Wernicke脑病综合征10例临床分析

目的:探讨Wernicke脑病(WE)的病因、临床表现特点、MRI表现及预后。方法:对10例WE患者的病因、临床表现、头颅MRI特点和预后进行了回顾性分析。结果:WE病因多与维生素B1缺乏有关,主要临床表现为眼肌麻痹,共济失调、精神异常三联症,但也可以表现为周围神经病、言语障碍等。头颅MRI有特异性部位的异常信号改变。及时予以维生素B1治疗者预后多良好。结论:提高临床对WE的认识,有利于早诊断、早干预,有助于改善WE的预后。     

Wernicke脑病的罕见临床表现(附1例报告)

Wernicke脑病(Wernicke encephalopathy,WE)是硫胺素(thiamine),即维生素B1缺乏引起的急性代谢性脑病。WE以精神意识障碍、眼肌麻痹和共济失调“三联征”为典型临床表现,但临床上“三联征”均出现的患者仅占16%,多数患者只表现其中一种或两种,19%的患者不具备其中的任何症状[1],而以罕见不典型症状为主要表现,尤其在非酒精性WE的病程早期阶段[2]。     

磁共振对Wernicke脑病的诊断价值

<正>Wernicke脑病(Wernicke,sencephalopathy,WE)最初被称为上部出血性脑脊髓灰质炎,1881年由Carl Wernicke首先发现并报道。维生素B1(硫胺素)缺乏为WE的病因。WE是少数病因明确、可完全治愈的神经科疾病之一,如能早期诊断,可迅速逆转病情,避免严重神经功能缺失甚至危及生命。本文通过分析7例WE患者的临床资料及MRI表现,     

以精神意识障碍为主要表现的非酒精性Wernicke脑病二例

Wernicke脑病(Wernicke encephalopathy,WE)在1881年由Carl Wernicke首先报道,是维生素B1缺乏引起的严重的代谢异常性脑病,最常见于慢性酒精中毒患者[1],近年来非酒精性WE报道逐渐增多[2].WE的特征性表现为精神和意识障碍、小脑性共济失调和眼球运动障碍三联征,不到16%的患者具有经典的三联征,所以WE常被误诊和漏诊[2-3].我们结合收治的2例患者探讨WE的少见病因及非典型症状.     

Wernicke脑病的临床表现及影像学特点(附1例报告)

<正>Wernicke脑病(Wernicke’s encephalopathy,WE)是1881年由Carl Wernicke首先报道,因维生素B1缺乏引起的亚急性/慢性中枢神经系统代谢性脑病~([1,2])。WE典型的临床症状为麻痹性眼征、共济失调及精神意识障碍"三联征",也可出现多发性神经炎表现,有学者归为WE"四联征"。国外流     

非酒精性 Wernicke 脑病临床特征分析

目的：探讨非酒精性Wernicke脑病（Wernicke’s encephalopathy ,WE）的病因、临床表现、影像学检查及治疗。方法回顾性分析我院住院的18例非酒精性W E患者的临床资料。结果各种原因所引起的体内维生素B1缺乏均可导致非酒精性WE的发生;临床可表现为精神及意识障碍、眼外肌麻痹和共济失调三联征,但典型的 WE 三联征仅有3例（16.7％）,其余往往以其中1种或2种表现为主,精神及意识障碍最常见（77.8％）,而眼外肌麻痹（33.3％）和共济失调（27.8％）相对较少;影像学检查以头颅M RI为首选,病灶多对称性分布于脑中线结构附近,最常见累及部位是丘脑内侧、第三脑室周围（61.1％）、中脑导水管周围区域（55.6％）、乳头体（38.9％）;18例患者经及时的大剂量补充维生素B1,临床表现均有不同程度的改善。结论各种引起维生素B1缺乏的因素均可为W E的病因,识别不典型的临床表现及特征性头颅M RI改变,对WE早期诊断及治疗具有重要意义。     

淡漠型甲状腺功能亢进合并Wernicke脑病1例报告

正Wernicke脑病(WE)是维生素B1缺乏所致的代谢性脑病,急性WE死亡率17%[1],但如果早期诊断,疗效较好[2]。神经病学会欧洲联盟指南指出WE不是少见,而是诊断得太少;尸检病例中WE占0.4%~0.8%,68%酒精性WE被漏诊,94%非酒精性WE被漏诊[3]。本文报告1例非酒精性WE,以提高对该病的认识。1病例患者女性,74岁,农民。以"头晕伴走路不稳6个     

非酒精性Wernicke脑病1例并文献复习

Wemicke脑病(Wernicke encephalopathy,WE)是一种维生素B1(硫胺)缺乏引起的代谢异常性脑病,常见于慢性酒精中毒和妊娠剧吐者,近年来,各种原因所致的营养不良和胃肠外营养导致非酒精性Wemicke脑病的发病率明显升高[1]。早期诊断、及时给予大剂量维生素B1能有效阻止疾     

