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Central pontine myelinolysis

MedGen UID:
64511
•Concept ID:
C0206083
•
Disease or Syndrome
Synonyms: Central Pontine Myelinoclasis; Central Pontine Myelinolysis; Myelinoclasis, Central Pontine; Myelinolysis, Central Pontine; Pontine Myelinolysis, Central
SNOMED CT: Central pontine myelinolysis (6807001)
 
Monarch Initiative: MONDO:0006692

Definition

A central nervous system disorder caused by demyelination within the central basis pontis of the brain. It is characterized by spastic quadriplegia, pseudobulbar palsy and encephalopathy. It is observed in patients with severe hyponatremia, particularly when the hyponatremia is corrected too rapidly. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCentral pontine myelinolysis

Professional guidelines

PubMed

Neurologic conditions and disorders of uremic syndrome of chronic kidney disease: presentations, causes, and treatment strategies.
Hamed SA
Expert Rev Clin Pharmacol 2019 Jan;12(1):61-90. Epub 2019 Jan 11 doi: 10.1080/17512433.2019.1555468. PMID: 30501441
Differential diagnosis of T2 hyperintense brainstem lesions: Part 2. Diffuse lesions.
Guzmán-De-Villoria JA, Ferreiro-Argüelles C, Fernández-García P
Semin Ultrasound CT MR 2010 Jun;31(3):260-74. doi: 10.1053/j.sult.2010.03.002. PMID: 20483393
Management of hyponatremia.
Goh KP
Am Fam Physician 2004 May 15;69(10):2387-94. PMID: 15168958

Recent clinical studies

Etiology

Hyponatremia in Cirrhosis.
Rondon-Berrios H, Velez JCQ
Clin Liver Dis 2022 May;26(2):149-164. Epub 2022 Apr 1 doi: 10.1016/j.cld.2022.01.001. PMID: 35487602Free PMC Article
Electrolyte Disorders and the Nervous System.
Jacoby N
Continuum (Minneap Minn) 2020 Jun;26(3):632-658. doi: 10.1212/CON.0000000000000872. PMID: 32487900
Central Pontine Myelinosis and Osmotic Demyelination Syndrome.
Lambeck J, Hieber M, Dreßing A, Niesen WD
Dtsch Arztebl Int 2019 Sep 2;116(35-36):600-606. doi: 10.3238/arztebl.2019.0600. PMID: 31587708Free PMC Article
Risk Factors and Outcomes of Rapid Correction of Severe Hyponatremia.
George JC, Zafar W, Bucaloiu ID, Chang AR
Clin J Am Soc Nephrol 2018 Jul 6;13(7):984-992. Epub 2018 Jun 5 doi: 10.2215/CJN.13061117. PMID: 29871886Free PMC Article
Central pontine and extrapontine myelinolysis: a systematic review.
Singh TD, Fugate JE, Rabinstein AA
Eur J Neurol 2014 Dec;21(12):1443-50. Epub 2014 Sep 15 doi: 10.1111/ene.12571. PMID: 25220878

Diagnosis

Alcohol and the central nervous system.
Wolfe M, Menon A, Oto M, Fullerton NE, Leach JP
Pract Neurol 2023 Aug;23(4):273-285. Epub 2023 Jun 16 doi: 10.1136/pn-2023-003817. PMID: 37328277
Central Pontine Myelinosis and Osmotic Demyelination Syndrome.
Lambeck J, Hieber M, Dreßing A, Niesen WD
Dtsch Arztebl Int 2019 Sep 2;116(35-36):600-606. doi: 10.3238/arztebl.2019.0600. PMID: 31587708Free PMC Article
Neurological and neuropsychiatric manifestations of porphyria.
Suh Y, Gandhi J, Seyam O, Jiang W, Joshi G, Smith NL, Ali Khan S
Int J Neurosci 2019 Dec;129(12):1226-1233. Epub 2019 Sep 1 doi: 10.1080/00207454.2019.1655014. PMID: 31402774
Central pontine and extrapontine myelinolysis: a systematic review.
Singh TD, Fugate JE, Rabinstein AA
Eur J Neurol 2014 Dec;21(12):1443-50. Epub 2014 Sep 15 doi: 10.1111/ene.12571. PMID: 25220878
Differential diagnosis of T2 hyperintense brainstem lesions: Part 2. Diffuse lesions.
Guzmán-De-Villoria JA, Ferreiro-Argüelles C, Fernández-García P
Semin Ultrasound CT MR 2010 Jun;31(3):260-74. doi: 10.1053/j.sult.2010.03.002. PMID: 20483393

