妊娠剧吐致低钠血症并脑桥中央髓鞘溶解症1例并文献复习

目的探讨妊娠剧吐与低钠血症、脑桥中央髓鞘溶解症关系。方法报告我院收治的1例妊娠剧吐致低钠血症并脑桥中央髓鞘溶解症患者的诊治经过并进行文献复习。结果该患者发病过程中存在严重低钠血症及补钠过快情况,复查MRI可见脑桥呈蝠翼状,符合脑桥中央溶解综合症。结论妊娠剧吐可至低钠血症,低钠血症补钠过快可致脑桥中央髓鞘溶解症。     

脑桥外髓鞘溶解症1例

渗透性髓鞘溶解症( osmotic myelinolysis,OM)是一种严重的神经系统脱髓鞘疾病。根据病变部位的不同,分为脑桥中央髓鞘溶解症和脑桥外髓鞘溶解症( extrapontine myelinosis, EPM),EPM属于少见疾病,病变累及基底节区、丘脑及皮质下蛋白质等。     

脑桥外髓鞘溶解症的临床特点和MRI表现

目的探讨脑桥外髓鞘溶解症的临床特点和MRI表现。方法分析2例脑桥外髓鞘溶解症患者的临床特点,包括起病前诱因、临床表现、头颅MRI特点。结果2例患者均有慢性形成低钠血症后被快速纠正的病史,以意识改变、构音和吞咽困难、四肢瘫痪等为临床表现,MRI表现为基底节区、大脑半球皮质对称性DWI和T2加权高信号。结论提高髓鞘溶解症的认识对于本病的防治非常重要,缓慢纠正慢性形成的低钠血症是预防的关键。     

桥脑中央髓鞘溶解症及其临床观察

渗透性脱髓鞘综合征是由于慢性低钠血症患者血液中低渗状态迅速纠正,予以迅速补钠,造成有毒损害。渗透性脱髓鞘综合征的主要临床表现为脑桥中央髓鞘溶解症,其特点是急性麻痹、构音障碍、吞咽困难等神经系统症状。本文介绍了渗透性脱髓鞘综合征的病因和病理及临床。为提高对渗透性脱髓鞘综合征的认识和诊断水平,现对收治的2例桥脑中央髓鞘溶解症的临床资料及CT、MRI表现进行回顾性分析。     

脑桥中央髓鞘溶解症诊疗进展

脑桥中央髓鞘溶解症(CPM)是以脑桥基底部对称性脱髓鞘为病理特征的可致死性疾病,多在电解质紊乱、营养不良的疾病基础上发生[1, 2] ,因髓鞘脱失不伴有炎症反应,与常规脱髓鞘病变不同,一般将其归类于酒精中毒相关性神经系统疾病.该病的发病率尚不清楚,自1959年Adams等[2]首次描述该疾病(4例)以来,CPM的诊断和治疗已经取得了很大的进展.     

渗透性脱髓鞘综合征12例临床分析

目的：总结低钠血症患者发生渗透性脱髓鞘综合征（ ODS ）的临床、影像学特点以及预后情况。方法纳入发生ODS的低钠血症患者，收集起病前诱因、临床表现、影像学特征、治疗措施及预后情况的数据并进行总结分析。结果12例ODS患者均为重度低钠血症，且为慢性低钠血症，其中10例纠正低钠速度过快。最常见的临床表现为肢体乏力、吞咽困难以及意识障碍。头颅MR显示桥脑中央髓鞘溶解症最多见。预后方面，2例临床康复，2例病情加重死亡。结论大部分ODS患者存在典型的临床表现及影像学特征，ODS总体预后较差。     

桥脑中央髓鞘溶解症的临床分析

目的：探讨桥脑中央髓鞘溶解症的临床特征及预后,提高其疾病的确诊率和治愈率。方法：对2例患者采用电极法检测患者血清生化（K^＋、Na^＋、Ca^2＋、Cl^-）、同时行头颅MRI和TCD检查,治疗后再次检测。结果：2例患者均确诊为桥脑中央髓鞘溶解症,经治疗后症状明显改善,肌力基本恢复正常出院。结论：急性低钠血症老年患者血钠快速被纠正后,发生桥脑中央髓鞘溶解现象,短期内快速纠正急性低钠血症老年患者的血钠,可能同样易发生桥脑中央髓鞘溶解症,对急性低钠血症老年患者,补钠速度应相对缓慢。     

