Magnetic resonance imaging lesion analysis in neurofibromatosis type 1
Magnetic resonance imaging lesion analysis in neurofibromatosis type 1
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DOI：
10.1001/archneur.55.4.500
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发表时间：
1998-04-01
影响因子：
--
通讯作者：
Ramsby, G
中科院分区：
文献类型：
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作者：
DiMario, FJ;Ramsby, G
目的：明确1型神经纤维瘤病(NF-1)患者在磁共振成像(MRI)研究中发现的高信号脑实质病变的演变。设计：前瞻性地确定一组接受MRI检查的NF-1患者，并对其影像结果进行分析。患者：所有符合美国国立卫生研究院共识标准的NF-1患者均符合条件。在123名接受病情评估的NF-1患者中，30名患者接受了59次核磁共振检查。男22例，女8例，年龄1~53岁，平均12.5岁。确定了两组患者，有脑损伤(WBL)的患者和没有脑损伤的患者。结果：在19例WBL患者中，19例病变位于大脑半球19例，脑干和小脑各10例。病变部位以小脑和苍白球最多(129个病灶中87个)。在有系列研究的WBL患者中，在最初的研究中总共发现了97个病变，相当于197个单位(平均每个病变2.03个单位)。随访评估(间隔0.5-4.5年；平均2.3年)显示，病变总数(68个[-29%])和大小(132个单位；平均每个病变1.86个单位[-33%])均有所减少。重要的是，在连续研究的13例WBL患者中，7例在相同的时间间隔内，脑干病变的数量(+36%)和大小(+6.4%)都有所增加，而大脑半球和小脑的病变更加模糊。结论：随着时间的推移，NF-1患者MRI上的高信号T-2病变逐渐演变。核因子-1损伤的演变是区域特异性的，可能与核因子-1基因产物的优先区域特异性效应有关。
Objective: To define the evolution of identified high-signal brain parenchymal lesions on magnetic resonance imaging (MRI) studies in patients with neurofibromatosis type 1 (NF-1).Design: A cohort of patients with NF-1 who underwent MRI were identified prospectively and their imaging studies analyzed.Patients: All referred patients with NF-1 (as defined by National Institutes of Health consensus criteria), who had undergone imaging with MRI were eligible. Of 123 patients with NF-1 whose conditions were evaluated, 30 patients had undergone 59 MRIs. There were 22 males and 8 females, aged 1 to 53 years with mean age of 12.5 years. Two groups of patients were identified, those with brain lesions (WBL) and those with no brain lesions. All initial and subsequently obtained MRIs from the WBL group were analyzed and tallied for number, size, and location of lesions over serial studies.Results: Of the 19 patients with WBL, lesions were in hemispheres in 19 patients, and in the brainstem and the cerebellum in 10 patients each, respectively. Lesions were located in the cerebellum and globus pallidus most often (87 of 129 lesions). Of the patients with WBL having serial studies, a total of 97 lesions equaling 197 units (mean, 2.03 units per lesion) were identified at initial study. Follow-up evaluation (interval, 0.5-4.5 years; mean, 2.3 years), showed a decrease in both total number of lesions (68 [-29%]) and size (132 units; mean, 1.86 units per lesion [-33%]). Importantly, brainstem lesions increased in both number (+36%) and size (+6.4%) over the same intervals in 7 of 13 patients with WBL studied serially, whereas hemispheric and cerebellar lesions were more evanescent.Conclusions: High-signal T-2 lesions on MRI in patients with NF-1 evolve over time. The evolution of the NF-1 lesion is region specific and may relate to preferential region-specific effects of the NF-1 gene product.