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戴克-大卫多夫-马森综合征——典型影像学特征

DYKE-DAVIDOFF-MASSON SYNDROME - TYPICAL IMAGING FEATURES.

作者信息

Bjelan Mladen, Kozic Dusko, Njagulj Vesna, Ragaji Aleksandar, Koprivsek Katarina, Lucic Milos

出版信息

Med Pregl. 2016 Nov;69(11-12):373-375. doi: 10.2298/mpns1612373b.

Abstract

INTRODUCTION

Dyke-Davidoff-Masson syndrome is an uncommon neurological disorder clinically presented with seizures, various degrees of mental retardation, motor weakness and sometimes body asymmetry. Typical neuroimaging features include cerebral hemiatrophy with ipsilateral hyper pneumatization of paranasal sinuses. The purpose of this report was to present a rare cause of seizures revealed by Magnetic resonance imaging.

CASE REPORT

We report a case of a 17-year-old boy admitted to hospital due to a severe headache. He had been treated because of partial epileptic seizures for six years. Neuropsychological examination revealed mild mental retardation, mild speech and reading difficulties and discrete right-sided hemiparesis. Typical magnetic resonance imaging features confirmed clinical suspicion of Dyke-Davidoff-Masson syndrome. revealing left frontal lobe atrophy, with consecutive widening of the left lateral ventricle frontal horn, thickening of the nearby frontal squama and hypertrophy of left frontal sinus.

CONCLUSION

Magnetic resonance imaging is the key imaging modality that confirms clinical suspicion of Dyke-Davidoff-Masson syndrome based on a proper physical and neurological examination.

摘要

引言

戴克-戴维多夫-马森综合征是一种罕见的神经系统疾病,临床表现为癫痫发作、不同程度的智力迟钝、运动无力,有时还伴有身体不对称。典型的神经影像学特征包括脑半球萎缩伴同侧鼻窦过度气化。本报告的目的是介绍磁共振成像揭示的一种罕见的癫痫病因。

病例报告

我们报告一例17岁男孩因严重头痛入院。他因部分性癫痫发作已接受治疗六年。神经心理学检查显示轻度智力迟钝、轻度言语和阅读困难以及轻度右侧偏瘫。典型的磁共振成像特征证实了临床对戴克-戴维多夫-马森综合征的怀疑,显示左额叶萎缩,左外侧脑室额角连续增宽,附近额骨增厚以及左额窦肥大。

结论

磁共振成像是基于适当的体格检查和神经学检查来证实临床对戴克-戴维多夫-马森综合征怀疑的关键影像学检查方法。

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