李俊敏,彭涛,付振强,马兴荣,王建平,卢宏

• 1:郑州大学第一附属医院

摘要(Abstract)：

目的分析渗透性脱髓鞘综合征(osmotic demyelination syndromes,ODS)的临床表现,影像学特点及预后。方法对2010-01—2017-12收住郑州大学一附院ODS患者临床资料进行回顾性分析。结果 9例患者中8例有低钠血症补钠史,另1例肝移植患者术后持续高钠血症。临床主要表现为肢体无力(8/9)、精神异常(7/9)、延髓性麻痹(5/9)。病变累及脑桥6例,脑桥外病变8例,脑桥外病变主要累及双侧基底节区、丘脑、皮层灰白质交界区。MRI多表现为等或稍长T_1、长T_2信号。FLAIR像、DWI像高信号,ADC像根据时期不同可呈低或等信号,增强多无强化。9例患者均使用类固醇激素治疗,基本痊愈2例,好转5例,无明显缓解1例,死亡1例。结论 ODS包括脑桥中央髓鞘溶解和脑桥外髓鞘溶解,患者多有血钠浓度快速波动史,经类固醇激素治疗,多数患者预后良好。

关键词(KeyWords)： 渗透性脱髓鞘综合征;脑桥中央髓鞘溶解;脑桥外髓鞘溶解;磁共振;低钠血症

Abstract:

Keywords:

基金项目(Foundation):