脑桥中央和脑桥外髓鞘溶解症的临床分析和影像学特点

目的 探讨脑桥中央和脑桥外髓鞘溶解症的临床及神经影像学特点.方法 分析5例脑桥中央髓鞘溶解症和2例脑桥外髓鞘溶解症患者的临床特点,包括基础疾病、起病前诱因、临床表现、头颅MRI特点、治疗及预后情况.结果 7例患者均有慢性形成低钠血症后被快速纠正的病史,以意识改变、构音和吞咽困难、四肢瘫痪等为临床表现.5例脑桥中央髓鞘溶解症的MRI表现为脑桥部位对称性的T1加权低信号灶、T2加权高信号灶,均呈典型"蝙蝠翅"样的改变;2例脑桥外髓鞘溶解症者分别在丘脑、基底节区域有对称性的T1加权低信号、T2加权高信号病灶.5例患者基本痊愈.结论 髓鞘溶解症的发病与低钠血症及过快补钠有关,缓慢纠正慢性形成的低钠血症是预防的关键. Abstract： Objective To investigate the clinical features and neuroimaging features of myelinolysis in central pontine and extrapontine. Methods The clinical features of 5 patients with central pontine myelinolysis and 2 cases with extrapontine myelinolysis were analyzed,including the inducing factors,clinicalmanifestations,cranial MRI,treatrnent and prognosis. Results All the seven patients had the history of rapidity of correcting chronic hyponatraemia, The common clinicalrranifestations included change of consciousness, dysphasia,dysphagia tetraplegia etc. The MRI of 5 cases of central pontine myelinolysis showed pontine parts of the symmetry T1-weighed low signal lesions,T2-weighed high signal lesions,all showed typical change like "Bat Wing". Two cases of extrapontine myelinolysis respectively in the hypothalamus、basical ganglia region of summetry T1-weighed low signal,T2-weighed high signal lesions.Five examples were basically cured. Conclusions The incidence of myelinolysis is related to hyponatremia and rapidity of correcting chronic hyponatraemia,and avoiding the rapidity of correcting chronic hyponatraemia is the key point for preventing its happening.     

脑桥外髓鞘溶解症合并缺氧性脑病1例并文献分析

脑桥外髓鞘溶解症（extrapontine myelinolysis EPM）是脑桥中央髓鞘溶解症（central pontine myelinolysis CPM）的一种特殊类型,临床比较少见,约占CMP的10％,容易误诊,现我院收治1例脑桥外髓鞘溶解症合并缺氧性脑病分析报告如下：     

多发伤伴桥脑及桥脑外髓鞘溶解症1例护理体会

桥脑中央髓鞘溶解症（CPM）是一种原因不明的以脑桥基底部对称性脱髓鞘病变为病理特征的致死性疾病,是一种罕见的疾病。根据发生部位的不同,分为CPM和桥脑外髓鞘溶解症（extrapontin emy elinolysis,EPM）。近十年来,随着神经影像学技术的发展,对本病有了进一步的认识,但由于其临床表现的非特异性,临床仍易误诊。2008年6月我科收住了1例全身多发伤伴脑桥中央髓鞘溶解症患者,经积极的治疗和护理,基本痊愈,恢复独立生活能力,住院122d,现将护理体会报告如下。     

Treating hyponatremia in an empty sella syndrome patient complicated with possible myelinolysis

Hyponatremia as the presenting manifestation of empty sella syndrome is rare. There is little clinical experience in the management of this problem and its possible therapeutic complications. We herein report on a 44-year-old woman with a past history of massive postpartum hemorrhage who was admitted because of hyponatremia and disturbed consciousness. Initial biochemical data suggested the effects of antidiuretic hormone, but fluid restriction alone offered limited benefit. Later, hormonal levels indicated hypopituitarism. Magnetic resonance imaging and cisternography led to a diagnosis of empty sella. Although glucocorticoid substitution was initiated and the clinical condition initially improved, possible myelinolysis subsequently became a complication. With early recognition and immediate replacement of hypotonic fluid, the patient completely recovered. We report this case to illustrate the fact that glucocorticoid substitution and concurrent fluid restriction can probably lead to myelinolysis in empty sella syndrome patients. We suggest that the serum sodium level should be frequently monitored and that much more attention should be paid to the neurologic signs when substituting glucocorticoids in these patients, even though the increment in the serum sodium level is acceptable. Once possible myelinolysis develops, early recognition is critical, and the immediate replacement of hypotonic fluid is suggested.     