Wernicke脑病的临床进展

正Wernicke脑病(Wernicke Encephalopathy,WE)是一种由维生素B1缺乏引起的中枢神经系统代谢性疾病。最常见的病因是慢性酒精中毒,但临床上许多Wernicke脑病患者并没有酒精中毒史~([1])。国外研究结果提示,该病总的生前诊断率仅为20%,多于尸检时发现。其中非酒精性WE的误诊率更高,生前诊断仅为16%~([2,3])。现对近年来WE尤其是非酒精性     

韦尼克脑病20例临床分析

目的探讨韦尼克脑病(Wernicke’s encephalopathy,WE)的病因、发病机制、临床表现及治疗,提高对韦尼克脑病的认识和早期临床诊断率,减少临床误诊率。方法对济宁医学院附属医院肾内科、消化科、产科、神经内科、胃肠外科2005-01—2015-12收治的20例(男12例,女8例,年龄21～72岁)WE患者的病史、临床特征、诊断、治疗以及预后进行分析。结果饮酒为韦尼克脑病最常见的病因,临床表现多种多样,典型的临床表现为眼球运动麻痹、共济失调和意识精神障碍三大主征,但典型的WE"三联征"很少同时出现,20例患者中只有2例(10%)表现为典型的三联征。绝大多数患者经大剂量补充维生素B_1后疗效显著。结论 WE病因及临床表现复杂多样,早期诊断十分困难,颅脑MRI可作为诊断此病的重要辅助检查。及时大量补充维生素B_1可以明显改善患者预后情况。     

Wernicke's encephalopathy in patients on peritoneal dialysis or hemodialysis

Although the occurrence of Wernicke's encephalopathy (WE) in patients on dialysis is frequently alluded to, review of the literature reveals only 3 described cases. We describe 5 patients on dialysis who developed WE in the absence of alcoholism or other predisposing factors. The clinical diagnoses included uremic encephalopathy (2 patients), dysequilibrium syndrome (1), dialysis dementia (1), and brainstem hemorrhage (1). At postmortem examination, classic findings of WE were evident. The rarity of WE in patients on dialysis may in part be explained by studies indicating a genetic defect in transketolase activity. Patients on dialysis are also potentially at risk for thiamine deficiency because of anorexia, vomiting, and intravenous alimentation. Other factors altering thiamine requirements, such as glucose load or infections, may also contribute. Preventable and potentially curable, WE should be suspected in all patients on dialysis who have an unexplained neurological picture.     

Wernicke's encephalopathy associated with hemodialysis: report of two cases and review of the literature.

Two rare cases of Wernicke's encephalopathy (WE) in non-alcoholic patients on hemodialysis (HD) are reported. They presented with the clinical triad of WE (ophthalmoplegia, ataxia and disturbance of consciousness) and intravenous administration of thiamine led to complete elimination of these manifestations. Reduced plasma thiamine levels prior to the administration confirmed the diagnosis of WE. Interestingly, a reduction in plasma thiamine levels by about half was seen in one of the patients on HD, suggesting that thiamine, a water-soluble vitamin, can be depleted with HD. In the literature, nine HD-dependent patients have been reported to develop WE, seven of whom were diagnosed postmortem. Their premortem diagnoses included uremic encephalopathy, dysequilibrium syndrome and dialysis dementia, which can often complicate HD and present symptoms similar to those of WE. We therefore emphasize that WE, even though a rare complication, should be suspected in all patients on HD who present with at least one of the clinical triad of WE.     

非酒精性Wernicke脑病二例

目的探讨Wernicke脑病患者的临床和影像学特点。方法分析2例非酒精性Wernicke脑病患者的临床资料。结果 2例患者均为消化道晚期肿瘤行手术治疗、术后有营养不均衡、发病前有葡萄糖注射液补液史。2例患者均有意识障碍和典型的头颅MRI信号改变,即双侧丘脑内侧、中脑顶盖及导水管周围的高T_2信号;维生素B_1补充治疗后患者的症状有不同程度的好转。结论导致维生素B_1缺乏的高危因素、临床表现及典型的头颅MRI表现是临床上Wernicke脑病早期诊断的重要线索。早期足量维生素B_1治疗效果好。高危患者应注意预防Wernicke脑病的发生。     

Wernicke encephalopathy after gastrointestinal surgery for cancer: causes of diagnostic failure or delay

Wernicke encephalopathy (WE) is a neurological emergency due to thiamine deficiency. We aimed to identify clinical course and causes of diagnostic delay or failure of WE in a group of patients who underwent surgery for gastrointestinal tumors. A retrospective review of clinical, laboratory, neuroimaging, and therapeutic features of 10 patients with WE following abdominal surgery for cancer was carried out. Four patients died; in these subjects, diagnosis was delayed and supplementation of vitamin was absent or likely inadequate. Diagnostic delay or failure was also related to the coexistence of several medical complications at presentation masking typical symptoms of WE. In the surviving patients, outcome was influenced by promptness and type of therapy. Postoperative abdominal bleeding and number of subsequent operations may also had an effect. Postsurgical patients with gastrointestinal tumors may develop a subtle WE. The number of subsequent operations and the severity of postoperative complications may increase the risk of unrecognized WE. The disease should be suspected in postsurgical patients who have unexpected mental status changes, even under prophylactic treatment with vitamins. We suggest that prophylaxis with high doses of thiamine should be undertaken in patients with gastrointestinal tumors before surgery.     

