人类Creutzfeldt-Jakob病临床进展

Creutzfeldt—Jakob病(克一雅氏病)是较为罕见的中枢神经系统变性疾病，其发病率约为每年每百万人口1～2例。现被证实它是由一种传染性朊蛋白所造成的。该病已经有很长的历史了。早在1920年，Creutzfeldt和Jakob两位医生就报道共6例不明原因的进行性痴呆，肌阵挛，小脑共济失调的病人，他们病程多短于1年。后经病理解剖发现这6例病人大脑都有不同程度的海绵状变的共同病理改变。     

MR DWI与常规MRI对Creutzfeldt-Jakob病的诊断价值比较

目的比较MR扩散加权像(DWI)与常规MRI对Creutzfeldt-Jakob病(CJD)的诊断价值。方法总结我院10例散发性CJD患者的临床资料及MRI资料,比较DWI与常规MRI结果。结果 T1WI上仅1例显示两侧额叶大脑皮层带状稍低信号;T2WI上2例显示双侧大脑皮层带状高信号,伴或不伴基底节稍高信号,1例显示脑干、小脑、两侧基底节、两侧丘脑高信号,1例仅双侧基底节高信号;液体衰减反转恢复(FLAIR)序列4例显示双侧大脑皮层带状高信号,其中3例伴基底节高信号;1例显示脑干、小脑、两侧基底节、两侧丘脑高信号;1例仅显示两侧基底节高信号。5例6次增强扫描均未见明显异常强化。5例行DWI,其中4例示双侧大脑皮层带状高信号,伴或不伴基底节高信号;1例仅表现为两侧基底节高信号。病灶部位平均表观扩散系数(ADC)值为0.687×10-3mm2/s,较正常脑实质明显减低。结论 DWI显示CJD病变优于常规MRI,可为CJD的早期发现、早期诊断提供有价值的信息。     

MRI诊断Creutzfeldt-Jakob病1例

患者男,63岁。因行走不稳伴记忆力下降月余,加重10天入院。患者1月前无诱因感行走不稳,并感近期记忆力减退,当时未引起重视,病情呈进行性加重,半月后出现行走蹒跚,动作迟缓,反应迟钝,懒言少语,理解力、记忆力均有不同程度下降,空间定向能力降低,偶有肢体阵发性抽搐。MRI检查：两侧大脑半球皮质区及右侧尾状核头部稍肿胀,呈稍长T1长T2信号（图1）,Flair序列呈稍高信号,DWI序列显示额顶枕叶脑回样明显高信号,以右侧为主,且右侧尾状核头部亦表现为高信号（图2）,增强扫描后病灶未见强化,结合临床,MRI诊断：考虑为Creutzfeldt-Jakob病（皮质-纹状体-脊髓变性）可能性大。     

以皮质盲为首发症状的散发型Creutzfeldt-Jakob病1例

患者男性,66岁。因“双眼视物模糊2月余,右侧肢体无力半月”,于2012年8月29日入院。患者于2月前无明显诱因下出现双眼视物模糊,休息后无法缓解,无畏寒发热,无意识障碍,无恶心、呕吐,无四肢抽搐,无大小便失禁,在当地医院就诊,诊断为“双眼目系卒病,双眼球后视神经炎,双眼白内障”,予住院治疗,期间予“弥可保,强的松”等药物治疗。治疗半月后症状无明显好转,遂来我院就诊,门诊拟“脑卒中”收住入院。既往史：患者平素体键,否认“糖尿病、高血压“等病史,否认”肝炎、结核”等病史。否认有外伤及手术史。家族史：否认两系三代以内家族性传染病、遗传病,否认家族中有类似病史。入院查体：血压：120／80mmHg ,神志清,GCS评分15分,双眼瞳孔等大等圆,直径3mm ,对光反射灵敏,颈软,无抵抗,胸廓挤压痛阴性,腹平软,右侧肢体肌力 IV级,感觉减退,肌张力无异常,双侧巴氏征阳性。辅助检查：头颅CT ：颅内未见异常。查肝功能：直接胆红素10．8umol／L ,谷氨酰转肽酶40IU／L ;神经元特异性烯醇化酶26．59ng／ml ,D二聚体定量1050ug／L ,全血低切相对指数16．00。脑电图：轻中度异常,与疾病有关。超声检查心脏：左室舒张功能减退。超声检查血管：双侧颈部动脉内膜增厚。心电图：窦性心律,正常范围心电图。头颅M RI检查：双侧枕顶叶脑回表面多发T1WI低信号,T2WI高信号,脑沟增深加宽,上述病变在FLAIR及DWI上明显高信号（图1～4）,考虑炎症。予阿昔洛韦针抗病毒、醒脑静针保护脑细胞对症支持治疗一周后,症状无明显好转。且近来出现缄默、智能减退明显,反应较迟钝,回答不切题或回答极慢,语言欠流利,与患者接触时可见患者时有惊慌表情。患者双侧上肢出现肌阵挛征象。复查脑电图     

