Wernicke's encephalopathy

韦尼克脑病
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    目的:严格通过低血维生素B1(VB1)水平证实的Wernicke脑病(WE)的临床特征有限。这项研究旨在分析磁共振成像(MRI)的发现,和临床特征,在已确认低血VB1水平的WE患者中。
    方法:回顾了过去11年来我院收治的12例WE患者的临床和实验室记录。WE诊断是基于低血液VB1水平和至少一个经典三联征的存在而确认的。
    结果:记录了75%和50%的患者眼肌麻痹和眼球震颤,分别。12例患者中有11例出现意识障碍/记忆丧失。所有患者均出现步态障碍。12例患者中有8例在典型部位(背侧中脑[n=7],丘脑内侧[n=6],乳头体[n=5],和背桥[n=5])。在12名患者中,六个在非典型部位显示异常(call体的脾[n=4],穹窿[n=3],大脑皮层[n=2],小脑疣[n=2],和背髓质[n=1])。MRI异常阳性的患者血液VB1水平明显低于无异常的患者(9.5vs.16.0ng/mL)。
    结论:在证实WE具有低血VB1水平的病例中,call体,穹窿,与以前的研究相比,大脑皮层的参与频率更高。典型和非典型部位的MRI异常与WE中的低血液VB1水平相关,提示较低的血液VB1水平与WE患者更严重的脑损伤相关。
    OBJECTIVE: Clinical features of Wernicke\'s encephalopathy (WE) confirmed strictly through the low blood vitamin B1 (VB1) levels are limited. This study aimed to analyse magnetic resonance imaging (MRI) findings, and clinical characteristics, in patients with WE who have confirmed low blood VB1 levels.
    METHODS: Clinical and laboratory records of 12 consecutive patients with WE admitted to our hospital during the past 11 years were reviewed. The WE diagnosis was confirmed based on low blood VB1 levels and the presence of at least one of the classical triad.
    RESULTS: Ophthalmoplegia and nystagmus were recorded in 75% and 50% of the patients, respectively. Eleven of 12 patients presented with consciousness disturbance/memory loss. All patients experienced gait disturbances. Eight of the 12 patients exhibited MRI abnormalities at typical sites (the dorsal midbrain [n = 7], medial thalamus [n = 6], mammillary bodies [n = 5], and dorsal pons [n = 5]). Of the 12 patients, six showed abnormalities at atypical sites (the splenium of the corpus callosum [n = 4], fornix [n = 3], cerebral cortex [n = 2], cerebellar vermis [n = 2], and dorsal medulla [n = 1]). Patients with positive MRI abnormalities had significantly lower blood VB1 levels than those without abnormalities (9.5 vs. 16.0 ng/mL).
    CONCLUSIONS: In cases of confirmed WE with low blood VB1 levels, the corpus callosum, fornix, and cerebral cortex were more frequently involved than in previous studies. MRI abnormalities at both typical and atypical sites were correlated with low blood VB1 levels in WE, suggesting that lower blood VB1 levels are associated with more severe brain damage in patients with WE.
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  • 文章类型: Case Reports
    背景:韦尼克脑病(WE)是一种由硫胺素(维生素B1)缺乏引起的急性神经综合征。它在非酒精患者中得到了越来越多的认可,比如在营养不良的情况下。最近的文献揭示了罕见的症状和神经影像学发现。
    方法:我们报告了一例44岁男性,最初表现为双侧听力损失,并在磁共振成像(MRI)扩散加权成像序列中显示出call体的脾异常。第二天,患者出现了新的症状,包括不稳定的行走,双重视力和幻觉.随后的脑MRI显示病变涉及导水管周围灰质和双侧内侧丘脑,说明WE的诊断。静脉内硫胺素的经验性治疗可实现完整的临床和放射学分辨率。
    结论:据我们所知,本病例是WE在文献中首次报道的罕见但可逆的表现.这个案例警告我们在营养不良的神经功能缺损患者中对WE的怀疑程度更高,尽管可能出现非典型表现,包括双侧听力障碍和异常的神经放射学结果。在WE中早期诊断和及时施用硫胺素可能导致有利的结果和完全康复。
    BACKGROUND: Wernicke\'s encephalopathy (WE) is an acute neurological syndrome resulting from thiamine (vitamin B1) deficiency. It has been recognized increasingly in non-alcoholic patients, such as in the condition of malnutrition. Recent literature has shed light on uncommon symptoms and neuroimaging findings.
