Magnetic Resonance Reflects the Pathological Evolution of Wernicke Encephalopathy

BACKGROUND AND PURPOSE: Wernicke encephalopathy (WE) is an acute phase of Wernicke-Korsakoff syndrome. Pathologic findings change between acute and chronic phases. Only a few magnetic resonance imaging (MRI) studies have been done to date. METHODS: To correlate the MRI findings in acute and chronic stages of WE with the known pathologic information, 15 consecutive patients with WE were examined with MRI: 3 before thiamine treatment, 7 within 24 hours of thiamine treatment, 4 between the second and sixth day after thiamine treatment, and 1 fifty-five days after thiamine treatment. Nine of the patients had follow-up MRI between 2 days and 33 months. T1-weighted, proton, and T2-weighted axial images were obtained with additional 5-mm-thick T1-weighted sagittal and coronal images to better visualize the mammillary bodies. RESULTS: In the acute WE, MRI showed high signal intensityon T2-weighted images in periaqueduct and medial thalamic regions. In a few patients with alcoholism, vermian and mammillary body atrophies and third ventricular enlargements were noted. In the chronic phase of WE, T2 hyperintensity disappeared but mammillary bodies and cerebellar vermis became atrophic and third ventricular enlargements were evident. High signal intensity on T2-weighted images disappeared as early as 2 days, and atrophic changes appeared as early as 1 week. CONCLUSION: MRI is useful for in vivo monitoring and reflects the pathological evolution in acute and chronic phases 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。     

Diffusion-weighted imaging abnormalities in wernicke encephalopathy: reversible cytotoxic edema?

BACKGROUND: Wernicke encephalopathy (WE) is a metabolic disorder of the central nervous system resulting from vitamin B(1) deficiency. The exact mechanisms underlying the pathogenesis of the lesions in WE are not completely understood. Vitamin B1 deficiency is associated with intracellular and extracellular edema by glutamate(N-methyl-D-aspartate) receptor-mediated excitotoxicity. Conventional magnetic resonance imaging (MRI) cannot differentiate the types of edema. Diffusion-weighted imaging (DWI) has been reported to detect early ischemic damage (cytotoxic edema) as bright areas of high signal intensity (SI) and vasogenic edema as areas of heterogeneous SI. OBJECTIVES: To describe the DWI findings and to characterize the types of edema in WE using DWI. SETTING: Tertiary referral center. DESIGN AND METHODS: Two patients with WE underwent DWI and conventional MRI with gadolinium enhancement. Wernicke encephalopathy was diagnosed with salient conventional MRI findings (high SIs in the paramedian thalamus, periaqueductal gray matter, and mamillary bodies) and typical clinical history and symptoms. Apparent diffusion coefficient (ADC) values were measured in abnormal lesions by visual inspection of DWIs and T2-weighted echo planar images. RESULTS: T2-weighted and fluid-attenuated inversion recovery MRIs showed high SIs in the bilateral paramedian thalamus, mamillary bodies, and periaqueductal gray matter. The DWIs showed bright high SI in the corresponding lesions, and ADC values were decreased (patient 1: 512-545 x 10(-6)mm2/s; patient 2: 576-612 x 10(-6)mm2/s). The ADC decrease and the DWI high SI were normalized in 2 weeks with administration of thiamine hydrochloride. CONCLUSIONS: Abnormalities on DWI and ADC decrease became normalized with adequate therapy. The MRI abnormalities in WE might be owing to the "reversible cytotoxic edema" caused by vitamin B1 deficiency.     

Clinical and pathological features of alcohol-related brain damage

One of the sequelae of chronic alcohol abuse is malnutrition. Importantly, a deficiency in thiamine (vitamin B(1)) can result in the acute, potentially reversible neurological disorder Wernicke encephalopathy (WE). When WE is recognized, thiamine treatment can elicit a rapid clinical recovery. If WE is left untreated, however, patients can develop Korsakoff syndrome (KS), a severe neurological disorder characterized by anterograde amnesia. Alcohol-related brain damage (ARBD) describes the effects of chronic alcohol consumption on human brain structure and function in the absence of more discrete and well-characterized neurological concomitants of alcoholism such as WE and KS. Through knowledge of both the well-described changes in brain structure and function that are evident in alcohol-related disorders such as WE and KS and the clinical outcomes associated with these changes, researchers have begun to gain a better understanding of ARBD. This Review examines ARBD from the perspective of WE and KS, exploring the clinical presentations, postmortem brain pathology, in vivo MRI findings and potential molecular mechanisms associated with these conditions. An awareness of the consequences of chronic alcohol consumption on human behavior and brain structure can enable clinicians to improve detection and treatment of ARBD.     

