脑桥中央髓鞘溶解症和脑桥外髓鞘溶解症5例临床分析

目的 探讨脑桥中央髓鞘溶解症（CPM）和/或脑桥外髓鞘溶解症（EPM）的发病机制及早期预防和诊治原则。方法 对5例CPM/EPM患者的临床表现，影像学资料及治疗转归进行分析。结果 临床观察发现：（1）5例患者均存在严重的基础疾病，特别是严重的电解质紊乱（低钠血症），不同程度的意识障碍，吞咽困难，构音障碍。（2）3例有四肢瘫，锥体束征阳性；1例表现为帕金森综合征。（3）5例头颅MRI均阳性。（4）5例均临床好转出院，生活自理。结论（1）CPM和/或EPM的发生与低钠血症及快速纠正低钠血症有关。（2）CPM和/或EPM并非致死性疾病，无论病情多严重，均不应放弃治疗。     

脑桥中央合并脑桥外髓鞘溶解症一例

脑桥中央髓鞘溶解症(central pontine myelinolysis,CPM)是原因不明的以脑桥基底部对称性脱髓鞘为病理特征的综合征,常发生在快速纠正低钠血症之后.病灶若出现在脑内的其他部位,如双侧豆状核、尾状核、丘脑和皮层下白质等,则称为脑桥外髓鞘溶解症(extrapontine myelinolysis,EPM),其临床相对少见.现将收集的一例CPM合并EPM报道如下.     

桥脑中央髓鞘溶解症

桥脑中央髓鞘溶解症是一种少见的脱髓鞘疾病,目前的发病机制尚不清,主要与慢性酒精中毒、营养不良、过快纠正低钠血症有关,临床以假性球麻痹和四肢痉挛性瘫痪为典型的表现,头颅MRI是诊断该疾病的重要手段,尤其是功能头颅MRI对早期诊断有重要价值。临床治疗缺乏有效方法。预后与并发症发生密切相关,强直状态、构音不清和记忆力障碍是其共有的后遗症。     

中枢神经系统新型冠状病毒感染合并脑桥外髓鞘溶解症(附1例报告及文献复习)

目的 探讨中枢神经系统新型冠状病毒(新冠病毒)感染合并脑桥外髓鞘溶解症(EPM)的致病机制、临床特点及治疗方案。方法 对1例中枢神经系统新冠病毒感染合并EPM患者进行临床观察,分析临床特点,复习文献进行回顾性分析。结果 患者确诊为新冠病毒感染后脑炎有意识障碍表现,头颅MRI可见双侧基底节少许脱髓鞘,在快速纠正低钠血症后临床症状加重,复查头颅MRI可见双侧对称性基底节脱髓鞘及颞叶脑回信号异常,预后不佳。结论 新冠病毒感染脑炎及EPM发病机制需进一步研究和讨论,对于有新冠病毒感染者接触史、低钠血症、有明显意识改变及神经系统症状患者,应警惕新冠病毒感染后继发脑炎可能,合并低钠血症患者应严格把控补钠速度、严密监测血钠纠正程度,一旦确诊应及早干预。     

脑桥外髓鞘溶解症合并中央型肺癌1例

<正>脑桥外髓鞘溶解症(extrapontine myelinolysis,EPM)是一种少见的中枢神经系统脱髓鞘疾病,与脑桥中央髓鞘溶解症(central pontine myelinolysis,CPM)合称为渗透性髓鞘溶解综合征(osmotic demyelination syndrome,ODS)。ODS多发生于快速纠正缓慢形成的低钠血症的过程,可单独发生CPM或EPM,也可二者合并发生,目前其发生机制尚不完全明确。该病在临床中较为少见,重在预防,一旦发生无特效治疗。现将我院收治的1例患者报道如下。1资料患者,男,49岁,因"精神行为异常12 d"入院。15 d前     

脑桥中央髓鞘溶解症和脑桥外髓鞘溶解症5例临床分析

目的　探讨脑桥中央髓鞘溶解症 (CPM)和 /或脑桥外髓鞘溶解症 (EPM)的发病机制及早期预防和诊治原则。方法　对 5例CPM/EPM患者的临床表现、影像学资料及治疗转归进行分析。结果　临床观察发现 :(1)5例患者均存在严重的基础疾病 ,特别是严重的电解质紊乱 (低钠血症 ) ,不同程度的意识障碍 ,吞咽困难 ,构音障碍。 (2 ) 3例有四肢瘫 ,锥体束征阳性 ;1例表现为帕金森综合征。 (3) 5例头颅MRI均阳性。 (4) 5例均临床好转出院 ,生活自理。结论　 (1)CPM和 /或EPM的发生与低钠血症及快速纠正低钠血症有关。 (2 )CPM和 /或EPM并非致死性疾病 ,无论病情多严重 ,均不应放弃治疗。     

