MRI of Creutzfeldt-Jakob disease: imaging features and recommended MRI protocol

Creutzfeldt-Jakob Disease (CJD) is a rare, progressive and invariably fatal neurodegenerative disease characterized by specific histopathological features. Of the four subtypes of CJD described, the commonest is sporadic CJD (sCJD). More recently, a new clinically distinct form of the disease affecting younger patients, known as variant CJD (vCJD), has been identified, and this has been causally linked to the bovine spongiform encephalopathy (BSE) agent in cattle. Characteristic appearances on magnetic resonance imaging (MRI) have been identified in several forms of CJD; sCJD may be associated with high signal changes in the putamen and caudate head and vCJD is usually associated with hyperintensity of the pulvinar (posterior nuclei) of the thalamus. These appearances and other imaging features are described in this article. Using appropriate clinical and radiological criteria and tailored imaging protocols, MRI plays an important part in the in vivodiagnosis of this disease.     

Sequential MRI in a case of Creutzfeldt-Jakob disease

A 48-year-old man suddenly developed clinically and electroencephalographically nonspecific dementia. On MRI sequences, only diffusion-weighted images (DWI) of the cortex were unequivocally pathological. Obvious atrophy and basal ganglia signal changes appeared only 9 months after the onset. Brain biopsy confirmed Creutzfeldt-Jakob disease (CJD). In rapidly progressive dementia, we recommend DWI for early diagnosis of CJD.     

Creutzfeldt—Jakob病3例临床及病理分析

分析3例CJD的临床、EEG、MRI、CSF中14－3－3蛋白、脑病理组织学及PrP的免疫组化资料。西文本组CJD发病年龄平均在64岁,均有急性超病,除痴呆和肌阵挛表现明显外,视觉改变及小脑征出现相对较早,而锥体束损害相对不明显。3例EEG显示周期性兴慢复合波,1例脑CT显示底节钙化,3例脑MRI示脑萎缩不明显,1例T2加权像示双侧脑室旁信号增加,额叶及枕叶各1例脑活检证实为海绵状脑病、1例额叶活检的病例PrP免疫组化检查阳性而CSF中14－3－3蛋白检测为阴性,另1例临床诊断为可能CJD而14－3－3S检测1次为阴性,1次为可疑。国人CJD存在着以下特点：①发病年龄相对较早;②多数为急性起病;③除痴呆和肌阵挛表现明显外,视力损伤和小脑征出现相对较早,而锥体束损害相对不太明显;④脑萎缩不明显;⑤脑MRI的改变尚需进一步研究;⑥14－3－3蛋白阴性者也不能排除CJD的诊断。     

Neuroradiology of human prion diseases,diagnosis and differential diagnosis

Human transmissible spongiform encephalopathies (TSEs), or prion diseases, are invariably fatal conditions associated with a range of clinical presentations. TSEs are classified as sporadic [e.g. sporadic Creutzfeldt–Jakob disease (sCJD), which is the most frequent form], genetic (e.g. Gerstmann–Straussler–Scheinker disease, fatal familial insomnia, and inherited CJD), and acquired or infectious (e.g. Kuru, iatrogenic CJD, and variant CJD). In the past, brain imaging played a supporting role in the diagnosis of TSEs, whereas nowadays magnetic resonance imaging (MRI) plays such a prominent role that MRI findings have been included in the diagnostic criteria for sCJD. Currently, MRI is required for all patients with a clinical suspicion of TSEs. Thus, MRI semeiotics of TSEs should become part of the cultural baggage of any radiologist. The purposes of this update on the neuroradiology of CJD are to (i) review the pathophysiology and clinical presentation of TSEs, (ii) describe both typical and atypical MRI findings of CJD, and (iii) illustrate diseases mimicking CJD, underlining the MRI key findings useful in the differential diagnosis.     

1例Creutzfeldt-Jakob病的神经影像学与脑电图长期跟踪研究

目的探讨散发性Creutzfeldt-Jakob病(sCJD)患者神经影像学、脑电图的特点和动态规律性。方法利用CT、MRI以及动态脑电图设备先后对1例病理确诊的sCJD患者行5次神经影像学和6次脑电图监测,并分析其规律性。结果①在辅助诊断CJD方面,弥散加权像(DWI)较头颅CT、常规MRI的T1加权像(T1WI)、T2加权像(T2WI)、FLAIR像以及MRI增强扫描更敏感;②CJD患者DWI表现为双侧底节区及皮层的异常高信号;病程晚期,皮层DWI异常信号可以消失;③CJD患者脑电图周期性尖慢复合波(PSD)的出现有明显的病程区间性及正弦曲线性特征;PSD在病程早期可以不出现,病程晚期可以消失。结论反复监测DWI和脑电图,对于确诊CJD是十分重要的。     

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 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 follow-up in a case of clinically diagnosed Creutzfeld-Jakob disease

We report the case of a 75-year-old woman suffering from Creutzfeld-Jakob disease (CJD). As brain biopsy was refused, diagnosis had to be based on clinical examination, EEC and findings on cranial MRI. Over a 4-month period MRI examinations demonstrated progressive cortical atrophy and bilateral enhanced signal intensity on T2-weighted images of caudate nuclei and putamina indicating development of spongioform degeneration. As clinical course and the characteristic pattern of brain lesions corresponded to cases of neuropathologically confirmed CJD, we suggest that MRI should be considered a valuable diagnostic tool in clinical diagnosis of the disease. Received 22 July 1997; Revision received 21 August 1997; Accepted 26 August 1997     

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.     

