Osmotic demyelination syndrome
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Osmotic demyelination syndrome, abbreviated ODS, is a demyelination associated with osmotic imbalances.
It classically affect the pons and was previously known as central pontine myelinolysis.[1] The name was changed as it is now recognized that it can occur extrapontine.[2]
General
- Classically in the pons.
- Classically midline, ergo "central" was previously in the name.
- May occur elsewhere - previously referred to as extrapontine myelinolysis.
Etiology:
- Rapid correction of hyponatremia.[3]
- Tacrolimus post-liver transplant.[4]
- Associated with alcoholism and malnourishment.
Clinical:[5]
- Decreased level of consciousness - most common.
- Quadriplegia.
- Poor prognosis.
- May cause locked-in syndrome - inability to produce voluntary responses with preservation of intellectual capacity.[6]
Microscopic
Features:[7]
- Myelin loss.
- No inflammation.
- Relative preservation of neurons.
Images
Luxol fast blue (LFB stain) in a case of central pontine myelinolysis (WC/jensflorian)
Myelinophages in CPM showing intracellular myelin fragments (WC/jensflorian)
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DDx:
- MS /ADEM.
- PACNS - Primary angiitis of the central nervous system.
- CLIPPERS - Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids.
See also
References
- ↑ Alleman, AM. (Apr 2014). "Osmotic demyelination syndrome: central pontine myelinolysis and extrapontine myelinolysis.". Semin Ultrasound CT MR 35 (2): 153-9. doi:10.1053/j.sult.2013.09.009. PMID 24745890.
- ↑ Zunga, PM.; Farooq, O.; Dar, MI.; Dar, IH.; Rashid, S.; Rather, AQ.; Basu, JA.; Ashraf, M. et al. (Jan 2015). "Extra pontine osmotic demyelination syndrome.". Ann Neurosci 22 (1): 51-3. doi:10.5214/ans.0972.7531.220212. PMID 26124552.
- ↑ Chang, Y.; An, DH.; Xing, Y.; Qi, X. (Nov 2011). "Central pontine and extrapontine myelinolysis associated with acute hepatic dysfunction.". Neurol Sci. doi:10.1007/s10072-011-0838-3. PMID 22080394.
- ↑ Fukazawa, K.; Nishida, S.; Aguina, L.; Pretto, E. (Sep 2011). "Central pontine myelinolysis (CPM) associated with tacrolimus (FK506) after liver transplantation.". Ann Transplant 16 (3): 139-42. PMID 21959523.
- ↑ Lai, CC.; Tan, CK.; Lin, SH.; Chen, HW. (Jun 2011). "Central pontine myelinolysis.". CMAJ 183 (9): E605. doi:10.1503/cmaj.090186. PMC 3114939. PMID 21543311. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3114939/.
- ↑ Sohn, MK.; Nam, JH. (Oct 2014). "Locked-in Syndrome due to Central Pontine Myelinolysis: Case Report.". Ann Rehabil Med 38 (5): 702-6. doi:10.5535/arm.2014.38.5.702. PMID 25379502.
- ↑ 7.0 7.1 URL: http://neuropathology-web.org/chapter6/chapter6dCPM.html. Accessed on: 20 December 2011.