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     

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     

Short TE Quantitative Proton Magnetic Resonance Spectroscopy in Variant Creutzfeldt-Jakob Disease

Variant Creutzfeldt-Jakob disease (vCJD) is a fatal neurodegerative disorder. Clinical diagnosis is difficult in the early stages as the disease often presents with non-specific psychiatric and neurological symptoms. To investigate the diagnostic potential of quantitative short TE in vivo MRS, and the nature and anatomical distribution of biochemical abnormalities in vCJD, localised single-voxel spectra (TE/TR 30 ms/2,000 ms) were acquired from three brain regions: thalami, caudate nuclei and frontal white matter. Metabolite concentrations and ratios from three patients with definite or probable vCJD were compared with eight normal age-matched controls. Abnormal signal on T2-weighted MRI was apparent in the pulvinar region in all vCJD patients; this region also showed greatly increased myo-inositol [MI] (mean 2.5-fold, P=0.01) and decreased N-acetyl-aspartate (NAA; mean 2-fold, P=0.01). Two patients also showed increased [MI] (z=17, 11; one with decreased NAA, z=-12) in normal-appearing caudate nuclei. The magnitude of metabolite abnormalities in the thalami in moderately advanced vCJD suggests a potential role in earlier diagnosis. Short TE protocols allow the measurement of MI, which adds discriminant power to the MRS examination.     

Computed tomography during Creutzfeldt-Jakob disease

Summary A case of extremely advanced Creutzfeldt-Jakob disease of the cortico-striato-cerebellar type is described. The different stages of the disease are documented by computer-tomography.     

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。     

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.     

Serial computed tomography findings in Creutzfeldt-Jakob disease

Summary Serial CT investigations of 3 patients with histologically confirmed Creutzfeldt-Jakob disease revealed persisting slight brain atrophy to progressive extreme atrophy corresponding to the absolute, not the individual duration of illness. No correlation was observed between CT findings and the patients' condition or electroencephalographic results. In one case with a duration of about 16 months and a terminal brain weight of 750 g a massive bilateral, later unilateral subdural hygroma appeared which probabely was caused by retraction of the brain showing an enormous atrophy.     

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     

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.     

临床很可能诊断的Creutzfeldt-Jakob病分析

目的 探讨Creutzfeldt-Jakob病（CJD）的临床和脑电图演变特征.方法 分析4例CJD临床、脑电图、MRI、CSF资料.结果 4例CJD患者的脑电图特点如下：弥漫性低波幅慢波背景上,间断出现或不对称或在某一局部突出的双相或三相性慢波,逐渐变为双侧广泛同步的周期性三相波,随着病情进展,逐渐变得明显而持续,最终成为持续的电静息.EEG早期异常不典型,需反复检测,方可见周期性三相复合波.结论 充分认识CJD的临床和脑电图演变特点,CJD的EEG演变过程与临床病情变化具有一致性.     

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.     

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。     

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.     

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     

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.     

Radiological assessment of Crohn disease

Fifty-eight consecutive cases of patients affected by Crohn's disease, with ileum (23/58), colon (10/58) and ileo-colic (25/58) involvement were studied. A good overall sensitivity was reached by the radiological procedures employed (barium meal, barium enema, enteroclysis). Enteroclysis is proposed as a second-step method for the study of ileum involvement, because it provides a quite precise assessment of disease stage and extent. Some criteria for a rational use of current radiological procedures in the follow-up of both surgically and medically treated patients are proposed. Moreover it is suggested that better coordination of anatomo-radiological and clinical aspects could improve the therapeutic approach and prognostic judgement in such cases.     

Radiological assessment of aluminium-related bone disease

Two hundred and fifty-nine radiological skeletal surveys were reviewed in 67 cases of end-stage renal failure. Fractures were identified in 16 patients, of whom 12 (17.9% of total) had aluminium-related bone disease. Moderate or severe fracturing osteopathy with more than five fractures not explained by trauma was 100% specific for aluminium intoxication. It is sufficient to perform radiological skeletal surveys in the assessment of renal osteodystrophy annually. They should include radiographs of the fingers, a lateral view of the lumbar spine and oblique views of the ribs. The primary aim of reporting on such surveys should be to grade the severity of fracturing osteopathy and of subperiosteal erosions.     

