MCID: EXT039
MIFTS: 18

Extrapontine Myelinolysis

Categories: Neuronal diseases

Aliases & Classifications for Extrapontine Myelinolysis

MalaCards integrated aliases for Extrapontine Myelinolysis:

Name: Extrapontine Myelinolysis 53 71

Classifications:



External Ids:

UMLS 71 C0393667

Summaries for Extrapontine 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 : Extrapontine Myelinolysis is related to central pontine myelinolysis and alcohol dependence. Affiliated tissues include liver, brain and eye.

Related Diseases for Extrapontine Myelinolysis

Diseases related to Extrapontine Myelinolysis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 119)
# Related Disease Score Top Affiliating Genes
1 central pontine myelinolysis 12.0
2 alcohol dependence 10.8
3 dysphagia 10.6
4 ocular motor apraxia 10.5
5 ataxia and polyneuropathy, adult-onset 10.5
6 pseudobulbar palsy 10.5
7 tremor 10.5
8 quadriplegia 10.5
9 demyelinating disease 10.5
10 dystonia 10.5
11 diabetes mellitus, ketosis-prone 10.5
12 alcohol use disorder 10.5
13 hypopituitarism 10.5
14 diabetes insipidus 10.5
15 encephalopathy 10.5
16 chorea, childhood-onset, with psychomotor retardation 10.4
17 choreatic disease 10.4
18 pituitary adenoma 10.4
19 craniopharyngioma 10.4
20 mutism 10.4
21 hypokalemia 10.4
22 hyperglycemia 10.4
23 visual epilepsy 10.3
24 nephrotic syndrome 10.3
25 neutropenia 10.3
26 locked-in syndrome 10.3
27 acute cystitis 10.3
28 gastroenteritis 10.3
29 uremia 10.3
30 movement disease 10.3
31 adenoma 10.3
32 pulmonary embolism 10.3
33 hypoglycemia 10.3
34 dipsogenic diabetes insipidus 10.3
35 spastic paraparesis 10.3
36 brain injury 10.3
37 head injury 10.3
38 pituitary tumors 10.3
39 seizure disorder 10.3
40 traumatic brain injury 10.3
41 esophageal cancer 10.1
42 systemic lupus erythematosus 10.1
43 thrombophilia due to thrombin defect 10.1
44 arachnoid cysts, intracranial 10.1
45 cystic fibrosis 10.1
46 pituitary hormone deficiency, combined, 2 10.1
47 wernicke-korsakoff syndrome 10.1
48 wilson disease 10.1
49 pituitary adenoma, prolactin-secreting 10.1
50 yemenite deaf-blind hypopigmentation syndrome 10.1

Graphical network of the top 20 diseases related to Extrapontine Myelinolysis:



Diseases related to Extrapontine Myelinolysis

Symptoms & Phenotypes for Extrapontine Myelinolysis

Drugs & Therapeutics for Extrapontine Myelinolysis

Search Clinical Trials , NIH Clinical Center for Extrapontine Myelinolysis

Genetic Tests for Extrapontine Myelinolysis

Anatomical Context for Extrapontine Myelinolysis

MalaCards organs/tissues related to Extrapontine Myelinolysis:

40
Liver, Brain, Eye, Pons, Pituitary, Spinal Cord, Lung

Publications for Extrapontine Myelinolysis

Articles related to Extrapontine Myelinolysis:

(show top 50) (show all 277)
# Title Authors PMID Year
1
A rare case of pontine and extrapontine myelinolysis in a pediatric patient with chronic renal failure. 61
32504171 2020
2
Electrolyte Disorders and the Nervous System. 61
32487900 2020
3
Extensive subpial cortical demyelination is specific to multiple sclerosis. 61
31916298 2020
4
Colchicine poisoning complicated by medulla oblongata myelinolysis: a case report. 61
32527125 2020
5
Extrapontine myelinolysis caused by rapid correction of pituitrin-induced severe hyponatremia: A case report. 61
32190632 2020
6
Central pontine (and extrapontine) myelinolysis despite appropriate sodium correction. 61
31467150 2020
7
Rehabilitation of anterior pituitary dysfunction combined with extrapontine myelinolysis: A case report. 61
31911927 2019
8
Central Pontine/Extrapontine Myelinolysis Presenting with Manic and Catatonic Symptoms. 61
31548777 2019
9
Osmotic Demyelination Syndrome in Children. 61
31128892 2019
10
Idiopathic Central Pontine and Extrapontine Myelinolysis in a Child. 61
31516629 2019
11
Acute parkinsonism as an unexpected consequence of pituitary adenoma resection: A case report. 61
31027077 2019
12
Successful treatment of an 86-year-old patient with severe hyponatremia leading to central and extrapontine myelinolysis: A case report. 61
31942513 2019
13
Extrapontine Myelinolysis following Extreme Hypernatremia and Hyperosmolarity. 61
30800484 2019
14
[Osmotic demyelination syndrome complicating the quick correction of a severe hyponatremia associated with hypokalemia]. 61
32180882 2019
15
Extrapontine Myelinolysis and Microhemorrhages: Rare Finding in Pediatric Diabetic Ketoacidosis. 61
30396831 2018
16
Extrapontine Myelinolysis and Reversible Parkinsonism After Hyponatremia Correction in a Case of Pituitary Adenoma: Hypopituitarism as a Predisposition for Osmotic Demyelination. 61
30055367 2018
17
Treatment response in osmotic demyelination syndrome presenting as severe parkinsonism, ptosis and gaze palsy. 61
30344146 2018
18
Sequential MRI in pontine and extrapontine myelinolysis following rapid correction of hyponatremia. 61
30290836 2018
19
Extrapontine myelinolysis effects in intracranial langerhans cell histiocytosis: Case report. 61
30211327 2018
20
Posterior Reversible Encephalopathy Syndrome as a Postpartum Complication. 61
29875859 2018
21
Posterior reversible encephalopathy syndrome with spinal cord involvement (PRES-SCI) as a rare complication of severe diabetic ketoacidosis: a case report and review of the literature. 61
29330587 2018
22
B/L Basal Ganglia Lesions in a Child Leading to a Diagnosis of Glucose-6-Phosphate Dehydrogenase Deficiency. 61
29887684 2018
23
Therapeutic Effect of Steroids in Osmotic Demyelination of Infancy. 61
29687030 2018
24
Letter to the Editor: Presentation of extrapontine myelinolysis associated with hypernatremia in an infant: a case report. 61
28905219 2017
25
Extrapontine Myelinolysis in a Child with Salt Intoxication. 61
28427777 2017
26
Osmotic Demyelination Syndrome Revisited: Review With Neuroimaging. 61
28850553 2017
27
Secondary parkinsonism in a patient of psychogenic polydipsia. 61
28446482 2017
28
[Image Findings in Central Pontine Myelinolysis (CPM) and Extrapontine Myelinolysis (EPM)]. 61
28142166 2017
29
Newly described additional sites of extrapontine myelinolysis along with typical pontine and extrapontine myelinolysis. 61
28084269 2017
30
[A case report of central pontine and extrapontine myelinolysis in the combination with spinal cord damage in a patient with tuberculosis in the lung]. 61
28374704 2017
31
A case of central pontine and extrapontine myelinolysis after surgery for a pituitary tumor. 61
27980288 2017
32
Acute Psychosis as Main Manifestation of Central Pontine Myelinolysis. 61
28392953 2017
33
Liver Transplant Recipient With Tumefactive Demyelinating Lesions: A Case Report and Literature Review. 61
27932180 2016
34
Extrapontine myelinolysis manifested selectively by acute severe parkinsonian syndrome. Case Report. 61
28315624 2016
35
Rapid Correction of Chronic Hyponatremia Secondary to Psychogenic Polydipsia: Hypoxic Injury or Extrapontine Myelinolysis? 61
27835732 2016
36
Extrapontine myelinolysis associated with severe hypernatremia in infancy. 61
27463219 2016
37
Poster 365 Traumatic Brain Injury Resulting from Pontine and Extrapontine Myelinolysis due to Acute Onset of Pregnancy Induced Diabetes Insipidus: A Case Report. 61
27673118 2016
38
Fatal hepatic failure and pontine and extrapontine myelinolysis in XMEA. 61
27566744 2016
39
Treatable Leukoencephalopathy in a Patient with Hypophosphatemia. 61
28203182 2016
40
Wine Glass Sign and Empty Delta Sign: A Rare Imaging Presentation of Postpartum Encephalopathy in Dehydration. 61
27504377 2016
41
Pontine and extrapontine myelinolysis secondary to glycemic fluctuation. 61
25440064 2016
42
Central and Extrapontine Myelinolysis. 61
30151467 2016
43
Preoperative Extrapontine Myelinolysis with Good Outcome in a Patient with Pituitary Adenoma. 61
26962424 2016
44
[Extrapontine myelinolysis developed after aneurysmal subarachnoid hemorrhage (a case report and literature review)]. 61
28139577 2016
45
Delayed chorea after recovery from severe akinetic-rigid parkinsonism caused by extrapontine myelinolysis. 61
26384614 2015
46
[Is physiological saline really physiological? Hyponatreamia treatment--small deviations from the rules of appropriate therapy create serious complications and side effects]. 61
26608493 2015
47
Central pontine myelinolysis in a case of alcohol dependence syndrome. 61
27212829 2015
48
[Case Report of a Suspected Extrapontine Myelinolysis due to Hyperglycemic Hyperosmolar Syndrome]. 61
26015381 2015
49
Risk factors for central pontine and extrapontine myelinolysis after liver transplantation: a single-center study. 61
25427166 2015
50
Reversible Cerebellar Ataxia Related to Extrapontine Myelinolysis without Hyponatremia after Cisplatin-Based Chemotherapy for Cholangiocarcinoma. 61
25358385 2015

Variations for Extrapontine Myelinolysis

Expression for Extrapontine Myelinolysis

Search GEO for disease gene expression data for Extrapontine Myelinolysis.

Pathways for Extrapontine Myelinolysis

GO Terms for Extrapontine Myelinolysis

Sources for Extrapontine Myelinolysis

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63 QIAGEN
68 SNOMED-CT via HPO
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70 Tocris
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72 UMLS via Orphanet
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