21
Hypertensive brainstem encephalopathy mimicking central pontine myelinolysis: a potential pitfall. ( 26935223 )
2016
22
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
23
Psychiatric symptoms and limb tremors associated with central pontine myelinolysis: A case of alcoholism without hyponatremia. ( 27882183 )
2016
24
Asymptomatic central pontine myelinolysis without hyponatriemia in diffuse large B cell lymphoma. ( 27488303 )
2016
25
Central pontine myelinolysis in Sjogren's syndrome with hypokalemia. ( 26929036 )
2016
26
Central pontine myelinolysis caused by hypernatremia. ( 27772775 )
2016
27
The owl and the monkey - The varied faces of pons in central pontine myelinolysis. ( 26588651 )
2015
28
Diffuse large B-cell lymphoma presenting with central pontine myelinolysis: a case report. ( 26044457 )
2015
29
A Case of Central Pontine Myelinolysis Caused by Hypophosphatemia Secondary to Refeeding Syndrome. ( 26557081 )
2015
30
Urinary incontinence a first presentation of central pontine myelinolysis: a case report. ( 26209689 )
2015
31
Central pontine myelinolysis: a rare presentation secondary to hyperglycaemia. ( 25917480 )
2015
32
Central pontine myelinolysis-induced mania: A case study. ( 25732324 )
2015
33
Central Pontine Myelinolysis with Minimal Hyponatremia in the Setting of AIDS. ( 26605097 )
2015
34
Central pontine myelinolysis in a case of alcohol dependence syndrome. ( 27212829 )
2015
35
Central pontine myelinolysis presenting as isolated sixth nerve palsy in third trimester of pregnancy. ( 25745319 )
2015
36
A Rare Case of Central Pontine Myelinolysis in Overcorrection of Hyponatremia with Total Parenteral Nutrition in Pregnancy. ( 26798530 )
2015
37
Osmotic myelinolysis: Does extrapontine myelinolysis precede central pontine myelinolysis? Report of two cases and review of literature. ( 25969642 )
2015
38
Plasma exchange successfully treats central pontine myelinolysis after acute hypernatremia from intravenous sodium bicarbonate therapy. ( 24708786 )
2014
39
Osmotic demyelination syndrome: central pontine myelinolysis and extrapontine myelinolysis. ( 24745890 )
2014
40
Locked-in Syndrome due to Central Pontine Myelinolysis: Case Report. ( 25379502 )
2014
41
Central pontine myelinolysis without electrolyte disorder, alcoholism or denutrition. ( 24906708 )
2014
42
Central pontine myelinolysis following pediatric living donor liver transplantation: a case report and review of literature. ( 24725019 )
2014
43
Central pontine myelinolysis: electrolytes and beyond. ( 24682140 )
2014
44
Central pontine myelinolysis. ( 25395281 )
2014
45
Can central pontine myelinolysis be prevented through non-rapid serum sodium correction? ( 23065440 )
2013
46
Central pontine myelinolysis in advanced HIV infection with tuberculosis and multicentric Castleman's disease. ( 23970776 )
2013
47
Central pontine myelinolysis in a patient with persistent mild hypernatremia following cadaver donor liver transplantation. ( 23904947 )
2013
48
Central pontine myelinolysis associated with Wilson disease in a 7-year-old child. ( 23704419 )
2013
49
Central pontine myelinolysis following acute hypoglycemia. ( 23948485 )
2013
50
Recovery of locked-in syndrome in central pontine myelinolysis. ( 23826473 )
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:

# Name GO ID Score Top Affiliating Genes
1 transmembrane transport GO:0055085 9.65 AQP1 AQP4 BCL2
2 negative regulation of apoptotic process GO:0043066 9.58 AQP1 AVP BCL2
3 cellular response to hypoxia GO:0071456 9.46 AQP1 BCL2
4 response to toxic substance GO:0009636 9.43 BCL2 MBP
5 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.37 AVP BCL2
6 cellular response to mechanical stimulus GO:0071260 9.32 AQP1 MAG
7 negative regulation of cysteine-type endopeptidase activity involved in apoptotic process GO:0043154 9.26 AQP1 AVP
8 multicellular organismal water homeostasis GO:0050891 9.16 AQP1 AQP4
9 renal water homeostasis GO:0003091 9.13 AQP1 AQP4 AVP
10 water transport GO:0006833 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
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 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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