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1.
Objective To investigate the changes of brain volumes in neuromyelitis optica (NMO)patients using voxel-based morphometry (VBM) method, and preliminarily explore the pattern of cerebral anatomical impairment. Methods Twenty-three clinically defined NMO patients and 15 gender and age matched healthy volunteers underwent 3-dimensional (3D) fast spoiled gradient echo (FSPGR) sequence scanning on 3.0 Tesla MR systen. Raw data was processed and analyzed using statistical parametric mapping (SPM) 5. Whole brain volumes included grey matter volume (GMV), white matter volume (WMV), total intracranial volume (TIV), grey matter fraction (GMF), white matter fraction (WMF),brain tissue fraction (BTF) and regional brain volumes between the two groups were compared by independent samples t-test and an Pearson were performed to compare the regional brain volumes and the ages. Results GMV of NMO group[(610. 2 ± 55.0) ml] was significantly decreased comparing to healthy control group[(657. 2 ± 36. 3) ml] (t = - 2. 915, P < 0. 05). The age of NMO patients [(40 ± 9) years old] showed negative correlation with GMF [(42. 5 ± 2. 6) %] (r = - 0. 673, P < 0. 05). Regional brain volume analysis showed decreased GMV in left insula and bilateral posterior cingutates in NMO patients,while decreased WMV was found in left frontal and left parietal white matter. Conclusion VBM could detect brain volume changes sensitively. Total grey matter volume in NMO patients was decreased comparing to HC group. Regional grey matter atrophy in NMO patients occurred in left insular and bilateral posterior cingutates, regional white matter atrophy occurred in left frontal and left parietal lobe.  相似文献   

2.
Objective To investigate the changes of brain volumes in neuromyelitis optica (NMO)patients using voxel-based morphometry (VBM) method, and preliminarily explore the pattern of cerebral anatomical impairment. Methods Twenty-three clinically defined NMO patients and 15 gender and age matched healthy volunteers underwent 3-dimensional (3D) fast spoiled gradient echo (FSPGR) sequence scanning on 3.0 Tesla MR systen. Raw data was processed and analyzed using statistical parametric mapping (SPM) 5. Whole brain volumes included grey matter volume (GMV), white matter volume (WMV), total intracranial volume (TIV), grey matter fraction (GMF), white matter fraction (WMF),brain tissue fraction (BTF) and regional brain volumes between the two groups were compared by independent samples t-test and an Pearson were performed to compare the regional brain volumes and the ages. Results GMV of NMO group[(610. 2 ± 55.0) ml] was significantly decreased comparing to healthy control group[(657. 2 ± 36. 3) ml] (t = - 2. 915, P < 0. 05). The age of NMO patients [(40 ± 9) years old] showed negative correlation with GMF [(42. 5 ± 2. 6) %] (r = - 0. 673, P < 0. 05). Regional brain volume analysis showed decreased GMV in left insula and bilateral posterior cingutates in NMO patients,while decreased WMV was found in left frontal and left parietal white matter. Conclusion VBM could detect brain volume changes sensitively. Total grey matter volume in NMO patients was decreased comparing to HC group. Regional grey matter atrophy in NMO patients occurred in left insular and bilateral posterior cingutates, regional white matter atrophy occurred in left frontal and left parietal lobe.  相似文献   

