Osmotic Demyelination Syndrome – MRI Diagnosis: a Case Report
Authors:
M. Slanina 1; J. Žižka 1; L. Klzo 1; J. Novotný 1; P. Ryška 1; Z. Špriňar 1; J. Hons 2; M. Šenkeřík 3; P. Kunc 4
Authors‘ workplace:
Radiologická klinika LF UK a FN
Hradec Králové
1; Psychiatrická klinika LF UK a FN
Hradec Králové
2; Dětská klinika LF UK a FN
Hradec Králové
3; Neurologická klinika LF UK a FN
Hradec Králové
4
Published in:
Cesk Slov Neurol N 2007; 70/103(3): 322-327
Category:
Case Report
Overview
Central pontine myelinolysis (CPM) together with extrapontine myelinolysis (EPM) are also named osmotic demyelination syndrome (ODMS). The osmotic destruction of myelin sheaths in the pons and/or extrapontine localization occurs in this disease while neurons and their axons remain intact. The mechanism of the myelinolysis development is not well known yet. It is commonly found in association with mineral dysbalance (particularly hyponatraemia or its rapid correction), therefore chronic alcoholics or seriously malnourished patients are typically afflicted. Common symptoms are spastic quadruparesis, pseudobulbar paralysis, pseudocoma (locked-in syndrome), coma, and a few cases may also progress to death. MRI is of the main importance in the ODMS diagnosis. In the case of CPM, we can find T1 hypointense, T2, PD (proton density weighed images) and FLAIR (fluid attenuated inversion recovery) hyperintense lesion in the central pons whereas peripheral area and pyramidal tracts remain intact. There is either no enhancement or a ring-like peripheral enhancement after a contrast agent administration. DWI (diffusion weighed images) with ADC (apparent diffusion coefficient) allow to differentiate cytotoxic oedema, which can be present in ODMS, and vasogenic oedema. Similar imaging findings as those in ODMS can be also observed in ischaemia, astrocytoma, multiple sclerosis, some metabolic diseases, posterior reversible encephalopathy syndrome or mesial temporal sclerosis. The authors have presented their experience with 7 cases of ODMS diagnosed by MRI in their institution.
Key words:
central pontine myelinolysis (CPM) – extrapontine myelinolysis (EPM) – osmotic demyelination syndrome (ODMS) – magnetic resonance imaging (MRI)
Sources
1. Adams RD, Victor M, Mancall EL. Central pontine myelinolysis: a hitherto undescribed disease occuring in alcoholic and malnourished patients. AMA Arch Neurol Psychiatry 1959; 81: 154–72.
2. Lampl C, Yazdi K. Central pontine myelinolysis. Eur Neurol 2002; 47: 3–10.
3. Žižka J. Onemocnění bílé hmoty. In: Černoch Z et al. Neuroradiologie. Hradec Králové: Nucleus 2000: 383–414.
4. Osborn AG. Osmotic Demyelination Syndrome. In: Osborn AG et al. Diagnostic Imaging: Brain. 1st ed. Salt Lake City: Amirsys Inc 2004: 42–5.
5. Nusbaum AO, Fung KM, Atlas SW. White Matter Diseases and Inherited Metabolic Disorders. In: Atlas SW. Magnetic Resonance Imaging of the Brain and Spine. 3rd ed. Philaladelphia: Lippincott Williams & Wilkins 2002: 457–563.
6. Yu J, Zheng SS, Liang TB, Shen Y, Wang WL, Ke QH. Possible causes of central pontine myelinolysis after liver transplantation. World J Gastroenterol 2004; 10: 2540–3.
7. Kozáková S, Zamrazil V, Tomek A, Charvát J, Kvapil M. Centrální pontinní myelinolýza u diabetes mellitus 1. typu. Praha: GEUM: Kazuistiky v diabetologii 2003; 1: 28–30.
8. Shintani M, Yamashita M, Nakano A, Aotani D, Maeda K, Yamamoto T et al. Central pontine and extrapontine myelinolysis associated with type 2 diabetic patient with hypokalemia. Diabetes Res Clin Pract 2005; 68: 75–80.
9. Moritani T, Ekholm S, Westesson PL, Zhong J. Toxic and Metabolic Disease. In: Moritani T, Ekholm S, Westesson PL. Diffusion Weighted MR Imaging of the Brain. Berlin, Heidelberg: Springer-Verlag 2003: 119–30.
10. Lasheen I, Doi SA, Al-Shoumer KA. Glucocorticoid replacement in panhypopituitarism complicated by myelinolysis. Med Princ Pract 2005; 14: 115–7.
11. Santinelli R, Tolone C, D'Avanzo A, del Giudice EM, Perrone L, D'Avanzo M. Pontine myelinolysis in a child with carbamate poisoning. Clin Toxicol (Phila) 2006; 44: 327–8.
12. Sugimoto T, Murata T, Omori M, Wada Y. Central pontine myelinolysis associated with hypokalaemia in anorexia nervosa. J Neurol Neurosurg Psychiatry 2003; 74: 353–5.
13. Grossman RI, Yousem DM. White Matter Diseases. In: Grossman RI, Yousem DM. The Requisites: Neuroradiology. St. Louis: Mosby-Year Book, Inc 1994: 201–24.
14. Cramer SC, Stegbauer KC, Schneider A, Mukai J, Maravilla KR. Decreased diffusion in central pontine myelinolysis. AJNR Am J Neuroradiol 2001; 22: 1476–9.
15. Michell AW, Burn DJ, Reading PJ. Central pontine myelinolysis temporally related to hypophosphataemia. J Neurol Neurosurg Psychiatry 2003; 74: 820.
16. Chua GC, Sitoh YY, Lim CC, Chua HC, Ng PY. MRI findings in osmotic myelinolysis. Clin Radiol 2002; 57: 800–6.
17. Kumar SR, Mone AP, Gray LC, Troost BT. Central pontine myelinolysis: delayed changes on neuroimaging. J Neuroimaging 2000; 10: 169–72.
18. Bartoš A, Rusina R. Centrální pontinní myelinolýza v klinicko-neuroanatomických souvislostech. Česk Slov Neurol N 2000; 63: 422–5.
19. Chu K, Kang DW, Ko SB, Kim M. Diffusion-weighted MR findings of central pontine and extrapontine myelinolysis. Acta Neurol Scand 2001; 104: 385–8.
20. Popov P. Návykové poruchy. In: Raboch J, Zvolský P et al. Psychiatrie. Praha: Galén 2001: 182–226.
21. Horáček J, Zadák Z, Živný P. Metabolismus. In: Bureš J, Horáček J. Základy vnitřního lékařství. Praha: Galén 2003: 611–54.
22. Achiwa S, Ando K, Ishikura R, Takada Y, Takahashi Y. A case of extrapontine myelinolysis precipitated by correction of a hyperosmolar state. Nippon Igaku Hoshasen Gakkai Zasshi 2004; 64: 310–2.
23. DeLuca GC, Nagy Z, Esiri MM, Davey P. Evidence for a role for apoptosis in central pontine myelinolysis. Acta Neuropathol (Berl) 2002; 103: 590–8.
24. Hinchey J, Chaves C, Appignani B, Breen J, Pao L, Wang A et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996; 334: 494–500.
25. Keswani SC. Central pontine myelinolysis associated with hypokalaemia in anorexia nervosa. J Neurol Neurosurg Psychiatry 2004; 75: 663.
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Paediatric neurology Neurosurgery NeurologyArticle was published in
Czech and Slovak Neurology and Neurosurgery
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