

Osmotic demyelination syndrome
https://doi.org/10.30629/2658-7947-2024-29-5-4-12
Abstract
Introduction. Osmotic demyelinating syndrome(ODS) is a life-threatening condition caused by water and electrolyte disturbances, usually associated with rapid correction of hyponatremia, manifested by acute demyelination of the brain. The average age of development of patients with ODS varies from 30 to 50 years. The incidence of unfavorable outcome (mortality/complete dependence on outside help) varies from 33 to 55%.
Objective. Systematization of data on the etiology, pathophysiology, clinical manifestations, diagnosis and treatment of osmotic demyelinating syndrome.
Material and methods. The literature search was performed in the electronic search engines Scopus, eLibrary, PubMed using the keywords: osmotic demyelinating syndrome, central pontine myelinolysis, extrapontine myelinolysis. Scientific articles published between 1959 and 2024 were selected for analysis.
Results. The main reason for the development of ODS is water and electrolyte disturbances that occur secondary to severe somatic pathology. Clinical manifestations of ODS are intermittent. Сlinical improvement is typical after rapid correction of osmolarity, followed by the development after 3–5 days of a clinical picture of acute myelinolysis. Currently, there is no specific therapy for ODS. However, early diagnosis of this disease is extremely important due to the high incidence of disorders of consciousness and breathing that require artificial ventilation. The polymorphism of clinical symptoms, as well as the low awareness of doctors, complicate the diagnosis and timely initiation of therapy for ODS. The optimal method of neuroimaging in patients with ODS is MRI in DWI, T2 and T2 FLAIR modes. The rate of correction of hyponatremia by no more than 10 mmol/l in the first 24 hours and no higher than 8 mmol/l every subsequent day is the main way to prevent the development of ODS.
About the Authors
G. R. RamazanovRussian Federation
Moscow
E. A. Kovaleva
Russian Federation
Moscow
K. R. Akchurina
Russian Federation
Moscow
M. E. Bykova
Russian Federation
Moscow
References
1. Lambeck J, Hieber M, Dreßing A, Niesen WD. Central Pontine Myelinosis and Osmotic Demyelination Syndrome. Dtsch Arztebl Int. 2019;116(35–36):600–606. https://doi.org/10.3238/arztebl.2019.0600
2. Ramazanov G.R., Kovaleva E.A., Stepanov V.N., Korigova H.V., Shevchenko E.V., Zabrodskaya Y.V., Petrikov S.S. Clinical cases of Wernicke’s encephalopathy. Journal named after N.V. Sklifosovsky «Emergency medical care».2020;9(2):292–297 (in Russ.). https://doi.org/10.23934/2223-9022-2020-9-2-292-297
3. Rao PB, Azim A, Singh N, Baronia AK, Kumar A, Poddar B. Osmotic demyelination syndrome in Intensive Care Unit. Indian J Crit Care Med. 2015;19(3):166–169. https://doi. org/10.4103/0972-5229.152760
4. Singh TD, Fugate JE, Rabinstein AA. Central pontine and extrapontine myelinolysis: a systematic review. Eur J Neurol. 2014;21(12):1443–1450. https://doi.org/10.1111/ene.12571
5. Berlit P. Die zentrale pontine Myelinolyse [Central pontine myelinolysis]. Nervenarzt. 1986;57(11):624–633. German.
6. Adams RD, Victor M, Mancall EL. Central pontine myelinolysis: a hitherto undescribed disease occurring in alcoholic and malnourished patients. AMA Arch Neurol Psychiatry. 1959;81(2):154–172.
7. Tomlinson BE, Pierides AM, Bradley WG. Central pontine myelinolysis. Two cases with associated electrolyte disturbance. Q J Med. 1976;45(179):373–386.
