CPM
MCID: CNT025
MIFTS: 46

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 52 53 15 17 32
Myelinolysis, Central Pontine 43 71
Osmotic Demyelination Syndrome 12
Myelinolysis Central Pontine 54
Cpm 52

Classifications:



External Ids:

Disease Ontology 12 DOID:636
MeSH 43 D017590
NCIt 49 C84623
SNOMED-CT 67 6807001
ICD10 32 G37.2
UMLS 71 C0206083

Summaries for Central Pontine Myelinolysis

NINDS : 53 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 demyelinating disease and neuromyelitis optica, and has symptoms including dysphagia, dysarthria and acute paralysis. An important gene associated with Central Pontine Myelinolysis is MBP (Myelin Basic Protein), and among its related pathways/superpathways are Sudden Infant Death Syndrome (SIDS) Susceptibility Pathways and Aquaporin-mediated transport. The drugs tannic acid and Benzocaine have been mentioned in the context of this disorder. Affiliated tissues include liver, pons and brain, and related phenotypes are behavior/neurological and hematopoietic system

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 : 52 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 : 74 Central pontine myelinolysis (CPM) is a neurological condition involving severe damage to 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 285)
# Related Disease Score Top Affiliating Genes
1 demyelinating disease 30.8 MBP MAG AQP4
2 neuromyelitis optica 30.7 MBP AQP4 AQP1
3 cranial nerve palsy 30.2 AVP AQP4
4 diabetes insipidus 30.2 AVPR2 AVP AQP2
5 optic nerve disease 29.9 MBP MAG AQP4 AQP1
6 hydrocephalus 29.7 MBP AVPR2 AQP4 AQP1
7 diabetes insipidus, nephrogenic, autosomal 29.2 AVPR2 AVP AQP4 AQP2 AQP1
8 extrapontine myelinolysis 11.9
9 wernicke encephalopathy 11.9
10 liposarcoma 11.4
11 well-differentiated liposarcoma 11.4
12 esophagus sarcoma 11.2
13 dentine erosion 11.2
14 lateral medullary syndrome 11.2
15 brain stem infarction 11.2
16 alcohol dependence 10.9
17 quadriplegia 10.8
18 encephalopathy 10.7
19 ataxia and polyneuropathy, adult-onset 10.7
20 locked-in syndrome 10.7
21 pseudobulbar palsy 10.7
22 ocular motor apraxia 10.6
23 alcohol use disorder 10.6
24 tremor 10.6
25 dystonia 10.6
26 anorexia nervosa 10.5
27 hypokalemia 10.5
28 subacute delirium 10.5
29 dysphagia 10.5
30 movement disease 10.5
31 hepatic encephalopathy 10.5
32 hyperglycemia 10.5
33 specific language disorder 10.5
34 hypophosphatemia 10.4
35 hepatic coma 10.4
36 liver cirrhosis 10.4
37 myoclonus 10.4
38 acute retrobulbar neuritis 10.4 MBP AQP4
39 metabolic acidosis 10.4
40 hypoglycemia 10.4
41 pseudobulbar affect 10.4
42 autoimmune optic neuritis 10.4 MBP AQP4
43 chiari malformation 10.4 MBP AQP1
44 internuclear ophthalmoplegia 10.4 MBP AQP4
45 subependymoma 10.4 AQP4 AQP1
46 dysferlinopathy 10.4 AQP4 AQP1
47 transverse myelitis 10.3 MBP AQP4
48 traumatic brain injury 10.3 MBP AQP4
49 acute hemorrhagic leukoencephalitis 10.3 MBP AQP4
50 benign mammary dysplasia 10.3 BCL2 BAK1

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
  • dysphagia
  • dysarthria
  • acute paralysis