作者(Author): 李俊敏,彭涛,付振强,马兴荣,王建平,卢宏

参考文献(References)：

• [1] SINGH T D,FUGATE J E,RABINSTEIN A A.Central pontine and extrapontine myelinolysis:a systematic review[J].Eur J Neurol,2014,21(12):1 443-1 450.
• [2] ZUNGA P M,FAROOQ O,DAR M,I et al.Extra pontine osmotic demyelination syndrome[J].ANN NEUROL,2015,22(1):51-53.
• [3] VARANDA S,COSTA S,CARVALHO R,et al.Central pontine myelinolysis caused by hypernatremia[J].J Neurol Sci,2016,370:274-276.
• [4] TAN A H,LIM S Y,NG R X.Osmotic Demyelination Syndrome With Evolving Movement Disorders[J].JAMA Neurol,2018,75(7):888-889.
• [5] DE SOUZA A,DE SOUZA R J.Choreiform dyskinesias following levodopa treatment of parkinsonism due to osmotic demyelination syndrome[J].PARKINSONISM RELAT,2017,43:120-121.
• [6] HERATH H M M T B,PAHALAGAMAGE S P,SENANAYAKE S.Tongue fasciculations with denervation pattern in osmotic demyelination syndrome:a case report of diagnostic dilemma[J].BMC research notes,2018,11(1):177.
• [7] ABDUL HALIM S,MOHD AMIN N A.Treatment response in osmotic demyelination syndrome presenting as severe parkinsonism,ptosis and gaze palsy[J].BMJ Case Rep,2018 Oct 21;2018-225751.
• [8] ADAMS R A,VICTOR M,MANCALL E L.Central pontine myelinolysis:a hitherto underdescribed disease occurring in alcoholics and malnourished patients[J].AMA Arch Neurol Psychiatry,1959,81:154-172.
• [9] LEE E M,KANG J K,YUN S C,et al.Risk factors for central pontine and extrapontine myelinolysis following orthotopic liver transplantation[J].Eur Neurol,2009,62(6):362-368.
• [10] CRISMALE J F,MELIAMBRO K A,DEMARIA S,et al.Prevention of the Osmotic Demyelination Syndrome After Liver Transplantation:A Multidisciplinary Perspective[J].AM J TRANSPLANT,2017,17(10):2 537-2 545.
• [11] HSU Y C,LEE Y C,HSU C W.Osmotic demyelina-tion syndrome after slow sodium administration in a hyponatremic patient with concurrent hypokalemia[J].AM J EMERG MED,2017,35(2):378.e3-378.e4.
• [12] TARHAN N C,AGILDERE A M,BENLI U S,et al.Osmotic demyelination syndrome in end stage renal disease after recent hemodialysis:MRI of the brain[J].AJR Am J Roentgenol,2004,182:809-816.
• [13] CHATTERJEE K,FERNANDES A B,GOYAL S,et al.Central pontine myelinolysis in a case of alcohol dependence syndrome[J].Ind Psychiatry J,2015,24(2):198-201.
• [14] HAN M J,KIM D H,KIM Y H,et al.A Case of Osmotic Demyelination Presenting with Severe Hypernatremia[J].Electrolyte Blood Press,2015,13(1):30-36.
• [15] HERRAIZ L,CUESTA M,RUNKLE I.Osmotic demyelination syndrome in a patient with uncontrolled hyperglycemia[J].Medicina Clinica,2017,149(8):370-371.
• [16] HIROSAWA T,SHIMIZU T.Osmotic demyelination syndrome due to hyperosmolar hyperglycemia[J].CLEV CLIN J MED,2018,85(7):511-513.
• [17] PERIKAL P J,JAGANNATHA A T,KHANAPURE K S,et al.Extrapontine Myelinolysis and Reversible Parkinsonism After Hyponatremia Correction in a Case of Pituitary Adenoma:Hypopituitarism as a Predisposition for Osmotic Demyelination[J].World Neurosurgery,2018,118:304-310.
• [18] ODIER C,NGUYEN D K,PANISSET M.Central pontine and extrapontine myelinolysis:from epileptic and other manifestations to cognitive prognosis[J].J Neurol,2010,257(7):1 176-1 180.
• [19] W NEWELL K L,KLEINSCHMIDT-DEMASTERS B K.Central pontine myelinolysis at autopsy;a twelve year retrospective analysis[J].J Neurol Sci,1996,142:134-139.
• [20] SULLIVAN E V,PFEFFERBAUM A.Magnetic resonance relaxometry reveals central pontine abnormalities in clinically asymptomatic alcoholic men[J].Alcohol Clin Exp Res,2001,25:1 206-1 212.
• [21] BEH S C.Temporal evolution of the trident and piglet signs of osmotic demyelination syndrome[J].J Neurol Sci,2017,373:268-273.
• [22] ALLEMAN A M.Osmotic demyelination syndrome:central pontine myelinolysis and extrapontine myelinolysis[J].Semin Ultrasound CT MR,2014,35(2):153-159.
• [23] GARG PRERNA,AGGARWAL ABHISHT,MALHOTRA RAJEEV,et al.Osmotic Demyelination Syndrome-Evolution of Extrapontine Before Pontine Myelinolysis on Magnetic Resonance Imaging[J].J Neurosci Rural Pract,2019,10(1):126-135.
• [24] FENG X M,ZHAO T,ZHOU C K,et al.Psychiatric symptoms and limb tremors associated with central pontine myelinolysis:A case of alcoholism without hyponatremia[J].Exp Ther Med,2016,12(5):3 485-3 487.
• [25] XU D H,YUAN M,WANG J W,et al.Osmotic demyelination syndrome after correction of severe hyponatremia associated with pituitrin[J].Int J Clin Pharm th,2015,53(5):408-411.
• [26] 郭筱华,赵忠新.脑桥中央髓鞘溶解症的流行病学、发病机制和临床特点[J].中华神经科杂志,2006,39(4):275-277.
• [27] HOORN E J,ZIETSE R.Diagnosis and Treatment of Hyponatremia:Compilation of the Guidelines[J].J Am Soc Nephrol,2017,28(5):1 340-1 349.
• [28] GEORGE J C,ZAFAR W,BUCALOIU I D,et al.Risk Factors and Outcomes of Rapid Correction of Severe Hyponatremia[J].Clin J Am Soc Nephr,2018,13(7):984-992.
• [29] NORENBERG M D.Central pontine myelinolysis:historical and mechanistic considerations[J].Metab Brain Dis,2010,25:97-106.
• [30] HEGAZI M O.Treatment of the osmotic demyelina-tion syndrome:the earlier the better?[J].Intern Emerg Med,2017,12(1):133-134.