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.     

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。     

脑桥中央及脑桥外髓鞘溶解症1例

脑桥中央及脑桥外髓鞘溶解症是常见的渗透性脱髓鞘疾病,近年来报道的病例逐渐增多,随着重症监护的发展、核磁共振的早期诊断,患者预后得到较大改善。现将解放军总医院神经内科重症监护室收治的1例脑桥中央及脑桥外髓鞘溶解症及诊治经验报道如下。     

脑桥中央髓鞘溶解症的临床和MRI特点(8例报告)

目的 探讨脑桥中央髓鞘溶解症及脑桥外髓鞘溶解的发病原因、临床特点、治疗、疾病预后及急性期、恢复期MRI影像学特点.方法 对2002年～2006年吉林大学中日联谊医院收治的8例CPM患者的病因、临床表现、MRI影像学特点及治疗转归进行回顾性分析.结果 8例患者均有基础疾病.患者表现不同程度的意识障碍、肢体瘫痪、球麻痹;其中1例无相应的临床症状.6例患者在发病早期行头部MRI检查,表现脑桥基底部对称分布的病灶;其中的3例伴有脑桥外的髓鞘溶解,病灶主要分布在丘脑、壳核;其中的2例在发病后的1个月复查头部MRI,发现脑桥病灶有不同程度的缩小,表现T1W1低信号、T2W2高信号;丘脑、壳核的病灶表现T1 W1高信号、T2W2高信号.除1例无临床症状及1例肝移植术后患者外,其余6例一经明确诊断,均立即行激素治疗.6例患者基本恢复正常,生活可以自理.结论 肝功能损伤、慢性肾功能不全的患者容易出现脑桥中央及脑桥外的髓鞘溶解症;患者激素治疗有效;头部MRI是最有效的检查方法,是确诊的重要依据;疾病恢复期丘脑、壳核病灶的短T1、长T2的影像学改变考虑与髓鞘溶解后的脂类堆积有关.     

脑桥中央髓鞘溶解症1例

1 病例 患者, 男性, 32岁.常年每日饮酒史约1市斤左右.此次因发热, 在当地进行抗炎抗病毒治疗, 于第14日患者出现复视, 复视3日后出现尿潴留, 双下肢无力.     

脑桥中央髓鞘溶解症一例

患者男,30岁,因反复发热1月,四肢无力4 d于2008年11月7日入住南海人民医院神经内科.患者入院1月前无明显诱因出现畏寒发热,最高体温38.5℃,在外院予退热、抗感染等处理,仍反复发热.入我院前4d,患者出现四肢无力,伴头痛、言语不清,并进行性加重,不能站立.既往体健,无酗病史.查:体温36.5℃,脉搏60次/min.呼吸18次/min,血压90/60 mm Hg,心肺腹无明显异常.     

脑桥中央髓鞘溶解症MRI与临床

目的：观察MRI在脑桥中央髓鞘溶解症（CPM）早期诊断中的作用。方法：报告4例CPM,对其MRI、临床诊断、治疗、预后及发病机制进行回顾和分析。结果：4例CPM均有慢性消耗疾病、电解质紊乱或营养不良的基础;具有闭锁综合征表现,MRI示脑桥基底部对称分布的T_1长T_2信号的脱髓鞘改变仅1例,桥脑与脑其他部位同时存在脱髓鞘改变3例。经缓慢矫正电解质紊乱,给激素、脱水剂等治疗,3例好转,1例死亡。结论：CPM好发于有电解质紊乱或（和）营养不良的疾病,矫正电解质紊乱宜缓慢。脑MRI对本病早期诊断是最有效的检查手段,病因和发病机制未阐明。     

脑桥中央和脑桥外髓鞘溶解症的临床与影像学特征

目的 探索脑桥中央及脑桥外髓鞘溶解症的临床表现、发病机制及诊疗方法.方法 对2例诊断为脑桥中央及脑桥外髓鞘溶解症患者的临床资料进行分析.结果 脑桥中央及脑桥外髓鞘溶解症可表现为假性延髓麻痹、四肢瘫痪和意识障碍等.头颅磁共振是确立诊断的重要手段.结论 提高对脑桥中央及脑桥外髓鞘溶解症易感因素的认识及缓慢纠正电解质紊乱将有助于预防其发生,目前无特异性治疗方法.     

