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Central pontine myelinolysis

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48: 167: 391:, the extracellular tonicity increases, followed by an increase in intracellular tonicity. When the correction is too rapid, not enough time is allowed for the brain's cells to adjust to the new tonicity, namely by increasing the intracellular osmoles mentioned earlier. If the serum sodium levels rise too rapidly, the increased extracellular tonicity will continue to drive water out of the brain's cells. This can lead to cellular dysfunction and central pontine myelinolysis. 226: 437:
of critically ill patients may even be better than what is generally considered, despite severe initial clinical manifestations and a tendency by the intensivists to underestimate a possible favorable evolution. While some patients die, most survive and of the survivors, approximately one-third recover; one-third are disabled but are able to live independently; one-third are severely disabled. Permanent disabilities range from minor tremors and
241:). Apart from rapid correction of hyponatraemia, there are case reports of central pontine myelinolysis in association with hypokalaemia, anorexia nervosa when feeding is started, patients undergoing dialysis and burn victims. There is a case report of central pontine myelinolysis occurring in the context of 133:. 'Central pontine' indicated the site of the lesion and 'myelinolysis' was used to emphasise that myelin was affected. The authors intentionally avoided the term 'demyelination' to describe the condition, in order to differentiate this condition from multiple sclerosis and other neuroinflammatory disorders. 436:
Though traditionally the prognosis is considered poor, a good functional recovery is possible. All patients at risk of developing refeeding syndrome should have their electrolytes closely monitored, including sodium, potassium, magnesium, glucose and phosphate. Recent data indicate that the prognosis
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To minimise the risk of this condition developing from its most common cause, overly rapid reversal of hyponatremia, the hyponatremia should be corrected at a rate not exceeding 10 mmol/L/24 h or 0.5 mEq/L/h; or 18 mEq/L/48hrs; thus avoiding demyelination. No large clinical trials have
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Symptoms depend on the regions of the brain involved. Prior to its onset, patients may present with the neurological signs and symptoms of hyponatraemic encephalopathy such as nausea and vomiting, confusion, headache and seizures. These symptoms may resolve with normalisation of the serum sodium
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Once osmotic demyelination has begun, there is no cure or specific treatment. Care is mainly supportive. Alcoholics are usually given vitamins to correct for other deficiencies. The favourable factors contributing to the good outcome in central pontine myelinolysis without hyponatremia were:
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low plasma sodium (hyponatremia), the brain compensates by decreasing the levels of these osmolytes within the cells, so that they can remain relatively isotonic with their surroundings and not absorb too much fluid. The reverse is true in hypernatremia, in which the cells increase their
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concentration. Three to five days later, a second phase of neurological manifestations occurs correlating with the onset of myelinolysis. Observable immediate precursors may include seizures, disturbed consciousness, gait changes, and decrease or cessation of respiratory function.
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been performed to examine the efficacy of therapeutic re-lowering of serum sodium, or other interventions sometimes advocated such as steroids or plasma exchange. Alcoholic patients should receive vitamin supplementation and a formal evaluation of their nutritional status.
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It can be diagnosed clinically in the appropriate context, but may be difficult to confirm radiologically using conventional imaging techniques. Changes are more prominent on MRI than on CT, but often take days or weeks after acute symptom onset to develop. Imaging by
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Axial fat-saturated T2-weighted image showing hyperintensity in the pons with sparing of the peripheral fibers, the patient was an alcoholic admitted with a serum Na of 101 treated with hypertonic saline, he was left with quadriparesis, dysarthria, and altered mental
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concurrent treatment of all electrolyte disturbances, early intensive care unit involvement at the advent of respiratory complications, early introduction of feeding including thiamine supplements with close monitoring of the electrolyte changes and input.
271:, inadequate thirst reception leads to excessive water intake, severely diluting serum sodium. With this excessive thirst combined with psychotic symptoms, brain damage such as central pontine myelinolysis may result from 2157: 2142: 1961:
Louis G, Megarbane B, LavouĂ© S, Lassalle V, Argaud L, Poussel JF, et al. (March 2012). "Long-term outcome of patients hospitalized in intensive care units with central or extrapontine myelinolysis*".
