神经纤维瘤病 1 型患儿的囊性样脑白质病变进展
Growing Cyst-Like White Matter Lesions in Children With Neurofibromatosis Type 1.
机构信息
Department of Radiology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 03080, South Korea.
Department of Radiology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 03080, South Korea.
出版信息
Pediatr Neurol. 2017 Dec;77:84-88. doi: 10.1016/j.pediatrneurol.2017.07.013. Epub 2017 Aug 4.
BACKGROUND
Neurofibromatosis type 1 (NF1) is an autosomal dominant disease with prominent neurocutaneous manifestations. The most common intracranial imaging finding of NF1 on brain magnetic resonance imaging (MRI) is the high-signal intensity foci without a mass effect or growth in size.
PATIENT DESCRIPTION
We describe two children with NF1 in whom brain MRI showed growing cystic lesions and adjacent white matter signal abnormalities, which were confirmed as non-neoplastic cystic degeneration and reactive gliosis.
CONCLUSION
Growing cyst-like white matter lesions can be seen on serial brain MRI in children with NF1. Reactive gliosis with cystic degeneration could be a pathogenic basis of these cystic lesions.
背景
神经纤维瘤病 1 型（NF1）是一种常染色体显性疾病，具有明显的神经皮肤表现。脑磁共振成像（MRI）上 NF1 最常见的颅内影像学表现是高信号灶，无肿块效应或大小增大。
患者描述
我们描述了两名 NF1 患儿，其脑 MRI 显示囊性病变增大和相邻白质信号异常，经证实为非肿瘤性囊性变性和反应性神经胶质增生。
结论
NF1 患儿的连续脑 MRI 可观察到囊性病变增大。伴有囊性变性的反应性神经胶质增生可能是这些囊性病变的发病基础。
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