Central pontine myelinolysis

This appears to be the first report of a case of central pontine myelinolysis associated with chronic alcoholism and liver cirrhosis in the United Kingdom. The pathological features and theories of aetiology are briefly discussed.     

Central pontine myelinolysis following orthotopic liver transplant: association with cyclosporine toxicity.

Central pontine myelinolysis can occur after orthotopic liver transplantation leading to high mortality and serious morbidity. In our case, central pontine myelinolysis was associated with wide fluctuations in cyclosporine levels during an episode of hypocholesterolaemia, which may have precipitated central pontine myelinolysis.     

Central pontine myelinolysis complicating hyponatraemia

A case of severe hyponatraemia with long-term neurological sequelae is presented. The patient's electrolyte disturbance was associated initially with symptoms that were consistent with a hyponatraemic encephalopathy, but with correction of the electrolyte imbalance, after a short-term improvement, dramatic deterioration occurred. The neurological symptoms and signs, and associated computed tomographic (CT) scan abnormalities, were consistent with a diagnosis of central pontine myelinolysis. The relationship of central pontine myelinolysis to hyponatraemia and its correction is discussed, and the value of CT scanning in the diagnosis of central pontine myelinolysis is demonstrated.     

Central pontine myelinolysis: clinical and MRI correlates.

Central pontine myelinolysis (CPM) is a rare condition characterised by spastic tetraparesis, pseudobulbar palsy and the ''locked-in syndrome''. It is frequently fatal. We report a patient who developed CPM secondary to profound hyponatraemia and who recovered with no disability. Serial magnetic resonance imaging (MRI) demonstrated characteristic abnormalities within the pons at the onset of the disease, whereas computerised tomography was normal. Clinical improvement was followed six months later by progressive resolution of the MRI changes, with almost complete resolution after 18 months. Clinical and MRI findings correlate early in the course of CPM but clinical recovery predates MRI improvement by several months.     

脑桥中央髓鞘溶解症(附9例分析)

目的 研究脑桥中央髓鞘溶解症(CPM)的临床特点和影像学及电生理改变。方法对1984～2003年该院诊治的9例CPM患者的临床特点及影像学、电生理表现进行回顾性分析。结果CPM的病因主要与慢性酒精中毒、营养不良、快速纠正低钠血症等有关，临床以假性球麻痹和四肢痉挛性瘫痪为典型表现．MRI扫描可见脑桥中央髓鞘脱失灶，诱发电位也可见阳性发现。结论CPM是一种相对罕见的脱髓鞘疾病，临床症状及体征是诊断CPM的基础，头部MRI是诊断该病的重要手段，诱发电位可发现早期损害。     

肝移植后脑桥中央髓鞘溶解症5例

目的总结原位肝移植术后脑桥中央髓鞘溶解症(CPM)的诊治体会。方法回顾性分析5例原位肝移植术后并发CPM的临床资料。结果4例为肝炎后肝硬化失代偿期患者,1例为重型肝炎患者。术前均有持续低血钠,原位肝移植术后48 h内血钠纠正至正常范围或高于正常。CPM症状出现时间在术后1～2周,主要临床表现有精神异常、不同程度的意识障碍、言语不清及肢体活动障碍,1例伴有抽搐。5例患者均经头颅MRI检查证实为脑桥中央髓鞘溶解症,其中2例合并脑桥外髓鞘溶解(EPM)。经积极对症支持治疗,5例患者无1例死亡,4例患者神经系统症状明显改善,1例患者恢复较差,随访5个月仍处于昏迷状态。结论肝移植后发生CPM并非罕见,它可能是多种因素共同作用的结果,头颅MRI是主要确诊依据,脑电图可协助判断预后,脑脊液无特异性改变。CPM虽预后较差,但并非不可逆。     

Central pontine myelinolysis: a rare presentation secondary to hyperglycaemia

Central pontine myelinolysis (CPM) is classically described as a demyelinating condition that results from the rapid correction of hyponatraemia. CPM has also been reported to arise from hyperglycaemia in association with concomitant acidosis, hypernatraemia and hyperosmolar syndrome. Herein, we report a rare presentation of CPM, which was purely secondary to hyperosmolar hyperglycaemia. The patient presented with ataxia and pseudobulbar affect, which evolved subacutely over a duration of two weeks. It is important to note that, in addition to acute changes in osmolality, a subacute shift secondary to hyperglycaemia may also lead to CPM.     

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。     

Behavioural changes due to pontine and extrapontine myelinolysis.

OBJECTIVE: To report a case of central and extrapontine myelinolysis with unusual behavioural manifestations that developed after slow (not more than 0.5 mmol sodium per litre per hour) correction of hyponatraemia. CLINICAL FEATURES: A 51-year-old Caucasian woman with hyponatraemia caused by psychogenic polydipsia experienced a delayed onset of behavioural changes with some ataxia, but no pyramidal signs, after correction of the hyponatraemia. INTERVENTION AND OUTCOME: The hyponatraemia was corrected with a combination of hypertonic saline and fluid restriction at the currently recommended rate of not more than 0.5 mmol of sodium per litre per hour. CONCLUSION: Central and extrapontine myelinolysis may develop after slow correction of hyponatraemia. Behavioural manifestations may be the most prominent clinical feature.     

