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1型神经纤维瘤病患儿中枢神经系统表现的影像学考量

Imaging considerations of central nervous system manifestations in pediatric patients with neurofibromatosis type 1.

作者信息

Menor F, Martí-Bonmatí L, Mulas F, Cortina H, Olagüe R

机构信息

Department of Diagnostic Radiology, La Fe Children's Hospital, Valencia, Spain.

出版信息

Pediatr Radiol. 1991;21(6):389-94. doi: 10.1007/BF02026665.

DOI:10.1007/BF02026665
PMID:1749666
Abstract

CT and MRI were used in a prospective study of the central nervous system (CNS) manifestations in 41 consecutive children with neurofibromatosis type 1 (NF-1). Gadolinium-DTPA was used in 15 patients. MRI was more effective than CT in delimiting the extension of the optic pathway glioma and in evaluating associated cerebral malformations. MRI visualized lesions generally undetected by CT, in the form of iso- or hyperintense foci with respect to the cerebral cortex in T2-weighted sequences. Well-delimited lesions of high signal intensity were observed in the globus pallidus (22 cases), the internal capsule (6 cases), corpus callosum (2 cases), anterior commissure (1 case) and semioval center (2 cases). Poorly defined hyper- or isointense areas were also observed affecting the cerebellar white matter (21 cases) and brain stem (17 cases). None of these lesions showed Gadolinium-DTPA enhancement, and were of no clinical significance. MRI has displaced CT in the initial diagnosis of patients with NF-1. Periodic annual MRI controls are only justified in patients with MRI changes to evaluate the progression or stabilization of the lesions.

摘要

对41例连续性1型神经纤维瘤病(NF-1)患儿的中枢神经系统(CNS)表现进行了一项前瞻性研究,采用了CT和MRI检查。15例患者使用了钆喷酸葡胺。在界定视神经通路胶质瘤的范围以及评估相关脑畸形方面,MRI比CT更有效。MRI能显示出CT通常检测不到的病变,在T2加权序列中表现为相对于脑皮质呈等信号或高信号灶的形式。在苍白球(22例)、内囊(6例)、胼胝体(2例)、前连合(1例)和半卵圆中心(2例)观察到界限清楚的高信号强度病变。在小脑白质(21例)和脑干(17例)也观察到界限不清的高信号或等信号区。这些病变均未显示钆喷酸葡胺增强,且无临床意义。在NF-1患者的初始诊断中,MRI已取代CT。仅对MRI有改变的患者进行每年一次的定期MRI检查,以评估病变的进展或稳定情况才是合理的。

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