Stewart-Treves 综合征的 MRI 表现

目的：探讨 MRI 对 Stewart-Treves 综合征(STS)的诊断价值。方法回顾性分析48枚 STS 肿物的 MRI 表现,并与病理结果对照分析。结果48枚肿物均为实性,直径≥4 mm,根据 D2-40、CD31的表达结果将 STS 分为2型,STS-Ⅰ型(即血管肉瘤)47枚结节肿物,MRI 表现：T1 WI 呈稍低信号,T2 WI 呈稍高信号,与同层肌肉比较,结节信号明显增高,增强检查呈明显强化、且均匀。STS-Ⅱb 型(混合型淋巴管肉瘤)1枚肿块样肿物;MRI 表现：T1 WI、T2 WI 序列均呈高低混杂信号,以高信号为主,强化程度较低且不均匀。结论MR 能显示 STS 特征性信号表现,结合患者肢体慢性淋巴水肿病史,可以提高术前正确诊断率。     

特纳氏综合征儿童垂体MRI形态学表现

目的 探讨特纳氏综合征患儿MRI垂体形态学表现.方法 回顾性分析13例临床已确诊为特纳氏综合征的儿童垂体MRI的形态学表现.结果 13例中9例>10岁者垂体上缘呈对称性隆起,高7.1～10.3 mm,4例垂体明显均匀强化;4例<12岁者垂体形态正常.结论 MRI显示特纳氏综合征患儿青春期前垂体形态基本正常,进入青春期年龄后垂体呈病理性增生.     

尺骨撞击综合征的MRI表现

目的 分析尺骨撞击综合征引发的月骨和三角骨缺生坏死的ＭＲＩ表现,以确定定其像学特征。方法 对２３例尺侧腕痛病例行Ｘ线测量及ＭＲＩ检查。结果 尺骨阳性变异范围在１～３．８ｍｍ,平均２．１ｍｍ,阴性变异范围０．５～２．２ｍｍ,平均１．８ｍｍ。尺骨阳性变异１３例,伴月骨和三角骨坏死９例,阴性变异６例,伴月骨地不死２例;中性变异４例,伴月骨和三角骨坏死２例,ＭＲＩ示坏死区位于月骨尺侧部分近端和三角骨腰部。     

脑型肺吸虫病的MRI表现

目的 :探讨脑型肺吸虫病的MRI表现特征及其诊断价值。方法 :搜集 3例经临床治疗和手术病理证实的病例 ,回顾性分析MRI影像表现特征及其与肺吸虫病发病机制的关系。 3例均行常规SE序列MRI扫描 ,1例复查扫描共计 3次 ,1例行Gd DTPA增强扫描。结果 :3例患儿发病年龄为 6～ 9岁 ,肺吸虫抗原皮下试验均为强阳性。所有扫描病例共计 14个病灶 ,10个病灶位于大脑 ,4个位于脑干和小脑部位。MRI表现为囊状和片絮状长T1长T2 信号 ,囊状病变囊壁呈短T1短T2 出血信号 ,1例显示特征性的“隧道征”。增强扫描有一定的意义 ,可显示囊壁无出血的病灶。结论 :脑型肺吸虫病多发生于儿童 ,MR表现相对较为典型 ,出血信号及“隧道征”具有特征性 ,MRI诊断该病具有重要价值。     

艾滋病痴呆综合征的MRI表现

目的 分析艾滋病痴呆综合征(ADC)的MRI特点,提高对本病的认识.方法 回顾性分析35例ADC患者的临床及MRI资料. 结果 35例ADC患者中有31例脑组织总量减少,皮层及基底神经节弥漫性萎缩,脑沟增宽,脑室扩大;25例侧脑室周围白质信号异常;3例呈单纯脑萎缩.结论 ADC具有特征性的MRI表现,大部分患者出现脑萎缩征象,脑沟增宽,脑室扩大;侧脑室周围白质及半卵圆中心信号异常.结合临床病史,MRI检查对本病的早期诊断有重要意义.     