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Central pontine myelinolysis was first described as a disorder in 1959. The original paper described four cases with fatal outcomes, and the findings on autopsy. The disease was described as a disease of
252:. In these instances, occurrence may be entirely unrelated to hyponatremia or rapid correction of hyponatremia. It could affect patients who take some prescription medicines that are able to cross the 897:
Bernsen HJ, Prick MJ (September 1999). "Improvement of central pontine myelinolysis as demonstrated by repeated magnetic resonance imaging in a patient without evidence of hyponatremia".
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Leroy S, Gout A, Husson B, de Tournemire R, Tardieu M (June 2012). "Centropontine myelinolysis related to refeeding syndrome in an adolescent suffering from anorexia nervosa".
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Since this original description, demyelination in other areas of the central nervous system associated with osmotic stress has been described outside the pons (extrapontine).
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Kato T, Hattori H, Nagato M, Kiuchi T, Uemoto S, Nakahata T, Tanaka K (April 2002). "Subclinical central pontine myelinolysis following liver transplantation".
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Odier C, Nguyen DK, Panisset M (July 2010). "Central pontine and extrapontine myelinolysis: from epileptic and other manifestations to cognitive prognosis".
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Kleinschmidt-DeMasters BK, Norenberg MD (March 1981). "Rapid correction of hyponatremia causes demyelination: relation to central pontine myelinolysis".
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McKee AC, Winkelman MD, Banker BQ (August 1988). "Central pontine myelinolysis in severely burned patients: relationship to serum hyperosmolality".
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Central pontine myelinolysis, and osmotic demyelination syndrome, present most commonly as a complication of treatment of patients with profound
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following treatment in individuals with chronic, severe hyponatremia who have made intracellular adaptations to the prevailing hypotonicity.
992:"Psychogenic polydipsia: the result, or cause of, deteriorating psychotic symptoms? A case report of the consequences of water intoxication" 925: 1548:"Rapid development of central pontine myelinolysis after recovery from Wernicke encephalopathy: a non-alcoholic case without hyponatremia" 1043:"Central pontine myelinolysis in a patient with acute lymphoblastic leukemia after hematopoietic stem cell transplantation: a case report" 2215: 213:
In about ten per cent of people with central pontine myelinolysis, extrapontine myelinolysis is also found. In these cases symptoms of
151:), which can result from a varied spectrum of conditions, based on different mechanisms. It occurs as a consequence of a rapid rise in 2603: 2707: 2240: 202:), with other more variable neurological features associated with brainstem damage. These result from a rapid myelinolysis of the 287: 2743: 2712: 2588: 2535: 2208: 1898:"Myoinositol administration improves survival and reduces myelinolysis after rapid correction of chronic hyponatremia in rats" 2722: 2527: 2687: 2613: 2573: 1096:"Central nervous system lesions in adult liver transplant recipients: clinical review with implications for management" 2748: 2717: 2646: 2593: 2583: 2540: 2394: 2361: 2341: 644: 2682: 2314: 2598: 2510: 471:"Central pontine myelinolysis: a hitherto undescribed disease occurring in alcoholic and malnourished patients" 1853:
Brown WD (December 2000). "Osmotic demyelination disorders: central pontine and extrapontine myelinolysis".
449:. Some improvements may be seen over the course of the first several months after the condition stabilizes. 2641: 2336: 1080: 850:"Pontine and extrapontine myelinolysis: a neurologic disorder following rapid correction of hyponatremia" 2200: 401: 2578: 2463: 2253: 424:
reduces the severity of osmotic demyelination syndrome if given before attempting to correct chronic
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and cause abnormal thirst reception - in this scenario the central pontine myelinolysis is caused by
2172: 1448:"Wernicke's encephalopathy and central pontine myelinolysis associated with hyperemesis gravidarum" 616:
Gocht A, Colmant HJ (1987). "Central pontine and extrapontine myelinolysis: a report of 58 cases".
230: 2298: 1736:"Intracranial Lesions with Low Signal Intensity on T2-weighted MR Images - Review of Pathologies" 352: 253: 1937: 1186:
Martinez AJ, Estol C, Faris AA (May 1988). "Neurologic complications of liver transplantation".