急性肾上腺皮质功能减退症伴低钠血症临床分析

目的探讨原发或继发性急性肾上腺皮质功能减退症伴低钠血症的发生和防治。方法收集1970~2006年间急性肾上腺皮质功能减退症伴低钠血症患者的病史资料共48例,分为脑水肿组(n=23)和非脑水肿组(n=25)。回顾两组患者病因和诱因、临床症状、实验室指标以及治疗和预后等情况。分别以慢性肾上腺皮质功能减退症患者(n=48)和健康体检者(n=48)作为慢性疾病对照组和正常对照组。结果48例急性肾上腺皮质功能减退症伴低钠血症的最常见诱因为感染;脑水肿组和非脑水肿组的血钠和尿皮质醇水平均明显低于慢性疾病对照组和正常对照组(P<0.01和P<0.05);48例中死亡7例,均伴休克。脑水肿组中16例使用生理盐水,7例使用3%高渗盐水(血钠<110 mmol/L);非脑水肿组25例患者中5例使用高渗盐水治疗(血钠均在110 mmol/L左右),其中4例发生中心性桥脑脱髓鞘。结论当急性肾上腺皮质功能减退症的低钠血症伴脑水肿时,为迅速提高血浆渗透压及改善脑水肿可酌情使用高渗盐水;无脑水肿则提示脑细胞存在自身代偿,应以治疗基础疾病(激素替代)为主而慎用高渗盐水,以免引起中心性桥脑脱髓鞘。     

关于“渗透性脱髓鞘综合征”的临床进展——肝移植术后并发症机理的探讨

脑桥中央髓鞘溶解症(central pontine myelinolysis,CPM)自1970年代后期被联系于低血钠的快速纠正,约10年后又拓展至"渗透性脱髓鞘综合征(osmotic demyelination syndrome,ODS)[1]",及至2008年个案文献甚至提出了"可能的周围神经ODS[2]"。但不少迹象提示,诸如后述的问题或许尚无满意答案:CPM最可能的病因是什么?文献肝移植术后或严重呕吐相关的CPM或"ODS"预后极差之原因为何,如何优化其围手术期处理、如何优化重度低钠血症的治疗原则、改善预后?"ODS"累及的主要部位为何?病理机制为何?如何在纠正血钠时避免之?等等。兹对早期的、经典的或有一定代表性的病例文献的具体资料进行重新分析及商榷;在此基础上,对上述诸问题进行初步的探索和反思。     

老年颅脑损伤后并发低钠血症的治疗体会

目的通过总结老年颅脑损伤后并发低钠血症患者的治疗体会,探讨老年患者颅脑损伤后并发低钠血症的病因、处理方法及治疗效果。方法对神经外科于2009—2012年所收治的36例老年颅脑损伤后治疗过程中并发低钠血症患者的病因、发病机制、临床表现、治疗过程等临床资料进行回顾性分析。结果在36名患者中,其中按低钠程度分类有轻度低钠血症16例,中度低钠血症15例,重度低钠血症5例;按病因分类有营养性低钠血症22例,抗利尿激素分泌异常综合征（SIADH）7例,脑性盐耗综合征（CSWS）5例,中枢性尿崩2例;根据不同病因给予相应治疗方案治疗后,其中30例患者血钠于1～2周内逐渐恢复正常,3例患者低血钠经纠正后又反复出现低钠血症持续约一个半月才恢复,另3例患者低钠血症纠正但死于原发脑损伤过重或其他并发症。结论低钠血症是老年颅脑损伤后患者的常见并发症,是脑外伤后加重脑水肿和神经功能损害的重要原因,在对老年颅脑损伤患者的治疗过程中应密切监测血钠浓度,及时发现低钠血症并鉴别其类型,同时根据低钠血症的发生机制及类型,采取不同的针对性的治疗方法,并且积极治疗控制原发病,均能及时纠正低钠血症,显著改善患者的预后。     