3.
Objective To evaluate the microstructural integrity of white matter (WM) in patients with amnestic mild cognitive impairment (aMCI) and mild Alzheimer's disease (AD) using voxel-based analysis (VBA), and investigate the relationship between WM abnormalities and gray matter(GM) atrophy.Methods Thirty-three cases with aMCI, 32 cases with mild AD and 31 normal aging volunteers as control subjects were scanned on a 3.0 T MR system using diffusion tensor imaging (DTI) and three-dimensional spoiled gradient-recalled(3DSPGR) sequences. Fractional anisotropy (FA) maps and morphological images were preprocessed by SPM5 and voxel-based comparisons between the 2 patient groups and the control group were performed by t test. Results Relative to the control group, patients with aMCI showed significantly reduced FA value in bilateral frontal, temporal and left occipital WM, left anterior part of cingulum, left inferior parietal lobule, and the W M adjacent to the triangular part of the right lateral ventricle(k≥20 voxels).In mild AD,significantly reduced FA value was found in bilateral hippocampal,inferior parietal lobular,frontal,temporal,and occipital WM,bilateral corpus callosum,anterior part of cingulums,the WM adjacent to the triaangular part of the bilateral lateral ventricles,left temporal stem,left thalamus,right precuneus(k≥20 voxels).Significantly reduced GM volume was found in left hippocampus,parahippocampal gyrus,lingual gyrus and superior temporal gyrus,bilateral insulae and middle temporal gyri in aMCl group whencompared with control group(k≥50 voxels).In mild AD,significantly reduced GM volume was found in bilateral hippoeampi,parahippocampal gyri,amygdalae,thalami,temporal,parietal,frontal,occipital cortex(k≥50 voxels).The pattern of areas with reduced FA differs;from that of the GM volumetric reduction.No areas with significantlv reduced FA was detected in aMCl compared with mild AD. There was no significant correlation between FA value of WM in patient groups and Mini-Mental State Examination(MMSE)scores.Conclusions Voxel-based MRI DTI analysis of whole brain white matter can objectively reveal widespread white matter abnormalities in early-stage AD.The difierence between WM FA reduction pattern and GM volumetric reduction pattern indicates that the pathological WM changes in earlyslage AD were caused by multiple mechanisms. FA did not vary significantly in patients pr0gressing from aMCI to mild AD and can hardly reflect the severitv of cognitive function damage in these patients.  相似文献   

4.
目的 通过基于体素的形态测量学(VBM)技术研究视神经脊髓炎(NMO)患者的脑体积变化情况,初步从结构方面探讨NMO的脑损害模式.方法 对23例NMO患者和15名健康志愿者进行脑部3D快速扰相梯度回波(FSPGR)序列扫描.原始数据用统计参数图(SPM)5软件进行处理.采用t检验比较两组全脑体积的差别,应用Pearson相关分析评价全脑体积和年龄的相关性.结果 NMO组患者的全商灰质体积[(610.2±55.0)ml]明显小于健康对照组[(657.2±36.3)ml],差异有统计学意义(t=-2.915,P<0.05).NMO患者的年龄[(40±9)岁]与脑灰质分数[(42.5±2.6)%]呈负相关(r=-0.673,P<0.05).局部脑体积分析结果显示,与健康对照组相比,NMO灰质体积减少主要集中在左侧岛叶和双侧后扣带回,白质体积减少区主要位于左侧额叶和左侧顶叶皮层下白质.结论 VBM技术可以敏感地检测NMO的脑体积变化情况.NMO患者的脑体积较健康对照组有减少的趋势,以灰质萎缩更明显.
Abstract:
Objective To investigate the changes of brain volumes in neuromyelitis optica (NMO)patients using voxel-based morphometry (VBM) method, and preliminarily explore the pattern of cerebral anatomical impairment. Methods Twenty-three clinically defined NMO patients and 15 gender and age matched healthy volunteers underwent 3-dimensional (3D) fast spoiled gradient echo (FSPGR) sequence scanning on 3.0 Tesla MR systen. Raw data was processed and analyzed using statistical parametric mapping (SPM) 5. Whole brain volumes included grey matter volume (GMV), white matter volume (WMV), total intracranial volume (TIV), grey matter fraction (GMF), white matter fraction (WMF),brain tissue fraction (BTF) and regional brain volumes between the two groups were compared by independent samples t-test and an Pearson were performed to compare the regional brain volumes and the ages. Results GMV of NMO group[(610. 2 ± 55.0) ml] was significantly decreased comparing to healthy control group[(657. 2 ± 36. 3) ml] (t = - 2. 915, P < 0. 05). The age of NMO patients [(40 ± 9) years old] showed negative correlation with GMF [(42. 5 ± 2. 6) %] (r = - 0. 673, P < 0. 05). Regional brain volume analysis showed decreased GMV in left insula and bilateral posterior cingutates in NMO patients,while decreased WMV was found in left frontal and left parietal white matter. Conclusion VBM could detect brain volume changes sensitively. Total grey matter volume in NMO patients was decreased comparing to HC group. Regional grey matter atrophy in NMO patients occurred in left insular and bilateral posterior cingutates, regional white matter atrophy occurred in left frontal and left parietal lobe.  相似文献   

