朗讀老師：馮璐霏 諸暨市中心醫(yī)院
翻譯老師：朱芳梅 浙江省立同德醫(yī)院
審校老師：姜春雷 青島市第九人民醫(yī)院
History and CT images
病史及CT圖像
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歲，女性，既往高血壓、胃炎、偏頭痛及大量飲酒史，因下肢無(wú)力、意識(shí)模糊急診就診。患者自述右下肢無(wú)力入院前一天，身體一直健康。她特別提出，最近她母親去世，恰逢自己胃炎惡化，伴有極度的惡心、嘔吐和食欲減退。體格檢查，她在四個(gè)基本方向有無(wú)消退性眼球震顫和輕度下肢無(wú)力。
An unenhanced head CT was performed in the ED. Shown below are axial images at three different levels. Click to enlarge.
患者進(jìn)行了顱腦CT平掃檢查。下面顯示的是三個(gè)不同水平的軸向圖像。
MR圖像
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.
患者進(jìn)行了顱腦和脊柱MR成像檢查。下面顯示的是大腦三個(gè)不同水平的軸位 FLAIR和T2加權(quán)圖像，其中兩個(gè)不同水平的擴(kuò)散加權(quán)圖像，以及同一層面的T1增強(qiáng)前和增強(qiáng)后圖像。
結(jié)果和診斷
Findings
結(jié)果
Head CT: Normal examination.
顱腦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.
顱腦MRI：雙側(cè)丘腦、乳頭體、導(dǎo)水管周圍和第四腦室/延髓背側(cè)灰質(zhì)，于T2/FLAIR呈高信號(hào)，DWI信號(hào)略高，增強(qiáng)掃描無(wú)強(qiáng)化。
Differential diagnosis
鑒別診斷
Leigh disease Leigh病
Metronidazole-induced encephalopathy 甲硝唑誘發(fā)的腦病
Creutzfeldt-Jakob disease 克雅氏病
Bilateral thalamic glioma 雙側(cè)丘腦膠質(zhì)瘤
Artery of Percheron infarction Percheron動(dòng)脈梗塞
Wernicke encephalopathy 韋尼克腦病
Diagnosis: Wernicke encephalopathy
診斷：韋尼克腦病
討論
Wernicke encephalopathy
韋尼克腦病
Pathophysiology
病理生理學(xué)
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.
韋尼克腦病是由硫胺素缺乏引起的。硫胺素是Krebs循環(huán)和磷酸戊糖途徑中幾種酶的輔助因子。缺乏硫胺素會(huì)導(dǎo)致能量產(chǎn)生途徑中的幾個(gè)關(guān)鍵底物減少，從而導(dǎo)致嚴(yán)重的代謝失衡。這反過(guò)來(lái)又導(dǎo)致神經(jīng)系統(tǒng)并發(fā)癥，包括神經(jīng)元細(xì)胞死亡。
Epidemiology
流行病學(xué)
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.
韋尼克腦病的發(fā)病率因地區(qū)而異，由于維生素缺乏和營(yíng)養(yǎng)不良，發(fā)展中國(guó)家的發(fā)病率較高。在發(fā)達(dá)國(guó)家，患病率數(shù)據(jù)主要來(lái)自尸檢研究，比例在人口的1%到3%之間。
Clinical presentation
臨床表現(xiàn)
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.
韋尼克腦病的經(jīng)典三聯(lián)征包括精神狀態(tài)改變、眼球運(yùn)動(dòng)異常（眼肌麻痹或眼球震顫）和共濟(jì)失調(diào)。其他特征包括譫妄、低血壓、疲勞、易怒和腹部不適。以前觀點(diǎn)認(rèn)為韋尼克腦病見于嚴(yán)重酗酒的情況。然而，已經(jīng)認(rèn)識(shí)到許多其他重要原因，包括但不限于嚴(yán)重營(yíng)養(yǎng)不良、胃腸手術(shù)、透析、妊娠劇吐、反復(fù)嘔吐/腹瀉、長(zhǎng)期腸外營(yíng)養(yǎng)、惡性腫瘤、免疫缺陷綜合征、肝病、甲狀腺功能亢進(jìn)和嚴(yán)重的神經(jīng)性厭食癥。
Imaging features
成像特點(diǎn)
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對(duì)韋尼克腦病的檢測(cè)不敏感，通常顯示正常。MRI是首選的成像方式，通常顯示以乳頭體、導(dǎo)水管周圍和腦室周圍灰質(zhì)、上下丘、丘腦T2/FLAIR高信號(hào)為特征的水腫/炎癥變化。也可以看到這些大腦結(jié)構(gòu)的萎縮。還可以看到這些區(qū)域的強(qiáng)化和彌散受限，但這不是診斷的必要條件。增強(qiáng)后強(qiáng)化最常見于乳頭體。
Treatment
治療
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.
雖然它可能會(huì)危及生命，但韋尼克腦病被認(rèn)為可以通過(guò)胃腸外硫胺素的迅速給藥來(lái)逆轉(zhuǎn)。硫胺素在葡萄糖溶液之前或與葡萄糖溶液一起給藥很重要，因?yàn)槠咸烟茄趸瘯?huì)導(dǎo)致硫胺素水平暫時(shí)降低和癥狀惡化。因?yàn)榱虬匪厝狈?jīng)常伴發(fā)其他營(yíng)養(yǎng)缺乏，所以患者需要經(jīng)常服用鎂劑和其他維生素。
聯(lián)系客服