Imaging dementias

Dementia is the progressive loss of intellectual functions due to involvement of cortical or subcortical areas. Specific involvement of certain brain areas in the different diseases leads to impairment of different functions, e. g., memory, language, visuospatial abilities, and behavior. Magnetic resonance imaging and other neuroradiological studies may indicate which structures are mainly or selectively involved in a demented patient, thus allowing clinical–radiological correlations. Clinical presentation and evolution of the disease, supported by imaging studies, may lead to a highly probable diagnosis. The most common disorders, or the most relevant from the neuroradiological point of view, such as Alzheimer's disease, frontotemporal dementia, vascular dementias, dementia associated with parkinsonism, Huntington's disease, Creutzfeldt-Jakob disease, and normal-pressure hydrocephalus, are briefly discussed. Received: 16 June 2000 Accepted: 4 July 2000     

Regional brain metabolite abnormalities in inherited prion disease and asymptomatic gene carriers demonstrated in vivo by quantitative proton magnetic resonance spectroscopy

Introduction Inherited prion diseases are caused by mutations in the gene which codes for prion protein (PrP), leading to proliferation of abnormal PrP isomers in the brain and neurodegeneration; they include Gerstmann–Sträussler–Scheinker disease (GSS), fatal familial insomnia (FFI) and familial Creutzfeldt–Jakob disease (fCJD).Methods We studied two patients with symptomatic inherited prion disease (P102L) and two pre-symptomatic P102L gene carriers using quantitative magnetic resonance spectroscopy (MRS). Short echo time spectra were acquired from the thalamus, caudate region and frontal white matter, metabolite levels and ratios were measured and z-scores calculated for individual patients relative to age-matched normal controls. MRS data were compared with structural magnetic resonance imaging.Results One fCJD case had generalised atrophy and showed increased levels of myo-inositol (MI) in the thalamus (z=3.7). The other had decreased levels of N-acetylaspartate (z=4) and diffuse signal abnormality in the frontal white matter. Both asymptomatic gene carriers had normal imaging, but increased frontal white matter MI (z=4.3, 4.1), and one also had increased MI in the caudate (z=5.3).Conclusion Isolated MI abnormalities in asymptomatic gene carriers are a novel finding and may reflect early glial proliferation, prior to significant neuronal damage. MRS provides potential non-invasive surrogate markers of early disease and progression in inherited prion disease.     

Rapid echoplanar diffusion imaging in a case of variant Creutzfeldt-Jakob disease; where speed is of the essence

Neuroimaging with magnetic resonance imaging (MRI) is important in the diagnosis of Creutzfeldt-Jakob disease (CJD), but is frequently frustrated by patient movement. Diffusion-weighted imaging (DWI) has previously shown markedly restricted diffusion in grey matter structures of patients with CJD, and may add to diagnostic sensitivity. Echoplanar imaging (EPI) sequences, which are usually used for DWI, are also very rapid, and typically allow imaging of the whole brain in less than 1 min. A case of histologically proven variant CJD (vCJD) in which conventional MRI was difficult to interpret confidently owing to motion artefact, but EPI was diagnostic, emphasises the utility of rapid imaging in agitated patients. Comparison of the regional quantitative apparent diffusion coefficient (ADC) with a control group (n=5) showed restricted diffusion in the caudate (vCJD: 0.63×10^–3 mm²/s; controls: mean 0.722×10^–3 mm²/s, SD 0.017) and lentiform (vCJD: 0.65×10^–3 mm²/s; controls: mean 0.707×10^–3 mm²/s, SD 0.011) nuclei. T2 effects dominated the signal abnormality on DWI in the pulvinar; ADC was increased (vCJD: 0.87–0.95×10^–3 mm²/s; controls: mean 0.773×10^–3 mm²/s, SD 0.038). Our data emphasise variation in diffusion patterns in vCJD, and illustrate the value in using all the components available from the DWI examination for maximum diagnostic information. EPI-DWI provides both rapid T2- and diffusion-dependent information, and is recommended for those patients in whom confusion and agitation is likely to confound standard MRI protocols.