5.
Objective To evaluate the microstructural integrity of white matter (WM) in patients with amnestic mild cognitive impairment (aMCI) and mild Alzheimer's disease (AD) using voxel-based analysis (VBA), and investigate the relationship between WM abnormalities and gray matter(GM) atrophy.Methods Thirty-three cases with aMCI, 32 cases with mild AD and 31 normal aging volunteers as control subjects were scanned on a 3.0 T MR system using diffusion tensor imaging (DTI) and three-dimensional spoiled gradient-recalled(3DSPGR) sequences. Fractional anisotropy (FA) maps and morphological images were preprocessed by SPM5 and voxel-based comparisons between the 2 patient groups and the control group were performed by t test. Results Relative to the control group, patients with aMCI showed significantly reduced FA value in bilateral frontal, temporal and left occipital WM, left anterior part of cingulum, left inferior parietal lobule, and the W M adjacent to the triangular part of the right lateral ventricle(k≥20 voxels).In mild AD,significantly reduced FA value was found in bilateral hippocampal,inferior parietal lobular,frontal,temporal,and occipital WM,bilateral corpus callosum,anterior part of cingulums,the WM adjacent to the triaangular part of the bilateral lateral ventricles,left temporal stem,left thalamus,right precuneus(k≥20 voxels).Significantly reduced GM volume was found in left hippocampus,parahippocampal gyrus,lingual gyrus and superior temporal gyrus,bilateral insulae and middle temporal gyri in aMCl group whencompared with control group(k≥50 voxels).In mild AD,significantly reduced GM volume was found in bilateral hippoeampi,parahippocampal gyri,amygdalae,thalami,temporal,parietal,frontal,occipital cortex(k≥50 voxels).The pattern of areas with reduced FA differs;from that of the GM volumetric reduction.No areas with significantlv reduced FA was detected in aMCl compared with mild AD. There was no significant correlation between FA value of WM in patient groups and Mini-Mental State Examination(MMSE)scores.Conclusions Voxel-based MRI DTI analysis of whole brain white matter can objectively reveal widespread white matter abnormalities in early-stage AD.The difierence between WM FA reduction pattern and GM volumetric reduction pattern indicates that the pathological WM changes in earlyslage AD were caused by multiple mechanisms. FA did not vary significantly in patients pr0gressing from aMCI to mild AD and can hardly reflect the severitv of cognitive function damage in these patients.  相似文献   

6.
Objective To characterize the regular and the dynamic contrast enhancement MR imaging in choroidal hemangioma.Methods MR imaging findings of 30 cases (31eyes, 32 lesions) with choroidal hemangioma confirmed by follow-up results were retrospectively analyzed.Among them, postcontrasted T1-weighted imaging was performed in 30 patients and dynamic contrast enhancement scanning was performed in 26 cases.MRI findings and the time-intensity curve of dynamic contrast enhancement were analyzed.Results Among the 32 choroidal hemangiomas, 26 of them were at the temporal side of optic disc and 28 lesions were fusiform.Before enhancement, 23 lesions showed isointense T1-weighted signal and 31 lesions were isointense on T2-weighted imaging.All the lesions showed strong enhancement on postcontrast T1-weighted imaging, including 31 homogenously enhanced lesions and one heterogeneously enhanced lesion.Retinal detachments were found in 18 eyes.Fill-in sign were observed in 12 lesions during dynamic contrast enhancement.The time-intensity curve of dynamic contrast enhancement in 28 lesions suggested a pattern with rapid enhancement and slow washout, time to peak (91.00±25.27) s, slope ratio 3.03±1.13, the median of washout ratio 17.06%, enhancement ratio 2.87±0.79.Conclusion MRI showed a few features of the location, shape, signal characteristics, and enhancement pattern in choroidal hemangioma, which may contribut to diagnosis and treatment plan of this disease.  相似文献   

