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     

散发型Creutzfeldt-Jakob病的MRI表现

目的探讨散发型Creutzfeldt-Jakob病（sCJD）的MR特点。方法回顾性分析3例临床诊断为sCJD的患者资料，MR采用SE T1 WI、快速自旋回波（FSE）T2 WI、扩散加权成像（DWI）扫描，观察其MR表现特征。结果3例SE TI WI和FSE T2 WI序列对基底节区和皮质的病变显示不佳，DWI则可清晰地显示病变，额、顶、枕叶皮质最常受累，表现为高信号，病变可对称，也可不对称，皮层下区脑白质信号未见异常。双侧尾状核、丘脑也可受累，DWI上呈高信号。晚期脑实质广泛萎缩，以皮质为著。结论sCJD采用DWI序列结合其特征性的临床表现可作出较为准确的诊断。     

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     

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     

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.     

Radiological assessment of Creutzfeldt-Jakob disease

Creutzfeldt-Jakob disease is a rare fatal neurodegenerative disorder, characterized by rapidly progressive dementia and neurological signs. There is a need for early and accurate clinical diagnosis in order to exclude any treatable disorder. Additionally, it is of public interest to differentiate the sporadic form of the disease from the variant CJD type (vCJD), which is probably transmitted from cattle infected with bovine spongiform encephalopathy (BSE). High signal in the striatum on T2-weighted, FLAIR and diffusion weighted (DW) MRI as well as cortical high signal in FLAIR and DW MRI are the classical findings in sCJD. The "pulvinar sign", defined as high signal in the pulvinar thalami that is brighter than potential additional high signal in the basal ganglia, is considered pathognomonic for vCJD.     

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.     

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。     

Serial MRI in early Creutzfeldt-Jacob disease with a point mutation of prion protein at codon 180

We report a 66-year-old woman with histologically diagnosed Creutzfeldt-Jacob disease (CJD), followed with MRI from an early clinical stage. MRI demonstrated expansion of the high cortical signal on T2-weighted images, which differs from previous MRI reports of CJD. This patient followed an atypical clinical course: 16 months had passed before she developed akinetic mutism, and periodic sharp waves had not been detected on EEG after 2 years in spite of her akinetic mutism. Brain biopsy showed primary spongiform changes in the grey matter, and a point mutation of the prion protein gene at codon 180 was discovered using polymerase chain reaction direct sequencing and Tth 111 I cutting. This is the first case with the point mutation of the codon 180 variant with an atypical clinical course and characteristic MRI findings.     

Diffusion-Weighted MR Imaging in Biopsy-Proven Creutzfeldt-Jakob Disease

Objective

To compare conventional and diffusion-weighted MR imaging in terms of their depiction of the abnormalities occurring in Creutzfeldt-Jakob disease.

Materials and Methods

We retrospectively analyzed the findings of conventional (T2-weighted and fluid-attenuated inversion recovery) and diffusion-weighted MR imaging in four patients with biopsy-proven Creutzfeldt-Jakob disease. The signal intensity of the lesion was classified by visual assessment as markedly high, slightly high, or isointense, relative to normal brain parenchyma.

Results

Both conventional and diffusion-weighted MR images demonstrated bilateral high signal intensity in the basal ganglia in all four patients. Cortical lesions were observed on diffusion-weighted MR images in all four, and on fluid-attenuated inversion recovery MR images in one, but in no patient on T2-weighted images. Conventional MR images showed slightly high signal intensity in all lesions, while diffusion-weighted images showed markedly high signal intensity in most.

Conclusion

Diffusion-weighted MR imaging is more sensitive than its conventional counterpart in the depiction of Creutzfeldt-Jakob disease, and permits better detection of the lesion in both the cerebral cortices and basal ganglia.     

Neuroimaging in human prion disease: Searching in the mist

Human prion disease is a rare, uniformly fatal neurodegenerative disorder.Its precise pathogenesis is obscure. The clinical profile of the disease differs among its various forms. There are no definitive diagnostic tests (except for brain biopsy) or proven treatment. To increase the clinical diagnostic sensitivity and specificity, three laboratory tests, including electroencephalogram, cerebrospinal fluid testing for 14-3-3 protein, and magnetic resonance imaging, are currently used. Additionally, proton magnetic resonance spectroscopy, positron emission tomography and single photon emission computed tomography can provide interesting and novel results in the research of human prion disease.     

散发性Creutzfeldt-Jakob病病程不同时期的辅助检查敏感性研究

目的 研究散发性Creutzfeldt-Jakob病(sCJD)患者在病程的不同时期时各项辅助检查手段的敏感性.方法 回顾性分析53例sCJD患者的临床资料,统计病程不同时期头颅核磁弥散加权像(DWI)、24h动态脑电图(EEG)、脑脊液14-3-3蛋白及正电子发射计算机断层显像(PET-CT)的敏感性.在计算某一检测手段的敏感性时,以特异性临床表现结合2项或以上辅助检查阳性结果作为诊断的公认标准.结果 在53例sCJD患者中,列入病程早期、中期及晚期统计的例数分别为24、53、22例(其中部分患者跨越2或3个时期).随病程进展,DWI敏感性(早期:58.3％;中期:84.6％;晚期:94.7％、EEG敏感性(早期:45.8％;中期:62.7％;晚期:77.8％、脑脊液14-3-3蛋白的敏感性(早期:l1.1％;中期:52.9％)及PET-CT的敏感性(早期:80％;中期:100％)均逐渐增高.除晚期未行PET-CT检查外,其他各期PET-CT的敏感性均优于其他辅助检查.结论 sCJD患者在病程不同时期各辅助检查的敏感性不同,在病程不同阶段进行多次复查可提高诊断的敏感性.PET-CT检查的敏感性较高,结合其他检查手段诊断sCJD可起到较为关键的作用.     

