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儿童韦尼克脑病的神经影像学表现:综述。

Neuroimaging findings in pediatric Wernicke encephalopathy: a review.

机构信息

Department of Radiology, Children's Hospital of Pittsburgh of UPMC, Children's Hospital of Pittsburgh, Pittsburgh, PA 15224, USA.

出版信息

Neuroradiology. 2010 Jun;52(6):523-9. doi: 10.1007/s00234-009-0604-x. Epub 2009 Oct 21.

Abstract

Wernicke encephalopathy (WE) is an acute neurological disease resulting from dietary thiamine (vitamin B1) deficiency. WE is characterized by changes in consciousness, ocular dysfunction, and ataxia. Neuroradiologic findings usually show symmetric signal intensity alterations in the mammillary bodies, medial thalami, tectal plate, and periaqueductal area. Selective involvement of the cranial nerve nuclei, cerebellum, red nuclei, dentate nuclei, fornix, splenium, cerebral cortex, and basal ganglia characterize nonalcoholic WE patients. Furthermore, symmetric basal ganglia alterations with involvement of the putamen have only been observed in children. The incidence of WE is underestimated in both adult and pediatric patients. Interestingly, the frequency of WE in children appears to be similar to that observed in adults. The prognosis of the disease largely depends on the time from diagnosis to thiamine supplementation. The aim of this pediatric literature review is to provide an update on neuroradiologic findings in children affected by WE in an effort to determine pertinent clinical and imaging findings that can improve the detection and early identification of the disease. A thorough knowledge of the MRI findings of WE will assist in arriving at an early diagnosis, thereby reducing the morbidity and mortality associated with this disease in children.

摘要

威尼克脑病(WE)是一种由饮食中硫胺素(维生素 B1)缺乏引起的急性神经系统疾病。WE 的特征是意识改变、眼部功能障碍和共济失调。神经影像学表现通常显示在乳头体、内侧丘脑、脑桥被盖和导水管周围灰质有对称的信号强度改变。非酒精性 WE 患者的特征还包括颅神经核、小脑、红核、齿状核、穹窿、穹窿体、大脑皮质和基底节的选择性受累。此外,仅在儿童中观察到对称性基底节改变,累及壳核。WE 在成人和儿科患者中的发病率均被低估。有趣的是,儿童中 WE 的发生率似乎与成人观察到的相似。疾病的预后在很大程度上取决于从诊断到补充硫胺素的时间。本儿科文献综述的目的是提供儿童 WE 的神经影像学发现的最新信息,努力确定有助于提高疾病检测和早期识别的相关临床和影像学发现。对 WE 的 MRI 表现有透彻的了解将有助于早期诊断,从而降低儿童中与该疾病相关的发病率和死亡率。

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