渗透性脱髓鞘综合征的临床和影像学研究(附四例报道)

目的 探讨渗透性脱髓鞘综合征的临床和神经影像特点.方法 对4例渗透性脱髓鞘综合征患者的临床演变过程、CSF、头颅CT和MRI、EEG动态变化特点、治疗及预后进行分析.结果 4例患者均存在低钠血症,纠正后出现精神意识改变、构音和吞咽困难、四肢瘫痪、肌张力障碍等症状,临床过程有双相性.EEG出现一过性的重度异常.头颅CT及CSF均未见异常.MRI特征性影像晚于临床表现10 d以后出现,4例患者首次MRI均为阴性,7～13 d后复查才显示病灶.MRI示4例患者均存在脑桥外髓鞘溶解症病灶,T1WI加权低信号,T2WI加权高信号,对称性地累及双侧尾状核、豆状核、丘脑、脑岛叶皮质、海马头部等部位,其中3例同时存在脑桥中央髓鞘溶解症改变,呈脑桥基底部位对称性T1低、T2高信号的蝶形病灶;Flair加权异常信号更清楚.3例有好转或痊愈,其中1例遗留明显肌张力障碍.结论 渗透性脱髓鞘综合征与慢性低钠血症有关,合并低血钾、低血氯时可能更易发生.治疗时应尽量避免过快纠正,临床病程具有双相性.MRI的特征性改变出现较迟,复查MRI是非常必要的.     

桥脑中央髓鞘溶解症(附3例报告)

目的：探讨桥脑中央髓鞘溶解症(CPM)的病因、临床表现和诊断。方法：回顾性分析3例经MRI证实为CPM的临床资料，并系统复习国内文献报告的12例患者的资料。结果：15例均表现为四肢瘫、延髓麻痹，8例不完全闭锁综合征，7例意识不清，3例共济失调，2例幻觉。12例发生在低血钠症背景下。14例MRI检查显示脑干脱髓鞘病灶，8例显示脑干外病灶。11例预后良好。结论：低血钠是CPM的常见原因，在脑干损害临床症状的基础上，MRI出现脑干以及脑干外的脱髓鞘病灶有助于诊断。     

渗透性脱髓鞘综合征的临床和影像学研究(附四例报道)

目的 探讨渗透性脱髓鞘综合征的临床和神经影像特点.方法 对4例渗透性脱髓鞘综合征患者的临床演变过程、CSF、头颅CT和MRI、EEG动态变化特点、治疗及预后进行分析.结果 4例患者均存在低钠血症,纠正后出现精神意识改变、构音和吞咽困难、四肢瘫痪、肌张力障碍等症状,临床过程有双相性.EEG出现一过性的重度异常.头颅CT及CSF均未见异常.MRI特征性影像晚于临床表现10 d以后出现,4例患者首次MRI均为阴性,7～13 d后复查才显示病灶.MRI示4例患者均存在脑桥外髓鞘溶解症病灶,T1WI加权低信号,T2WI加权高信号,对称性地累及双侧尾状核、豆状核、丘脑、脑岛叶皮质、海马头部等部位,其中3例同时存在脑桥中央髓鞘溶解症改变,呈脑桥基底部位对称性T1低、T2高信号的蝶形病灶;Flair加权异常信号更清楚.3例有好转或痊愈,其中1例遗留明显肌张力障碍.结论 渗透性脱髓鞘综合征与慢性低钠血症有关,合并低血钾、低血氯时可能更易发生.治疗时应尽量避免过快纠正,临床病程具有双相性.MRI的特征性改变出现较迟,复查MRI是非常必要的.     

渗透性脱髓鞘综合征的临床和影像学研究(附四例报道)

目的 探讨渗透性脱髓鞘综合征的临床和神经影像特点.方法 对4例渗透性脱髓鞘综合征患者的临床演变过程、CSF、头颅CT和MRI、EEG动态变化特点、治疗及预后进行分析.结果 4例患者均存在低钠血症,纠正后出现精神意识改变、构音和吞咽困难、四肢瘫痪、肌张力障碍等症状,临床过程有双相性.EEG出现一过性的重度异常.头颅CT及CSF均未见异常.MRI特征性影像晚于临床表现10 d以后出现,4例患者首次MRI均为阴性,7～13 d后复查才显示病灶.MRI示4例患者均存在脑桥外髓鞘溶解症病灶,T1WI加权低信号,T2WI加权高信号,对称性地累及双侧尾状核、豆状核、丘脑、脑岛叶皮质、海马头部等部位,其中3例同时存在脑桥中央髓鞘溶解症改变,呈脑桥基底部位对称性T1低、T2高信号的蝶形病灶;Flair加权异常信号更清楚.3例有好转或痊愈,其中1例遗留明显肌张力障碍.结论 渗透性脱髓鞘综合征与慢性低钠血症有关,合并低血钾、低血氯时可能更易发生.治疗时应尽量避免过快纠正,临床病程具有双相性.MRI的特征性改变出现较迟,复查MRI是非常必要的.     