Castleman's disease in the neck: MRI

We describe a case of Castleman's disease in a 8-year-old-boy who presented with a slowly growing mass in the upper neck. MRI showed a well-defined homogeneous mass with nonspecific signal features. Unusual features were a kidney-bean shape and the deep location in the suprahyoid region, the mass originating in the left retropharyngeal space. Received: 7 August 1996 Accepted: 9 October 1996     

MRI in acute neuropathic Gaucher's disease

We present the cranial MRI findings in a 6-month-old girl with biopsy-proven acute neuropathic Gaucher's disease, which include unilateral cerebral atrophy and dural thickening with contrast enhancement. Received: 4 March 1999/Accepted: 18 March 1999     

Lyme disease in a child presenting with bilateral facial nerve palsy: MRI findings and review of the literature

We report a 7-year-old boy with neuroborreliosis presenting with headache and bilateral facial nerve palsy. MRI demonstrated tentorial and bilateral facial and trigeminal nerve enhancement. Received: 13 February 1998 Accepted: 10 April 1998     

脑胶质肉瘤的MRI表现及临床病理对照研究

目的 评价MRI对脑胶质肉瘤的诊断价值. 资料与方法 经病理证实的脑胶质肉瘤8例,回顾分析其MRI表现及病理学表现. 结果 病灶基本上表现为长T1 、长T2信号,边界不清,多数形态不规则,伴有囊变和坏死,个别可见瘤内出血,生长具有侵袭性,少数为结节状.周围脑组织水肿较轻,占位效应明显.增强扫描7例明显强化,1例轻中度强化,5例呈不规则环状强化,2例呈结节状强化,3例内部可见丝瓜瓤样不规则条状强化;2例直接侵犯邻近脑叶,3例发现瘤内出血,5例发生于脑凸面,1例发生于深部脑白质,1例发生于左侧侧脑室. 结论 脑胶质肉瘤通常发生于额、顶叶脑凸面,MRI能够清楚地显示病变及其边界,但信号改变易与多形胶质母细胞瘤、恶性胶质瘤、转移瘤等相混淆.确诊仍依赖于组织病理学诊断.     

小儿重症手足口病并发脑炎的MRI表现

目的:探讨小儿重症手足口病(HFMD)并发脑炎的MRI表现.方法:回顾性分析24例小儿手足口病并发脑炎的MRI表现.结果:24例小儿脑部病变共69处病灶.病变多发13例,对称性分布10例,非对称性3例;多发病灶以脑白质病变为主,共37处病变;病变单发11例,其中累及丘脑2例,累及基底节区4例,累及脑干2例,累及颞叶1例...     

Correlation of MRI findings with clinical findings of trochanteric pain syndrome

OBJECTIVE: Greater trochanter pain syndrome due to tendinopathy or bursitis is a common cause of hip pain. The previously reported magnetic resonance (MR) findings of trochanteric tendinopathy and bursitis are peritrochanteric fluid and abductor tendon abnormality. We have often noted peritrochanteric high T2 signal in patients without trochanteric symptoms. The purpose of this study was to determine whether the MR findings of peritrochanteric fluid or hip abductor tendon pathology correlate with trochanteric pain. MATERIALS AND METHODS: We retrospectively reviewed 131 consecutive MR examinations of the pelvis (256 hips) for T2 peritrochanteric signal and abductor tendon abnormalities without knowledge of the clinical symptoms. Any T2 peritrochanteric abnormality was characterized by size as tiny, small, medium, or large; by morphology as feathery, crescentic, or round; and by location as bursal or intratendinous. The clinical symptoms of hip pain and trochanteric pain were compared to the MR findings on coronal, sagittal, and axial T2 sequences using chi-square or Fisher's exact test with significance assigned as p < 0.05. RESULTS: Clinical symptoms of trochanteric pain syndrome were present in only 16 of the 256 hips. All 16 hips with trochanteric pain and 212 (88%) of 240 without trochanteric pain had peritrochanteric abnormalities (p = 0.15). Eighty-eight percent of hips with trochanteric symptoms had gluteus tendinopathy while 50% of those without symptoms had such findings (p = 0.004). Other than tendinopathy, there was no statistically significant difference between hips with or without trochanteric symptoms and the presence of peritrochanteric T2 abnormality, its size or shape, and the presence of gluteus medius or minimus partial thickness tears. CONCLUSIONS: Patients with trochanteric pain syndrome always have peritrochanteric T2 abnormalities and are significantly more likely to have abductor tendinopathy on magnetic resonance imaging (MRI). However, although the absence of peritrochanteric T2 MR abnormalities makes trochanteric pain syndrome unlikely, detection of these abnormalities on MRI is a poor predictor of trochanteric pain syndrome as these findings are present in a high percentage of patients without trochanteric pain.