MGI Mouse Phenotypes related to Central Pontine Myelinolysis:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 10.17 AQP1 AQP2 AQP4 AVP AVPR2 BAK1
2 hematopoietic system MP:0005397 10.11 AQP1 AQP2 AQP4 AVP AVPR2 BAK1
3 homeostasis/metabolism MP:0005376 10.02 AQP1 AQP2 AQP4 AVP AVPR2 BAK1
4 mortality/aging MP:0010768 9.93 AQP1 AQP2 AQP4 AVP AVPR2 BAK1
5 immune system MP:0005387 9.91 AQP4 AVP BAK1 BCL2 FOXM1 GJC2
6 hearing/vestibular/ear MP:0005377 9.8 AQP1 AQP4 BAK1 BCL2 MBP
7 nervous system MP:0003631 9.61 AQP1 AQP4 AVP BAK1 BCL2 GJC2
8 renal/urinary system MP:0005367 9.17 AQP1 AQP2 AQP4 AVP AVPR2 BAK1

Drugs & Therapeutics for Central Pontine Myelinolysis

Drugs for Central Pontine Myelinolysis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 11)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
tannic acid Approved Phase 4 1401-55-4
2
Benzocaine Approved, Investigational Phase 4 94-09-7, 1994-09-7 2337
3
carbamide peroxide Approved 124-43-6
4
Aspartic acid Approved, Nutraceutical 56-84-8 5960
5 Anesthetics
6 Liver Extracts
7 N-Methylaspartate
8 Calcium, Dietary
9
Bilirubin 69853-43-6, 635-65-4 21252250 5280352
10
L-Alanine Nutraceutical 56-41-7 5950
11
Calcium Nutraceutical 7440-70-2 271

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Efficacy and Safety of Rapid Intermittent Correction Compared With Slow Continuous Correction With Hypertonic Saline in Patient With Moderately Severe or Severe Symptomatic Severe Hyponatremia (SALSA Trial) Completed NCT02887469 Phase 4 3% hypertonic saline
2 Changes in Plasma Osmolality Related to the Use of Cardiopulmonary Bypass With Hyperosmolar Prime, an Observational Study. Completed NCT03060824
3 Evaluation of Preoperative and Postoperative S100β and Neuron Specific Enolase Levels in Patients Undergoing Liver Transplantation Recruiting NCT03453047

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:

40
Liver, Pons, Brain, Eye, Heart, Pituitary, B Cells

Publications for Central Pontine Myelinolysis

Articles related to Central Pontine Myelinolysis:

(show top 50) (show all 804)
# Title Authors PMID Year
1
Osmotic demyelination and hypertonic dehydration in a 9-year-old girl: changes in cerebrospinal fluid myelin basic protein. 61 54
17095502 2006
2
Electrolyte Disorders and the Nervous System. 61
32487900 2020
3
Central pontine myelinolysis in a patient with bulimia: Case report and literature review. 61
32097845 2020
4
Pontine Neoplasm or Myelinolysis Despite Normal Sodium Levels. 61
32416240 2020
5
Osmotic Demyelination Syndrome Despite Appropriate Hyponatremia Correction. 61
32577327 2020
6
Subacute central pontine myelinolysis secondary to hyperglycaemia. 61
29169807 2020
7
Orthostatic Myoclonus Secondary to Central Pontine Myelinolysis. 61
32258236 2020
8
Magnetic resonance spectroscopy findings of central pontine myelinolysis in an alcohol abuser. 61
32097207 2020
9
Wernicke encephalopathy associated with hyperemesis gravidarum. 61
31784394 2020
10
Neurological Complications in Recipients after Living Donor Liver Transplantation. 61
32129264 2020
11
Neurological and neuropsychiatric manifestations of porphyria. 61
31402774 2019
12
Incidence of osmotic demyelination syndrome in Sweden: A nationwide study. 61
31343728 2019
13
Central Pontine Myelinosis and Osmotic Demyelination Syndrome. 61
31587708 2019
14
Central Pontine Myelinolysis After Living-Donor Liver Transplant: A Report of 2 Cases. 61
29137593 2019
15
Osmotic Demyelination Syndrome in Children. 61
31128892 2019
16
Occult central pontine myelinolysis post liver transplant: A consequence of pre-transplant hyponatremia. 61
31056363 2019
17
Histopathological changes of organ dysfunction in sepsis. 61
31346833 2019
18
Real-World Effectiveness and Tolerability of Tolvaptan in Patients With Heart Failure - Final Results of the Samsca Post-Marketing Surveillance in Heart Failure (SMILE) Study. 61
31118355 2019
19
Severe hypokalaemia in diabetic ketoacidosis: a contributor to central pontine myelinolysis? 61
31368676 2019
20
Localized atrophy of the pontine base as a sequela of prolonged ischemia: Report of an autopsy case. 61
30834588 2019
21
The role of the interdisciplinary team in subacute rehabilitation for central pontine myelinolysis. 61
30950662 2019
22
Neurologic conditions and disorders of uremic syndrome of chronic kidney disease: presentations, causes, and treatment strategies. 61
30501441 2019
23
[Osmotic demyelination syndrome complicating the quick correction of a severe hyponatremia associated with hypokalemia]. 61
32180882 2019
24
Extrapontine Myelinolysis and Reversible Parkinsonism After Hyponatremia Correction in a Case of Pituitary Adenoma: Hypopituitarism as a Predisposition for Osmotic Demyelination. 61
30055367 2018
25
[A case of liver cirrhosis with central pontine myelinolysis]. 61
30481888 2018
26
Normal Correction of Sodium Leading to Central Pontine Demyelinosis: A Rare Occurrence. 61
30416903 2018
27
[Disturbances of Sodium Homeostasis]. 61
30036896 2018
28
Latent systemic lupus erythematosus presenting with hypokalaemic quadriparesis and central pontine myelinolysis. 61
29429118 2018
29
Desmopressin-Induced Severe Hyponatremia with Central Pontine Myelinolysis: A Case Report. 61
29696555 2018
30
Tongue fasciculations with denervation pattern in osmotic demyelination syndrome: a case report of diagnostic dilemma. 61
29540223 2018
31
A case of chronic asymptomatic central pontine myelinolysis with histological evidence of remyelination. 61
29177819 2018
32
Simultaneous acute Marchiafava-Bignami disease and central pontine myelinolysis: A case report of a challenging diagnosis. 61
29465574 2018
33
Central Pontine Myelinolysis in Pediatric Diabetic Ketoacidosis. 61
29973999 2018
34
Astroglial Modulation of Hydromineral Balance and Cerebral Edema. 61
29946238 2018
35
Central pontine myelinolysis and poorly controlled diabetes: MRI's hints for pathogenesis. 61
28914383 2018
36
Central pontine myelinolysis in a type 1 diabetes patient with chronic hepatitis. 61
28583270 2018
37
Utility of Tolvaptan in the Perioperative Management of Severe Hyponatremia During Liver Transplantation: A Case Report. 61
29198689 2017
38
Central pontine myelinolysis in Sjogren's syndrome with hypokalemia. 61
26929036 2017
39
Wilson's disease presenting as central pontine myelinolysis. 61
28785820 2017
40
Rapid-onset obesity with hypothalamic dysregulation, hypoventilation, and autonomic dysregulation (ROHHAD syndrome): A case report and literature review. 61
27340018 2017
41
Abrus Precatorius Poisoning and Central Pontine Myelinolysis. 61
29675076 2017
42
Acute necrotic pancreatitis: A rare and not always fatal cause of central pontine myelinolysis. 61
28757177 2017
43
Localized Pontine Uptake in Fluorine-18-Fuorodeoxyglucose Positron Emission Tomography/Computed Tomography in a Case of Hyponatremia: A Case Report and Review of Literature. 61
29142352 2017
44
Osmotic Demyelination Syndrome Revisited: Review With Neuroimaging. 61
28850553 2017
45
Demyelinating diseases as a result of cerebral edema? 61
28673564 2017
46
Central pontine myelinolysis associated with hypokalemia in a diabetic patient with sepsis. 61
28488657 2017
47
Osmotic demyelination syndrome in type 1 diabetes in the absence of dyselectrolytaemia: an overlooked complication? 61
28500261 2017
48
Central pontine myelinolysis during pregnancy: Pathogenesis, diagnosis and management. 61
27922286 2017
49
Does Accidental Overcorrection of Symptomatic Hyponatremia in Chronic Heart Failure Require Specific Therapeutic Adjustments for Preventing Central Pontine Myelinolysis? 61
28270885 2017
50
[Antypsychotic-induced central pontine myelinolysis in a congenital adrenal hyperplasia subject]. 61
28229445 2017

