Variations and natural history of primary intraparenchymal lesions associated with neurofibromatosis type 2
Variations and natural history of primary intraparenchymal lesions associated with neurofibromatosis type 2
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2 型神经纤维瘤病相关原发性实质内病变的变异和自然史
DOI：
10.1007/s00234-021-02809-5
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发表时间：
2021
期刊：
影响因子：
2.8
通讯作者：
Fujimura Miki
中科院分区：
文献类型：
--
作者：
Ishi Yukitomo;Harada Taisuke;Kameda Hiroyuki;Okada Hiromi;Yokota Isao;Okamoto Michinari;Sawaya Ryosuke;Motegi Hiroaki;Yamaguchi Shigeru;Terasaka Shunsuke;Kudo Kohsuke;Fujimura Miki
本研究旨在探讨2型神经纤维瘤病患者原发性脑实质内病变的临床意义和自然史。回顾性分析15例2型神经纤维瘤病的X线表现。在15例患者中的7例（47%）中观察到27处原发性脑实质内病变。皮质/皮质下T2高信号病变和Virchow-Robin间隙扩大分别是5例和4例患者最常见的结果。在随访期间（中位84个月），发现1例新的原发性脑实质内病变，在对比增强MRI上观察到2例病变增加。1例经手术病理证实为非典型脑膜瘤，行手术切除。其他25个没有增强的病灶在随访期间没有明显的生长。虽然大多数原发性脑实质内病变是良性的，但在增强MRI上，一部分病例会出现新的或增加的病变。对此类病例需要仔细监测，并应考虑病理学证实。
The study aimed to investigate the clinical implications and natural history of primary intraparenchymal lesions in patients with neurofibromatosis type 2. Radiological findings of 15 neurofibromatosis type 2 cases were retrospectively collected. Twenty-seven primary intraparenchymal lesions were observed in 7 out of 15 patients (47%). Cortical/subcortical T2 hyperintense lesions and enlarged Virchow–Robin spaces were the most common findings in five and four patients, respectively. During the follow-up period (median 84 months), one new primary intraparenchymal lesion was identified and increased lesions were observed in two cases on contrast-enhanced MRI. Surgical resection was performed in one case pathologically diagnosed with atypical meningioma. Twenty-five other lesions without contrast enhancement presented no apparent growth during follow-up. Although most primary intraparenchymal lesions are benign, a subset of cases would present newly developed or increased lesions on contrast-enhanced MRI. Careful monitoring is necessary for such cases, and pathological confirmation should be considered.
双侧听神经纤维瘤病（神经纤维瘤病 2）脑微错构瘤的分布和免疫反应性
影响因子：
12.7
作者：
Otmar D. Wiestler;K. Siebenthal;H. Schmitt;W. Feiden;P. Kleihues
通讯作者：
P. Kleihues
令人惊讶的癫痫病因：局灶性皮质发育不良儿童的全外显子组测序鉴定出 2 型神经纤维瘤病。
影响因子：
3.8
作者：
K. Muir;M. Sargent;C. Boelman
通讯作者：
C. Boelman