单囊型脑囊虫病的MRI表现

目的:探讨MRI对单囊型脑囊虫病的诊断价值.方法:回顾性分析15例经手术病理证实的单囊型脑囊虫病的MRI表现.结果:本组15例中7例位于脑实质,7例位于脑室,1例位于脑池;病灶形态尚规则,多呈圆形或类圆形(n=14),少数可见浅分叶(n=1);囊肿最大径为13～56mm,平均28mm;15例常规MRI平扫均呈长T1、长...     

MRI in sporadic Creutzfeldt-Jakob disease: Correlation with clinical and neuropathological data

To ascertain whether increased grey matter signal intensity on T2-weighted images in patients with sporadic Creutzfeldt-Jakob disease (CJD) corresponds to the stage and severity of this disease, we correlated MRI findings in four of our own and previously reported patients with sporadic CJD with the clinical variants, neuropathological changes at autopsy, duration of the disease and survival time after MRI examination. Of 15 patients with the extrapyramidal type of CJD, 10 showed increased signal in the basal ganglia on T2-weighted images. One of seven patients with the Heidenhain variant had increased signal in the occipital cortex. Patients without increased grey matter signal intensity had a longer overall duration of CJD (P = 0.035). Although the interval between onset of neurological symptoms and MRI was not different, patients without increased grey matter signal also survived longer after MRI examination (P = 0.022). Received: 21 February 1997 Accepted: 4 June 1997     

MRI abnormalities in Creutzfeldt-Jakob disease

Two patients with biopsy-proven Creutzfeldt-Jakob disease had MRI studies that revealed increased signal in the basal ganglia on T2-weighted images, suggesting that MRI can be a useful diagnostic instrument in Creutzfeldt-Jakob disease.     

MRI evidence of cerebellar and hippocampal involvement in Creutzfeldt-Jakob disease

We report a 51-year-old woman with the Brownell-Oppenheimer (cerebellar) variant of Creutzfeldt-Jakob disease (CJD). She had the typical findings of bilateral basal ganglion changes on MRI, as well as changes in the cerebellum and hippocampus. This case adds further information to the known imaging characteristics of CJD. Received: 29 November 2000/Accepted: 11 January 2001     

MR扩散加权成像对Creutzfeldt-Jakob病的诊断意义

目的评价MR扩散加权像(DWI)对Creutzfeldt-Jakob病(CJD)的诊断价值。方法8例散发性CJD(4例确诊，3例临床很可能，1例临床可能)，比较其常规MRI及DWI检查结果。结果T1WI及LWI除4例显示脑萎缩外，未见异常信号；而8例DWI均异常，其中2例为单纯大脑皮层高信号改变，6例为大脑皮层合并尾状核、壳核高信号改变，5例呈对称性，3例呈非对称性；1例液体衰减反转恢复(FLAIR)序列成像显示大脑皮层呈稍高信号，但不如DWI明显。结论DWI显示的大脑皮层和(或)纹状体的高信号改变是CJD的特征之一，其诊断价值明显优于常规MRI，是早期诊断CJD的重要方法。     

CT and MRI in iatrogenic and sporadic Creutzfeldt-Jakob disease: as far as imaging perceives

Creutzfeldt-Jakob Disease (CJD), an invariably fatal dementing illness, affects patients in middle and old age (sporadic form). However, the association of CJD with certain treatments (iatrogenic form) has been described in younger patients. The clinical onset of the two forms seems to differ; in the iatrogenic form a high frequency of the ataxic CJD variant has been reported. Nowadays, a definitive diagnosis of CJD is exclusively histological. We present five cases of CJD, one sporadic and the others iatrogenic, following dura mater grafts and analyse their CT and MRI features. CT typically demonstrates brain atrophy, generally progressive, but in sporadic CJD midfield MRI also showed abnormal signal, with predominant deep grey matter involvement. The use of narrow windows with proton-density sequences may reveal subtle cortical signal abnormalities not clearly visible with conventional windows. The early demonstration of these changes, in the appropriate clinical context, may suggest CJD and this supports the use of mid- or high magnetic fields in the diagnosis of CJD and other forms of dementia. In our cases of iatrogenic CJD, low-field MRI did not reveal more than the progressive atrophy displayed by CT, and raises the question on the one hand of possible differences, based on imaging, from the sporadic form, and on the other of the lack of sensitivity of low-field magnets to signal changes in CJD.Presented in part at the 6th annual meeting of the Sociedad Ibero-Latinoamericana de Neuroradiología Diagnóstica y Terapéutica (SILAN) Madrid, June 1994     

New MRI findings in Creutzfeldt-Jakob disease: high signal in the globus pallidus on T 1-weighted images

