CPM
MCID: CNT025
MIFTS: 44

Central Pontine Myelinolysis (CPM)

Categories: Neuronal diseases, Rare diseases
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Aliases & Classifications for Central Pontine Myelinolysis

MalaCards integrated aliases for Central Pontine Myelinolysis:

Name: Central Pontine Myelinolysis 11 19 52 14 16 75 31 33
Myelinolysis, Central Pontine 43 71
Osmotic Demyelination Syndrome 11
Myelinolysis Central Pontine 53
Central Pontine Myelinosis 33
Cpm 19

Classifications:



External Ids:

Disease Ontology 11 DOID:636
MeSH 43 D017590
NCIt 49 C84623
SNOMED-CT 68 6807001
ICD10 31 G37.2
ICD11 33 558060012
UMLS 71 C0206083

Summaries for Central Pontine Myelinolysis

NINDS: 52 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 alcohol use disorder and alcohol dependence, 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 Transport of inorganic cations/anions and amino acids/oligopeptides and Neuroscience. Affiliated tissues include pons, brain and liver, and related phenotypes are Negative genetic interaction between PTEN-/- and PTEN+/+ and nervous system

GARD: 19 The part of the brain called the pons is especially sensitive and too much sodium can damage nerve fibers. Symptoms usually 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.

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.

Wikipedia: 75 Central pontine myelinolysis is a neurological condition involving severe damage to the myelin sheath of... 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 505)
# Related Disease Score Top Affiliating Genes
1 alcohol use disorder 31.2 POMC INS AVP ALB
2 alcohol dependence 31.1 POMC INS AVP ALB
3 wernicke encephalopathy 31.1 AQP4 ALB
4 demyelinating disease 30.9 MBP MAG AQP4
5 acquired immunodeficiency syndrome 30.7 POMC MBP ALB
6 metabolic acidosis 30.6 REN AQP2 ALB
7 kwashiorkor 30.6 INS ALB
8 diabetes mellitus, ketosis-prone 30.5 REN INS ALB
9 bell's palsy 30.5 MBP ALB
10 central diabetes insipidus 30.4 POMC AVPR2 AVP AQP2
11 abducens palsy 30.4 POMC AQP4
12 facial paralysis 30.3 MBP AQP4 ALB
13 bartter disease 30.3 REN AVPR2 AVP AQP2
14 sheehan syndrome 30.3 POMC INS AVP
15 neuromyelitis optica 30.3 MBP AQP4 AQP1
16 hypertensive encephalopathy 30.2 REN AVP ALB
17 hypoglycemic coma 30.2 POMC INS
18 pituitary apoplexy 30.2 POMC INS AVP
19 beriberi 30.2 INS ALB
20 liver cirrhosis 30.2 REN INS AVP AQP2 ALB
21 polyneuropathy 30.1 MBP MAG ALB
22 autonomic neuropathy 30.0 REN INS ALB
23 urinary tract infection 30.0 REN INS ALB
24 aspiration pneumonia 30.0 POMC INS ALB
25 hypokalemia 29.9 REN POMC INS AVPR2 AVP AQP2
26 uremia 29.9 REN INS ALB
27 graves disease 1 29.9 POMC INS ALB
28 cranial nerve palsy 29.9 POMC AVP AQP4 ALB
29 lipoid congenital adrenal hyperplasia 29.9 REN POMC INS
30 hypothyroidism 29.9 REN POMC INS ALB
31 chronic kidney disease 29.8 REN INS AQP2 ALB
32 inappropriate adh syndrome 29.8 REN POMC AVPR2 AVP AQP4 AQP2
33 bilirubin metabolic disorder 29.7 INS ATP7B ALB
34 diabetes insipidus, neurohypophyseal 29.7 REN POMC AVPR2 AVP AQP2
35 prostatic hypertrophy 29.7 INS ALB
36 nephrotic syndrome 29.7 REN INS AQP2 ALB
37 major depressive disorder 29.7 POMC INS AVP ALB
38 diabetes insipidus 29.5 REN POMC INS AVPR2 AVP AQP4
39 migraine with or without aura 1 29.5 REN POMC INS AQP4 ALB
40 amyotrophic lateral sclerosis 1 29.3 MBP MAG INS AQP4 ALB
41 nephrogenic diabetes insipidus 29.2 REN AVPR2 AVP AQP4 AQP2 AQP1
42 peripheral nervous system disease 29.0 REN POMC MBP MAG INS AQP4
43 pneumoconiosis 11.1
44 cleft palate, isolated 11.1
45 liposarcoma 11.1
46 esophagus sarcoma 10.9
47 hereditary angioedema 10.9
48 lateral medullary syndrome 10.9
49 brain stem infarction 10.9
50 well-differentiated liposarcoma 10.9

