CPM
MCID: CNT025
MIFTS: 42

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 20 53 15 17 32
Myelinolysis, Central Pontine 44 70
Osmotic Demyelination Syndrome 12
Myelinolysis Central Pontine 54
Cpm 20

Classifications:



External Ids:

Disease Ontology 12 DOID:636
MeSH 44 D017590
NCIt 50 C84623
SNOMED-CT 67 6807001
ICD10 32 G37.2
UMLS 70 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 wernicke encephalopathy and demyelinating 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 Transport of glucose and other sugars, bile salts and organic acids, metal ions and amine compounds and Aquaporin-mediated transport. Affiliated tissues include brain, pons and kidney, 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.

GARD : 20 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 : 73 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 316)
# Related Disease Score Top Affiliating Genes
1 wernicke encephalopathy 32.1 AQP4 ALB
2 demyelinating disease 30.8 MBP MAG AQP4
3 neuromyelitis optica 30.7 MBP AQP4 AQP1
4 hypokalemia 30.3 AVPR2 AQP2 ALB
5 diabetes insipidus 30.1 AVPR2 AVP AQP2
6 polyneuropathy 30.0 MBP MAG ALB
7 acquired immunodeficiency syndrome 29.9 MBP IL2RB ALB
8 cranial nerve palsy 29.9 AVP AQP4 ALB
9 bartter disease 29.9 AVPR2 AVP AQP2
10 peripheral nervous system disease 29.8 MBP MAG AQP4 ALB
11 diabetes insipidus, nephrogenic, autosomal 29.2 AVPR2 AVP AQP4 AQP2 AQP1
12 extrapontine myelinolysis 11.4
13 cleft palate, isolated 11.0
14 liposarcoma 11.0
15 well-differentiated liposarcoma 11.0
16 esophagus sarcoma 10.9
17 dentine erosion 10.9
18 tooth erosion 10.9
19 lateral medullary syndrome 10.9
20 brain stem infarction 10.9
21 alcohol dependence 10.9
22 quadriplegia 10.7
23 ataxia and polyneuropathy, adult-onset 10.7
24 locked-in syndrome 10.7
25 pseudobulbar palsy 10.7
26 encephalopathy 10.7
27 alcohol use disorder 10.6
28 tremor 10.6
29 dystonia 10.6
30 ocular motor apraxia 10.5
31 anorexia nervosa 10.5
32 subacute delirium 10.5
33 movement disease 10.5
34 dysphagia 10.5
35 hepatic encephalopathy 10.4
36 hyperglycemia 10.4
37 specific language disorder 10.4
38 hypophosphatemia 10.4
39 hepatic coma 10.4
40 myoclonus 10.4
41 metabolic acidosis 10.4
42 renal tubular acidosis 10.4
43 hypoglycemia 10.4
44 distal renal tubular acidosis 10.4
45 pseudobulbar affect 10.4
46 acute retrobulbar neuritis 10.3 MBP AQP4
47 autoimmune optic neuritis 10.3 MBP AQP4
48 internuclear ophthalmoplegia 10.3 MBP AQP4
49 sjogren syndrome 10.3
50 diabetes mellitus, ketosis-prone 10.3

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 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 ALB AQP1 AQP2 AQP4 AVP AVPR2
4 immune system MP:0005387 9.96 ALB AQP1 AQP4 AVP BAK1 BCL2
5 mortality/aging MP:0010768 9.9 ALB AQP1 AQP2 AQP4 AVP AVPR2
6 hearing/vestibular/ear MP:0005377 9.88 AQP1 AQP4 BAK1 BCL2 MAG MBP
7 nervous system MP:0003631 9.56 AQP1 AQP4 AVP BAK1 BCL2 GJC2
8 renal/urinary system MP:0005367 9.23 ALB AQP1 AQP2 AQP4 AVP AVPR2

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:

40
Brain, Pons, Kidney, Liver, Pituitary, Heart, Bone Marrow

Publications for Central Pontine Myelinolysis

Articles related to Central Pontine Myelinolysis:

