儿童神经皮肤黑变病累及杏仁体的MRI表现及文献复习  

MRI findings and literature review of amygdala involvement in neurocutaneous melanosis in children

作　　者：苏晓然 陈琬 时胜利 郑彬 陈志平 SU Xiaoran;CHEN Wan;SHI Shengli;ZHENG Bin;CHEN Zhiping(Department of Radiology,Children's Hospital Affiliated of Zhengzhou University,Zhengzhou 450000,China)

机构地区：[1]郑州大学附属儿童医院放射科,河南郑州450000

出　　处：《实用放射学杂志》2021年第10期1685-1688,共4页Journal of Practical Radiology

摘　　要：目的探讨儿童神经皮肤黑变病累及杏仁体的MRI表现.方法结合相关文献,回顾性分析8例经病理活检确诊的神经皮肤黑变病累及杏仁体患儿的MRI特点.结果患儿临床均以无明显诱因抽搐就诊,皮肤均出现大面积伴浓密毛发黑斑.本组8例均有脑膜及杏仁体受累,其中7例累及双侧杏仁体,MRI平扫示受累脑膜增厚,T_(1)WI呈等稍高信号,增强明显强化;受累脑实质呈团片状、结节状,边界清晰,T_(1)WI呈高信号,T_(2)WI呈等、稍低信号,液体衰减反转恢复(FLAIR)呈等信号,扩散加权成像(DWI)未见异常,增强扫描病变呈轻度强化.8例均行临床复查,1例杏仁体及脑膜受累范围逐渐增大,临床症状加重,最终死亡,1例颅内病变范围略减小,T_(1)WI信号强度略减低,另外6例颅内情况变化不明显.结论儿童神经皮肤黑变病杏仁体受累罕见,对称发病为主,MRI信号特点为T_(1)WI高、T_(2)WI等或稍低信号,扩散不受限,增强轻度强化,患儿预后差.总结其影像特点并进行相关文献复习可提高本病的诊断能力.Objective To investigate the MRI findings of neurocutaneous melanosis involving the amygdala in children.Methods In combination with relevant literature,MRI features of 8 cases of neurocutaneous melanosis with amygdala involvement confirmed by pathological biopsy were analyzed retrospectively.Results Clinically,all the children were diagnosed with convulsion without obvious inducement,with large areas of skin accompanied by dense hair black spots.Meningeal and amygdala were involved in all 8 cases,7 of which involved bilateral amygdala.MRI plain scan showed thickening of the involved meninges,with isohyperintensity on T_(1)WI and significant enhancement.The affected brain parenchyma was patchy and nodular,with clear boundaries.T_(1)WI showed hyperintensity,T_(2)WI showed isointensity and slightly hypointensity,and fluid attenuated inversion recovery(FLAIR)showed isointensity.Diffusion weighted imaging(DWI)showed no abnormality,and the lesion was slightly enhanced on enhanced scan.All the 8 cases underwent clinical reexamination,and 1 case gradually increased the scope of amygdala and meningeal involvement,with worsening clinical symptoms,and finally died.1 case slightly decreased the scope of intracranial lesions,slightly decreased T_(1)WI signal intensity,and the other 6 cases showed no change in intracranial conditions.Conclusion The amygdala is rarely involved in neurocutaneous melanosis in children,and the incidence is mainly symmetrical.MRI signals are characterized by high T_(1)WI signal,equal or slightly low T_(2)WI signal,with unrestricted dispersion,mild enhancement and poor prognosis.Summarizing the imaging characteristics and reviewing the relevant literature can improve the diagnostic ability of this disease.

关 键 词：神经皮肤黑变病 杏仁体 磁共振成像 

分 类 号：R758.3[医药卫生—皮肤病学与性病学] R445.2[医药卫生—临床医学]