7.
Objective To characterize the regular and the dynamic contrast enhancement MR imaging in choroidal hemangioma.Methods MR imaging findings of 30 cases (31eyes, 32 lesions) with choroidal hemangioma confirmed by follow-up results were retrospectively analyzed.Among them, postcontrasted T1-weighted imaging was performed in 30 patients and dynamic contrast enhancement scanning was performed in 26 cases.MRI findings and the time-intensity curve of dynamic contrast enhancement were analyzed.Results Among the 32 choroidal hemangiomas, 26 of them were at the temporal side of optic disc and 28 lesions were fusiform.Before enhancement, 23 lesions showed isointense T1-weighted signal and 31 lesions were isointense on T2-weighted imaging.All the lesions showed strong enhancement on postcontrast T1-weighted imaging, including 31 homogenously enhanced lesions and one heterogeneously enhanced lesion.Retinal detachments were found in 18 eyes.Fill-in sign were observed in 12 lesions during dynamic contrast enhancement.The time-intensity curve of dynamic contrast enhancement in 28 lesions suggested a pattern with rapid enhancement and slow washout, time to peak (91.00±25.27) s, slope ratio 3.03±1.13, the median of washout ratio 17.06%, enhancement ratio 2.87±0.79.Conclusion MRI showed a few features of the location, shape, signal characteristics, and enhancement pattern in choroidal hemangioma, which may contribut to diagnosis and treatment plan of this disease.  相似文献   

8.
Objective To characterize the regular and the dynamic contrast enhancement MR imaging in choroidal hemangioma.Methods MR imaging findings of 30 cases (31eyes, 32 lesions) with choroidal hemangioma confirmed by follow-up results were retrospectively analyzed.Among them, postcontrasted T1-weighted imaging was performed in 30 patients and dynamic contrast enhancement scanning was performed in 26 cases.MRI findings and the time-intensity curve of dynamic contrast enhancement were analyzed.Results Among the 32 choroidal hemangiomas, 26 of them were at the temporal side of optic disc and 28 lesions were fusiform.Before enhancement, 23 lesions showed isointense T1-weighted signal and 31 lesions were isointense on T2-weighted imaging.All the lesions showed strong enhancement on postcontrast T1-weighted imaging, including 31 homogenously enhanced lesions and one heterogeneously enhanced lesion.Retinal detachments were found in 18 eyes.Fill-in sign were observed in 12 lesions during dynamic contrast enhancement.The time-intensity curve of dynamic contrast enhancement in 28 lesions suggested a pattern with rapid enhancement and slow washout, time to peak (91.00±25.27) s, slope ratio 3.03±1.13, the median of washout ratio 17.06%, enhancement ratio 2.87±0.79.Conclusion MRI showed a few features of the location, shape, signal characteristics, and enhancement pattern in choroidal hemangioma, which may contribut to diagnosis and treatment plan of this disease.  相似文献   