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T2 weighted magnetic resonance scan image showing bilaterally symmetrical hyperintensities in
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Spasovski G, Vanholder R, Allolio B, Annane D, Ball S, Bichet D, et al. (March 2014).
1266:"Central nervous system complications of thermal burns. A postmortem study of 139 patients" 307: 293:
Central pontine myelinolysis may also occur in patients prone to hyponatremia affected by:
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Musana AK, Yale SH (August 2005). "Central pontine myelinolysis: case series and review".
8: 2449: 2371: 2090: 347: 199: 1914: 1897: 1796: 2550: 2434: 2429: 2424: 2414: 2066: 2041: 1987: 1938:"INOSITOL: Overview, Uses, Side Effects, Precautions, Interactions, Dosing and Reviews" 1878: 1760: 1735: 1621: 1588: 1523: 1496: 1472: 1447: 1428: 1380: 1363: 1339: 1314: 1295: 1246: 1168: 1125: 1067: 1018: 991: 879: 830: 719: 672: 446: 388: 380: 276: 268: 242: 195: 182:(broad arrow), suggestive of extrapontine myelinolysis (osmotic demyelination syndrome) 1497:"Wernicke's encephalopathy and central pontine myelinolysis in hyperemesis gravidarum" 1199: 1156: 140:
is the term used for both central pontine myelinolysis and extrapontine myelinolysis.
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intracellular osmolytes so as not to lose too much fluid to the extracellular space.
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It has also been known to occur in patients suffering withdrawal symptoms of chronic
61: 1882: 1432: 1299: 1250: 1172: 1129: 883: 834: 723: 676: 2351: 2061: 2053: 2014: 1971: 1909: 1862: 1800: 1755: 1747: 1706: 1660: 1616: 1600: 1559: 1518: 1508: 1467: 1459: 1412: 1375: 1334: 1326: 1277: 1230: 1195: 1152: 1107: 1062: 1054: 1013: 1003: 962: 861: 814: 750: 703: 656: 588: 540: 482: 328: 166: 2661: 2631: 2177: 2018: 1975: 179: 171: 2636: 2326: 2288: 1604: 1364:"Central pontine myelinolysis associated with hypokalaemia in anorexia nervosa" 1315:"Central pontine myelinolysis associated with hypokalaemia in anorexia nervosa" 1058: 2166: 2005:
Young GB (March 2012). "Central pontine myelinolysis: a lesson in humility*".
818: 428:. Further study is required before using inositol in humans for this purpose. 190:
The classical clinical presentation is the progressive development of spastic
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The most common cause is overly-rapid correction of low blood sodium levels (
207: 203: 191: 152: 92: 1804: 951:"Psychotic disorder in a patient with central and extrapontine myelinolysis" 755: 738: 545: 528: 452:
The degree of recovery depends on the extent of the original axonal damage.
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Laureno R (1980). "Experimental pontine and extrapontine myelinolysis".
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Kishimoto Y, Ikeda K, Murata K, Kawabe K, Hirayama T, Iwasaki Y (2012).
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Lim KH, Kim S, Lee YS, Kim KH, Kim J, Rhee J, et al. (April 2008).
739:"Central pontine and extrapontine myelinolysis after alcohol withdrawal" 529:"Central pontine and extrapontine myelinolysis after alcohol withdrawal" 2503: 2439: 2419: 2404: 2378: 2263: 1733: 364: 363:
The currently accepted theory states that the brain cells adjust their
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typically demonstrates areas of hyperintensity on T2-weighted images.
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Sutamnartpong P, Muengtaweepongsa S, Kulkantrakorn K (January 2013).
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Research has led to improved outcomes. Animal studies suggest that
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Babar SM (October 2013). "SIADH associated with ciprofloxacin".
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Silver SM, Schroeder BM, Sterns RH, Rojiani AM (January 2006).
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Zimny A, Neska-Matuszewska M, Bladowska J, SÄ…siadek MJ (2015).