非酒精性韦尼克脑病的临床与MRI影像特征

目的非酒精性韦尼克脑病(Wernicke encehalopathy,WE)易误诊,本文旨在提高对该病的认识。方法回顾性分析6例非酒精性WE患者临床及MRI特征。结果 6例患者均出现不同程度的意识障碍,其中仅2例表现为经典的三联征。6例患者均出现双侧对称性丘脑内侧、脑室及导水管周围、中脑顶盖异常信号典型表现,同时2例深昏迷患者分别表现出弥漫性皮层及面神经核受累。随访患者平均恢复时间为7.5个月,而MRI则为2.8个月。2例深昏迷患者预后较差,1例患者死亡,另1例2年后仍遗留严重四肢痉挛性瘫痪,并伴智能低下。2例深昏迷患者DWI上表现为广泛高信号。结论 MRI可为非酒精性WE提供早期诊断,而病变累及广泛皮层及颅神经核可能提示较差的预后,同时DWI序列可能有一定的预后作用。     

MRI in non-alcoholic Wernicke's encephalopathy

Wernicke's encephalopathy (WE) is a potentially reversible disorder. It is often not considered in non-alcoholic patients unless MRI demonstrates lesions in the appropriate sites. However, specific MRI sequences only highlight some areas of abnormality and hence WE may not be considered unless a more complete study is performed and this is highlighted in the case described herein. The neuropathological basis for the imaging findings is also discussed.     

35例韦尼克脑病临床分析

目的探讨韦尼克脑病(Wernicke’s encephalopathy,WE)的病因、临床表现、磁共振特征、误诊原因和治疗转归。方法回顾性分析我院2012年10月~2015年6月收治的35例WE患者的临床资料。结果饮酒是WE最常见原因,其次是胃、胆囊、胰腺病变导致呕吐和进食差。具有典型的精神意识障碍、眼肌麻痹、共济失调三联征者占11.4%,具备三联征中两项者占42.9%,仅有三联征中一项者占45.7%。头部MRI可见双侧丘脑、侧脑室周围、导水管周围、第三脑室、四脑室旁、乳头体、皮质、胼胝体等部位对称性异常信号。本组患者的误诊率达60%,其中酒精中毒性WE误诊率为54.17%,非酒精中毒性WE误诊率为72.73%。住院期间91.42%患者(32/35)好转,8.58%患者无好转。出院5 m时9例失访,随访的26例中6例死亡(死亡率23.07%),13例痊愈(50%),5例遗留记忆力障碍,2例完全卧床。结论 WE病因及临床表现多样,MRI有特征性改变,但早期误诊率高,预后与是否诊断和治疗及时密切相关。     

颅脑MRI检查对Wernicke脑病的诊断价值

目的 探讨颅脑MRI检查对Wemicke脑病(WE)的诊断价值.方法 回顾性分析8例WE患者的临床资料及MRI检查结果.结果 8例WE患者MRI示脑部T2 WI、Flair成像及弥散加权成像(DWI)有对称性异常高信号影,其中出现在丘脑内侧6例、中脑导水管周围灰质4例、第三脑室周围灰质3例、乳头体2例及壳核、视交叉、小脑上蚓部、皮质下白质各1例;2例有增强效应.2例发病早期T2 WI、Flair无异常信号影,DWI示丘脑内侧对称性异常高信号影.结论 MRI对WE具有诊断价值,DWI对WE的早期诊断价值更高.     

MRI对非乙醇中毒性韦尼克脑病的诊断价值(附五例临床分析)

目的 分析非乙醇中毒性韦尼克脑病(WE)患者的临床和颅脑MRI成像特点,探讨颅脑MRI对其的诊断价值. 方法 深圳市第二人民医院放射科自2007年6月至2010年2月应用MRI检查非乙醇中毒性WE患者5例,回顾性分析患者的临床特征、颅脑MRI成像特点及治疗转归等资料. 结果 非乙醇中毒性WE患者缺乏特征性临床表现,颅脑MRI主要表现为丘脑内侧,侧脑室,第三脑室,中脑导水管周围脑组织对称性高信号,2例患者可见大脑皮层受累.增强扫描后部分病变可见强化.2例患者死亡,3例患者应用维生素B1治疗后预后良好. 结论 颅脑MRI对非乙醇中毒性WE具有诊断价值,其显示的损害范围可反映WE的疾病严重程度.