    METHODS: We reported a case of a 44-year-old male who initially presented with bilateral hearing loss, and exhibited abnormality in the splenium of the corpus callosum on magnetic resonance imaging (MRI) diffusion-weighted imaging sequence. On the following day the patient developed new symptoms, including unstable walking, double vision and hallucination. The subsequent brain MRI demonstrated lesions involving periaqueductal grey matter and bilateral medial thalamus, indicating the diagnosis of WE. Empirical treatment with intravenous thiamine resulted in complete clinical and radiological resolution.
    CONCLUSIONS: To the best of our knowledge, the current case is the first report of WE in literature with uncommon but reversible manifestations. This case warns us to maintain a heightened level of suspicion for WE in malnourished patients with neurological deficits, despite the possibility of atypical presentations encompassing bilateral hearing disturbances and unusual neuroradiological results. Early diagnosis and timely administration of thiamine in WE are likely to lead to a favorable outcome and full recovery.
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  • 文章类型: Journal Article
    背景:韦尼克脑病(WE)是一种严重的神经系统疾病,未被诊断。尽管临床指南有限,静脉(IV)硫胺素的标准使用未得到充分利用,仍然是值得关注的研究领域。
    目的:我们使用Medline进行了系统评价,Embase,和CENTRAL数据库,以鉴定和总结WE中IV硫胺素治疗的文献。对接受≥100mg硫胺素IV的WE患者的人体研究符合纳入标准。随机对照试验,横断面研究,并纳入病例报告。
    结果:共纳入27项研究:20例病例报告,五项回顾性研究,一项前瞻性研究和一项随机对照试验。在病例报告中,11例(55%)为女性,所有病例的平均年龄为45岁(SD=15)。其他7项研究包括688例患者;平均年龄为52岁(SD=9),266名(38.7%)为女性。在病例报告中,在16例(80%)病例中,使用神经和临床表现诊断WE.MRI用于诊断15例(75%)。在12例病例报告中报告了500mg静脉注射硫胺素TID(60%)。18例(90%)的病例报告在静脉注射硫胺素后症状部分或完全缓解。
    结论:IV硫胺素可以缓解神经系统症状,认知功能障碍,和与WE相关的脑成像病变。我们发现静脉硫胺素的证据和WE的诊断标准存在关键局限性。未来的针对性研究应为WE建立明确的诊断和治疗指南,以防止这种严重的疾病被诊断不足或治疗不足。
    Wernicke\'s encephalopathy (WE) is a serious neurological disorder that is underdiagnosed. Despite limited clinical guidelines, the standard use of intravenous (IV) thiamine is underutilized and remains an area of research deserving much attention.
    We conducted a systematic review using Medline, Embase, and CENTRAL databases to identify and summarize the literature on IV thiamine treatment in WE. Human studies with WE patients who received ≥100 mg of thiamine IV met inclusion criteria. Randomized controlled trials, cross-sectional studies, and case reports were included.
    A total of 27 studies were included: 20 case reports, five retrospective studies, one prospective study and one randomized control trial. Of the case reports, 11 (55%) cases were female, and the average age of all cases was 45 years (SD = 15). The other seven studies included 688 patients; the average age was 52 years (SD = 9), and 266 (38.7%) were female. Among the case reports, neurological and clinical findings were used to diagnose WE in 16 (80%) cases. MRI was utilized to diagnose 15 (75%) cases. 500 mg IV thiamine TID was reported in 12 case reports (60%). 18 (90%) of case reports had partial or complete resolution of symptoms following IV thiamine.