9.
Objective To characterize the regular and the dynamic contrast enhancement MR imaging in choroidal hemangioma.Methods MR imaging findings of 30 cases (31eyes, 32 lesions) with choroidal hemangioma confirmed by follow-up results were retrospectively analyzed.Among them, postcontrasted T1-weighted imaging was performed in 30 patients and dynamic contrast enhancement scanning was performed in 26 cases.MRI findings and the time-intensity curve of dynamic contrast enhancement were analyzed.Results Among the 32 choroidal hemangiomas, 26 of them were at the temporal side of optic disc and 28 lesions were fusiform.Before enhancement, 23 lesions showed isointense T1-weighted signal and 31 lesions were isointense on T2-weighted imaging.All the lesions showed strong enhancement on postcontrast T1-weighted imaging, including 31 homogenously enhanced lesions and one heterogeneously enhanced lesion.Retinal detachments were found in 18 eyes.Fill-in sign were observed in 12 lesions during dynamic contrast enhancement.The time-intensity curve of dynamic contrast enhancement in 28 lesions suggested a pattern with rapid enhancement and slow washout, time to peak (91.00±25.27) s, slope ratio 3.03±1.13, the median of washout ratio 17.06%, enhancement ratio 2.87±0.79.Conclusion MRI showed a few features of the location, shape, signal characteristics, and enhancement pattern in choroidal hemangioma, which may contribut to diagnosis and treatment plan of this disease.  相似文献   

10.
Objective To characterize the regular and the dynamic contrast enhancement MR imaging in choroidal hemangioma.Methods MR imaging findings of 30 cases (31eyes, 32 lesions) with choroidal hemangioma confirmed by follow-up results were retrospectively analyzed.Among them, postcontrasted T1-weighted imaging was performed in 30 patients and dynamic contrast enhancement scanning was performed in 26 cases.MRI findings and the time-intensity curve of dynamic contrast enhancement were analyzed.Results Among the 32 choroidal hemangiomas, 26 of them were at the temporal side of optic disc and 28 lesions were fusiform.Before enhancement, 23 lesions showed isointense T1-weighted signal and 31 lesions were isointense on T2-weighted imaging.All the lesions showed strong enhancement on postcontrast T1-weighted imaging, including 31 homogenously enhanced lesions and one heterogeneously enhanced lesion.Retinal detachments were found in 18 eyes.Fill-in sign were observed in 12 lesions during dynamic contrast enhancement.The time-intensity curve of dynamic contrast enhancement in 28 lesions suggested a pattern with rapid enhancement and slow washout, time to peak (91.00±25.27) s, slope ratio 3.03±1.13, the median of washout ratio 17.06%, enhancement ratio 2.87±0.79.Conclusion MRI showed a few features of the location, shape, signal characteristics, and enhancement pattern in choroidal hemangioma, which may contribut to diagnosis and treatment plan of this disease.  相似文献   

11.
A case of Marchiafava-Bignami disease (MBD) is presented using magnetic resonance imaging (MRI). A patient with a long history of alcoholism developed a gait disturbance with involuntary movements at the lower extremities. MRI scans taken at the onset showed no particular abnormalities. He progressed to a coma 10 days later. MRI scans taken 20 days after the onset showed a focal lesion at the genu of the corpus callosum and he was diagnosed as having MBD. In addition, multiple lesions were observed in bilateral frontoparietal subcortical white matter. These lesions demonstrated similar intense MRI signals as the corpus callosum.  相似文献   

12.

Objective

The purpose of this study was to explore brain MRI findings in neuromyelitis optica (NMO) and to investigate specific brain lesions with respect to the localization of aquaporin-4 (AQP-4).

Materials and methods

Forty admitted patients (36 women) who satisfied the 2006 criteria of Wingerchuk et al. for NMO were included in this study. All patients received a neurological examination and MRI scanning including brain and spinal cord. MRIs were classified as normal, nonspecific, multiple sclerosis-like, typical abnormalities. MS-like lesions were too few to satisfy the Barkhof et al. criteria for MS. Confluent lesions involving high AQP-4 regions were considered typical. Non-enhancing deep white matter lesions other than MS-like lesions or typical lesions were classified as nonspecific.