EFNS guidelines for diagnosis,therapy and prevention of Wernicke encephalopathy

Background: Although Wernicke encephalopathy (WE) is a preventable and treatable disease it still often remains undiagnosed during life. Objectives: To create practical guidelines for diagnosis, management and prevention of the disease. Methods: We searched MEDLINE, EMBASE, LILACS, Cochrane Library. Conclusions and recommendations:

• 1 The clinical diagnosis of WE should take into account the different presentations of clinical signs between alcoholics and non alcoholics (Recommendation Level C); although prevalence is higher in alcoholics, WE should be suspected in all clinical conditions which could lead to thiamine deficiency (good practice point – GPP).
• 2 The clinical diagnosis of WE in alcoholics requires two of the following four signs; (i) dietary deficiencies (ii) eye signs, (iii) cerebellar dysfunction, and (iv) either an altered mental state or mild memory impairment (Level B).
• 3 Total thiamine in blood sample should be measured immediately before its administration (GPP).
• 4 MRI should be used to support the diagnosis of acute WE both in alcoholics and non alcoholics (Level B).
• 5 Thiamine is indicated for the treatment of suspected or manifest WE. It should be given, before any carbohydrate, 200 mg thrice daily, preferably intravenously (Level C).
• 6 The overall safety of thiamine is very good (Level B).
• 7 After bariatric surgery we recommend follow‐up of thiamine status for at least 6 months (Level B) and parenteral thiamine supplementation (GPP).
• 8 Parenteral thiamine should be given to all at‐risk subjects admitted to the Emergency Room (GPP).
• 9 Patients dying from symptoms suggesting WE should have an autopsy (GPP).

     

Wernicke脑病的临床、影像学及病理特点

目的探讨Wernicke脑病的临床、影像学及病理特点。方法回顾性分析10例Wernicke脑病患者的临床、影像学及病理资料。结果本组10例均非乙醇中毒患者,临床表现为不同程度的精神及意识障碍9例,首发症状为眩晕、恶心和呕吐6例,眼肌瘫痪5例,低血压3例,共济失调2例,严重的周围神经病变1例。5例行头颅MRI检查,3例表现为第三、四脑室及中脑导水管周围对称性的长T1长T2异常信号,2例无阳性发现。经补充维生素B1明显好转4例,死亡5例,放弃治疗1例。5例尸检脑部表现为第三、四脑室及中脑导水管周围灰质充血、水肿和点状出血。结论Wernicke脑病临床表现不典型,MRI检查可为Wernicke脑病的早期诊断提供帮助,及早补充维生素B1是治疗的关键。     

Wernicke’s encephalopathy in a patient with acute pancreatitis: unusual cortical involvement and marvelous prognosis

Wernicke's encephalopathy (WE) is a severe neurological disorder caused by thiamine deficiency. Clinically, it is most frequently observed in people with alcohol abuse. WE, however, can occur in any clinical condition associated with malnutrition or thiamine deficiency. We present the case of a 47-year-old woman with prolonged therapeutic fasting who presented with ophthalmoplegia, ataxia and deep coma. MRI showed unusual symmetric cortical abnormalities in the frontal and parietal lobes, as well as typical lesions surrounding the third ventricle and aqueduct. Although the patient entered a vegetative state, she finally regained consciousness after thiamine supplementation unexpectedly. To the best of our knowledge, it has never been reported to date that the patient with WE in a vegetative state with cortical damage shows a marvelous prognosis, which prompts us to report this case. In the present report, we highlight the role of MRI in the diagnosis of acute WE.     

中枢神经系统孤立性纤维瘤的诊断与治疗（附28例报告）

目的探讨中枢神经系统孤立性纤维瘤（cSFT）的病理学、影像学和临床特征,以及手术对其的治疗效果。方法自2003年3月至2007年2月共有28例cSFT瘤患者在我科手术治疗。对此28例cSFT患者的临床资料进行回顾性分析。结果本组cSFT患者的发病中位年龄39．5岁。cSFT起源于硬脑膜23例,非硬脑膜5例;良性肿瘤23例,非典型性5例。5例非典型性者CD34阴性表达或弱表达。磁共振T1多为等信号,T2多为混杂信号。全切21例;7例部分或次全切除,术后均行伽玛刀治疗。随访19例,全切14例无复发,5例部分切除预后不佳。结论cSFT多附着于大脑镰与小脑幕;非典型性肿瘤CD34阴性表达或弱表达;磁共振B多为混杂信号,而T1多为等信号;肿瘤全切者预后较好;未全切肿瘤者术后伽玛刀放射治疗的效果尚不明确。