“烟雾病”表象之下：1型神经纤维瘤病与烟雾病综合征相关
'Moya' than meets the eye: neurofibromatosis type 1 associated with Moyamoya syndrome.
作者信息
Tan R M, Chng S M, Seow W T, Wong J, Lim C C
机构信息
Department of Paediatric Surgery, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore 229899.
出版信息
Singapore Med J. 2008 Apr;49(4):e107-9.
Moyamoya syndrome (MMS) is an uncommon association of neurofibromatosis type 1 (NF1). We describe a seven-year-old chinese girl with NF1 and unilateral MMS with multiple hyperintensities on T2-weighted magnetic resonance (MR) images. The ischaemic lesions in the ipsilateral white matter were hypointense on fluid attenuated inversion recovery (FLAIR) MR images, in contrast to the hyperintense "unidentified bright objects" (UBOs) of NF1. Neuroradiologists should be aware of associated MMS in NF1 patients, and distinguish the effects of ischaemia from UBOs, especially on FLAIR MR imaging.
烟雾综合征（MMS）是1型神经纤维瘤病（NF1）的一种罕见关联疾病。我们描述了一名7岁中国女孩，患有NF1和单侧MMS，其T2加权磁共振（MR）图像上有多处高信号。与NF1的高信号“不明亮物体”（UBO）形成对比的是，同侧白质的缺血性病变在液体衰减反转恢复（FLAIR）MR图像上呈低信号。神经放射科医生应意识到NF1患者中存在相关的MMS，并区分缺血与UBO的影响，尤其是在FLAIR MR成像上。
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