8. Laureno R, Karp BI. Myelinolysis after correction of hyponatremia. Ann Intern Med. 1997;126(1):57–62. https://doi. org/10.7326/0003-4819-126-1-199701010-00008
9. de Souza A. Movement disorders and the osmotic demyelination syndrome. Parkinsonism Relat Disord. 2013;19(8):709–716. https://doi.org/10.1016/j.parkreldis.2013.04.005
10. Estol CJ, Faris AA, Martinez AJ, Ahdab-Barmada M. Central pontine myelinolysis after liver transplantation. Neurology. 1989;39(4):493–498. https://doi.org/10.1212/wnl.39.4.493
11. Menger H, Jörg, J. Outcome of central pontine and extrapontine myelinolysis (n = 44). J Neurol. 1999;246(8):700–705. https:// doi.org/10.1007/s004150050435
12. Danyalian A, Heller D. Central Pontine Myelinolysis. [Updated 2023 Aug 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan. Available from: https://www.ncbi. nlm.nih.gov/books/NBK551697/
13. Lampl C, Yazdi K. Central pontine myelinolysis. Eur Neurol. 2002;47(1):3–10. https://doi.org/10.1159/000047939
14. Sugimoto T, Murata T, Omori M, Wada Y. Central pontine myelinolysis associated with hypokalaemia in anorexia nervosa. J Neurol Neurosurg Psychiatry. 2003;74(3):353–355. https://doi. org/10.1136/jnnp.74.3.353
15. Giuliani C, Peri A. Eff ects of Hyponatremia on the Brain. J Clin Med. 2014;3(4):1163–1177. https://doi.org/10.3390/jcm3041163
16. Pasantes-Morales H, Franco R, Ordaz B, Ochoa LD. Mechanisms counteracting swelling in brain cells during hyponatremia. Arch Med Res. 2002;33(3):237–244. https://doi.org/10.1016/ s0188-4409(02)00353-3
17. Sterns RH, Silver SM. Brain volume regulation in response to hypo-osmolality and its correction. Am J Med. 2006;119(7 Suppl 1):S12–S16. https://doi.org/10.1016/j.amjmed.2006.05.003
18. Arieff AI, Llach F, Massry SG. Neurological manifestations and morbidity of hyponatremia: correlation with brain water and electrolytes. Medicine (Baltimore). 1976;55(2):121–129. https:// doi.org/10.1097/00005792-197603000-00002
19. Alleman AM. Osmotic demyelination syndrome: central pontine myelinolysis and extrapontine myelinolysis. Semin Ultrasound CT MR. 2014;35(2):153–159. https://doi.org/10.1053/j. sult.2013.09.009
20. Popescu BF, Bunyan RF, Guo Y, Parisi JE, Lennon VA, Lucchinetti CF. Evidence of aquaporin involvement in human central pontine myelinolysis. Acta Neuropathol Commun. 2013;1:40. https://doi.org/10.1186/2051-5960-1-40
21. Odier C, Nguyen DK, Panisset M. Central pontine and extrapontine myelinolysis: from epileptic and other manifestations to cognitive prognosis. J Neurol. 2010;257(7):1176–1180. https:// doi.org/10.1007/s00415-010-5486-7
22. de Souza A, Desai PK. More often striatal myelinolysis than pontine? A consecutive series of patients with osmotic demyelination syndrome. Neurol Res. 2012;34(3):262–271. https://doi.org/10.1 179/1743132812Y.0000000009
23. Seiser A, Schwarz S, Aichinger-Steiner MM, Funk G, Schnider P, Brainin M. Parkinsonism and dystonia in central pontine and extrapontine myelinolysis. J Neurol Neurosurg Psychiatry. 1998;65(1):119–121. https://doi.org/10.1136/jnnp.65.1.119
24. Garzon T, Mellibovsky L, Roquer J, Perich X, Diez-Perez A. Ataxic form of central pontine myelinolysis. Lancet Neurol. 2002;1(8):517–518. https://doi.org/10.1016/s1474-4422(02)00224-7
25. Roh JH, Kim JH, Oh K, Kim SG, Park KW, Kim BJ. Cortical laminar necrosis caused by rapidly corrected hyponatremia. J Neuroimaging. 2009;19(2):185–187. https://doi.org/10.1111/j.1552-6569.2008.00244.x
26. Graff -Radford J, Fugate JE, Kaufmann TJ, Mandrekar JN, Rabinstein AA. Clinical and radiologic correlations of central pontine myelinolysis syndrome. Mayo Clin Proc. 2011;86(11):1063– 1067. https://doi.org/10.4065/mcp.2011.0239
27. Kallakatta RN, Radhakrishnan A, Fayaz RK, Unnikrishnan JP, Kesavadas C, Sarma SP. Clinical and functional outcome and factors predicting prognosis in osmotic demyelination syndrome (central pontine and/or extrapontine myelinolysis) in 25 patients. J Neurol Neurosurg Psychiatry. 2011;82(3):326–331. https://doi.org/10.1136/jnnp.2009.201764