    IV thiamine can alleviate neurological symptoms, cognitive dysfunction, and brain imaging lesions associated with WE. We found key limitations in the evidence for IV thiamine and diagnostic standards for WE. Future targeted research should establish clear diagnostic and treatment guidelines for WE to prevent this serious condition from being underdiagnosed or undertreated.
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  • 文章类型: Case Reports
    视神经脊髓炎谱系障碍(NMOSD)是一种自身免疫性疾病,其特征在于视神经炎(ON)和横贯性脊髓炎的反复发作。本病例报告旨在强调在遇到MRI检测到的Wernicke脑病时考虑NMOSD的非典型表现的重要性。NMOSD的主要靶标是水通道蛋白4(AQP4)蛋白,主要位于星形胶质细胞表面。与AQP4结合的抗体可导致星形胶质细胞功能障碍和损伤,有助于NMOSD的独特病理学。相关的免疫反应和炎症会对中枢神经系统的各种组件造成继发性伤害,包括少突胶质细胞和神经元轴突。这种炎症过程导致血管周围脱髓鞘和轴突损伤,进一步加重NMOSD的神经功能缺损。在这种情况下,我们介绍了一名39岁女性,既往无病史或手术史,她因3周的进行性眼睑沉重感和嗜睡病史而寻求医疗护理.NMOSD是一种主要针对AQP4蛋白的自身免疫性疾病,导致复发性ON和横贯性脊髓炎。患者最初被误诊为重症肌无力,原因是嗜睡和上睑下垂。由于对弥漫性疲劳和双侧下垂引起的重症肌无力的担忧,患者最初接受静脉注射免疫球蛋白(IVIG)治疗,并进入神经内科.她住院的第一天,有和没有对比的MRI显示广泛,非增强T2加权液体衰减倒置恢复(T2-FLAIR)高信号围绕第三脑室并影响导水管周围灰色,内侧丘脑,和乳头体。右内侧颞叶T2-FLAIR高强度也有间隔增加,向后和向下延伸,邻接颞角。随后的CSF脑炎小组结果显示西尼罗河病毒(WNV)IgG阳性,但WNVIgM阴性,AQP4抗体阳性。鉴于AQP4抗体的高特异性,患者被诊断为视神经脊髓炎(NMO)脑炎.此病例强调了在遇到MRI检测到的Wernicke脑病时考虑NMO非典型表现的重要性。由于我们的病人主要表现为嗜睡和眼部相关症状,鉴别诊断最初未考虑NMO和Wernicke脑病。此外,尽管MRI显示Wernicke脑病,由于缺乏硫胺素缺乏症和家庭成员对饮酒的一贯否认,因此被认为可能性较小,肠胃问题,或口服摄入不足。该病例强调了在症状不典型的患者中考虑NMOSD的重要性,即使最初的陈述暗示了其他条件。及时诊断对于防止管理不善和改善患者预后至关重要。临床医生应该对NMOSD保持高度怀疑,尤其是当MRI检查结果与最初诊断不一致时,早期识别和治疗可以显著影响患者的护理和预后。
    Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune condition characterized by recurrent episodes of optic neuritis (ON) and transverse myelitis. This case report aims to highlight the importance of considering atypical presentations of NMOSD when confronted with MRI-detected Wernicke\'s encephalopathy. The primary target in NMOSD is the aquaporin-4 (AQP4) protein, predominantly located on astrocyte surfaces. Antibodies binding to AQP4 can lead to astrocyte dysfunction and damage, contributing to NMOSD\'s distinctive pathology. The associated immune response and inflammation can cause secondary harm to various components of the central nervous system, including oligodendrocytes and neuronal axons. This inflammatory process results in perivascular demyelination and axonal injury, further aggravating neurological deficits in NMOSD. In this case, we present a 39-year-old female with no prior medical or surgical history who sought medical attention due to a three-week history of progressive eyelid heaviness and somnolence. NMOSD is an autoimmune condition primarily targeting the AQP4 protein, resulting in recurrent ON and transverse myelitis. The patient was initially misdiagnosed with myasthenia gravis due to somnolence and ptosis. Due to concerns about myasthenia gravis due to diffuse fatigue and bilateral ptosis, the patient was initially treated with intravenous immunoglobulin (IVIG) and admitted to the neurology service. On the first day of her hospitalization, MRI with and without contrast revealed extensive, non-enhancing T2-weighted-fluid-attenuated inversion recovery (T2-FLAIR) hyperintensities surrounding the third ventricle and affecting the periaqueductal grey, medial thalami, and mammillary bodies. There was also an interval increase in T2-FLAIR hyperintensity within the right medial temporal lobe, extending more posteriorly and inferiorly, abutting the temporal horn. Subsequent CSF encephalitis panel results showed positive West Nile virus (WNV) IgG but negative WNV IgM, and AQP4 antibodies were positive. Given the high specificity of AQP4 antibodies, the patient was diagnosed with neuromyelitis