Atypical forms of the osmotic demyelination syndrome

Osmotic demyelination syndrome (ODS) is the damage over the central nervous system caused by several electrolytes, metabolic and toxic disorders. We aimed to describe cases of unusual forms of ODS. In a 9-year period, 25 consecutive patients with ODS (15 men; mean age 42 years) were registered in our referral institution, among them, four (16 %) with atypical neuroimaging findings were abstracted for this communication. None of them presented cardiorespiratory arrest, head trauma, seizures, neuromyelitis optica spectrum or contact with toxic chemicals. Case 1 was a 33-year-old alcoholic man without hypertension or electrolyte imbalance, who presented a classic central pontine myelinolysis (CPM) and a hemorrhage within the pons. Case 2 was a 34-year-old alcoholic man with hypoglycemia and hyponatremia who presented CPM and diffuse bihemispheric extrapontine myelinolysis (EPM) after correction of serum sodium. Case 3 was a 52-year-old woman with mild hypokalemia and hyponatremia (inadequately corrected), who presented a peduncular and cerebellar EPM. Case 4 was a 67-year-old woman who had a suicidal attempt with antidepressants and carbamazepine without impaired consciousness, who complicated with mild hyponatremia associated with a classical CPM and a spinal cord EPM. Case 2 died and the rest remained with variable neurological impairments at last follow-up visit. With modern neuroimaging, the so-called atypical forms of ODS may not be as rare as previously thought; however, they could have a more adverse outcome than the classical ODS.     

Belly dancer's syndrome following central pontine and extrapontine myelinolysis.

We report on a woman with delayed-onset of belly dancer's syndrome 5 months after central pontine and extrapontine myelinolysis (CPM/EPM) and severe hyponatriemia. This case demonstrates that basal ganglia lesions in EPM can be the underlying pathoanatomic substrate for the rarely observed belly dancer's syndrome. The sequential appearance of extrapyramidal symptoms might reflect an ongoing but ineffective or deficient remyelination process. The presence of CPM/EPM should be considered in patients with involuntary dyskinesias of the abdominal wall.     

Overlapping features of extrapontine myelinolysis and acquired chronic (non-Wilsonian) hepatocerebral degeneration

Central pontine myelinolysis (CPM, osmotic demyelination syndrome) and acquired chronic hepatocerebral degeneration (ACHD) both occur in patients with liver failure, but are not thought to be caused by similar etiopathogenic mechanisms despite the fact that occasional patients exhibit both disorders. In our autopsy practice we have recently encountered three patients with the pontine lesions of acute or subacute osmotic demyelination syndrome, coupled with superimposed non-Wilsonian ACHD. All three patients had well-documented rapid elevations in serum sodium proximate to their demise, as well as terminal liver failure. A close intermingling and juxtaposition of lesions with severe demyelination and macrophage breakdown [thought to represent extrapontine myelinolysis (EPM)] to those with vacuolization of myelin but no cellular reaction or myelin loss (ACHD) was noted within some of the same anatomic areas. Particular overlap was seen in lesions at the cerebral cortical gray–white junction and in pencil fibers of the striatum. In these areas it was difficult to be certain whether the lesions were due to EPM or ACHD. We concluded that there was a synergism between the two disorders and raise the possibility that there may be factors common to both disorders that lead to similar anatomic sites for involvement.Presented (in abstract format) at XVIth International Congress of Neuropathology, San Francisco, Sept 10–15, 2006.     

Symptomatic hyponatraemia: can myelinolysis be prevented by treatment?

The treatment of hyponatraemia is controversial because of the risk of causing central or extrapontine myelinolysis (EPM). Rapid correction with hypertonic saline to a low normal sodium level has its proponents; others feel that slow correction to below normal sodium values is preventative. Most investigators feel that overcorrection should be avoided. It is not known whether the magnitude of serum sodium change is more important than the actual rate of correction. We present three patients with hyponatraemia ranging from 103 to 105 mmol/l who were corrected slowly with normal saline, corrected quickly with hypertonic saline, or rapidly overcorrected with hypertonic saline. All became comatose and died; all had EPM with or without central pontine myelinolysis (CPM). The rate of correction, the solution used, or the magnitude of correction did not seem to protect against demyelination. In a review of 67 reported CPM cases since 1983, no patients documented as having CPM or EPM by radiological studies or necropsy were treated with water restriction only. A group of 27 hyponatraemic patients treated only with water restriction and 35 with diuretic cessation alone did not develop CPM or EPM. This may be a reasonable approach to patients with symptomatic hyponatraemia and normal renal function.     