脑桥中央髓鞘溶解症1例报道

患者 ,女 ,63岁 ,因“反复中上腹隐痛 3年 ,加重 1月”入当地医院。当时查体神经系统无明显阳性体征。患者因长期慢性胃炎导致营养不良。患者入院后第 2天大便后 ,出现四肢无力 ,进行性加重 ,初尚能言语 ,后发展致四肢迟缓性瘫痪 ,不能言语 ,精神科会诊 ,诊断为“亚木僵状态” ,头颅ＭＲＩ提示脑桥基底部基本对称的长Ｔ1,长Ｔ2信号 ,转入神经内科治疗 ,再次头颅ＭＲＩ检查提示脑桥基底部对称性的长Ｔ1长Ｔ2信号 ,诊断为桥脑中央髓鞘溶解症。给予营养神经 ,改善循环 ,清除自由基等治疗。出院时 ,患者构音不良 ,双下肢肌力Ⅴ级 ,右上肢为Ⅴ级 …     

Central myelinolysis in a patient with hyponatraemia

We present a case of a 50-year old man who developed mutism and a flaccid quadriparesis within 48 hours of presentation to hospital with severe hyponatraemia. A diagnosis of central pontine myelinolysis was made based on the clinical features and typical appearances on magnetic resonance imaging.     

早期应用地塞米松预防大鼠桥脑中央髓鞘溶解症的发生

目的：探讨地塞米松（DEX）对大鼠中央髓鞘溶解症（CPM）的预防作用及机理。方法：通过皮下注射长效尿崩停针和腹腔注射2．5％葡萄糖液诱导大鼠低钠血症3d，第4天腹腔注射1mol／L氯化钠液（高渗盐水）快速补钠的方法诱导大鼠CPM模型。DEX早期治疗组大鼠在注射高渗盐水同时肌注5mg／kg DEX；DEX延迟治疗组大鼠在注射高渗盐水后24h肌注5mg／kg DEX；生理盐水治疗组大鼠在注射高渗盐水同时肌注生理盐水；另设正常对照组。观察大鼠脑组织脱髓鞘病变发生情况；测定脑内伊文思兰（EB）的含量变化；Western blot印迹法测定脑内一氧化氮合酶（iNOS）的表达变化。结果：通过诱导低钠血症、快速补钠的方法成功建立了大鼠CPM模型。DEX早期治疗组、DEX延迟治疗组、生理盐水治疗组3组大鼠在快速补钠后0h时点，脑内EB含量与正常对照组无明显差异（P〉0．05）。生理盐水治疗组大鼠在快速补钠后6h，脑内EB含量比0h时点明显增加（P〈0．05），24h达高峰，同时脑内iNOS在快速补钠后3h开始表达增强，36h仍呈较强表达，脱髓鞘发生率为66．7％。DEX早期治疗组大鼠快速补钠后脑内EB含量及iN0s表达，均较同时点生理盐水治疗组明显下降，未见明显脱髓鞘病变。DEX延迟治疗组脱髓鞘病变发生率为75％，与生理盐水治疗组无明显差异（P〉0．05）。结论：早期应用DEX能够通过保护血脑屏障和抑制脑内iNOS表达，起到预防CPM的作用。     

桥脑中央髓鞘溶解症MRI表现及其在诊断中的应用

目的:探讨5例桥脑中央髓鞘溶解症(CPM)患者的MRI表现,阐明MRI在诊断CPM方面的应用,以提高其诊断水平。方法:收集5例CPM患者的主要病史和临床表现。应用Philip Achieva 3.0T超导MR,采集从后颅窝底到颅顶的T1WI、T2WI、Fiair和弥散加权像(DWI)序列。结果:CPM的MRI表现为桥脑中央受累,皮质脊髓束的轴索无损害,无占位及水肿; T1WI呈低信号,T2WI及T2 Flair呈高、稍高信号,DWI呈高信号或等信号,增强后周边轻度强化或无强化;病灶呈"凸"字形,栗形或斑片状;少数累及基底节、皮层、小脑等脑桥外结构。结论:CPM的MRI表现较具特征性,MRI检查可为CPM的早期诊断提供帮助。     

Hodgkin's lymphoma with exuberant granulomatous reaction

We report a 36-year-old woman, presented with cervical lymphadenopathy and low-grade fever. Two fine needle aspiration cytology and one excisional biopsy were performed in the referral hospital, all showed granulomatous lesions without necrosis. A tentative diagnosis of tuberculosis was made, and she started on antituberculous treatment. However, there was no clinical improvement. She presented to our institution one year after the initial diagnosis, and a new biopsy from the cervical lymph node revealed effacement of the whole node by marked non-necrotizing granulomatous reaction. However, there were scattered large cells with few classic Reed-Sternberg cells between the granulomas. Immunohistochemistry reveals strong reaction of CD15 and CD30, and negative staining for CD45RB, CD45RO, and CD20. These findings confirmed the diagnosis of Hodgkin's lymphoma with remarkable granulomatous reaction that almost masked the malignant component. She was treated with chemotherapy, and she showed an excellent response.     

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.     

Intracranial involvement in a patient with Hodgkin's lymphoma

Intracranial and intraspinal involvement is a rare complication of Hodgkin's lymphoma. Intracranial involvement is observed in 0.2 to 0.5 percent of patients with Hodgkin's lymphoma. No specific risk factors associated with intracranial involvement have been found. We report intracranial involvement of Hodgkin's lymphoma in a patient who had previously undergone thyroidectomy due to thyroid papillary carcinoma.