Abstract：

Objective To analyze the clinical features and MR imaging features of patients with nonalcoholic Wemicke's encephalopathy (WE). Methods A retrospective review of the data,consisting of clinical and cranial MRI features, and the treatment results, was conducted on 5 patients with nonalcoholic WE, who admitted to our hospital fiom June 2007 to February 2010. Results The clinical features of nonalcoholic WE were non-characterized and most of them had no specific value for diagnosis. MR imaging showed symmetrical high signal in the medial thalamus, lateral ventricle, third ventricle and surrounding area of the aqueduct of midbrain; involvement of the cerebral cortex was found in 2 patients. Enhancement in some of the lesions was noted after performing contrast-enhanced scan.Favorable prognosis was given to the 3 patients treated with vitamin B1; 2 patients died. Conclusion Cerebral MRI enjoys great value in diagnosing nonalcoholic WE and reflects appropriately the pathological severity of this disease by demonstrating the scope of the lesions.     

MR Atypical Wernicke Encephalopathy Showing Extensive Brain Stem and Diencephalic Involvement

Wernicke encephalopathy (WE) is a serious neurological disorder caused by thiamine (vitamin B1) deficiency. We report a case of atypical and extensive location of abnormal signal lesions on magnetic resonance imaging (MRI) in a man with alcohol abuse with WE. MRI performed on the first hospital day showed signal intensity alterations extending in the whole brain stem and diencephalon; the mismatch between diffusion‐weighted images and apparent diffusion coefficient map was highly suggestive of vasogenic edema. This report further supports the view that WE may represent a spectrum of radiological entities and can have a wide spectrum of manifestations on MRI; thus, clinical features are essential to diagnose it.     

Proton magnetic resonance imaging in ischemic cerebrovascular disease

Proton magnetic resonance imaging (MRI) using a 0.6- or 1.5-Tesla superconductive magnet was compared with high-resolution computed tomography (CT) in 60 patients with transient ischemic attacks (TIAs) or brain infarction. MRI showed focal parenchymal changes in 84% of patients with TIAs, whereas CT showed similar changes in 42%. The sensitivity of MRI was also greater in patients with infarcts, but the difference between CT and MRI was not as great. Infarcts were usually better delineated by MRI regardless of location. However, MRI failed to reveal cortical infarcts that were clearly seen on contrast-enhanced CT scans and was unable to clearly distinguish subacute from chronic hemorrhagic infarcts. MRI changes were best detected with T2-weighted images and usually appeared as multiple areas of increased signal intensity in the subcortical and periventricular white matter. MRI changes often could not be correlated with the clinical history and neurological findings; identical changes have been seen in patients with no history of cerebrovascular disease.     

Magnetic resonance imaging findings in patients with severe neonatal indirect hyperbilirubinemia.

The aim of this study was to document the magnetic resonance imaging (MRI) findings of cases with a history of severe neonatal indirect hyperbilirubinemia. Ten cases (eight cases with neurologic findings, two normal cases) with a history of severe neonatal indirect hyperbilirubinemia were studied. Neurologic findings and MRI results were described and correlated. Seven of eight cases with neurologic findings demonstrated symmetric and uniform increased T2 signal changes limited to globus pallidi. MRI scans of two cases without neurologic findings showed no abnormality. Severe neonatal indirect hyperbilirubinemia should be considered in the differential diagnosis of bilateral symmetric hyperintense signal changes in the globus pallidus on MRI. However, high levels of unconjugated bilirubin concentrations in the neonatal period may not always cause such lesions of globus pallidus on MRI despite the presence of neurologic findings.     