Results

Brain MRI lesions were delineated in 12 patients (25%). Four patients (10%) had hypothalamus, brainstem or periventricle lesions. Six (15%) patients were nonspecific, and 2 (5%) patients had multiple sclerosis-like lesions.

Conclusion

Brain MRIs are negative in most NMO, and brain lesions do not exclude the diagnosis of NMO. Hypothalamus, brainstem or periventricle lesions, corresponding to high sites of AQP-4 in the brain, are indicative of lesions of NMO.  相似文献   

13.
目的:分析放射性脑损伤的MRI表现及MRI诊断的意义。方法:回顾性分析25例放射性脑损伤患者的MRI资料,观察并比较病变的部位、范围及增强表现。结果:急性放射性脑损伤1例,深部脑白质及胼胝体压部对称性长T2信号,脑白质和胼胝体压部肿胀,增强扫描无强化;早期迟发性放射性脑损伤18例,深部脑白质对称性长T2信号,增强扫描无强化;晚期迟发性放射性脑损伤6例,双侧颞叶不均匀长T1长T2信号,增强扫描呈明显花环样强化。结论:放射性脑损伤具有特征性MRI表现,MRI对诊断放射性脑损伤具有重要价值。  相似文献   

14.
目的研究颅脑创伤后持续植物状态(PVS)的磁共振影像(MRI)特征。方法分析36例颅脑创伤后持续植物状态患者MRI资料,按病灶位置、性质大小进行统计处理。结果所有患者都有脑实质损害,共发现脑软化灶312处.平均每例8.67处,主要分布在大脑皮层、脑室旁白质、基底节、胼胝体、脑干。结论MRI检查对颅脑创伤后持续植物状态的诊治有重要意义。  相似文献   

15.
目的探讨视神经脊髓炎(neuromyelitis optica,NMO)患者脑部及脊髓MRI影像学表现及特征。方法回顾性分析符合2006年Wingerchuk诊断标准23例NMO患者的脑部、脊髓MRI表现,分析NMO患者脑部及脊髓病灶的分布及信号特点。结果典型的NMO脑内病灶位于脑室室管膜下、下丘脑及脑干等部位,表现为斑点、斑片及线状异常信号。5例脑部增强检查均未见异常强化病灶。NMO患者脊髓常呈长节段横贯性或次横贯性损伤,且以脊髓中央灰质受累为主,可见线样征。结论 NMO患者脊髓及脑部MRI出现异常信号有较特异的好发部位,病灶的分布有其自身特征。  相似文献   

16.
目的 探讨成人起病的脑白质病伴轴索球样变和色素胶质细胞(ALSP)的临床及影像特征,以期提高对该病的认识及对早期影像的诊断能力。 方法 回顾性分析3例经临床诊断的ALSP病人,分析其临床表现、神经影像学特征和基因检查结果,并行文献复习。 结果 ALSP病人的临床表现多样,3例病人均有认知障碍和锥体束征,存在精神行为异常和帕金森病样表现者2例,存在癫痫、共济失调和步态异常者2例。MRI显示3例病人双侧侧脑室旁和深部白质多发病变,呈额、顶叶分布,可累及胼胝体;2例累及锥体束,均未累及脑干;3例病变均呈不对称分布,其中2例病灶呈大片状、融合分布;3例均未见病灶强化及皮质下U纤维受累;除白质病变外,3例病人均可见侧脑室扩大和病变区扩散加权成像(DWI)点片状高信号,2例病人伴脑叶萎缩。2例病人CT伴点状微小钙化灶。2例病人基因诊断发现CSF1R突变。3例病人实验室检查均未见明显异常。 结论 中青年ALSP病人可表现为认知障碍,影像表现具有一定特征,可见以额顶叶受累为主的白质异常信号,伴受累部位脑叶萎缩、胼胝体萎缩,DWI上呈高信号和/或点状钙化,结合临床、影像特征及基因检查可考虑本病。  相似文献   