optica (NMO) encephalitis. This case underscores the importance of considering atypical presentations of NMO when confronted with MRI-detected Wernicke\'s encephalopathy. Since our patient primarily displayed somnolence and eye-related symptoms, neither NMO nor Wernicke\'s encephalopathy were initially considered in the differential diagnosis. Furthermore, despite MRI findings suggestive of Wernicke\'s encephalopathy, it was considered less likely due to the absence of thiamine deficiency and consistent denials by family members regarding alcohol use, gastrointestinal issues, or inadequate oral intake. This case underscores the importance of considering NMOSD in patients with atypical symptoms, even when initial presentations suggest other conditions. Timely diagnosis is crucial to prevent mismanagement and improve patient outcomes. Clinicians should maintain a high level of suspicion for NMOSD, especially when MRI findings do not align with the initial diagnosis, as early recognition and treatment can significantly impact patient care and prognosis.
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  • 文章类型: Journal Article
    背景与目的维生素B1缺乏可引起多种神经精神异常,心血管,和其他系统。通过补充维生素B1,可以迅速纠正和防止这种情况发展为不可逆的后遗症。因此,早期发现和干预维生素B1缺乏至关重要。我们先前已经证明了日本农村住院的老年患者维生素B1缺乏与食欲减退之间的关联。这项研究旨在研究食欲不振和其他提示维生素B1缺乏症状的患者维生素B1缺乏的其他预测因素。材料和方法这项横断面研究涉及2020年4月至2022年3月期间在农村医院住院的519名患者。维生素B1水平的数据,年龄,性别,BMI,白蛋白水平,功能独立性度量(FIM),血红蛋白水平,Charlson合并症指数(CCI),从电子病历中收集药物。维生素B1缺乏定义为血清维生素B1水平<20µg/dL。数据使用Mann-WhitneyU检验进行分析,学生t检验,和卡方检验,其次是多因素逻辑回归,以检查维生素B1缺乏的预测因素。结果113例(21.5%)患者发现维生素B1缺乏。多因素logistic回归分析显示贫血与维生素B1缺乏显著相关[校正比值比(AOR):1.71,95%置信区间(CI):1.07~2.73,p<0.05]。结论根据我们的发现,在居住在农村地区的日本住院患者中,贫血与维生素B1缺乏显著相关.因此,医师应注意住院贫血患者维生素B1缺乏的可能性.
    Background and objective Vitamin B1 deficiency can cause a variety of abnormalities in the neuropsychiatric, cardiovascular, and other systems. This condition can be rapidly corrected and prevented from progressing to irreversible sequelae through vitamin B1 supplementation. Therefore, early detection of and intervention in vitamin B1 deficiency are essential. We have previously demonstrated an association between vitamin B1 deficiency and appetite loss in hospitalized older adult patients in rural Japan. This study aimed to examine the additional predictors of vitamin B1 deficiency in patients with appetite loss and other symptoms suggestive of vitamin B1 deficiency. Material and methods This cross-sectional study involved 519 patients admitted to a rural hospital between April 2020 and March 2022. Data on vitamin B1 levels, age, sex, BMI, albumin levels, functional independence measure (FIM), hemoglobin levels, Charlson Comorbidity Index (CCI), and medications were collected from electronic medical records. Vitamin B1 deficiency was defined as serum vitamin B1 level <20 µg/dL. Data were analyzed using the Mann-Whitney U test, Student\'s t-test, and chi-square test, followed by multivariate logistic regression to examine the predictors of vitamin B1 deficiency. Results A total of 113 patients (21.5%) were found to be vitamin B1-deficient. Multivariate logistic regression showed that anemia was significantly associated with vitamin B1 deficiency [adjusted odds ratio (AOR): 1.71, 95% confidence interval (CI): 1.07-2.73, p<0.05]. Conclusion Based on our findings, anemia is significantly associated with vitamin B1 deficiency in hospitalized Japanese patients living in rural areas. Therefore, physicians should be mindful of the possibility of vitamin B1 deficiency in hospitalized patients with anemia.