AIDS脑部病变影像学表现分析

目的总结AIDS脑部病变影像学表现,为AIDS脑部病变的影像诊断提供依据。方法回顾性分析19例神经系统型AIDS患者脑部CT和MRI表现。结果5例HIV脑炎中,3例表现为双侧大脑半球白质区对称性异常信号,2例表现为脑萎缩。2例弓形体脑炎影像学表现为基底节区和额顶叶多发密度或/和信号异常,增强T1WI呈多发环状高信号。3例脑淋巴瘤分别表现为双侧大脑半球单发或多发结节,周围伴水肿,增强T1WI呈结节状或不均匀强化。3例脑梗塞表现为基底节区局限性低密度影。2例进行性多灶性白质脑病表现为额顶叶脑白质区多发斑片状异常信号。1例霉菌感染表现为脑干信号异常,增强T1WI呈环状强化;1例霉菌性脑膜炎和2例单纯颅内压增高患者,影像学无异常表现。结论CT和MRI检查可以发现大部分AIDS脑部病变,并可根据影像学表现和其它相关检查做出定性诊断。     

Movement disorders: role of imaging in diagnosis

Magnetic resonance imaging (MRI and single-photon emission computed tomography (SPECT) have a considerable role in the diagnosis of the single patient with movement disorders. Conventional MRI demonstrates symptomatic causes of parkinsonism but does not show any specific finding in Parkinson's disease (PD). However, SPECT using tracers of the dopamine transporter (DAT) demonstrates an asymmetric decrease of the uptake in the putamen and caudate from the earliest clinical stages. In other degenerative forms of parkinsonism, including progressive supranuclear palsy (PSP), multisystem atrophy (MSA), and corticobasal degeneration (CBD), MRI reveals characteristic patterns of regional atrophy combined with signal changes or microstructural changes in the basal ganglia, pons, middle and superior cerebellar peduncles, and cerebral subcortical white matter. SPECT demonstrates a decreased uptake of tracers of the dopamine D2 receptors in the striata of patients with PSP and MSA, which is not observed in early PD. MRI also significantly contributes to the diagnosis of some inherited hyperkinetic conditions including neurodegeneration with brain iron accumulation and fragile-X tremor/ataxia syndrome by revealing characteristic symmetric signal changes in the basal ganglia and middle cerebellar peduncles, respectively. A combination of the clinical features with MRI and SPECT is recommended for optimization of the diagnostic algorithm in movement disorders.     

Neuroradiological findings in primary progressive aphasia: CT,MRI and cerebral perfusion SPECT

Primary progressive aphasia (PPA) is defined as progressive decline in language for 2 or more years with preservation of activities of daily living and general cognitive functions. Whereas the clinical features of this syndrome have been well documented, the neuroradiological findings have not been studied systematically. We studied 13 patients with PPA retrospectively: 10 underwent CT, 12 MRI and 12 cerebral perfusion studies using^99mTc-HMPAO SPECT. CT and MR images were scored for focal atrophy by two independent assessors. Initial qualitative assessment of SPECT images was confirmed by quantitative analysis. CT was normal in 5 patients. Focal atrophy, affecting predominantly the left temporal lobe, was seen in 4 of 10 patients on CT, and 10 of 12 on MRI. Atrophy was localised primarily to the superior and middle temporal gyri on MRI. All 12 patients who underwent SPECT had unilateral temporal lobe perfusion defects, in 2 patients of whom MRI was normal. CT is relatively insensitive to focal abnormalities in PPA; MRI and SPECT are the imaging modalities of choice. MRI allows accurate, specific localisation of atrophy within the temporal neocortex. SPECT may reveal a functional decrease in cerebral perfusion prior to establishment of structural change.     

Masthead,Volume 35, Issue 2

Magnetic resonance imaging (MRI and single‐photon emission computed tomography (SPECT) have a considerable role in the diagnosis of the single patient with movement disorders. Conventional MRI demonstrates symptomatic causes of parkinsonism but does not show any specific finding in Parkinson's disease (PD). However, SPECT using tracers of the dopamine transporter (DAT) demonstrates an asymmetric decrease of the uptake in the putamen and caudate from the earliest clinical stages. In other degenerative forms of parkinsonism, including progressive supranuclear palsy (PSP), multisystem atrophy (MSA), and corticobasal degeneration (CBD), MRI reveals characteristic patterns of regional atrophy combined with signal changes or microstructural changes in the basal ganglia, pons, middle and superior cerebellar peduncles, and cerebral subcortical white matter. SPECT demonstrates a decreased uptake of tracers of the dopamine D2 receptors in the striata of patients with PSP and MSA, which is not observed in early PD. MRI also significantly contributes to the diagnosis of some inherited hyperkinetic conditions including neurodegeneration with brain iron accumulation and fragile‐X tremor/ataxia syndrome by revealing characteristic symmetric signal changes in the basal ganglia and middle cerebellar peduncles, respectively. A combination of the clinical features with MRI and SPECT is recommended for optimization of the diagnostic algorithm in movement disorders. J. Magn. Reson. Imaging 2012 © 2012 Wiley Periodicals, Inc.     

