神经元核内包涵体病脑MRI特征  

MRI Features of Neuronal Intranuclear Inclusion Disease

作　　者：张子璇 谢媛 李建瑞 张超 白卓杰[4] 柴学[5] 徐凯 肖朝勇[5] 陈峰[6] 刘涛[7] 卢光明 张志强 ZHANG Zixuan;XIE Yuan;LI Jianrui(School of Medical Imaging,Xuzhou Medical University,Xuzhou,Jiangsu Province 221004,P.R.China)

机构地区：[1]徐州医科大学医学影像学院,221004 [2]东部战区总医院放射诊断科放射诊断科,南京210002 [3]徐州医科大学附属医院医学影像科,221004 [4]南京江北人民医院医学影像科,210002 [5]南京脑科医院医学影像科,210002 [6]海南省人民医院医学影像科,海口 [7]海南省人民医院神经内科,海口

出　　处：《临床放射学杂志》2022年第9期1625-1630,共6页Journal of Clinical Radiology

基　　金：国家重点研发计划项目(编号:2018YFA0701703);江苏省医学重点人才计划项目(编号:ZDRCA2016093)。

摘　　要：目的 总结及分析神经元核内包涵体病(NIID)的脑部MRI特征性表现。方法 回顾性分析29例经临床诊断为神经元核内包涵体病患者的临床及脑MRI资料。在大体观察的基础上,依据DWI和TWI病变空间分布形态制作频次热图。采用单因素方差分析比较皮层、DWI及TWI异常信号区磁共振表观扩散系数(ADC)的差异,组间两两比较方差齐者采用LSD检验,不齐者采用Game-Howell检验;运用磁共振动脉自旋标记灌注加权成像(ASL)、扩散张量成像(DTI)及磁共振波谱成像(MRS),大体分析灌注、弥散及波谱代谢的改变,总结该病的影像特点。结果 散发性21例,家族性8例。临床症状包括中枢神经、周围神经、自主神经受累等多样化表现,其中家族性患者有较一致的临床症状。影像上,通过大体观察,提出“鸡冠花征”形容特征性皮髓交界区DWI高信号。空间分布频次热图显示出特征性异常信号好发的位置,并且展示出DWI-TWI位置不匹配现象:DWI高信号位于较外层皮髓交界区,而TWI高信号位于较深部白质区。ADC定量分析显示,皮髓交界区DWI高信号的ADC值表现为高于皮层而低于深部白质TWI高信号区。部分病例显示:DTI发现全脑广泛白质纤维减少;ASL及MRS未见明显异常。结论 NIID特征MRI表现为:DWI皮髓交界区高信号可能为血管源性水肿,形态上呈“鸡冠花征”,且DWI靠外分布的皮髓交界区高信号与TWI较深部白质区高信号空间分布位置不匹配,这些影像特征有助于NIID的诊断及鉴别诊断。Objective To analyze and summarize MRI characteristics of the Neuronal Intranuclear Inclusion Disease(NIID). Methods Clinical and MRI data of 29 patients with NIID clinically confirmed were analyzed retrospectively.DWI and TWI data were used to generate lesion frequency heat maps.ADC values in the regions of cortex, DWI and TWI lesions were compared using one way ANOVA,the LSD test was used for two way comparisons between groups where the variance was the same, and the Game Howell test was used for those in which data variance were not the same.The manifestations of arterial spin labeling perfusion weighted imaging(ASL),diffusion tensor imaging(DTI) and magnetic resonance spectroscopy(MRS) were observed. Results Among the patients, 21 cases were sporadic and 8 were familial.The symptoms involved central nervous system, peripheral nervous system and autonomic nervous system.Clinical manifestations were consistent in family cases.DWI showed high intensity signal resembling “Coxcomb” in cerebral corticomedullary junction.The lesion frequency maps revealed high probability location of the lesions with mismatched DWI and TWI.Distinctive clues to the diagnosis: high intensity signal on DWI located at corticomedullary junction, while high intensity area was widely observed from the corticomedullary junction to deep brain white matter.The ADC values of high signal areas on DWI were intermediate between the normally lower ADC of adjacent cerebral cortex and higher ADC of adjacent abnormal deep white matter on TWI.MRI data in a portion of cases did not show abnormalities in ASL and MRS.DTI showed white matter tracts lesions. Conclusion The radiological characteristics of NIID on MRI included :High signal called “Coxcomb sign”on DWI may correlated with vasogenic edema, mismatched locations of lesions on DWI(cerebral corticomedullary junction)and TWI( deep brain white matter).These image findings are helpful for diagnosis of NIID.

关 键 词：神经元核内包涵体病 多模态磁共振成像 热图 

分 类 号：R445.2[医药卫生—影像医学与核医学] R741[医药卫生—诊断学]