脑桥中央髓鞘溶解症

目的:提高脑桥中央髓鞘溶解症(CPM)的早期诊断率。方法:报告6例经MRI证实的CPM,对其临床诊断、治疗、预后及发病机制进行回顾和分析。结果:男女各3例,平均年龄35岁。6例CPM均有慢性消耗疾病、营养不良或电解质紊乱的基础;具有闭锁综合征表现、MRI示脑桥中央病灶(长T_1、长T_2信号)仅1例,桥脑与脑其他部位同时存在髓鞘溶解病灶(本组5/6例)。经缓慢矫正电解质紊乱,给激素、脱水剂等治疗,5例好转出院,1例死亡。结论:营养不良或(和)电解质紊乱是常见的基础性疾病,矫正电解质紊乱不宜过快;脑MRI对本病早期确诊意义重大;病因和发病机制尚未完全明了。脑的某些部位对代谢紊乱或(和)电解质紊乱等可能具有特殊易感性。     

原发性干燥综合征引起脑桥中央髓鞘溶解症的临床与影像学特征分析

目的：探讨原发性干燥综合征（pSS）引起的脑桥中央髓鞘溶解综合征（CPM）的临床及影像学特征。方法：分析1例pSS引起的CPM临床资料并与国外报道的1例pSS患者进行比较。结果：pSS患者表现为吞咽困难、构音障碍及四肢瘫痪，MRI示桥脑异常信号；给予甲泼尼龙冲击冶疗后，症状明显缓解。国外报道的1例表现为四肢瘫痪和缄默症，MRI示桥脑异常信号，给予甲泼尼龙及静脉注射丙种球蛋白治疗后，症状缓解。结论：pSS引起的CPM是极罕见疾病，经免疫抑制治疗可获得良好预后。     

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 Signal Changes in Wilson's Disease: Distinct MRI Morphology and Sequential Changes with De-Coppering Therapy

BACKGROUND AND PURPOSE: Reports of central pontine myelinolysis (CPM)-like changes in Wilson's disease (WD) and its sequential changes are exceptional. The aim was to study the MRI characteristics of CPM-like changes in WD and the serial changes. METHODS: Among the 121 patients of WD, twenty (M:F:9:11, age at onset: 14.2 +/- 4.6 years) had features similar to CPM. All had progressive neuropsychiatric form of WD. All except five were on de-coppering treatment. None had acute deterioration or hepatic failure. Ten patients underwent repeat studies. RESULTS: Twenty patients with CPM-like changes manifested with characteristic phenotype of WD. Three distinct patterns of CPM-like changes were observed: (a) characteristic round shape -7, (b) "bisected" -9, and (c) "trisected" -4. Only one had signal changes suggesting extra-pontine myelinolysis. All patients had contiguous involvement of midbrain. Serial MRI evaluation in 10 patients, at mean interval period of 17.4 +/- 13.2 months, revealed complete reversal in one, partial improvement in five, and no change in three. Clinical and MRI improvement occurred pari passu, except in one. CONCLUSIONS: CPM-like changes in WD are perhaps under-recognized and are distinct from the commonly known "osmotic demyelination." It is potentially reversible similar to other MRI features of WD.     