17.
苯丙酮尿症的颅脑MRI研究   总被引:7,自引:0,他引:7  
目的 分析苯丙酮尿症颅脑MRI异常表现。方法 用0.5T超导磁共振机对临床检查和生化测定已确诊的73例苯丙酮尿症患儿头颅进行扫描。结果 MRI发现异常者71例。T2WI上脑白质内散在斑片状高信号灶最为常见(69例,94.5%);沿胼胝低向前后延伸并向双侧放射冠和半卵圆中心白质对称性扩展的弥漫性主信号病灶在T2WI上也很常见(51例,69.9%);MRI还发现无胼胝体(3例,4.1%)、胼胝体发育不  相似文献   

18.
 目的 探讨急性原发性胼胝体变性的MRI表现,提高对该病的认识和诊断水平。方法 回顾性分析2016-01至2017-05经临床证实的急性原发性胼胝体变性31例患者的临床资料及影像资料。结果 急性原发性胼胝体变性多见于胼胝体压部 (13/31, 41.9%),MRI示呈等长T1、长T2信号改变,DWI呈高信号及稍高信号。20例出现胼胝体以外脑实质病变,多见于半卵圆中心(20/31, 64.5%)和额顶叶白质(18/31, 58.1%)。结论 MRI能清晰显示胼胝体及以外病灶,影像表现具有一定的特异性,MRI对其早期诊断、治疗及预后具有重要价值,可作为临床首选检查。  相似文献   

19.
脑脂肪栓塞的MRI及CT诊断   总被引:7,自引:0,他引:7  
目的 总结脑脂肪栓塞(CFE)的临床及MRI及CT的影像特点。方法 分析3例急性CFE的临床表现、影像特点。结果 (1)3例均为长骨骨折,在外伤后或骨折固定、复位数小时后突发精神状态改变。(2)醒状昏迷是主要临床表现。(3)3例患者MRI能明确显示病灶,1例CT显示了病灶。(4)MRI、CT显示脑内病灶均呈基本对称性分布,为边缘模糊的点、片状长T1、长T2信号,CT呈低密度。病灶均累及脑干、分水岭区脑白质、基底节区、胼胝体压部。2例病灶累及小脑。(5)1例患者发病康复治疗3个月后MRI复查示脑内病灶完全消失。结论 急性脑脂肪栓塞的临床及MRI、CT影像改变具有特征性,MRI在病灶显示上优于CT。  相似文献   

20.
佩梅病的头颅MRI表现及其与临床、基因分型的关系   总被引:2,自引:0,他引:2  
目的 探讨佩梅病( PMD)患儿的头颅MRI特点,以及与临床及基因分型的关系.方法 回顾性分析16例经临床诊断为佩梅病患儿的临床和影像资料.患儿均为男性,年龄5个月至9岁8个月.由儿科神经医师对患儿的症状和体征进行检查,将其按照临床症状进行临床分型.由影像科医师对头颅MRI图像的特点进行分析,病灶的位置包括苍白球、锥体束、胼胝体、小脑白质、半卵圆中心,判断大、小脑是否萎缩、是否有“豹纹征”.结果 临床诊断经典型佩梅病8例,中间型7例,先天型1例.16例佩梅病患儿MRI均以广泛的脑白质髓鞘化延迟为特点,病灶累及苍白球13例、锥体束7例、胼胝体11例、小脑白质7例、半卵圆中心12例、脑萎缩5例、小脑萎缩1例,5例有“豹纹征”.锥体束和小脑白质受累在点突变患儿中多见;临床症状较重的中间型及先天型患儿出现小脑白质病变频率较高;“豹纹征”多见于经典型患儿,提示患儿的髓鞘化程度相对较高.结论 佩梅病患儿的头颅MRI具有典型的影像特点,其影像学表现与临床分型和基因突变类型有一定的相关性.  相似文献   

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