1692: 438: 148: 113:(treatment-induced), and is characterized by acute paralysis, 96: 468: 41:
Osmotic demyelination syndrome, central pontine demyelination
1960: 1895: 1402: 926:"Psychogenic Polydipsia (Excessive Fluid seeking Behaviour)" 2293: 581:
The Journal of the Royal College of Physicians of Edinburgh
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in response to varying serum osmolality. In the context of
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is a neurological condition involving severe damage to the
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Megalencephalic leukoencephalopathy with subcortical cysts
1545: 121:(difficulty speaking), and other neurological symptoms. 1312: 1142: 574: 2039: 2367:
Lesional demyelinations of the central nervous system
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Abbott R, Silber E, Felber J, Ekpo E (October 2005).
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Transactions of the American Neurological Association
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and have a long history of mental health conditions.
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Journal of Neuropathology and Experimental Neurology
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Sugimoto T, Murata T, Omori M, Wada Y (March 2003).
1220: 577:"Central pontine myelinolysis without hyponatraemia" 575:
Bose P, Kunnacherry A, Maliakal P (September 2011).
1586: 804: 260:leading to low blood sodium levels (hyponatremia). 1587:Gankam Kengne, Fabrice; Decaux, Guy (2017-09-01). 1368:Journal of Neurology, Neurosurgery, and Psychiatry 1319:Journal of Neurology, Neurosurgery, and Psychiatry 1185: 2556:Chronic inflammatory demyelinating polyneuropathy 1263: 2735: 2652:Leukoencephalopathy with vanishing white matter 1688: 1686: 1684: 1642: 1640: 1093: 570: 568: 566: 564: 2216: 736: 526: 367:by changing levels of certain osmolytes like 2033: 1998: 1954: 1846: 1681: 1637: 1539: 1488: 1445: 1396: 1355: 1306: 1264:Winkelman MD, Galloway PG (September 1992). 1136: 1040: 1034: 989: 931:. American Psychological Society Divisions. 896: 890: 615: 507:: CS1 maint: multiple names: authors list ( 1646: 1439: 1257: 1214: 1179: 1087: 847: 841: 771: 636: 561: 462: 2223: 2209: 1501:Journal of Neurosciences in Rural Practice 948: 777: 642: 609: 46: 2604:Experimental autoimmune encephalomyelitis 2065: 1913: 1759: 1710: 1664: 1620: 1563: 1522: 1512: 1471: 1379: 1338: 1281: 1111: 1094:Singh N, Yu VL, Gayowski T (March 1994). 1066: 1017: 1007: 966: 865: 754: 592: 544: 441:to signs of severe brain damage, such as 387:With correction of the hyponatremia with 2708:List of multiple sclerosis organizations 487:10.1001/archneurpsyc.1959.02340140020004 233:in the brainstem (Luxol-Fast blue stain) 224: 165: 1825: 1361: 469:Adams RD, Victor M, Mancall EL (1959). 288:hematopoietic stem cell transplantation 14: 2736: 2713:List of people with multiple sclerosis 2589:Neuromyelitis optica spectrum disorder 2536:Neuromyelitis optica spectrum disorder 2088: 522: 520: 518: 317:People with serum sodium <105 mEq/L 2204: 2004: 1852: 955:Psychiatry and Clinical Neurosciences 693: 161: 2688:Mitochondrial DNA depletion syndrome 2574:Acute disseminated encephalomyelitis 848:Karp BI, Laureno R (November 1993). 687: 275:caused by excess intake of fluids, ( 1915:10.1097/01.jnen.0000195938.02292.39 1446:Bergin PS, Harvey P (August 1992). 