MCID: CNT025
MIFTS: 48

Central Pontine Myelinolysis

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Central Pontine Myelinolysis

MalaCards integrated aliases for Central Pontine Myelinolysis:

Name: Central Pontine Myelinolysis 12 54 55 15 17
Myelinolysis, Central Pontine 45 74
Osmotic Demyelination Syndrome 12
Myelinolysis Central Pontine 56
Cpm 54

Classifications:



External Ids:

Disease Ontology 12 DOID:636
MeSH 45 D017590
NCIt 51 C84623
SNOMED-CT 69 6807001
ICD10 34 G37.2
UMLS 74 C0206083

Summaries for Central Pontine Myelinolysis

NINDS : 55 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. The drugs Tacrolimus and Immunosuppressive Agents have been mentioned in the context of this disorder. Affiliated tissues include pons, brain and liver, 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 : 54 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 : 77 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 138)
# Related Disease Score Top Affiliating Genes
1 neuromyelitis optica 30.1 AQP1 AQP4 MBP
2 optic nerve disease 30.0 AQP4 MBP
3 diabetes insipidus, nephrogenic, autosomal 29.7 AQP1 AQP4 AVP
4 extrapontine myelinolysis 11.8
5 cleft palate, isolated 11.3
6 dentine erosion 11.1
7 well-differentiated liposarcoma 11.1
8 encephalopathy 10.6
9 alcohol dependence 10.5
10 anorexia nervosa 10.5
11 wernicke encephalopathy 10.5
12 lymphoma 10.4
13 movement disease 10.4
14 dystonia 10.4
15 aceruloplasminemia 10.3
16 hepatitis 10.3
17 leukemia 10.3
18 hypophosphatemia 10.3
19 hypoglycemia 10.3
20 myoclonus 10.3
21 tremor 10.3
22 myelitis 10.2 AQP4 MBP
23 alexia 10.2 AQP4 MBP
24 cranial nerve disease 10.2 AQP4 MBP
25 balo concentric sclerosis 10.2 AQP4 MBP
26 sjogren syndrome 10.2
27 wilson disease 10.2
28 diabetes mellitus, ketosis-prone 10.2
29 diffuse large b-cell lymphoma 10.2
30 locked-in syndrome 10.2
31 quadriplegia 10.2
32 hyperglycemia 10.2
33 hypokalemia 10.2
34 pancreatitis 10.2
35 transverse myelitis 10.2 AQP4 MBP
36 internuclear ophthalmoplegia 10.2 AQP4 MBP
37 irritable bowel syndrome 10.2
38 colorectal adenoma 10.2
39 acute disseminated encephalomyelitis 10.2 AQP4 MBP
40 traumatic brain injury 10.2 AQP4 MBP
41 autoimmune optic neuritis 10.2 AQP4 MBP
42 subependymoma 10.2 AQP1 AQP4
43 dysferlinopathy 10.2 AQP1 AQP4
44 spinal cord disease 10.1 AQP4 MBP
45 autoimmune disease of peripheral nervous system 10.1 MAG MBP
46 spindle cell synovial sarcoma 10.1 MAG MBP
47 neuromyelitis optica spectrum disorder 10.1
48 polyradiculoneuropathy 10.1 MAG MBP
49 intracranial hypertension 10.1 AQP1 AQP4
50 dentatorubral-pallidoluysian atrophy 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:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.85 AQP1 AQP4 AVP BCL2 MAG MBP
2 hematopoietic system MP:0005397 9.73 AQP1 AQP4 BCL2 MAG MBP RNF128
3 homeostasis/metabolism MP:0005376 9.7 AQP1 AQP4 AVP BCL2 MAG MBP
4 hearing/vestibular/ear MP:0005377 9.62 AQP1 AQP4 BCL2 MBP
5 nervous system MP:0003631 9.5 AQP1 AQP4 AVP BCL2 MAG MBP
6 renal/urinary system MP:0005367 8.92 AQP1 AQP4 AVP BCL2

Drugs & Therapeutics for Central Pontine Myelinolysis

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


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Tacrolimus Approved, Investigational 104987-11-3 6473866 445643 439492
2 Immunosuppressive Agents
3 Immunologic Factors
4 Liver Extracts
5 Calcineurin Inhibitors

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:

42
Pons, Brain, Liver, Eye, B Cells, Lung, Bone

Publications for Central Pontine Myelinolysis

Articles related to Central Pontine Myelinolysis:

(show top 50) (show all 398)
# Title Authors Year
1
The role of the interdisciplinary team in subacute rehabilitation for central pontine myelinolysis. ( 30950662 )
2019
2
Occult central pontine myelinolysis post liver transplant: A consequence of pre-transplant hyponatremia. ( 31056363 )
2019
3
Central pontine myelinolysis in a type 1 diabetes patient with chronic hepatitis. ( 28583270 )
2018
4
Central pontine myelinolysis and poorly controlled diabetes: MRI's hints for pathogenesis. ( 28914383 )
2018
5
A case of chronic asymptomatic central pontine myelinolysis with histological evidence of remyelination. ( 29177819 )
2018
6
Latent systemic lupus erythematosus presenting with hypokalaemic quadriparesis and central pontine myelinolysis. ( 29429118 )
2018
7
Simultaneous acute Marchiafava-Bignami disease and central pontine myelinolysis: A case report of a challenging diagnosis. ( 29465574 )
2018
8
Desmopressin-Induced Severe Hyponatremia with Central Pontine Myelinolysis: A Case Report. ( 29696555 )
2018
9
Central Pontine Myelinolysis in Pediatric Diabetic Ketoacidosis. ( 29973999 )
2018
10
Central pontine myelinolysis in Sjogren's syndrome with hypokalemia. ( 26929036 )
2017
11
Central pontine myelinolysis during pregnancy: Pathogenesis, diagnosis and management. ( 27922286 )
2017
12
Central Pontine Myelinolysis and Localized Fluorodeoxyglucose Uptake Seen on 18F-FDG PET/CT. ( 28217021 )
2017
13
Does Accidental Overcorrection of Symptomatic Hyponatremia in Chronic Heart Failure Require Specific Therapeutic Adjustments for Preventing Central Pontine Myelinolysis? ( 28270885 )
2017
14
Central Pontine Myelinolysis Induced by Alcohol Withdrawal: A Case Report. ( 28289647 )
2017
15
Acute Psychosis as Main Manifestation of Central Pontine Myelinolysis. ( 28392953 )
2017
16
Central pontine myelinolysis associated with hypokalemia in a diabetic patient with sepsis. ( 28488657 )
2017
17
Acute necrotic pancreatitis: A rare and not always fatal cause of central pontine myelinolysis. ( 28757177 )
2017
18
Wilson's disease presenting as central pontine myelinolysis. ( 28785820 )
2017
19
Atypical presentation of central pontine myelinolysis in hyperglycemia. ( 28924482 )
2017
20
Central Pontine Myelinolysis After Living-Donor Liver Transplant: A Report of 2 Cases. ( 29137593 )
2017
21
Subacute central pontine myelinolysis secondary to hyperglycaemia. ( 29169807 )
2017
22
Abrus Precatorius Poisoning and Central Pontine Myelinolysis. ( 29675076 )
2017
23
Striking Central Pontine Myelinolysis in a Patient With Alcohol Dependence Syndrome Without Hyponatremia. ( 26720620 )
2016
24
IMAGES IN CLINICAL MEDICINE. Central Pontine Myelinolysis. ( 26886548 )
2016
25
Hypertensive brainstem encephalopathy mimicking central pontine myelinolysis: a potential pitfall. ( 26935223 )
2016
26
Central pontine myelinolysis secondary to hyperglycaemia. ( 27407176 )
2016
27
Asymptomatic central pontine myelinolysis without hyponatriemia in diffuse large B cell lymphoma. ( 27488303 )
2016
28
Clinical Evolution of Central Pontine Myelinolysis in a Patient with Alcohol Withdrawal: A Blurred Clinical Horizon. ( 27610136 )
2016
29
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
30
Central pontine myelinolysis caused by hypernatremia. ( 27772775 )
2016
31
Psychiatric symptoms and limb tremors associated with central pontine myelinolysis: A case of alcoholism without hyponatremia. ( 27882183 )
2016
32
Is Central Pontine Myelinolysis Reversible? ( 29095576 )
2016
33
Central pontine myelinolysis in a case of alcohol dependence syndrome. ( 27212829 )
2015
34
Central pontine myelinolysis. ( 25395281 )
2015
35
Central pontine myelinolysis-induced mania: A case study. ( 25732324 )
2015
36
Central pontine myelinolysis presenting as isolated sixth nerve palsy in third trimester of pregnancy. ( 25745319 )
2015
37
Central pontine myelinolysis: a rare presentation secondary to hyperglycaemia. ( 25917480 )
2015
38
Osmotic myelinolysis: Does extrapontine myelinolysis precede central pontine myelinolysis? Report of two cases and review of literature. ( 25969642 )
2015
39
Diffuse large B-cell lymphoma presenting with central pontine myelinolysis: a case report. ( 26044457 )
2015
40
Urinary incontinence a first presentation of central pontine myelinolysis: a case report. ( 26209689 )
2015
41
A Case of Central Pontine Myelinolysis Caused by Hypophosphatemia Secondary to Refeeding Syndrome. ( 26557081 )
2015
42
The owl and the monkey - The varied faces of pons in central pontine myelinolysis. ( 26588651 )
2015
43
Central Pontine Myelinolysis with Minimal Hyponatremia in the Setting of AIDS. ( 26605097 )
2015
44
A Rare Case of Central Pontine Myelinolysis in Overcorrection of Hyponatremia with Total Parenteral Nutrition in Pregnancy. ( 26798530 )
2015
45
Central pontine myelinolysis presenting with tremor in a child with celiac disease. ( 23390116 )
2014
46
Brainstem somatosensory and auditory evoked responses in central pontine myelinolysis. ( 23975561 )
2014
47
Central pontine myelinolysis: electrolytes and beyond. ( 24682140 )
2014
48
Plasma exchange successfully treats central pontine myelinolysis after acute hypernatremia from intravenous sodium bicarbonate therapy. ( 24708786 )
2014
49
Central pontine myelinolysis following pediatric living donor liver transplantation: a case report and review of literature. ( 24725019 )
2014
50
Osmotic demyelination syndrome: central pontine myelinolysis and extrapontine myelinolysis. ( 24745890 )
2014

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 11)
# Name GO ID Score Top Affiliating Genes
1 response to toxic substance GO:0009636 9.49 BCL2 MBP
2 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.48 AVP BCL2
3 positive regulation of cell growth GO:0030307 9.46 AVP BCL2
4 cellular response to mechanical stimulus GO:0071260 9.43 AQP1 MAG
5 regulation of protein stability GO:0031647 9.4 BCL2 RNF128
6 negative regulation of cysteine-type endopeptidase activity involved in apoptotic process GO:0043154 9.37 AQP1 AVP
7 renal water homeostasis GO:0003091 9.33 AQP1 AQP4 AVP
8 response to nicotine GO:0035094 9.32 AVP BCL2
9 axon regeneration GO:0031103 9.26 BCL2 MAG
10 water transport GO:0006833 9.13 AQP1 AQP4 AVP
11 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 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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