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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
Hyponatremia: clinical diagnosis and management.
Lien YH, Shapiro JI
Am J Med 2007 Aug;120(8):653-8. doi: 10.1016/j.amjmed.2006.09.031. PMID: 17679119

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
Central pontine myelinolysis.
Lampl C, Yazdi K
Eur Neurol 2002;47(1):3-10. doi: 10.1159/000047939. PMID: 11803185

Diagnosis

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 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
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 myelinolysis.
Lampl C, Yazdi K
Eur Neurol 2002;47(1):3-10. doi: 10.1159/000047939. PMID: 11803185

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
Central pontine myelinolysis and the osmotic demyelination syndromes: an open and shut case?
Bose P
Acta Neurol Belg 2021 Aug;121(4):849-858. Epub 2021 Mar 13 doi: 10.1007/s13760-021-01634-0. PMID: 33713026
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
Osmotic Demyelination Syndrome in Children.
Bansal LR, Zinkus T
Pediatr Neurol 2019 Aug;97:12-17. Epub 2019 Mar 28 doi: 10.1016/j.pediatrneurol.2019.03.018. PMID: 31128892
Neurological complications of transplantation.
Pustavoitau A, Bhardwaj A, Stevens R
J Intensive Care Med 2011 Jul-Aug;26(4):209-22. doi: 10.1177/0885066610389549. PMID: 21764765

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
Osmotic Demyelination Syndrome in Children.
Bansal LR, Zinkus T
Pediatr Neurol 2019 Aug;97:12-17. Epub 2019 Mar 28 doi: 10.1016/j.pediatrneurol.2019.03.018. PMID: 31128892
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 myelinolysis.
Lampl C, Yazdi K
Eur Neurol 2002;47(1):3-10. doi: 10.1159/000047939. PMID: 11803185

Clinical prediction guides

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 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
Clinical and radiologic correlations of central pontine myelinolysis syndrome.
Graff-Radford J, Fugate JE, Kaufmann TJ, Mandrekar JN, Rabinstein AA
Mayo Clin Proc 2011 Nov;86(11):1063-7. Epub 2011 Oct 13 doi: 10.4065/mcp.2011.0239. PMID: 21997578Free PMC Article
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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