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与皮质带化和颅内出血相关的严重韦尼克脑病

Severe Wernicke's Encephalopathy Associated with Cortical Ribboning and Intracranial Hemorrhage.

作者信息

DePolo Daniel, Gillen Stefan, Marden Kyle, Rajagopalan Swarna, Thon Olga R, Siegler James E, Thon Jesse M

机构信息

Cooper Medical School of Rowan University, Camden, NJ, USA.

Cooper Neurological Institute, Cooper University Hospital, Camden, NJ, USA.

出版信息

Neurohospitalist. 2022 Oct;12(4):702-705. doi: 10.1177/19418744221098385. Epub 2022 Jul 5.

Abstract

Wernicke's encephalopathy (WE) is a neurological emergency that results from thiamine deficiency. It is most commonly associated with chronic alcohol consumption but can result from any cause of impaired thiamine absorption or dietary intake. The classic triad of ophthalmoparesis, ataxia, and altered sensorium is rarely seen in toto, and while certain radiographic findings strongly correlate with the disease, one should have a low threshold to suspect (and promptly treat) patients in order to mitigate the risk of morbidity and mortality. However, atypical presentations can result in delayed or missed diagnoses. In this report, we describe a case of severe non-alcoholic WE associated with atypical brain Magnetic resonance imaging (MRI) manifestations of both cortical diffusion restriction and intracranial hemorrhage, which have previously been associated with poor outcomes. Early treatment with high-dose parenteral thiamine resulted in rapid improvement in ocular motility and reversal of MRI abnormalities, and on long-term follow up, the patient had made a marked functional improvement. This case highlights the importance of recognizing these unusual imaging features of WE in a patient with a compatible clinical syndrome in order to make a timely diagnosis and initiate treatment, as there is potential for a good clinical outcome despite these imaging findings.

摘要

韦尼克脑病(WE)是一种由硫胺素缺乏引起的神经急症。它最常与慢性酒精摄入有关,但也可由任何导致硫胺素吸收受损或饮食摄入不足的原因引起。经典的眼肌麻痹、共济失调和意识改变三联征很少完全出现,虽然某些影像学表现与该疾病密切相关,但为了降低发病和死亡风险,对于疑似患者(并及时治疗)应保持较低的诊断阈值。然而,非典型表现可能导致诊断延迟或漏诊。在本报告中,我们描述了一例严重的非酒精性韦尼克脑病病例,其伴有皮质扩散受限和颅内出血的非典型脑磁共振成像(MRI)表现,这些表现之前与不良预后相关。早期大剂量肠外给予硫胺素治疗使眼球运动迅速改善,MRI异常逆转,长期随访显示患者功能有显著改善。该病例强调了在具有相符临床综合征的患者中识别韦尼克脑病这些不寻常影像学特征以进行及时诊断和启动治疗的重要性,因为尽管有这些影像学表现,但仍有可能获得良好的临床结局。

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本文引用的文献

1
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Thiamine Deficiency Presenting as Intraventricular Hemorrhage.以脑室内出血为表现的硫胺素缺乏症
Stroke. 2016 Jun;47(6):e95-7. doi: 10.1161/STROKEAHA.116.013048. Epub 2016 May 10.
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