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1型神经纤维瘤病中白质病变的演变:磁共振成像结果

Evolution of white matter lesions in neurofibromatosis type 1: MR findings.

作者信息

Sevick R J, Barkovich A J, Edwards M S, Koch T, Berg B, Lempert T

机构信息

Department of Radiology, University of California, San Francisco 94143.

出版信息

AJR Am J Roentgenol. 1992 Jul;159(1):171-5. doi: 10.2214/ajr.159.1.1609692.

Abstract

To characterize further the evolution of white matter lesions in neurofibromatosis type 1, we reviewed 68 MR images in 43 patients (age, 1-31 years), including 25 follow-up studies (mean interval, 27 months). Lesion number, location, morphology, signal characteristics, and contrast enhancement were assessed. Lesion characteristics and changes thereof were correlated with the patients' ages. Thirty-four patients (79%) had white matter lesions. These lesions were hyperintense on T2-weighted images, were isointense on T1-weighted images, and showed no mass effect or contrast enhancement in 31 patients; in three patients, T1-prolongation was observed (one with significant mass effect). None of the lesions evolved into a glioma. The most common locations were the cerebellum (49%), brainstem (22%), and internal capsule (19%). Nineteen patients had white matter lesions and follow-up studies. Lesions decreased in size or number in seven patients (average age, 13 years), showed no change in three (average age, 12 years), increased in size or number in four (average age, 5 years), and showed a mixed pattern (increased/decreased size/number) in four (average age, 7 years). White matter lesions in neurofibromatosis type 1 frequently increase in size or number early in childhood; this did not indicate neoplasia in our study. The lesions tend to resolve with increasing age. Lesion progression in a child more than 10 years old warrants close follow-up to rule out a neoplasm.

摘要

为了进一步明确1型神经纤维瘤病中白质病变的演变情况,我们回顾了43例患者(年龄1 - 31岁)的68份磁共振成像(MRI),其中包括25项随访研究(平均间隔27个月)。评估了病变的数量、位置、形态、信号特征及对比增强情况。将病变特征及其变化与患者年龄进行关联分析。34例患者(79%)存在白质病变。这些病变在T2加权图像上呈高信号,在T1加权图像上呈等信号,31例患者中未见占位效应或对比增强;3例患者观察到T1延长(1例有明显占位效应)。无一例病变演变为胶质瘤。最常见的部位是小脑(49%)、脑干(22%)和内囊(19%)。19例患者有白质病变并接受了随访研究。7例患者(平均年龄13岁)的病变大小或数量减少,3例(平均年龄12岁)无变化,4例(平均年龄5岁)大小或数量增加,4例(平均年龄7岁)呈混合模式(大小/数量增加/减少)。1型神经纤维瘤病中的白质病变在儿童早期常出现大小或数量增加;在我们的研究中这并不提示肿瘤形成。病变往往随着年龄增长而消退。10岁以上儿童的病变进展需要密切随访以排除肿瘤。

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