Therapy

Pseudo-Central Pontine Myelinolysis.
Rumschlag M, Prahlow JA, Castellani RJ, Fisher-Hubbard AO
Am J Forensic Med Pathol 2022 Jun 1;43(2):195-198. Epub 2021 Dec 14 doi: 10.1097/PAF.0000000000000731. PMID: 34907999
Equimolar doses of hypertonic agents (saline or mannitol) in the treatment of intracranial hypertension after severe traumatic brain injury.
Huang X, Yang L, Ye J, He S, Wang B
Medicine (Baltimore) 2020 Sep 18;99(38):e22004. doi: 10.1097/MD.0000000000022004. PMID: 32957318Free PMC Article
The neurology of solid organ transplantation.
Avila JD, Živković S
Curr Neurol Neurosci Rep 2015 Jul;15(7):38. doi: 10.1007/s11910-015-0560-2. PMID: 26008808
Identifying risk factors for central pontine and extrapontine myelinolysis after liver transplantation: a case-control study.
Morard I, Gasche Y, Kneteman M, Toso C, Mentha A, Meeberg G, Mentha G, Kneteman N, Giostra E
Neurocrit Care 2014 Apr;20(2):287-95. doi: 10.1007/s12028-013-9928-9. PMID: 24233816
Central nervous system complications after transplantation.
Dhar R, Human T
Neurol Clin 2011 Nov;29(4):943-72. doi: 10.1016/j.ncl.2011.07.002. PMID: 22032668

Prognosis

Central Pontine Myelinosis and Osmotic Demyelination Syndrome.
Lambeck J, Hieber M, Dreßing A, Niesen WD
Dtsch Arztebl Int 2019 Sep 2;116(35-36):600-606. doi: 10.3238/arztebl.2019.0600. PMID: 31587708Free PMC Article
Hyponatremia in Cirrhosis: Implications for Liver Transplantation.
Leise M, Cárdenas A
Liver Transpl 2018 Nov;24(11):1612-1621. doi: 10.1002/lt.25327. PMID: 30129266
Osmotic Demyelination Unrelated to Hyponatremia.
Shah MK, Mandayam S, Adrogué HJ
Am J Kidney Dis 2018 Mar;71(3):436-440. Epub 2017 Dec 23 doi: 10.1053/j.ajkd.2017.10.010. PMID: 29277507
Central pontine and extrapontine myelinolysis: a systematic review.
Singh TD, Fugate JE, Rabinstein AA
Eur J Neurol 2014 Dec;21(12):1443-50. Epub 2014 Sep 15 doi: 10.1111/ene.12571. PMID: 25220878
Demyelinating diseases.
Love S
J Clin Pathol 2006 Nov;59(11):1151-9. doi: 10.1136/jcp.2005.031195. PMID: 17071802Free PMC Article

Clinical prediction guides

Severe Hyponatremia Correction, Mortality, and Central Pontine Myelinolysis.
Seethapathy H, Zhao S, Ouyang T, Passos C, Sarang A, Cheung PW, Waikar SS, Steele DJR, Kalim S, Allegretti AS, Ayus JC, Nigwekar SU
NEJM Evid 2023 Oct;2(10):EVIDoa2300107. Epub 2023 Sep 26 doi: 10.1056/EVIDoa2300107. PMID: 38320180
Hyponatremia in Cirrhosis: Implications for Liver Transplantation.
Leise M, Cárdenas A
Liver Transpl 2018 Nov;24(11):1612-1621. doi: 10.1002/lt.25327. PMID: 30129266
Central pontine myelinolysis.
Pearce JM
Eur Neurol 2009;61(1):59-62. Epub 2008 Nov 25 doi: 10.1159/000175124. PMID: 19033724
Axonal damage: a key predictor of outcome in human CNS diseases.
Medana IM, Esiri MM
Brain 2003 Mar;126(Pt 3):515-30. doi: 10.1093/brain/awg061. PMID: 12566274
Hyponatremia and hypernatremia.
Fried LF, Palevsky PM
Med Clin North Am 1997 May;81(3):585-609. doi: 10.1016/s0025-7125(05)70535-6. PMID: 9167647

Recent systematic reviews

Central pontine and extrapontine myelinolysis: a systematic review.
Singh TD, Fugate JE, Rabinstein AA
Eur J Neurol 2014 Dec;21(12):1443-50. Epub 2014 Sep 15 doi: 10.1111/ene.12571. PMID: 25220878
Movement disorders in multiple sclerosis and other demyelinating diseases.
Mehanna R, Jankovic J
J Neurol Sci 2013 May 15;328(1-2):1-8. Epub 2013 Mar 19 doi: 10.1016/j.jns.2013.02.007. PMID: 23522528

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