神经皮肤综合征的磁共振成像及临床表现  

Neurocutaneous syndrome of MRI and clinical manifestations

作　　者：刘莹[1] 谷瓅[1] 于清太 

机构地区：[1]吉林油田总医院磁共振科,松原138000

出　　处：《实用医学影像杂志》2018年第1期39-41,共3页Journal of Practical Medical Imaging

摘　　要：目的分析几种神经皮肤综合征的磁共振成像(MRI)及临床表现,旨在进一步提高对本组疾病的认识。方法收集我院收治的14例神经皮肤综合征患者的临床及影像学资料,结合其他相关文献对本组疾病的MRI及临床特点进行回顾性分析。结果 14例患者包括结节性硬化7例,神经纤维瘤病Ⅰ型4例,颜面血管瘤病3例。结节性硬化MRI表现为脑实质内多发结节,呈稍长T_1长T_2信号,FLAIR呈高信号,伴钙化时呈短T_1短T_2信号,增强扫描非钙化成分不均匀强化。临床表现为结节性硬化三联征;神经纤维瘤病Ⅰ型MRI表现为等或等长T_1长T_2信号,信号不均匀,增强扫描呈均匀或不均匀强化。临床表现为颈胸部不适,皮肤牛奶咖啡斑;颜面血管瘤病MRI表现为皮层萎缩,皮层下伴长T_1长T_2信号,增强扫描呈明显线状强化。临床表现为同侧面部血管痣。结论神经皮肤综合征的MRI表现、特别是颅内病变具有一定特征性,结合其典型临床表现可对本类疾病做出明确诊断。Objective To analyze several kinds of neurocutaneous syndrome of MRI and clinical manifesta- tions, aims to further improve the understanding of this disease. Methods Collection of 14 cases admitted in our hospital neurocutaneous syndrome in patients with clinical and imaging data, combined with other related literature to the MRI and clinical manifestations of this disease were retrospectively analyzed, Results There were 7 cases of tuberous sclerosis, 4 cases of neurofibromatosis I and 3 cases of facial hemangioma. The nodular sclerosis MRI showed muhiple nodules in the brain parenchyma, with a slightly longer T~ long T2 signal, FLAIR showed high signal, accompanied with calcification in short T1 short T2 signal, enhanced scanning non-calcified composition unevenly strengthened. Clinical manifestation is tuberous sclerosis triad; Neurofibromatosis type I MRI showed a long T1 long T2 signal, and the signal was not uniform and enhanced the scanning light and moderate uneven enhancement. Clini- cal manifestations of cervical chest discomfort, skin milk coffee spot; MRI of facial hemangioma is characterized by cortical atrophy, with a long TI long T2 signal in the cortex, and enhanced scanning. The clinical manifestation is the blood vessel mole on the side. Conclusion The MRI findings of neurocutaneous syndrome, especially the intracranial lesions, are characteristic and can be diagnosed by the typical clinical manifestations.

关 键 词：神经皮肤综合征 磁共振成像 临床特点 

分 类 号：R445.2[医药卫生—影像医学与核医学] R596[医药卫生—诊断学]