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:

11
  • dysarthria
  • dysphagia
  • acute paralysis

GenomeRNAi Phenotypes related to Central Pontine Myelinolysis according to GeneCards Suite gene sharing:

25
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Negative genetic interaction between PTEN-/- and PTEN+/+ GR00255-A-3 9.02 AQP2 AQP4 ATP7B MBP SNAI1

MGI Mouse Phenotypes related to Central Pontine Myelinolysis:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 nervous system MP:0003631 10.14 AQP1 AQP4 ATP7B AVP GJC2 INS
2 homeostasis/metabolism MP:0005376 10.06 ALB AQP1 AQP2 AQP4 ATP7B AVP
3 behavior/neurological MP:0005386 10.03 AQP1 AQP2 AQP4 ATP7B AVP AVPR2
4 renal/urinary system MP:0005367 10.02 ALB AQP1 AQP2 AQP4 AVP AVPR2
5 normal MP:0002873 9.98 ALB AVP GJC2 INS MBP REN
6 immune system MP:0005387 9.9 ALB AQP1 AQP4 ATP7B AVP GJC2
7 hematopoietic system MP:0005397 9.73 AQP1 AQP2 AQP4 ATP7B AVP AVPR2
8 mortality/aging MP:0010768 9.44 ALB AQP1 AQP2 AQP4 ATP7B AVP

Drugs & Therapeutics for Central Pontine Myelinolysis

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Efficacy and Safety of Intermittent Bolus Comparing With Traditional Continuous Drip of 3% NaCl in Patients With Severe Symptomatic Hyponatremia at Rajavithi Hospital Unknown status NCT04561531

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

Organs/tissues related to Central Pontine Myelinolysis:

MalaCards : Pons, Brain, Liver, Kidney, Pituitary, Heart, Bone Marrow

Publications for Central Pontine Myelinolysis

Articles related to Central Pontine Myelinolysis:

(show top 50) (show all 1150)
# Title Authors PMID Year
1
Osmotic demyelination and hypertonic dehydration in a 9-year-old girl: changes in cerebrospinal fluid myelin basic protein. 53 62
17095502 2006
2
Sequential Brain Perfusion Findings in a Case of Osmotic Demyelination Syndrome. 62
36240840 2023
3
Pontine lesion in hyperglycemic crises: Relevance to osmotic demyelination syndrome and posterior reversible encephalopathy syndrome. 62
36458523 2022
4
Correction of severe hyponatremia by continuous veno-venous hemodialysis with regional citrate anticoagulation: A case series. 62
35114071 2022
5
A standard hemodialysis prescription to prevent osmotic demyelination in hyponatremic patients requiring dialysis. 62
35199958 2022
6
FDG PET/CT Aiding in Early Diagnosis of Central Pontine Myelinolysis. 62
36342803 2022
7
Incidence and risk factors of overcorrection in patients presenting with severe hyponatremia to the emergency department. 62
35870090 2022
8
Radio-Pathologic Correlation of the "Pig's Snout" Sign in Osmotic Demyelination Syndrome. 62
34511155 2022
9
Rapid correction of severe hyponatremia and control of subsequent overcorrection in operative hysteroscopy intravascular absorption syndrome: A case report. 62
36343073 2022
10
Parkinsonism with akinetic mutism following osmotic demyelination syndrome in a SARS-CoV-2 infected elderly diabetic woman: A case report. 62
34785833 2022
11
Parkinsonism with akinetic mutism following osmotic demyelination syndrome in a SARS-CoV-2 infected elderly diabetic woman: A case report. 62
36195379 2022
12
[Osmotic demyelination syndrome and overly rapid correction of hyponatremia]. 62
36314091 2022
13
Osmotic Demyelination Syndrome in a Patient With Tremors. 62
36381866 2022
14
Rare association of central pontine myelinolysis with intrasellar arachnoidocele - casual or correlated? 62
35923340 2022
15
Radiological Appearance and Imaging Techniques in the Diagnosis of Advanced Central Pontine Myelinolysis. 62
36407248 2022
16
Central Pontine Myelinolysis: A Case Report of Persistent Hyperglycemia With Normal Serum Sodium. 62
36312619 2022
17
A 44-Year-Old Alcohol-Dependent Man Who Recovered from Central Pontine Myelinolysis with Supportive Physical Therapy. 62
36081331 2022
18
Central Pontine Myelinolysis: A Case Report. 62
36213701 2022
19
Development of Parkinsonism in a Patient with Central Pontine Myelinolysis. 62
36135990 2022
20
Perioperative Delta Sodium and Post-Liver Transplant Neurological Complications in Liver Transplant Recipients. 62
35289775 2022
21
A case of Cotard syndrome associated with central pontine myelinolysis as a result of normally corrected hyponatremia. 62
34886708 2022
22
Neuroleptic malignant syndrome in a case of extra-pontine myelinolysis: On the horns of dilemma. 62
35753768 2022
23
Delirium Tremens and Central Pontine Myelinolysis in a Patient with Alcohol Use Disorder and Pneumonia: a Case Report and a Narrative Review. 62
35483887 2022
24
Central pontine myelinolysis in a chronic alcoholic patient with mild hyponatremia: A case report. 62
35585991 2022
25
Pseudo-Central Pontine Myelinolysis. 62
34907999 2022
26
Osmotic demyelination syndrome: novel risk factors and proposed pathophysiology. 62
35717664 2022
27
Refractory Hypernatremia and Osmotic Demyelination Syndrome After Liver Transplantation: A Case Report. 62
35760624 2022
28
Hyponatremia and Liver Transplantation: A Narrative Review. 62
34144870 2022
29
Overcorrection versus osmotic demyelination syndrome: what should we watch out for during management of symptomatic chronic hyponatremia? 62
35545222 2022
30
Risk factors for overcorrection of severe hyponatremia: a post hoc analysis of the SALSA trial. 62
35286796 2022
31
Osmotic demyelination syndrome despite appropriate gradual correction of moderate hyponatraemia. 62
35577547 2022
32
Acute diquat poisoning resulting in toxic encephalopathy: a report of three cases. 62
34982016 2022
33
A Case of Central Pontine Myelinolysis Unrelated Serum Sodium Level in Traumatic Brain Injury Patient. 62
35557639 2022
34
Osmotic Demyelination Syndrome in Hospitalized Patients With Cirrhosis: Analysis of the National Inpatient Sample (NIS). 62
33731600 2022
35
Pediatric osmotic demyelination syndrome in a case of type 1 diabetes mellitus with diabetic ketoacidosis. 62
35340640 2022
36
Tacrolimus-induced Central Pontine Myelinolysis in a Pediatric Liver Transplant Recipient. 62
35535097 2022
37
Osmotic Demyelination Syndrome: A New Mime in the Circus of Neurology. 62
33845937 2022
38
Unusual progression of osmotic demyelination after liver transplantation on MRI brain. 62
34987689 2022
39
Evaluation of Serum Sodium Correction Rates for Management of Hyponatremia in Hospitalized Patients. 62
34027713 2022
40
Bifocal lesions have a poorer treatment outcome than a single lesion in adult patients with intracranial germinoma. 62
35231071 2022
41
Congenital nephrogenic diabetes insipidus presenting as osmotic demyelination syndrome in infancy: A case report. 62
35060513 2022
42
Case Report: Hindlimb Ataxia Concurrent With Seizures by Presumed Osmotic Demyelination Syndrome in a Dog. 62
35782535 2022
43
Case Report: Osmotic Demyelination Syndrome After Transcatheter Aortic Valve Replacement: Case Report and Review of Current Literature. 62
35795632 2022
44
Imaging of extrapontine myelinolysis preceding central pontine myelinolysis in a case of ornithine transcarbamylase deficiency with hyperammonaemia and hypokalaemia. 62
36437853 2022
45
Acquired laryngomalacia as a cause of post-extubation respiratory failure in patient with postoperative seizure and central pontine myelinolysis after craniotomy. 62
36244788 2022
46
Postpartum Hypernatremia with Extrapontine Rhabdomyelinolysis: A Case Report. 62
36304065 2022
47
Occurrence of osmotic demyelination syndrome in diabetes mellitus: A case report and literature review of various etiologies for osmotic demyelination syndrome. 62
36337162 2022
48
Osmotic demyelination syndrome and thoughts on its prevention. 62
34089517 2022
49
Hyponatremia after neuroendoscopic skull base tumor surgery: Clinical characteristics and nursing management. 62
36406347 2022
50
[A geriatric case of hyperosmolar hyperglycemic state with osmotic demyelination syndrome that manifested with dysphagia]. 62
35650056 2022