Therapy with hypertonic saline in combination with anti-convulsants for hyponatremia-induced seizure: a case report and review of the literature

Seizures are an uncommon but serious complication of hyponatremia which can lead to permanent brain damage and even death. It is recommended that patients with hyponatremic-induced seizures be treated with 3% hypertonic saline, however, a rapid rate of correction may result in central pontine myelinolysis (CPM), a severe neurological disorder characterized by mutism, dysarthria, spastic quadriparesis, and pseudobulbar palsy. The patient in this case developed a hyponatremia-induced generalized tonic-clonic seizure which was aborted by rapid therapy with diazepam, followed by hypertonic saline and phenytoin. Subsequent replacement of hypertonic saline with normal saline and salt tabs in combination with phenytoin allowed gradual correction of serum sodium without any subsequent seizures or neurological complications.     

肝移植术后严重中枢神经系统并发症的病因探讨

目的 探讨肝移植术后早期严重中枢神经系统并发症的种类、发病原因及防治措施.方法 回顾性分析2001年7月至2006年7月实施的347例肝移植患者临床资料,比较分析肝移植后早期无神经系统并发症和有严重中枢神经系统并发症两组与原发疾病、血钠、血镁、血浆渗透压、凝血功能等的关系.结果 347例肝脏移植患者中71例(20.46%)出现与移植相关的精神神经系统并发症,其中6例(1.73%)出现严重中枢神经系统并发症,包括脑水肿、脑出血、脑桥中央髓鞘溶解症(central pontine myelinolysis,CPM),其中5例死亡.肝硬化失代偿、脾亢的患者术后严重中枢神经系统并发症发病率高.与无神经系统并发症组比较,脑出血患者术前均有脾亢、凝血功能不良明显;CPM和脑水肿患者移植前均存在严重的顽固性、慢性低钠血症,移植术前后48 h内血钠及血浆渗透压变化明显.结论 肝移植受体术后严重中枢神经系统并发症的发生与原发疾病有关,并可能与术前慢性低钠血症、围手术期血钠和血浆渗透压大幅波动、凝血功能未及时纠正有关.     

Clinics in diagnostic imaging (45). Osmotic myelinolysis ( central potine myelinolysis)

A 65-year-old woman developed progressive neurological deterioration following rapid correction of hyponatremia. Magnetic resonance imaging showed T2 hyperintense areas in the central pons, basal ganglia and deep white matter, typical of osmotic myelinolysis (OM). Previously thought to be uniformly fatal, there are increasing reports of non-fatal cases of OM. The recognition and understanding of this entity is important to prevent or reduce the incidence of its occurrence, as there is no specific treatment once it develops. The clinical and radiological features of OM are reviewed.     

Eight cases of Legionnaires' disease.

Eight patients with Legionnaires' disease were seen at one hospital in the summer of 1979. They presented in the same 12-day period with an illness of rapid onset characterized by fever, chills, malaise, profuse sweating and neurologic symptoms. Neutrophilia, a high erythrocyte sedimentation rate, proteinuria, hypoalbuminemia, hyponatremia, hypochloremia and abnormal liver enzyme levels in the serum were usually noted. The roentgenographic findings in the lungs ranged from segmental interstitial infiltration to panlobar pneumonia. Seven patients responded to erythromycin treatment, though one died suddenly, presumably of unrelated cardiac disease. The other patient died of a combination of renal and respiratory failure, with pulmonary edema.     

Self-induced water intoxication in a schizophrenic patient.

Water intoxication occurred in a 53-year-old woman with chronic simple schizophrenia and poorly controlled diabetes. For several years she had had a compulsive habit of drinking excessive amounts of water. Coma, fever, convulsions and other neurologic signs appeared suddenly, and she had severe hyponatremia. Her condition improved rapidly when the electrolyte abnormality was corrected.