Magnetic Resonance Imaging Detection of a Lesion Compatible With Central Pontine Myelinolysis in a Pregnant Patient With Recurrent Vomiting and Confusion

The authors report a patient who presented with unexplained confusion. She was 15 weeks pregnant and had been having recurrent vomiting for several weeks. This was felt to possibly represent hyperemesis gravidarum, but she had several other possible contributing factors. Her serum sodium was 146 mmol/L, and her potassium was 2.6 mmol/L. She was alert but had disorientation, visual hallucinations, memory impairment, and confabulation despite being a college graduate with no history of illicit drug use or excessive alcohol consumption. Her initial magnetic resonance imaging (MRI) brain scan was interpreted as being normal. However, her follow-up MRI brain scan revealed typical findings of central pontine myelinolysis, which correlated with hyperreflexia and positive Babinski reflexes. This patient illustrates the constellation of signs and symptoms that can be seen with a demyelinating lesion of the pons. In addition, our case illustrates how this MRI scan finding can be quite nonspecific but may help to explain the clinical findings.     

Extrapontine myelinolysis after surgical removal of a pituitary tumour

Salvesen R. Extrapontine myelinolysis after surgical removal of a pituitary tumour.
Acta Neurol Scand 1998: 98: 213–215. Munksgaard 1998.
Extrapontine myelinolysis is a rare and serious disorder characterized by patches of demyelination in certain areas of the brain. Common sites of involvement are the basal ganglia and the thalamus. The patient most often presents with behavioural abnormalities, including mutism, and extrapyramidal symptoms and signs. The diagnosis is established by magnetic resonance imaging. It usually carries a grave prognosis. The pathogenesis seems to be related to profound hyponatremia that is corrected with infusions of saline. We report a case that underwent surgical removal of a pituitary tumour and subsequently was treated with desmopressin. Probably due to an overdose of this antidiuretic hormone, she became obtunded and was found to have profound hyponatremia. This was corrected with infusions of saline, mostly isotonic. She later developed mental disorientation and mutism, and magnetic resonance imaging demonstrated myelinolysis in the basal ganglia. In the course of several months, she has made some recovery, though still demonstrating some memory deficit.     

Neuroimaging and Neurologic Complications after Organ Transplantation

Neurologic complications are common after transplantation and affect 30-60% of transplant recipients. The etiology of most of the posttransplant neurologic disorders is related to the opportunistic infections, both systemic and involving central nervous system (CNS), toxicity of immunosuppressive medications, and the metabolic insult created by the underlying primary disease and the transplant procedure. Neuroimaging studies are one of the key tools in the evaluation and enable early diagnosis of neurologic complications in transplant patients, especially posterior reversible leukoencephalopathy syndrome, central pontine myelinolysis, intracerebral hemorrhage, and fungal and bacterial abscesses. Magnetic resonance imaging (MRI) is the preferred technique, but each of the available neuroimaging techniques offers a unique insight into the pathophysiologic mechanisms underlying neurologic complications of transplantation. The role of neuroimaging in this population includes early detection of calcineurin inhibitor neurotoxicity, opportunistic infections, neoplasia, metabolic disorders, or cerebrovascular diseases. In addition, we can monitor longitudinal progression of disease and treatment response.     

Diffusion-weighted MR findings of central pontine and extrapontine myelinolysis

Diffusion-weighted MR (DWI) can detect changes in water diffusion associated with cellular dysfunction, which enables the differentiation of cytotoxic edema from vasogenic edema. In this study on DWI findings in central pontine (CPM) and extrapontine myelinolysis (EPM), DWI showed high signal intensities in the bilateral pons, midbrain, and genu of the corpus callosum. The corresponding apparent diffusion coefficient values were rather low. This suggests that cytotoxic edema does in fact exist in CPM and EPM and that DWI can be useful in the rapid diagnosis and prediction of the various types of edema occurring in active demyelinating diseases.     

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.     

桥脑梗死的临床及MRI研究

目的 :探讨桥脑梗死的临床特征及影像学改变。方法 :对 46例桥脑梗死患者的临床表现及头颅MRI所示异常的解剖相关性作一分析。结果 :发现大部分桥脑梗死患者其临床表现与大脑半球基底节区的梗死相似 ,及时进行头颅MRI的检查有助于责任病灶的确定 ,而典型的桥脑综合征并不多见