Osmotic demyelination syndrome

From Libre Pathology
(Redirected from Locked in syndrome)
Jump to navigation Jump to search

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)

www

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

  1. ↑ 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.
  2. ↑ 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.
  3. ↑ 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.
  4. ↑ 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.
  5. ↑ 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/.
  6. ↑ 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. ↑ 7.0 7.1 URL: http://neuropathology-web.org/chapter6/chapter6dCPM.html. Accessed on: 20 December 2011.
Retrieved from "https://librepathology.org/w/index.php?title=Osmotic_demyelination_syndrome&oldid=49129"