(show top 50) (show all 818)
# 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
Clinical utility of brain MRS imaging of patients with adult-onset non-cirrhotic hyperammonemia. 61
33763331 2021
3
Central pontine myelinolysis and the osmotic demyelination syndromes: an open and shut case? 61
33713026 2021
4
Continuous hemodialysis with citrate anticoagulation and standard dialysate for managing acute kidney injury in patients with moderate to severe hyponatremia-A retrospective study. 61
33595109 2021
5
Case report of anorexia nervosa showing periventricular gliosis at autopsy. 61
33474800 2021
6
[Central pontine myelinolysis during qualified alcohol withdrawal therapy. A case report]. 61
33230716 2020
7
Lack of astrocytes hinders parenchymal oligodendrocyte precursor cells from reaching a myelinating state in osmolyte-induced demyelination. 61
33357244 2020
8
A multidisciplinary advanced approach in central pontine myelinolysis recovery: considerations about a case report. 61
33290136 2020
9
Cerebral Imaging in Patients with COVID-19 and Neurological Symptoms: First Experience from two University Hospitals in Northern Germany. 61
33212537 2020
10
Central pontine myelinolysis during treatment of hyperglycemic hyperosmolar syndrome: a case report. 61
33292743 2020
11
A highly unusual case of osmotic demyelination syndrome and extrapontine myelinolysis in a 3-month-old infant with Bartter syndrome. 61
33107776 2020
12
The role of the interdisciplinary team in subacute rehabilitation for central pontine myelinolysis. 61
30950662 2020
13
Equimolar doses of hypertonic agents (saline or mannitol) in the treatment of intracranial hypertension after severe traumatic brain injury. 61
32957318 2020
14
Pontine Neoplasm or Myelinolysis Despite Normal Sodium Levels. 61
32416240 2020
15
Lethal diquat poisoning manifesting as central pontine myelinolysis and acute kidney injury: A case report and literature review. 61
32734801 2020
16
Electrolyte Disorders and the Nervous System. 61
32487900 2020
17
Central pontine myelinolysis in a patient with bulimia: Case report and literature review. 61
32097845 2020
18
Osmotic Demyelination Syndrome Despite Appropriate Hyponatremia Correction. 61
32577327 2020
19
Orthostatic Myoclonus Secondary to Central Pontine Myelinolysis. 61
32258236 2020
20
Magnetic resonance spectroscopy findings of central pontine myelinolysis in an alcohol abuser. 61
32097207 2020
21
Subacute central pontine myelinolysis secondary to hyperglycaemia. 61
29169807 2020
22
Wernicke encephalopathy associated with hyperemesis gravidarum. 61
31784394 2020
23
Pontine Myelinolysis Caused by Hypovolemic Hypernatremia. 61
32963856 2020
24
Unusual Presentation of Polyautoimmunity and Renal Tubular Acidosis in an Adolescent With Hashimoto's Thyroiditis and Central Pontine Myelinolysis. 61
33162932 2020
25
Neurological Complications in Recipients after Living Donor Liver Transplantation. 61
32129264 2020
26
Neurological and neuropsychiatric manifestations of porphyria. 61
31402774 2019
27
Incidence of osmotic demyelination syndrome in Sweden: A nationwide study. 61
31343728 2019
28
Central Pontine Myelinosis and Osmotic Demyelination Syndrome. 61
31587708 2019
29
Central Pontine Myelinolysis After Living-Donor Liver Transplant: A Report of 2 Cases. 61
29137593 2019
30
Osmotic Demyelination Syndrome in Children. 61
31128892 2019
31
Occult central pontine myelinolysis post liver transplant: A consequence of pre-transplant hyponatremia. 61
31056363 2019
32
Histopathological changes of organ dysfunction in sepsis. 61
31346833 2019
33
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
34
Severe hypokalaemia in diabetic ketoacidosis: a contributor to central pontine myelinolysis? 61
31368676 2019
35
Localized atrophy of the pontine base as a sequela of prolonged ischemia: Report of an autopsy case. 61
30834588 2019
36
Neurologic conditions and disorders of uremic syndrome of chronic kidney disease: presentations, causes, and treatment strategies. 61
30501441 2019
37
[Osmotic demyelination syndrome complicating the quick correction of a severe hyponatremia associated with hypokalemia]. 61
32180882 2019
38
Extrapontine Myelinolysis and Reversible Parkinsonism After Hyponatremia Correction in a Case of Pituitary Adenoma: Hypopituitarism as a Predisposition for Osmotic Demyelination. 61
30055367 2018
39
[A case of liver cirrhosis with central pontine myelinolysis]. 61
30481888 2018
40
Normal Correction of Sodium Leading to Central Pontine Demyelinosis: A Rare Occurrence. 61
30416903 2018
41
[Disturbances of Sodium Homeostasis]. 61
30036896 2018
42
Latent systemic lupus erythematosus presenting with hypokalaemic quadriparesis and central pontine myelinolysis. 61
29429118 2018
43
Desmopressin-Induced Severe Hyponatremia with Central Pontine Myelinolysis: A Case Report. 61
29696555 2018
44
Tongue fasciculations with denervation pattern in osmotic demyelination syndrome: a case report of diagnostic dilemma. 61
29540223 2018
45
A case of chronic asymptomatic central pontine myelinolysis with histological evidence of remyelination. 61
29177819 2018
46
Simultaneous acute Marchiafava-Bignami disease and central pontine myelinolysis: A case report of a challenging diagnosis. 61
29465574 2018
47
Central Pontine Myelinolysis in Pediatric Diabetic Ketoacidosis. 61
29973999 2018
48
Astroglial Modulation of Hydromineral Balance and Cerebral Edema. 61
29946238 2018
49
Central pontine myelinolysis and poorly controlled diabetes: MRI's hints for pathogenesis. 61
28914383 2018
50
Central pontine myelinolysis in a type 1 diabetes patient with chronic hepatitis. 61
28583270 2018