We report a 49-year-old woman with Creutzfeldt-Jakob disease (CJD). In addition to typical high-signal lesions on proton-density and T 2-weighted images there was high signal in the globus pallidus bilaterally on T 1-weighted images. The latter feature has not been described previously and probably due to deposition of prion protein, as found at autopsy. Received: 15 July 1998 Accepted: 28 September 1998     

Serial MR imaging in Creutzfeldt-Jakob disease

Summary Serial magnetic resonance (MR) imagings of two autopsied patients with Creutzfeldt-Jakob disease (CJD) are presented. Both patients showed a dramatic progression of brain atrophy. The initial MR imagings were, however, interpreted as normal except for localized mild cortical atrophy in one patient. When a normal MR image is obtained in a demented middle-aged or aged patient, CJD may still need to be ruled out: follow up MR imaging may be useful.     

Discordance of motion artifacts on magnetic resonance imaging in Creutzfeldt-Jakob disease: comparison of diffusion-weighted and conventional imaging sequences

Purpose Motion artifact is problematic in the diagnosis of Creutzfeldt-Jakob disease (CJD) because of dementia. The purpose was to compare the occurrence of this artifact between a diffusion-weighted (DW) magnetic resonance (MR) imaging sequence and conventional sequences. Materials and methods Ten MR examinations comprising T2-weighted, T1-weighted, DW, and fluid-attenuated inversion recovery imaging in seven CJD patients were retrospectively evaluated. The occurrence of motion artifacts on each sequence were assessed, and the examination was classified into four groups as follows: group A, motion artifact not revealed on DW imaging but revealed on one or more other sequences; group B, revealed on DW imaging and one or more other sequences; group C, not revealed on any sequences; and group D, revealed on DW imaging but not on any other sequences. Results The 10 MR examinations were classified as eight group A (80%), one B (10%), one C (10%), and zero D (0%). Conclusion Motion artifacts are likely to occur in any conventional imaging sequences in CJD, but the fast-imaging ability of DW imaging can reduce this artifact. The combination of an absence of motion artifact on DW imaging and the presence on conventional sequences may be one of the frequent findings of CJD.     

平山病的MRI诊断

目的 探讨平山病低位颈髓在自然位和过屈位特殊的动态变化对诊断的价值。方法 对18例临床确诊为平山病的患者和31例正常对照者进行MR自然位和过屈位的矢状面和轴面扫描，测量C6椎体上缘颈髓前后径（APD）和横径（TD），观察其动态改变。结果 （1）自然位，平山病组表现有低位颈髓萎缩（病例组11例，对照组2例，X^2=14．76，P=0．000）和变扁平（病例组7例，对照组1例，X^2=8．15，P=0．004），差异有统计学意义。（2）过屈位，平山病组颈髓均前移后显著变扁，脊膜后有“月牙”形异常信号影。（3）自然位平山病组和对照组APD分别为（5．6±0．7）、（6．7±0．5）mm，过屈位APD分别为（4．3±0．8）、（6．0±0．5）mm，差异均有统计学意义（t值分别为-5．802、-8．952，P值均为0．000）。结论 平山病低位颈髓在正常位和过屈位存在特殊的动态变化，过屈位MRI可协助诊断。     

韧带样纤维瘤病MRI特征表现及其病理基础

目的：探讨韧带样纤维瘤病（DF）的MRI特点,重点分析MRI特征影像及其病理基础,评价MRI在DF诊断中的价值。方法回顾性分析经手术病理证实14例韧带样纤维瘤病的MRI表现（14个病灶均行M RI增强及延迟扫描）,与术后病理结果进行比较,并探讨M RI特征影像的病理基础。结果14例均为单发病灶（腹外型7例、腹壁型4例、腹内型3例）,其中有2例为术后复发,肿瘤沿着肌肉长轴生长。T1 WI呈等或稍低于肌肉的信号,T2 WI呈高于肌肉信号,增强扫描病灶均呈不均匀强化,以延迟强化为明显,病灶内可见条状或带状 T1 WI及 T2 WI低信号,增强后未见强化。14例病灶内均未见坏死、囊变及瘤周水肿。病理组织学上肿瘤由不同比例的成纤维细胞、纤维组织和胶原纤维束组成,纤维细胞及成纤维细胞密集区域T2 WI信号增高,而肿瘤内胶原纤维区域在 T1 WI、T2 WI均为低信号。肿瘤内见较多血管壁完整的毛细血管,这是病灶延迟强化的病理基础。结论病灶内可见T1 WI、T2 WI均呈带状低信号且增强扫描不强化以及病灶延迟强化是DF的特征性MRI表现。MRI可以很好评估肿瘤侵犯范围及其与周围结构的关系,有助于DF的诊断和鉴别诊断。