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  • 文章类型: Journal Article
    酒精使用障碍(AUD)在重症监护病房(ICU)中很普遍。酒精滥用和/或依赖,导致酒精戒断综合征(AWS),高达10%或更多。用于管理这些患者的管理策略似乎存在很大差异,促使对知识差距进行评估,并找到障碍。注意到在印度环境中缺乏这种文献,进行了一项调查,以评估印度重症监护方案中酒精依赖/滥用和AWS的识别和管理的实践模式。调查的主要受访者是以麻醉为基础专业的独立从业者,并且绝大多数在多学科ICU中实践。他们估计AUD患病率低于10%。缺乏AUD文件的最主要原因是担心拒绝保险。很少有人在评估AUD和AWS时使用风险评估工具。对AWS诊断的ICD10/DSM-V组件的认识可以忽略不计。氯二氮卓和劳拉西m用于固定或基于症状的治疗。与现有文献相比,氟哌啶醇过量使用,而巴比妥类药物很少。AWS中硫胺素的剂量和频率差异很大,没有神经系统并发症。对死亡率和发病率的影响知之甚少。总之,与国际文献相比,该调查报告的患病率较低.保险拒绝是限制适当历史记录或记录AUD的主要因素之一。酒精戒断综合征风险评估,监测,管理是可变的和次优的。AUDs所有方面的变化归因于知识差距。需要进一步的研究来弥合研究差距。
    GopaldasJA,PadyanaM,RaiPP.印度重症监护病房酒精戒断综合征诊断和管理的实践模式。印度J暴击护理中心2023;27(11):816-820。
    Alcohol use disorders (AUDs) are prevalent in intensive care units (ICUs). Alcohol abuse and/or dependence, leading to alcohol withdrawal syndrome (AWS), is as high as 10% or more. There seem to be wide variations in management strategies used to manage these patients, prompting an evaluation of the knowledge gap as well as finding the barriers. Noting lack of such literature in the Indian setting, a survey is undertaken to evaluate practice patterns surrounding the identification and management of alcohol dependence/abuse and AWS in the Indian critical care scenario. The main respondents of the survey are independent practitioners with anesthesia as their base specialty and overwhelmingly practice in multidisciplinary ICUs. They estimated AUD prevalence to be under 10%. The reason most expressed for lack of AUD documentation is fear of insurance rejection. Very few used risk assessment tool in evaluation of AUDs and AWS. Awareness of ICD 10/DSM-V components of AWS diagnosis was negligible. Chlordiazepoxide and lorazepam were used either in a fixed- or symptom-based therapy. Compared to available literature, haloperidol use is excessive, while barbiturates rarely. The wide variation is seen with the dose and frequency of thiamine in AWS without neurological complications. The impact on mortality and morbidity is poorly understood. In conclusion, the survey reported a lower prevalence compared to international literature. Insurance rejection is one of the main factors in limiting adequate history taking or documenting AUDs. Alcohol withdrawal syndrome risk assessment, monitoring, and management is variable and suboptimal. Variability in all aspects of AUDs is attributable to the knowledge gap. Further studies are needed to bridge the research gap.
    UNASSIGNED: Gopaldas JA, Padyana M, Rai PP. Practice Patterns in the Diagnosis and Management of Alcohol Withdrawal Syndrome in Indian Intensive Care Units. Indian J Crit Care Med 2023;27(11):816-820.
