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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
Management of hyponatremia.
Goh KP
Am Fam Physician 2004 May 15;69(10):2387-94. PMID: 15168958
Changing concepts in treatment of severe symptomatic hyponatremia. Rapid correction and possible relation to central pontine myelinolysis.
Ayus JC, Krothapalli RK, Arieff AI
Am J Med 1985 Jun;78(6 Pt 1):897-902. doi: 10.1016/0002-9343(85)90209-8. PMID: 4014266

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
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
Management of hyponatraemia.
Mittal R, Sheftel H, Demssie Y
Br J Hosp Med (Lond) 2011 Feb;72(2):M22-5. doi: 10.12968/hmed.2011.72.sup2.m22. PMID: 21378608

Diagnosis

Central and Extrapontine Myelinolysis in the Setting of Hyperglycemia.
Qadir MS, Hafeez MU, Sheikh A, Hafeez K, Desai A, Hirzallah MI
Prim Care Companion CNS Disord 2022 Oct 11;24(5) doi: 10.4088/PCC.21cr03133. PMID: 36251978
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
Osmotic demyelination syndrome.
Epperla N, Landeck J, Sabbagh S
WMJ 2014 Oct;113(5):197-8. PMID: 25739163
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
Management of hyponatraemia.
Mittal R, Sheftel H, Demssie Y
Br J Hosp Med (Lond) 2011 Feb;72(2):M22-5. doi: 10.12968/hmed.2011.72.sup2.m22. PMID: 21378608

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
Osmotic demyelination syndrome.
Börnke C, Ellrichmann G, Schneider R, Lukas C
BMJ Case Rep 2014 Jul 8;2014 doi: 10.1136/bcr-2014-204742. PMID: 25006057Free PMC Article
Central pontine myelinolysis.
Hromanik K
J Emerg Nurs 2010 Jul;36(4):324-6. Epub 2009 Dec 8 doi: 10.1016/j.jen.2009.09.006. PMID: 20624565
Hyponatraemia.
Smith DM, McKenna K, Thompson CJ
Clin Endocrinol (Oxf) 2000 Jun;52(6):667-78. doi: 10.1046/j.1365-2265.2000.01027.x. PMID: 10848869
Central pontine myelinolysis.
McCormick WF, Danneel CM
Arch Intern Med 1967 May;119(5):444-78. PMID: 6024660

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
Central pontine myelinolysis.
Fersini F, Govi A, Tsokos M
Forensic Sci Med Pathol 2015 Mar;11(1):130-2. Epub 2014 Nov 14 doi: 10.1007/s12024-014-9628-6. PMID: 25395281
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
Central pontine and extrapontine myelinolysis: the osmotic demyelination syndromes.
Martin RJ
J Neurol Neurosurg Psychiatry 2004 Sep;75 Suppl 3(Suppl 3):iii22-8. doi: 10.1136/jnnp.2004.045906. PMID: 15316041Free 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
Monitoring and managing hepatic disease in anaesthesia.
Kiamanesh D, Rumley J, Moitra VK
Br J Anaesth 2013 Dec;111 Suppl 1:i50-61. doi: 10.1093/bja/aet378. PMID: 24335399
Central pontine and extrapontine myelinolysis in children: a review of 76 patients.
Ranger AM, Chaudhary N, Avery M, Fraser D
J Child Neurol 2012 Aug;27(8):1027-37. Epub 2012 May 30 doi: 10.1177/0883073812445908. PMID: 22647485
Central pontine myelinolysis.
Pearce JM
Eur Neurol 2009;61(1):59-62. Epub 2008 Nov 25 doi: 10.1159/000175124. PMID: 19033724
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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