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儿童和青少年韦尼克脑病。

Wernicke encephalopathy in children and adolescents.

机构信息

Division of Neurology, Children's Hospital Los Angeles, Los Angeles, California, USA.

出版信息

World J Pediatr. 2014 Nov;10(4):293-8. doi: 10.1007/s12519-014-0506-9. Epub 2014 Dec 17.

Abstract

BACKGROUND

Wernicke encephalopathy is caused by thiamine (vitamin B1) deficiency. It is generally considered to be a disease of adult alcoholics. However, it is known to occur in the pediatric population and in non-alcoholic conditions.

DATA SOURCES

We searched PubMed with the key words Wernicke, thiamine, pediatric, children and adolescents and selected publications that were deemed appropriate.

RESULTS

The global prevalence rates of hunger, poverty and resultant nutrient deprivation have decreased in the 21st century. However, several scenarios which may predispose to Wernicke encephalopathy may be increasingly prevalent in children and adolescents such as malignancies, intensive care unit stays and surgical procedures for the treatment of obesity. Other predisposing conditions include magnesium deficiency and defects in the SLC19A3 gene causing thiamine transporter-2 deficiency. The classic triad consists of encephalopathy, oculomotor dysfunction and gait ataxia but is not seen in a majority of patients. Treatment should be instituted immediately when the diagnosis is suspected clinically without waiting for laboratory confirmation. Common magnetic resonance findings include symmetric T2 hyperintensities in dorsal medial thalamus, mammillary bodies, periaqueductal gray matter, and tectal plate.

CONCLUSIONS

Wernicke encephalopathy is a medical emergency. Delay in its recognition and treatment may lead to significant morbidity, irreversible neurological damage or even death. This article aims to raise the awareness of this condition among pediatricians.

摘要

背景

Wernicke 脑病是由硫胺素(维生素 B1)缺乏引起的。它通常被认为是成年酗酒者的疾病。然而,它也已知发生在儿科人群和非酒精性疾病中。

资料来源

我们在 PubMed 上使用了关键词“Wernicke”、“硫胺素”、“儿科”、“儿童和青少年”进行搜索,并选择了认为合适的出版物。

结果

在 21 世纪,全球饥饿、贫困和由此导致的营养缺乏的发生率有所下降。然而,一些可能导致 Wernicke 脑病的情况在儿童和青少年中可能越来越普遍,例如恶性肿瘤、重症监护病房停留和治疗肥胖症的手术。其他易患疾病包括镁缺乏和 SLC19A3 基因缺陷导致的硫胺素转运体-2 缺乏。经典三联征包括脑病、眼球运动功能障碍和步态共济失调,但并非大多数患者都有。一旦临床怀疑诊断,应立即开始治疗,无需等待实验室确认。常见的磁共振成像发现包括背内侧丘脑、乳头体、导水管周围灰质和脑桥的对称 T2 高信号。

结论

Wernicke 脑病是一种医疗急症。如果不及时发现和治疗,可能会导致严重的发病率、不可逆的神经损伤甚至死亡。本文旨在提高儿科医生对这种疾病的认识。

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