[1]王春祥,李欣,候志彬.儿童结节性硬化症14例的磁共振表现[J].国际放射医学核医学杂志,2006,30(3):185-188.
　WANG Chun-xiang,LI Xin,HOU Zhi-bin.MRI findings of tuberous sclerosis complex in pediatrics[J].International Journal of Radiation Medicine and Nuclear Medicine,2006,30(3):185-188.
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《国际放射医学核医学杂志》[ISSN:1673-4114/CN:12-1381/R]

卷:
30
期数:
2006年第3期
页码:
185-188
栏目:
临床放射医学
出版日期:
1900-01-01

文章信息/Info

Title:
MRI findings of tuberous sclerosis complex in pediatrics
作者:
王春祥;  李欣;  候志彬
300074 天津, 天津市儿童医院核磁共振室
Author(s):
WANG Chun-xiang;  LI Xin;  HOU Zhi-bin
Department of Radiology, Tianjin Children’s Hospital, Tianjin 300074, China
关键词:
结节性硬化症; 磁共振成像; 儿童
Keywords:
Tuberous sclerosis; Magnetic resonance imaging; Child
分类号:
R445.2
摘要:
目的 探讨结节性硬化症(TSC)的磁共振影像表现。方法 收集2002年3月至2005年4月间临床证实的结节性硬化症14例,全部病例均进行MR检查,其中3例进行增强检查。不合作儿童检查前采用6.5%水合氯醛保留灌肠镇静睡眠。结果 全部病例均显示大小不等(1~5mm)的室管膜下结节。皮层或皮层下结节10例,表现为脑回核心样病灶和H型病灶。白质异常信号6例,分别呈线状、楔形、不规则形。室管膜下巨细胞星形细胞瘤2例,肾血管平滑肌脂肪瘤1例。结论 MRI对TSC诊断的敏感性高,是首选的影像学检查方法。室管膜下结节是TSC最重要的MR影像学表现之一,以T1加权成像(T1WI)及梯度回波序列显示效果最佳。T2加权成像(T2WI)及液体衰减反转恢复序列(FLAIR)对TSC大脑皮层或皮层下区结节、脑白质区病灶的显示优于T1WI。
Abstract:
Objective To analyze MRI characteristic features of tuberous sclerosis.Methods There are 14 patients were examined by MR routine scan and 3 patients with enhancement, all patients were proven by clinical examination.Results Subependymal nodules, were detected in all cases, which were most commonly along the lateral wall of the lateral ventricles. Cortical tubers were detected in 10 cases, the patterns of cortical tubers were gyral core lesion and H-shaped lesion on MRI. The abnormal findings of white matter were radial linear or wedge-shaped or irregular type on MR images. Subependymal giant cell astrocytomas were detected in 2 cases, renal angiomyolipoma was detected in 1 case.Conclusions MRI is sensitive to find the changes in imaging diagnosis of TSC and is the first choice among the diagnostic imaging modalities. T1-weighted imaging (T1WI) and gradient echo sequences are more sensitive to the subependymal nodules than T2-weighted imaging (T2WI). T2WI and fluid-attenuated inversion recovery (FLAIR) are more sensitive to find the cortical tubers and abnormal white matter than T1WI.

参考文献/References:

1 Roach ES, Gomez MR, Northrup H. Tuberous sclerosis complex consensus conference:revised clinical diagnostic criteria. J Child Neurol, 1998, 13(12):624-628.
2 Dabora SL Jozwiak S, Franz DN, et al. Mutational analysis in a cohort of 224 tuberous sclerosis patients indicates increased severity of TSC2, compared with TSC1, disease in multiple organs.Am J Hum Genet, 2001, 68(1):64-80.
3 Shepherd CW, Houser OW, Gomez MR. MR findings in tuberous sclerosis complex and correlation with seizure development and mental impairment. Am J Neuroradiol, 1995, 16(1):149-155.
4 Mukonoweshuro W, Wilkinson ID, Griffiths PD. Proton MR spectroscopy of cortical tubers in adults with tuberous sclerosis complex. AJNR, 2001, 22(10):1920-1925.
5 DiMario FJ Jr. Brain abnormalities in tuberous sclerosis complex. J Child Neurol, 2004, 19(9):650-657.
6 陶琨, 张福林, 周范民.9例室管膜下巨细胞星形细胞瘤临床病理观察.临床与实验病理学杂志,2002,18(3):291-294.

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备注/Memo

备注/Memo:
收稿日期:2006-03-14。
通讯作者:王春祥(E-mail:tjchct@sohu.com)
更新日期/Last Update: 1900-01-01