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  • 文章类型: Case Reports
    炎症性肠病(IBD),包括克罗恩病(CD)和溃疡性结肠炎(UC),是肠道的慢性炎症性疾病。两者都与肠道和肠道外表现(EIM)有关。EIM通常与肠道疾病活动有关,可能先于肠道症状或与肠道症状同时发展。虽然他们有据可查,CD与神经和神经肌肉受累的关联是罕见且有争议的,关于其患病率和频谱的零星和矛盾的数据。神经系统受累可影响中枢或周围神经系统,血栓性事件是最常见的并发症。韦尼克脑病(WE)是一般人群中发生的神经系统并发症之一,临床患病率为0.04%至0.13%。虽然没有IBD患者的具体数据,临床医生必须提高警惕,即使有轻微的神经系统症状,也要考虑这种情况的可能性,并在尝试进行任何生物学评估前及时服用维生素B1.及时治疗对于避免患者的不可逆损伤甚至死亡至关重要。我们在此报告了CD中WE的一个具有挑战性的案例,并讨论了文献。
    The inflammatory bowel diseases (IBD), including Crohn\'s disease (CD) and ulcerative colitis (UC), are chronic inflammatory disorders of the intestine. Both are associated with intestinal and extra-intestinal manifestations (EIM). EIM are usually related to intestinal disease activity and may precede or develop concurrently with intestinal symptoms. Although they are well documented, the association of CD with neurological and neuromuscular involvement is rare and controversial, with sporadic and contradictory data regarding its prevalence and spectrum. Neurological involvement can affect the central or peripheral nervous system, with thrombotic events being the most frequent complication. Wernicke\'s encephalopathy (WE) is one of the neurological complications that occurs in the general population with a clinical prevalence ranging from 0.04% to 0.13%. Although no specific data exists for IBD patients, it is imperative for clinicians to be vigilant and consider the possibility of this condition even with mild neurological symptoms and to administer vitamin B1 promptly before attempting any biological assessment. Timely treatment is essential to avoid irreversible damage or even the death of the patient. We report herein a challenging case of WE in CD and we discuss the literature.
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  • 文章类型: Systematic Review
    硫胺素(维生素B1)缺乏在肾脏疾病患者中相对常见。韦尼克脑病(WE)是由维生素B1缺乏引起的。我们的目的是系统地回顾肾脏疾病患者WE的体征和症状。我们对WE在肾脏疾病中的应用进行了系统的文献综述,并记录了临床和影像学特征,治疗和结果。总共审查了323份手稿,其中46例诊断为急性和慢性肾脏疾病,WE发表在37份报告中。WE的前驱特征是食欲不振,呕吐,减肥,腹痛,和腹泻。肠胃外硫胺素500毫克,每天3次,通常导致完全恢复,而Korsakoff综合征是在那些接受低剂量治疗的人中发现的。为了防止肾衰竭,我们建议对出现严重营养不良和(前驱)硫胺素缺乏症状的肾病患者给予高剂量的肠胃外硫胺素。
    Thiamine (vitamin B1) deficiency is relatively common in patients with kidney disease. Wernicke\'s encephalopathy (WE) is caused by vitamin B1 deficiency. Our aim was to systematically review the signs and symptoms of WE in patients with kidney disease. We conducted a systematic literature review on WE in kidney disease and recorded clinical and radiographic characteristics, treatment and outcome. In total 323 manuscripts were reviewed, which yielded 46 cases diagnosed with acute and chronic kidney disease and WE published in 37 reports. Prodromal characteristics of WE were loss of appetite, vomiting, weight loss, abdominal pain, and diarrhea. Parenteral thiamine 500 mg 3 times per day often led to full recovery, while Korsakoff\'s syndrome was found in those receiving low doses. To prevent WE in kidney failure, we suggest administering high doses of parenteral thiamine in patients with kidney disease who present with severe malnutrition and (prodromal) signs of thiamine deficiency.
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