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[散发性克雅氏病中丘脑枕和丘脑背内侧核的双侧高信号]

[Bilateral hyperintensity of the pulvinar and dorsomedial nucleus of the thalamus in sporadic Creutzfeldt-Jakob disease].

作者信息

Casimiro Carlos, Martins Joana, Parreira Tiago, Baldeiras Inês, Ribeiro Helena, Batista Sónia, Machado Egídio, Maduro Ana, Rebelo Olinda, Freitas Pedro

机构信息

Serviço de Imagiologia, Hospitais da Universidade de Coimbra, Coimbra, Portugal.

出版信息

Acta Med Port. 2012;25 Suppl 1:41-4. Epub 2012 Nov 2.

Abstract

INTRODUCTION

Creutzfedt-Jakob Disease (CJD) is a rapidly progressive neurodegenerative disease caused by prions. Early diagnosis and the determination of its form are epidemiologically important, with strong impact on public health. Bilateral pulvinar hyperintensity, either alone (pulvinar sign) or in association with the dorsomedial nucleus of the thalamus (double hockey stick sign) on T2, FLAIR and diffusion weighted imaging (DWI), is a criterion for the probable diagnosis of the variant CJD (vCJD). Bilateral hyperintensity of the caudate, putamina and cortex is the usual pattern found in the sporadic CJD (sCJD).

OBJECTIVE

Analysis of the imaging aspects on a sCJD patient showing T2 hyperintensity of the pulvinar and dorsomedial thalamic nucleus, in order to assess the magnetic resonance imaging (MRI) accuracy in the discrimination between vCJD and sCJD, when this lesion pattern is present.

METHODS

We performed a MRI on a 62-year-old female with definitive diagnosis of sCJD made by anatomopathologic study of the brain tissue. Qualitative analysis of MRI, including DWI, T2 and FLAIR sequences, as well as lesional patterns found.

RESULTS

Brain MRI showed hyperintensity of the caudate, putamina, pulvinar and dorsomedial nucleus of the thalamus, in DWI, T2 and FLAIR sequences; hypersignal of the caudate and putamina was greater than the signal intensity of the thalami. Hyperintensity of the hippocampus and frontal, temporal and parietal cortex were more obvious in FLAIR and DWI.

COMMENT

Hyperintensity of the pulvinar and dorsomedial nucleus of the thalamus on sCJD may complicate the differential diagnosis with vCJD. True pulvinar sign and double hockey stick sign, consistent with vCJD, must only be considered if the hyperintensity is greater than signal intensity of the caudate and putamina.

摘要

引言

克雅氏病(CJD)是一种由朊病毒引起的快速进展性神经退行性疾病。早期诊断及其类型的确定在流行病学上具有重要意义,对公共卫生有重大影响。在T2加权成像、液体衰减反转恢复序列(FLAIR)和扩散加权成像(DWI)上,双侧丘脑枕高信号,无论是单独出现(丘脑枕征)还是与丘脑背内侧核一起出现(双曲棍球棒征),是变异型克雅氏病(vCJD)可能诊断的标准。尾状核、壳核和皮质的双侧高信号是散发性克雅氏病(sCJD)常见的表现模式。

目的

分析一名表现为丘脑枕和丘脑背内侧核T2高信号的sCJD患者的影像学特征,以评估当出现这种病变模式时,磁共振成像(MRI)在鉴别vCJD和sCJD方面的准确性。

方法

我们对一名62岁女性进行了MRI检查,该患者经脑组织病理检查确诊为sCJD。对MRI进行定性分析,包括DWI、T2和FLAIR序列,以及发现的病变模式。

结果

脑部MRI显示,在DWI、T2和FLAIR序列上,尾状核、壳核、丘脑枕和丘脑背内侧核呈高信号;尾状核和壳核的高信号大于丘脑的信号强度。海马以及额叶、颞叶和顶叶皮质的高信号在FLAIR和DWI上更为明显。

评论

sCJD患者丘脑枕和丘脑背内侧核的高信号可能会使与vCJD的鉴别诊断复杂化。只有当高信号大于尾状核和壳核的信号强度时,才应考虑与vCJD一致的真正丘脑枕征和双曲棍球棒征。

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