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结节性硬化症中的囊肿样白质病变。

Cystlike white matter lesions in tuberous sclerosis.

作者信息

Van Tassel P, Curé J K, Holden K R

机构信息

Department of Radiology, Medical University of South Carolina, Charleston 29425, USA.

出版信息

AJNR Am J Neuroradiol. 1997 Aug;18(7):1367-73.

Abstract

PURPOSE

To investigate the presence of small cystlike structures in the cerebral hemispheric white matter on MR images of patients with tuberous sclerosis.

METHODS

The MR images of 18 consecutive patients with tuberous sclerosis were reviewed retrospectively.

RESULTS

Eight of the 18 patients were found to have cystlike structures in the cerebral white matter. The signal intensity of these lesions was isointense with cerebrospinal fluid on T1-, proton density-, and T2-weighted images. Four patients were imaged with a fluid-attenuated inversion recovery sequence, which in each case also showed fluid-type signal in these areas. Three of the patients had CT for correlation, and these scans supported the diagnosis of cystic lesions. Cysts ranged in number from one to 12 per patient and were usually smaller than 1 cm. The most common location was adjacent to the occipital horn or trigone of the lateral ventricle (six of eight patients). Less frequent sites were near the frontal horns, in the corpus callosum, and in the deep white matter near the body of the lateral ventricle. Cysts in five patients were either immediately adjacent to a cortical tuber or in the center of a white matter dysplastic lesion. A cyst in one patient had septa, and none of the cysts enhanced.

CONCLUSIONS

Cystlike structures in the cerebral hemispheric white matter were seen on the MR images of 44% of 18 patients with tuberous sclerosis. Whether these findings represent cystic degeneration of dysplastic tissue or are unrelated to the disease process of tuberous sclerosis is unknown. More than one pathogenesis may exist.

摘要

目的

研究结节性硬化症患者脑半球白质磁共振成像(MRI)上小囊肿样结构的存在情况。

方法

回顾性分析18例连续的结节性硬化症患者的MRI图像。

结果

18例患者中有8例在脑白质中发现囊肿样结构。这些病变在T1加权、质子密度加权和T2加权图像上的信号强度与脑脊液等信号。4例患者采用液体衰减反转恢复序列成像,在每种情况下,这些区域也显示出液体样信号。3例患者进行了CT检查以辅助诊断,这些扫描结果支持囊肿性病变的诊断。囊肿数量每位患者从1个到12个不等,通常小于1厘米。最常见的位置是邻近枕角或侧脑室三角区(8例患者中的6例)。较少见的部位是额叶角附近、胼胝体以及侧脑室体部附近的深部白质。5例患者的囊肿要么紧邻皮质结节,要么位于白质发育异常病变的中心。1例患者的囊肿有分隔,所有囊肿均无强化。

结论

在18例结节性硬化症患者中,44%的患者MRI图像上可见脑半球白质中的囊肿样结构。这些发现是代表发育异常组织的囊性退变还是与结节性硬化症的疾病进程无关尚不清楚。可能存在不止一种发病机制。

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