Central pontine and extrapontine myelinolysis: a systematic review

The purpose was to perform a systematic review of studies on central pontine and extrapontine myelinolysis [forms of osmotic demyelination syndrome (ODS)] and define the spectrum of causes, risk factors, clinical and radiological presentations, and functional outcomes of this disorder. A thorough search of the literature was conducted using multiple databases (PubMed, Ovid Medline and Google) and bibliographies of key articles to identify all case series of adult patients with ODS published from 1959 to January 2013. Only series with five or more cases published in English were considered. Of the 2602 articles identified, 38 case series were included comprising a total of 541 patients who fulfilled our inclusion criteria. The most common predisposing factor was hyponatremia (78%) and the most common presentation was encephalopathy (39%). Favorable recovery occurred in 51.9% of patients and death in 24.8%. Liver transplant patients with ODS had a combined rate of death and disability of 77.4%, compared with 44.7% in those without liver transplantation (P < 0.001). ODS is found to have a good recovery in more than half of cases and its mortality has decreased with each passing decade. Favorable prognosis is possible in patients of ODS, even with severe neurological presentation. Further research is required to confirm the differences found in liver transplant recipients.     

Central pontine myelinolysis in severely burned patients: relationship to serum hyperosmolality

The rapid correction or over-correction of hyponatremia is believed by many to be the crucial factor in the causation of central pontine myelinolysis (CPM). Over a 17-year period we found CPM in 10 (7%) of the 139 burn patients examined postmortem but in only 10 (0.28%) of the 3,528 patients in the general autopsy population (p less than 0.001). Each of the burn patients with CPM had suffered a prolonged, nonterminal episode of extreme serum hyperosmolality, whereas most burn patients without CPM had not suffered such an episode. The histologic age of the lesions correlated with the duration of time between the hyperosmolar episode and death. Hypernatremia, hyperglycemia, and azotemia, alone or combined, accounted for the hyperosmolality. No single electrolyte or metabolic derangement was essential, as in at least one burn patient with CPM the serum sodium, glucose, or blood urea nitrogen was normal during the hyperosmolar episode. Hyponatremia was not present in any burn patient with CPM. We conclude that severely burned patients, like alcoholics, are especially susceptible to CPM, and that in burn patients with CPM there is a striking association with serum hyperosmolality. The data also suggest that the rapid correction of hyponatremia exerts its effects by causing an osmotic shift and not because of any specific property of the sodium ion.     

Movement disorders and the osmotic demyelination syndrome

With the advent of MRI, osmotic demyelination syndromes (ODS) are increasingly recognised to affect varied sites in the brain in addition to the classical central pontine lesion. Striatal involvement is seen in a large proportion of cases and results in a wide variety of movement disorders. Movement disorders and cognitive problems resulting from ODS affecting the basal ganglia may occur early in the course of the illness, or may present as delayed manifestations after the patient survives the acute phase. Such delayed symptoms may evolve over time, and may even progress despite treatment. Improved survival of patients in the last few decades due to better intensive care has led to an increase in the incidence of such delayed manifestations of ODS. While the outcome of ODS is not as dismal as hitherto believed – with the acute akinetic-rigid syndrome associated with striatal myelinolysis often responding to dopaminergic therapy – the delayed symptoms often prove refractory to medical therapy. This article presents a review of the epidemiology, pathophysiology, clinical features, imaging, and therapy of movement disorders associated with involvement of the basal ganglia in ODS. A comprehensive review of 54 previously published cases of movement disorders due to ODS, and a video recording depicting the spectrum of delayed movement disorders seen after recovery from ODS are also presented.     

Morel's laminar necrosis like findings on MRI in a case of extra-pontine myelinolysis

A 39-year-old man developed disturbance of consciousness with hyponatremia during the treatment of schizophrenia in another hospital. He became alert after the correction of hyponatremia. But his consciousness deteriorated one day later in spite of normal serum sodium level, then he was referred to our hospital. The disturbance of consciousness, quadriparesis and rigidity were persisted even 4 months later. MRI (T2WI) showed well defined high intensity areas along the deep layer of the cerebral cortex and in the bilateral basal ganglia. But there were no lesions in the pons on MRI. Therefore, the diagnosis was made as extra-pontine myelinolysis (EPM) without apparent central pontine myelinolysis (CPM) according to the MRI findings. Recently, the EPM without CPM has been reported in 3 patients. Two cases were examined pathologically, findings of which were characterized by Morel's laminar necrosis at the deep layer of the cerebral cortex. But there is no report in the literature describing the detection of Morel's laminar necrosis on antemortem MRI. It seemed that the MRI findings of our case indicated Morel's laminar necrosis. Our case is suggestive in relation to the pathogenesis of EPM and CPM.