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结节性硬化症中的白质异常

White matter abnormalities in tuberous sclerosis complex.

作者信息

Griffiths P D, Bolton P, Verity C

机构信息

Academic Department of Radiology, University of Sheffield, UK.

出版信息

Acta Radiol. 1998 Sep;39(5):482-6. doi: 10.1080/02841859809172211.

Abstract

PURPOSE

The aim of this study was to investigate and describe the range of white matter abnormalities in children with tuberous sclerosis complex by means of MR imaging.

MATERIAL AND METHODS

A retrospective cross-sectional study was performed on the basis of MR imaging findings in 20 cases of tuberous sclerosis complex in children aged 17 years or younger.

RESULTS

White matter abnormalities were present in 19/20 (95%) cases of tuberous sclerosis complex. These were most frequently (19/20 cases) found in relation to cortical tubers in the supratentorial compartment. White matter abnormalities related to tubers were found in the cerebellum in 3/20 (15%) cases. White matter abnormalities described as radial migration lines were found in relation to 5 tubers in 3 (15%) children. In 4/20 (20%) cases, white matter abnormalities were found that were not related to cortical tubers. These areas had the appearance of white matter cysts in 3 cases and infarction in the fourth. In the latter case there was a definable event in the clinical history, supporting the diagnosis of stroke.

CONCLUSION

A range of white matter abnormalities were found by MR imaging in tuberous sclerosis complex, the commonest being gliosis and hypomyelination related to cortical tubers. Radial migration lines were seen infrequently in relation to cortical tubers and these are thought to represent heterotopic glia and neurons along the expected path of cortical migration.

摘要

目的

本研究旨在通过磁共振成像(MR成像)调查和描述结节性硬化症患儿白质异常的范围。

材料与方法

基于17岁及以下儿童的20例结节性硬化症的MR成像结果进行回顾性横断面研究。

结果

20例结节性硬化症患者中有19例(95%)存在白质异常。这些异常最常见于(19/20例)幕上脑叶的皮质结节。20例中有3例(15%)在小脑发现与结节相关的白质异常。在3名(15%)儿童中,与5个结节相关的白质异常表现为放射状移行线。20例中有4例(20%)发现与皮质结节无关的白质异常。其中3例表现为白质囊肿,第4例表现为梗死。在后一例中,临床病史中有明确事件,支持中风诊断。

结论

通过MR成像在结节性硬化症中发现了一系列白质异常,最常见的是与皮质结节相关的胶质增生和髓鞘形成不足。与皮质结节相关的放射状移行线很少见,这些被认为代表沿着预期皮质移行路径的异位胶质细胞和神经元。

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