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:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.83 AVPR2 AVP ATP7B AQP4 AQP2 AQP1
2 12.2 POMC MBP MAG AVP
3
Show member pathways
11.88 REN POMC INS
4
Show member pathways
11.84 POMC AVPR2 AVP
5 11.7 SNAI1 INS ALB
6 11.32 MBP MAG AQP4 AQP1
7
Show member pathways
11.06 AVPR2 AVP AQP4 AQP2 AQP1
8 10.73 POMC INS
9
Show member pathways
10.36 AVPR2 AVP
10 10.16 MBP MAG

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 plasma membrane GO:0005886 10.11 MAG AVPR2 ATP7B AQP4 AQP2 AQP1
2 plasma membrane GO:0005887 10.11 MAG AVPR2 ATP7B AQP4 AQP2 AQP1
3 basolateral plasma membrane GO:0016323 9.92 ATP7B AQP4 AQP2 AQP1
4 paranode region of axon GO:0033270 9.56 MAG GJC2
5 compact myelin GO:0043218 9.26 MBP MAG
6 myelin sheath GO:0043209 9.1 MBP MAG GJC2

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

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 cell-cell signaling GO:0007267 10.1 POMC INS GJC2 AVP
2 positive regulation of systemic arterial blood pressure GO:0003084 9.78 AVPR2 AVP
3 glycerol transmembrane transport GO:0015793 9.76 AQP2 AQP1
4 hyperosmotic salinity response GO:0042538 9.73 AVP AQP4
5 cellular water homeostasis GO:0009992 9.71 AQP4 AQP1
6 renal water homeostasis GO:0003091 9.63 AQP4 AQP2 AQP1
7 renal water transport GO:0003097 9.62 AQP1 AQP2
8 cellular response to mercury ion GO:0071288 9.56 AQP2 AQP1
9 water homeostasis GO:0030104 9.54 AQP4 AQP1
10 multicellular organismal water homeostasis GO:0050891 9.43 AVP AQP4 AQP1
11 water transport GO:0006833 9.23 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 9.56 AQP2 AQP1
2 glycerol transmembrane transporter activity GO:0015168 9.46 AQP2 AQP1
3 channel activity GO:0015267 9.13 AQP4 AQP2 AQP1
4 water channel activity GO:0015250 9.1 AQP4 AQP2 AQP1

Sources for Central Pontine Myelinolysis

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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