515: 24: 1647:Medana IM, Esiri MM (March 2003). 923: 737:Yoon B, Shim YS, Chung SW (2008). 527:Yoon B, Shim YS, Chung SW (2008). 358: 245:, in the absence of hyponatremia. 25: 2760: 2112: 1699:European Journal of Endocrinology 1047:Journal of Korean Medical Science 990:Gill M, McCauley M (2015-01-21). 229:Loss of myelinated fibers at the 2718:Multiple sclerosis drug pipeline 2594:Diffuse myelinoclastic sclerosis 2584:Marburg acute multiple sclerosis 2541:Diffuse myelinoclastic sclerosis 2395:Management of multiple sclerosis 2362:Radiologically isolated syndrome 2342:Expanded Disability Status Scale 2042:"Osmotic demyelination syndrome" 1867:10.1097/00019052-200012000-00014 1565:10.2169/internalmedicine.51.7498 1283:10.1097/00005792-199209000-00002 1113:10.1097/00005792-199403000-00004 968:10.1111/j.1440-1819.2007.01648.x 938:from the original on 2015-04-07. 867:10.1097/00005792-199311000-00001 683:from the original on 2012-03-06. 2315:Diagnosis of multiple sclerosis 2082: 1930: 1889: 1819: 1776: 1727: 1580: 983: 942: 917: 286:It has been observed following 2599:Tumefactive multiple sclerosis 2511:Research in multiple sclerosis 2231:Demyelinating diseases of the 2121:Images of Osmotic Myelinolysis 2091:"Central Pontine Myelinolysis" 949:Lim L, Krystal A (June 2007). 798: 730: 645:"Central pontine myelinolysis" 178:(long arrow), with sparing of 138:Osmotic demyelination syndrome 18:Osmotic demyelination syndrome 13: 1: 2744:Demyelinating diseases of CNS 2089:Luzzio C (17 November 2015). 1200:10.1016/S0733-8619(18)30873-9 1157:10.1016/S0387-7604(02)00013-X 696:The Annals of Pharmacotherapy 455: 2678:Central pontine myelinolysis 2647:Pelizaeus–Merzbacher disease 2642:Metachromatic leukodystrophy 2337:Clinically isolated syndrome 2308:Investigations and diagnosis 2019:10.1097/CCM.0b013e31823b8e0b 1976:10.1097/CCM.0b013e318236f152 1855:Current Opinion in Neurology 1593:Kidney International Reports 1589:"Hyponatremia and the Brain" 1374:(4): 663, author reply 663. 431: 407: 394: 333:Severe electrolyte disorders 297:Severe liver disease (e.g., 89:Central pontine myelinolysis 33:Central pontine myelinolysis 7: 2683:Marchiafava–Bignami disease 1740:Polish Journal of Radiology 10: 2765: 1605:10.1016/j.ekir.2017.08.015 1059:10.3346/jkms.2008.23.2.324 996:Case Reports in Psychiatry 475:AMA Arch Neurol Psychiatry 198:, and emotional lability ( 2700: 2670: 2612: 2579:Balo concentric sclerosis 2564: 2526: 2519: 2493: 2387: 2307: 2239: 2128: 1362:Keswani SC (April 2004). 819:10.1007/s00415-010-5486-7 643:Lampl C, Yazdi K (2002). 220: 210:tracts in the brainstem. 117:(difficulty swallowing), 72: 60: 54: 45: 37: 32: 2749:Electrolyte disturbances 2058:10.1136/bmj.331.7520.829 1514:10.4103/0976-3147.105608 1464:10.1136/bmj.305.6852.517 899:Acta Neurologica Belgica 708:10.1177/1060028013502457 231:basilar part of the pons 1805:10.1126/science.7466381 1145:Brain & Development 618:Clinical Neuropathology 353:Wernicke encephalopathy 109:). It is predominately 2520:Demyelinating diseases 2233:central nervous system 2007:Critical Care Medicine 1964:Critical Care Medicine 1417:10.1055/s-0032-1307458 743:Alcohol and Alcoholism 594:10.4997/JRCPE.2011.305 533:Alcohol and Alcoholism 342:hyperemesis gravidarum 269:psychogenic polydipsia 234: 183: 1331:10.1136/jnnp.74.3.353 1235:10.1212/wnl.38.8.1211 756:10.1093/alcalc/agn050 546:10.1093/alcalc/agn050 443:spastic quadriparesis 228: 174:(small, thin arrow), 169: 2622:Adrenoleukodystrophy 2546:MOG antibody disease 2348:Serological and CSF 2299:Uhthoff's phenomenon 1666:10.1093/brain/awg061 807:Journal of Neurology 346:Hyponatremia due to 2450:Monomethyl fumarate 1797:1981Sci...211.1068K 1791:(4486): 1068–1070. 