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 integral component of plasma membrane GO:0005887 9.8 MAG IL2RB AVPR2 AQP4 AQP2 AQP1
2 pore complex GO:0046930 9.26 BCL2 BAK1
3 paranode region of axon GO:0033270 9.16 MAG GJC2
4 compact myelin GO:0043218 8.96 MBP MAG
5 myelin sheath GO:0043209 8.92 MBP MAG GJC2 BCL2

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

(show all 23)
# Name GO ID Score Top Affiliating Genes
1 transmembrane transport GO:0055085 9.89 GJC2 BCL2 AQP4 AQP2 AQP1
2 regulation of cell cycle GO:0051726 9.76 FOXM1 BCL2 BAK1
3 membrane organization GO:0061024 9.73 MBP AVPR2 AVP
4 response to toxic substance GO:0009636 9.7 MBP GJC2 BCL2
5 cellular response to mechanical stimulus GO:0071260 9.63 MAG BAK1 AQP1
6 regulation of mitochondrial membrane potential GO:0051881 9.61 BCL2 BAK1
7 cellular response to copper ion GO:0071280 9.61 AQP2 AQP1
8 hair follicle morphogenesis GO:0031069 9.6 SNAI1 BCL2
9 response to gamma radiation GO:0010332 9.59 BCL2 BAK1
10 release of cytochrome c from mitochondria GO:0001836 9.58 BCL2 BAK1
11 B cell homeostasis GO:0001782 9.57 BCL2 BAK1
12 endoplasmic reticulum calcium ion homeostasis GO:0032469 9.55 BCL2 BAK1
13 axon regeneration GO:0031103 9.54 MAG BCL2
14 positive regulation of systemic arterial blood pressure GO:0003084 9.51 AVPR2 AVP
15 regulation of mitochondrial membrane permeability GO:0046902 9.48 BCL2 BAK1
16 glycerol transport GO:0015793 9.43 AQP2 AQP1
17 cellular water homeostasis GO:0009992 9.4 AQP4 AQP1
18 leukocyte homeostasis GO:0001776 9.37 BCL2 BAK1
19 multicellular organismal water homeostasis GO:0050891 9.33 AVP AQP4 AQP1
20 cellular response to mercury ion GO:0071288 9.32 AQP2 AQP1
21 renal water transport GO:0003097 9.26 AQP2 AQP1
22 water transport GO:0006833 9.26 AVP AQP4 AQP2 AQP1
23 renal water homeostasis GO:0003091 9.02 AVPR2 AVP AQP4 AQP2 AQP1

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

# Name GO ID Score Top Affiliating Genes
1 water transmembrane transporter activity GO:0005372 9.26 AQP2 AQP1
2 glycerol transmembrane transporter activity GO:0015168 9.16 AQP2 AQP1
3 water channel activity GO:0015250 9.13 AQP4 AQP2 AQP1
4 channel activity GO:0015267 8.92 BCL2 AQP4 AQP2 AQP1

Sources for Central Pontine Myelinolysis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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