Postmortem MRI and histopathology of white matter changes in Alzheimer brains. A quantitative,comparative study

To evaluate whether magnetic resonance imaging (MRI) of white matter changes in Alzheimer's disease either under- or overestimates the findings on neuropathology. Postmortem MRI and neuropathological examination were performed on 6 brains from elderly individuals with a postmortem diagnosis of AD. Using a specially designed brain slicer, the brains were cut corresponding to the MRI images, and stained by Luxol Fast Blue. Quantitative analysis of white matter changes on MRI and neuropathology was performed using stereological principles. Measures from MRI and pathology were highly correlated (r(2) = 0.71). However, pathology showed significantly more extensive changes than did MRI in all cases, with a mean of 54% larger areas. The lesions not identified with MRI represented, however, only minor changes with lower intensity of myelin staining and with an accentuation of the distance between fibres but with preserved axonal network and glial cell density.     

Wernicke脑病

Wernicke脑病（WE）是一种急性或亚急性起病的维生素B1缺乏症。表现为眼部体征、躯干性共济失调、意识障碍和情感淡漠及多发性周围神经病。M砌的表现有助于提示本病。WE是由于胃肠道手术、酗酒和胰腺炎等病因引起,补充维生素B1有助于恢复。     

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’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.     

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。     

Corpus Callosum Atrophy in Wernicke's Encephalopathy

Background and Purpose. Neuropathologic changes in Wernicke's encephalopathy (WE) involve variable brain structures. Corpus callosum involvement in WE, however, is largely unknown. The authors investigated the degree and the pattern of corpus callosum changes in WE according to the etiologies. Methods. Nineteen patients with WE (between 34 and 81 years) and 19 age‐ and sex‐matched control participants were included. The total cross‐sectional callosal area and 5 callosal subregions (C1‐C5) were measured by tracing outer margins in the midsagittal sections. Subregions were determined by placing radial dividers with 10 rays. The pixel numbers for corpus callosums were calculated, and the values obtained were adjusted for head size variations. Results. The causes of WE were alcoholism (10), intestinal surgery (5), anorexia (3), and hyperemesis gravidarum (1). The mean size of the total corpus callosum was significantly reduced in alcoholic WE (P< .001; 527.8 ± 70.8 mm 2for alcoholic WE; 664.6 ± 58.1 mm 2for the corresponding controls), but not in nonalcoholic WE. In subregion analysis, prefrontal callosum (C2) atrophy was the most prominent in alcoholic WE. In contrast, only splenium (C5) was atrophied in nonalcoholic WE. The degree of atrophy did not change throughout the follow‐up period (mean 5.3 weeks). Conclusion. This study suggests that the extent and location of corpus callosum atrophy differs between alcoholic WE and nonalcoholic WE, implying separate contribution of alcohol neurotoxicity and nutritional deficiency.     

Diffusion-weighted magnetic resonance imaging in Wernicke's encephalopathy

OBJECTIVE: To report diffusion-weighted imaging (DWI) findings and postulate the pathogenic mechanism of Wernicke's encephalopathy (WE). PATIENT: A 47-year-old-woman presented with altered consciousness, ophthalmoplegia, and ataxia. DWI revealed the abnormal signal changes in periaqueductal gray matter, mamillary bodies and bilateral medial thalami. Apparent diffusion coefficient (ADC) map revealed the high signal intensity lesions in bilateral medial thalami, suggestive of vasogenic edema. The abnormal signal intensity lesions disappeared on follow-up imaging with clinical improvement. CONCLUSIONS: Vasogenic edema plays an important role in the pathogenesis of WE and can be reversed by proper management. DWI findings in the early stage of WE may provide useful information about the prognosis.     

Restricted Diffusion of the Splenium in Acute Wernicke''s Encephalopathy

Acute Wernicke's encephalopathy (WE) is caused by profound vitamin B1 (thiamine) deficiency and commonly presents with the classic clinical triad of mental confusion, ataxia, and ophthalmoplegia. This characteristic presentation results from the propensity of acute thiamine deficiency to preferentially injure specific brain regions: the dorsomedial thalamus, periaqueductal gray, and mamillary bodies. In these regions, abnormal magnetic resonance signaling on conventional sequences has been well described; however, diffusion restriction has only recently been reported. The authors demonstrate diffusion-weighted imaging (DWI) abnormalities of the splenium of the corpus callosum in a patient with acute WE, which has not been reported previously, and suggest a potential pathological mechanism. With the recent addition of DWI, MRI is becoming more sensitive to the changes in acute WE. Furthermore, the use of apparent diffusion coefficient mapping to evaluate the extent of likely underlying cytotoxic injury may help determine long-term response to vitamin therapy and, thus, disability.