1752:10.12659/PJR.892146 1712:10.1530/eje-13-1020 1009:10.1155/2015/846459 348:peritoneal dialysis 254:blood-brain barrier 215:Parkinson's disease 200:pseudobulbar affect 2551:Multiple sclerosis 2435:Interferon beta-1b 2430:Interferon beta-1a 2425:Glatiramer acetate 2415:Diroximel fumarate 2388:Approved treatment 2241:Signs and symptoms 1188:Neurologic Clinics 649:European Neurology 447:locked-in syndrome 389:intravenous fluids 277:primary polydipsia 243:refeeding syndrome 235: 217:may be generated. 196:pseudobulbar palsy 184: 162:Signs and symptoms 2731: 2730: 2696: 2695: 2627:Alexander disease 2410:Dimethyl fumarate 2352:Oligoclonal bands 2320:McDonald criteria 2198: 2197: 2052:(7520): 829–830. 1659:(Pt 3): 515–530. 1558:(12): 1599–1603. 1552:Internal Medicine 1458:(6852): 517–518. 702:(10): 1359–1363. 661:10.1159/000047939 281:institutionalised 86: 85: 27:Medical condition 16:(Redirected from 2756: 2524: 2523: 2494:Other treatments 2372:Dawson's fingers 2225: 2218: 2211: 2202: 2201: 2126: 2125: 2106: 2105: 2103: 2101: 2086: 2080: 2079: 2069: 2037: 2031: 2030: 2013:(3): 1026–1027. 2002: 1996: 1995: 1958: 1952: 1951: 1949: 1948: 1934: 1928: 1927: 1917: 1893: 1887: 1886: 1850: 1844: 1843: 1823: 1817: 1816: 1780: 1774: 1773: 1763: 1731: 1725: 1724: 1714: 1690: 1679: 1678: 1668: 1644: 1635: 1634: 1624: 1584: 1578: 1577: 1567: 1543: 1537: 1536: 1526: 1516: 1492: 1486: 1485: 1475: 1443: 1437: 1436: 1400: 1394: 1393: 1383: 1359: 1353: 1352: 1342: 1310: 1304: 1303: 1285: 1261: 1255: 1254: 1229:(8): 1211–1217. 1218: 1212: 1211: 1183: 1177: 1176: 1140: 1134: 1133: 1115: 1091: 1085: 1084: 1079:. 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criteria 2324: 2323: 2322: 2311: 2309: 2305: 2304: 2302: 2301: 2296: 2291: 2289:Optic neuritis 2286: 2281: 2276: 2271: 2266: 2261: 2256: 2251: 2245: 2243: 2237: 2236: 2228: 2227: 2220: 2213: 2205: 2196: 2195: 2192: 2191: 2180: 2169: 2154: 2138: 2133: 2132: 2130: 2129:Classification 2123: 2122: 2114: 2113:External links 2111: 2108: 2107: 2081: 2032: 1997: 1970:(3): 970–972. 1953: 1929: 1888: 1861:(6): 691–697. 1845: 1818: 1775: 1726: 1680: 1636: 1579: 1538: 1487: 1438: 1411:(3): 152–154. 1395: 1354: 1325:(3): 353–355. 1305: 1276:(5): 271–283. 1256: 1213: 1194:(2): 327–348. 1178: 1151:(3): 179–182. 1135: 1106:(2): 110–118. 1086: 1083:on 2009-02-27. 1053:(2): 324–327. 1033: 982: 961:(3): 320–322. 941: 916: 905:(3): 189–193. 889: 860:(6): 359–373. 840: 797: 770: 749:(6): 647–649. 729: 686: 635: 624:(6): 262–270. 608: 587:(3): 211–214. 560: 539:(6): 647–649. 514: 460: 459: 457: 454: 433: 430: 409: 406: 396: 393: 360: 357: 356: 355: 350: 344: 339: 334: 331: 326: 321: 318: 315: 310: 305: 302: 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Index

Osmotic demyelination syndrome

Specialty
Neurology
Alcoholism
malnutrition
myelin sheath
nerve cells
pons
brainstem
iatrogenic
dysphagia
dysarthria
alcoholics
malnutrition
hyponatremia
sodium
serum
tonicity

caudate nucleus
putamen
globus pallidus
quadriparesis
pseudobulbar palsy
pseudobulbar affect
corticobulbar
corticospinal
Parkinson's disease

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