28. Chua GC, Sitoh YY, Lim CC, Chua HC, Ng PY. MRI Findings in Osmotic Myelinolysis. Clin Radiol. 2002;57(9):800–806.
29. Miller GM, Baker HL, Okazaki H, Whisnant JP. Central pontine myelinolysis and its imitators: MR fi ndings. Radiology. 1988;168(3):795–802. https://doi.org/10.1148/radiology.168.3.3406409
30. Martin RJ. Central pontine and extrapontine myelinolysis: the osmotic demyelination syndromes. J Neurol Neurosurg Psychiatry. 2004;75(Suppl 3):iii22–28. https://doi.org/10.1136/ jnnp.2004.045906
31. Wagner J, Mueller-Schunk S, Schankin Ch. The Piglet Sign: MRI Findings in Central Pontine Myelinolysis. Clinical Neuroradiology. 2016;18(3):191. https://doi.org/10.1007/s00062-008-8025-5
32. Spasovski G, Vanholder R, Allolio B, Annane D, Ball S, Bichet D, Decaux G, Fenske W, Hoorn EJ, Ichai C, Joannidis M, Soupart A, Zietse R, Haller M, van der Veer S, Van Biesen W, Nagler E. Clinical practice guideline on diagnosis and treatment of hyponatraemia. Hyponatraemia Guideline Development Group. Eur J Endocrinol. 2014;170(3):G1-G47. https://doi.org/10.1530/EJE-13-1020
33. Lohr JW. Osmotic demyelination syndrome following correction of hyponatremia: association with hypokalemia. Am J Med. 1994;96(5):408–413. https://doi.org/10.1016/0002-9343(94)90166-x
34. Sood L, Sterns RH, Hix JK, Silver SM, Chen L. Hypertonic saline and desmopressin: a simple strategy for safe correction of severe hyponatremia. Am J Kidney Dis. 2013;61(4):571–578. https://doi.org/10.1053/j.ajkd.2012.11.032
35. Bibl D, Lampl C, Gabriel C, Jüngling G, Brock H, Köstler G. Treatment of central pontine myelinolysis with therapeutic plasmapheresis. Lancet. 1999;353(9159):1155. https://doi. org/10.1016/S0140-6736(99)01145-9
36. Grimaldi D, Cavalleri F, Vallone S, Milanti G, Cortelli P. Plasmapheresis improves the outcome of central pontine myelinolysis. J Neurol. 2005;252(6):734–735. https://doi.org/10.1007/s00415-005-0738-7
37. Kumon S, Usui R, Kuzuhara S, Nitta K, Koike M. The improvement of the outcome of osmotic demyelination syndrome by plasma exchange. Intern Med. 2017;56(6):733–736. https://doi. org/10.2169/internalmedicine.56.7491
38. Sugimura Y, Murase T, Takefuji S, Hayasaka S, Takagishi Y, Oiso Y, Murata Y. Protective eff ect of dexamethasone on osmotic-induced demyelination in rats. Exp Neurol. 2005;192(1):178–183. https://doi.org/10.1016/j.expneurol.2004.10.018
39. Morard I, Gasche Y, Kneteman M, Toso C, Mentha A, Meeberg G, Mentha G, Kneteman N, Giostra E. Identifying risk factors for central pontine and extrapontine myelinolysis after liver transplantation: A case-control study. Neurocrit Care. 2014;20(2):287–295. https://doi.org/10.1007/s12028-013-9928-9
Review
For citations:
Ramazanov G.R., Kovaleva E.A., Akchurina K.R., Bykova M.E. Osmotic demyelination syndrome. Russian neurological journal. 2024;29(5):4-12. (In Russ.) https://doi.org/10.30629/2658-7947-2024-29-5-4-12