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结节性硬化症患者半侧巨脑症的纵向CT和MR表现。

Longitudinal CT and MR appearances of hemimegalencephaly in a patient with tuberous sclerosis.

作者信息

Balaji Ravikanth, Kesavadas Chandrasekharan, Ramachandran K, Nayak S Dinesh, Priyakumari T

机构信息

Division of Imageology, Regional Cancer Center, Trivandrum, India.

出版信息

Childs Nerv Syst. 2008 Mar;24(3):397-401. doi: 10.1007/s00381-007-0463-x. Epub 2007 Sep 18.

DOI:10.1007/s00381-007-0463-x
PMID:17876590
Abstract

CASE REPORT

A 3-day-old baby was referred to our institution with seizures since birth and a presumptive diagnosis of cerebral tumor detected by prenatal ultrasound. Computed tomography (CT) and Magnetic Resonance (MR) imaging performed immediately after birth revealed the presence of an enhancing mass in the left cerebral hemisphere.

DISCUSSION

The possibility of a congenital malignant brain tumor was considered. Although surgery was advised, the parents opted for conservative medical management. The infant was under treatment for seizures with antiepileptic drugs. At 7-month follow-up, there was a noticeable increase in the seizure activity with signs of developmental delay and increased head circumference. Repeat CT and MR imaging showed features of hemimegalencephaly with a calcified mass involving the left hemisphere. The patient was diagnosed to have tuberous sclerosis. This case report illustrates how an enhancing mass (possibly a tuber) over a period of time diminishes in size and becomes calcified in a patient with tuberous sclerosis and associated hemimegalencephaly.

摘要

病例报告

一名3天大的婴儿自出生起就因癫痫发作被转诊至我院,产前超声检查初步诊断为脑肿瘤。出生后立即进行的计算机断层扫描(CT)和磁共振成像(MR)显示左脑半球有一个强化肿块。

讨论

考虑到先天性恶性脑肿瘤的可能性。尽管建议进行手术,但家长选择了保守的药物治疗。婴儿正在接受抗癫痫药物治疗以控制癫痫发作。在7个月的随访中,癫痫发作活动明显增加,伴有发育迟缓迹象和头围增大。重复的CT和MR成像显示半侧巨脑症的特征,左半球有一个钙化肿块。该患者被诊断为结节性硬化症。本病例报告说明了在结节性硬化症和相关半侧巨脑症患者中,一个强化肿块(可能是一个结节)如何随着时间的推移缩小并钙化。

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本文引用的文献

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Contralateral hemimicrencephaly and clinical-pathological correlations in children with hemimegalencephaly.半侧巨脑症患儿的对侧半侧微脑回畸形及其临床病理相关性
Brain. 2006 Feb;129(Pt 2):352-65. doi: 10.1093/brain/awh681. Epub 2005 Nov 16.
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Longitudinal MR findings in a patient with hemimegalencephaly associated with tuberous sclerosis.一名患有与结节性硬化症相关的半侧巨脑症患者的纵向磁共振成像结果。
Brain Dev. 2005 Sep;27(6):458-61. doi: 10.1016/j.braindev.2004.11.004. Epub 2005 Jan 8.
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Molecular pathogenesis of tuber formation in tuberous sclerosis complex.
结节性硬化症中结节形成的分子发病机制。
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Hemimegalencephaly with tuberous sclerosis: a longitudinal imaging study.
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Cell output, cell cycle duration and neuronal specification: a model of integrated mechanisms of the neocortical proliferative process.细胞输出、细胞周期时长与神经元特化:一种新皮质增殖过程综合机制的模型
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