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
12.93 AVPR2 AVP AQP4 AQP2 AQP1 ALB
2
Show member pathways
11.77 AVPR2 AVP AQP4 AQP2 AQP1
3 11.54 MBP MAG AQP4 AQP1
4
Show member pathways
10.99 IL2RB BCL2
5 10.77 BCL2 BAK1
7 10.57 AVPR2 AVP AQP4 AQP2
8 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 integral component of membrane GO:0016021 10.02 MAG IL2RB GJC2 BCL2 BAK1 AVPR2
2 integral component of plasma membrane GO:0005887 9.8 MAG IL2RB AVPR2 AQP4 AQP2 AQP1
3 basolateral plasma membrane GO:0016323 9.58 AQP4 AQP2 AQP1
4 pore complex GO:0046930 9.26 BCL2 BAK1
5 paranode region of axon GO:0033270 9.16 MAG GJC2
6 compact myelin GO:0043218 8.96 MBP MAG
7 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 26)
# Name GO ID Score Top Affiliating Genes
1 transmembrane transport GO:0055085 9.88 GJC2 BCL2 AQP4 AQP2 AQP1
2 negative regulation of apoptotic process GO:0043066 9.77 IL2RB BCL2 AVP AQP1 ALB
3 membrane organization GO:0061024 9.74 MBP AVPR2 AVP
4 response to toxic substance GO:0009636 9.69 MBP GJC2 BCL2
5 cellular response to mechanical stimulus GO:0071260 9.67 MAG BAK1 AQP1
6 positive regulation of vasoconstriction GO:0045907 9.63 AVPR2 AVP
7 response to nicotine GO:0035094 9.62 BCL2 AVP
8 intrinsic apoptotic signaling pathway in response to endoplasmic reticulum stress GO:0070059 9.62 BCL2 BAK1
9 extrinsic apoptotic signaling pathway in absence of ligand GO:0097192 9.61 BCL2 BAK1
10 regulation of mitochondrial membrane potential GO:0051881 9.61 BCL2 BAK1
11 cellular response to copper ion GO:0071280 9.6 AQP2 AQP1
12 response to gamma radiation GO:0010332 9.59 BCL2 BAK1
13 release of cytochrome c from mitochondria GO:0001836 9.58 BCL2 BAK1
14 B cell homeostasis GO:0001782 9.58 BCL2 BAK1
15 endoplasmic reticulum calcium ion homeostasis GO:0032469 9.56 BCL2 BAK1
16 axon regeneration GO:0031103 9.55 MAG BCL2
17 positive regulation of systemic arterial blood pressure GO:0003084 9.54 AVPR2 AVP
18 regulation of mitochondrial membrane permeability GO:0046902 9.52 BCL2 BAK1
19 glycerol transport GO:0015793 9.46 AQP2 AQP1
20 cellular water homeostasis GO:0009992 9.43 AQP4 AQP1
21 leukocyte homeostasis GO:0001776 9.4 BCL2 BAK1
22 multicellular organismal water homeostasis GO:0050891 9.33 AVP AQP4 AQP1
23 cellular response to mercury ion GO:0071288 9.32 AQP2 AQP1
24 renal water transport GO:0003097 9.26 AQP2 AQP1
25 water transport GO:0006833 9.26 AVP AQP4 AQP2 AQP1
26 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
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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