双语病例——韦尼克脑病
朗读老师：冯璐霏 诸暨市中心医院
翻译老师：朱芳梅 浙江省立同德医院
审校老师：姜春雷 青岛市第九人民医院
History and CT images
病史及CT图像
History: A 44-year-old woman with a history of hypertension, gastritis, migraine, and prior heavy alcohol use presented to the emergency department (ED) with new diffuse lower-extremity weakness and confusion. The patient said she was in her usual state of health until the day prior to admission when she felt right lower-extremity weakness. She noted that her mother died recently, which coincided with worsening of her gastritis. She described excessive nausea, vomiting, and lack of appetite. On physical exam, she was noted to have nonextinguishing nystagmus in four cardinal directions and mild lower-extremity weakness, noting that lack of effort was apparent.
病史： 44岁，女性，既往高血压、胃炎、偏头痛及大量饮酒史，因下肢无力、意识模糊急诊就诊。患者自述右下肢无力入院前一天，身体一直健康。她特别提出，最近她母亲去世，恰逢自己胃炎恶化，伴有极度的恶心、呕吐和食欲减退。体格检查，她在四个基本方向有无消退性眼球震颤和轻度下肢无力。
An unenhanced head CT was performed in the ED. Shown below are axial images at three different levels. Click to enlarge.
患者进行了颅脑CT平扫检查。下面显示的是三个不同水平的轴向图像。
MR images
MR图像
An MRI of the brain and spine was performed. Shown below are axial fluid-attenuated inversion-recovery (FLAIR) and T2-weighted images at three different levels of the brain, diffusion-weighted images at two of these levels, and T1 precontrast and T1 postcontrast images at one of these levels. Click to enlarge.
患者进行了颅脑和脊柱MR成像检查。下面显示的是大脑三个不同水平的轴位 FLAIR和T2加权图像，其中两个不同水平的扩散加权图像，以及同一层面的T1增强前和增强后图像。
Findings and diagnosis
结果和诊断
Findings
结果
Head CT: Normal examination.
颅脑CT：正常。
Brain MRI: T2/FLAIR hyperintensities in the bilateral dorsomedial thalami, mammillary bodies, periaqueductal gray matter, and along the fourth ventricle/dorsal medulla, with associated subtle elevated DWI signal in these regions. There is no associated contrast enhancement.
颅脑MRI：双侧丘脑、乳头体、导水管周围和第四脑室/延髓背侧灰质，于T2/FLAIR呈高信号，DWI信号略高，增强扫描无强化。
Differential diagnosis
鉴别诊断
Leigh disease Leigh病
Metronidazole-induced encephalopathy 甲硝唑诱发的脑病
Creutzfeldt-Jakob disease 克雅氏病
Bilateral thalamic glioma 双侧丘脑胶质瘤
Artery of Percheron infarction Percheron动脉梗塞
Wernicke encephalopathy 韦尼克脑病
Diagnosis: Wernicke encephalopathy
诊断：韦尼克脑病
Discussion
讨论
Wernicke encephalopathy
韦尼克脑病
Pathophysiology
病理生理学
Wernicke encephalopathy is caused by thiamine deficiency. Thiamine is a cofactor for several enzymes in the Krebs cycle and pentose phosphate pathway. Deficiency in thiamine leads to reductions in several key substrates in the energy production pathways, causing severe metabolic imbalances. This, in turn, leads to neurological complications including neuronal cell death.
韦尼克脑病是由硫胺素缺乏引起的。硫胺素是Krebs循环和磷酸戊糖途径中几种酶的辅助因子。缺乏硫胺素会导致能量产生途径中的几个关键底物减少，从而导致严重的代谢失衡。这反过来又导致神经系统并发症，包括神经元细胞死亡。
Epidemiology
流行病学
The incidence of Wernicke encephalopathy varies by region, with higher incidences in developing countries due to vitamin deficiencies and malnutrition. In developed countries, prevalence data mainly come from autopsy studies, with rates ranging from 1% to 3% of the population.
韦尼克脑病的发病率因地区而异，由于维生素缺乏和营养不良，发展中国家的发病率较高。在发达国家，患病率数据主要来自尸检研究，比例在人口的1%到3%之间。
Clinical presentation
临床表现
The classic triad of Wernicke encephalopathy includes altered mental status, ocular motor abnormalities (ophthalmoplegia or nystagmus), and ataxia. Other features include delirium, hypotension, fatigue, irritability, and abdominal discomfort. Classically, Wernicke encephalopathy is seen in the context of severe alcohol abuse; however, numerous other important causes have been recognized, including but not limited to severe malnutrition, gastrointestinal surgery, dialysis, hyperemesis gravidarum, recurrent vomiting/diarrhea, prolonged parenteral nutrition, malignancies, immunodeficiency syndromes, liver disease, hyperthyroidism, and severe anorexia nervosa.
韦尼克脑病的经典三联征包括精神状态改变、眼球运动异常（眼肌麻痹或眼球震颤）和共济失调。其他特征包括谵妄、低血压、疲劳、易怒和腹部不适。以前观点认为韦尼克脑病见于严重酗酒的情况。然而，已经认识到许多其他重要原因，包括但不限于严重营养不良、胃肠手术、透析、妊娠剧吐、反复呕吐/腹泻、长期肠外营养、恶性肿瘤、免疫缺陷综合征、肝病、甲状腺功能亢进和严重的神经性厌食症。
Imaging features
成像特点
CT is not sensitive for the detection of Wernicke encephalopathy and usually appears normal. MRI is the preferred imaging modality and typically shows edematous/inflammatory changes characterized by increased T2/FLAIR signal in the mammillary bodies, periaqueductal and periventricular gray matter, collicular bodies, and thalamus. Atrophy of these brain structures can be seen as well. Contrast enhancement and restricted diffusion in these regions can be seen but is not a requisite for diagnosis. When contrast enhancement is present, it is most commonly seen in the mammillary bodies.
CT对韦尼克脑病的检测不敏感，通常显示正常。MRI是首选的成像方式，通常显示以乳头体、导水管周围和脑室周围灰质、上下丘、丘脑T2/FLAIR高信号为特征的水肿/炎症变化。也可以看到这些大脑结构的萎缩。还可以看到这些区域的强化和弥散受限，但这不是诊断的必要条件。增强后强化最常见于乳头体。
Treatment
治疗
Although it is potentially life-threatening, Wernicke encephalopathy is considered reversible with the prompt administration of parenteral thiamine. It is important that thiamine be administered before or together with glucose solutions, as glucose oxidation can lead to a transient decrease in thiamine levels and worsening of symptoms. Because thiamine deficiency often occurs in the setting of other nutritional deficiencies, magnesium and other vitamins and metabolites are frequently administered.
虽然它可能会危及生命，但韦尼克脑病被认为可以通过胃肠外硫胺素的迅速给药来逆转。硫胺素在葡萄糖溶液之前或与葡萄糖溶液一起给药很重要，因为葡萄糖氧化会导致硫胺素水平暂时降低和症状恶化。因为硫胺素缺乏经常伴发其他营养缺乏，所以患者需要经常服用镁剂和其他维生素。