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与脑炎/脑病相关的可逆性胼胝体压部病变综合征,临床表现具有高度异质性。

Reversible splenial lesion syndrome associated with encephalitis/encephalopathy presenting with great clinical heterogeneity.

作者信息

Zhu Yuanzhao, Zheng Junjun, Zhang Ling, Zeng Zhenguo, Zhu Min, Li Xiaobin, Lou Xiaoliang, Wan Hui, Hong Daojun

机构信息

Department of Neurology, the First Affiliated Hospital of Nanchang University, Yong Wai Zheng Street 17#, Nanchang, 330006, P. R. China.

Department of Critical Care Medicine, the First Affiliated Hospital of Nanchang University, Nanchang, China.

出版信息

BMC Neurol. 2016 Apr 18;16:49. doi: 10.1186/s12883-016-0572-9.

Abstract

BACKGROUND

Reversible splenial lesion syndrome (RESLES) is a disorder radiologically characterized by reversible lesion in the splenium of the corpus callosum (SCC). Most of patients with RESLES associated with encephalitis/encephalopathy were identified in Japanese population, but almost no Chinese patients were diagnosed as RESLES associated with encephalitis/encephalopathy.

METHODS

Possible patients with reversible isolated SCC lesions were retrieved from January 2012 to July 2015 using keyword "restricted diffusion and isolated SCC lesion" in MRI report system from a large academic center. The clinical, laboratory and radiological data were summarized.

RESULTS

A total of 15 encephalitis/encephalopathy patients (9 males and 6 females) were identified with a reversible isolated SCC lesion. Except for 13 patients with fever symptom, 8 patients also had cold symptoms before the onset of neurological symptoms. The neurological symptoms included headache, vertigo, seizure, disturbance of consciousness, and delirious behavior. Thirteen patients completely recovered within 1 month, but 2 patients who were subjected to mechanical ventilation had persistent neurological deficits. The initial MRI features showed isolated ovoid or extending SCC lesions with homogeneous hyperintense on diffusion weighted imaging (DWI) and decreased apparent diffusion coefficient (ADC) values. The follow-up MRI revealed that isolated SCC lesions with diffuse restriction disappeared at 10 to 32 days after the initial MRI study. Fractional anisotropy map revealed the decreased value of SCC lesion in a severe case with poor prognosis.

CONCLUSIONS

RESLES associated with encephalitis/encephalopathy is a reversible syndrome with an excellent prognosis in most patients, while a few patients required ventilator supporting at the early stage might have severe neurological sequelae. Reversible signal changes on DWI and ADC are identified in all patients, but fractional anisotropy values can be decreased in severe patient with neurological sequelae.

摘要

背景

可逆性胼胝体压部病变综合征(RESLES)是一种影像学表现为胼胝体压部(SCC)可逆性病变的疾病。大多数与脑炎/脑病相关的RESLES患者在日本人群中被发现,但几乎没有中国患者被诊断为与脑炎/脑病相关的RESLES。

方法

2012年1月至2015年7月期间,在一家大型学术中心的MRI报告系统中,使用关键词“扩散受限和孤立性SCC病变”检索可能患有可逆性孤立性SCC病变的患者。总结临床、实验室和影像学数据。

结果

共识别出15例伴有可逆性孤立性SCC病变 的脑炎/脑病患者(9例男性和6例女性)。除13例有发热症状外,8例患者在神经症状发作前还伴有感冒症状。神经症状包括头痛、眩晕、癫痫发作、意识障碍和谵妄行为。13例患者在1个月内完全康复,但2例接受机械通气的患者有持续性神经功能缺损。初始MRI特征显示孤立的椭圆形或延伸性SCC病变,在扩散加权成像(DWI)上呈均匀高信号,表观扩散系数(ADC)值降低。随访MRI显示,初始MRI检查后10至32天,伴有弥散受限的孤立性SCC病变消失。在一例预后较差的严重病例中,分数各向异性图显示SCC病变值降低。

结论

与脑炎/脑病相关的RESLES是一种可逆性综合征,大多数患者预后良好,而少数早期需要呼吸机支持的患者可能有严重的神经后遗症。所有患者在DWI和ADC上均有可逆性信号改变,但有神经后遗症的严重患者分数各向异性值可能降低。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/98b0/4835842/00a6799fff18/12883_2016_572_Fig1_HTML.jpg

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