High signal of the striatum in sporadic Creutzfeldt-Jakob disease: sequential change on T2-weighted MRI

The object of this study is to describe the sequential change of high signal of the striatum on T2-weighted MRI in sporadic Creutzfeldt-Jakob disease (CJD). Three cases of autopsy-proven sporadic CJD and a total of 18 serial MR images are included in this study. The degree of high signal of the striatum on T2-weighted MRI was evaluated by two neuroradiologists and divided into four grades by mutual agreement. Initial MRI of all three cases showed a slightly high signal of the bilateral striatum, and the conspicuity of the high signal became more prominent as the disease progressed. In each case the pathological change of striatum and globus pallidus was compared with the high signal on the last MR image.     

Cerebral MR and CT imaging in Creutzfeldt-Jakob disease

Magnetic resonance (MR) imaging and CT of three patients with Creutzfeldt-Jakob disease (CJD) showed bilateral cortical atrophy and no apparent white matter changes. Serial examinations revealed the progressive nature of the atrophy, findings compatible with the patients' clinical deterioration. At autopsy some white matter abnormalities were detected in one patient 6 months after MR imaging. The available data suggest that the white matter abnormalities, if present, develop during the final stage of CJD.     

Serial diffusion-weighted MRI of Creutzfeldt-Jakob disease

OBJECTIVE: The objective of our study was to evaluate the clinical usefulness of MRI findings, including diffusion-weighted imaging, in relation to the clinical signs and symptoms of Creutzfeldt-Jakob disease (CJD). MATERIALS AND METHODS: We reviewed nine cases of CJD in which MRI was performed from the early to terminal phase of the disease. MRI findings were correlated before (early phase) and after (intermediate phase) the onset of the characteristic clinical findings of myoclonus and periodic synchronous discharges on electroencephalograms. The chronologic changes in imaging findings were followed from the akinetic mutism to the terminal phase of the disease (terminal phase). T2-weighted images had been obtained in all the patients, and diffusion-weighted images and FLAIR images had been obtained in six patients. We evaluated the images for the presence and location of abnormal signal intensities. RESULTS: During the early phase, the T2-weighted images showed no abnormal findings. The diffusion-weighted images, however, revealed abnormal high signal intensities in the cortex in all patients and in the basal ganglia in five patients. In two cases, there were abnormal signals on FLAIR images that corresponded to diffusion-weighted imaging abnormalities. During the intermediate phase, the area of the high signal intensities on the diffusion-weighted images had expanded and progressive cerebral atrophy had become apparent. During the terminal phase, abnormal high signal intensities in the cerebral cortex and basal ganglia on the diffusion-weighted images in one patient disappeared. CONCLUSION: Diffusion-weighted imaging is extremely useful in detecting CJD during the very early phase-even before the onset of characteristic clinical findings.     

Magnetic resonance spectroscopic abnormalities in sporadic and variant Creutzfeldt-Jakob disease

AIM: To study the proton MR spectroscopic findings in Creutzfeldt-Jakob disease (CJD) (sporadic and variant). MATERIALS AND METHODS: MR imaging and proton MR spectra were acquired in two patients with sporadic CJD (biopsy proven) and one patient with variant CJD. RESULTS: The two patients with sporadic CJD demonstrated MR signal change within the basal ganglia and thalami and reduced N-acetylaspartate (NAA):creatine ratios. The patient with variant CJD showed characteristic signal change within the pulvinar of the thalami and a markedly reduced N-acetylaspartate:creatine ratio. CONCLUSION: All three patients with CJD demonstrated evidence of reduced N-acetylaspartate: creatine ratios on MR spectroscopy. These changes imply that neuronal loss and/or dysfunction is a consistent finding in established CJD.     

Progressive supranuclear palsy: MRI and pathological findings

Our purpose was to investigate brain atrophy and signal intensity changes on MRI in patients with progressive supranuclear palsy (PSP) and to correlate them with pathological features. We reviewed MRI and brain specimens of six patients with PSP, nine with Parkinson's disease (PD) and six with striatonigral degeneration (SND). Sagittal T1-weighted images showed that four patients with PSP had obvious reduction of anteroposterior midbrain diameter. T2-weighted images demonstrated diffuse high-signal lesions in the tegmentum and tectum of the midbrain of four patients, the upper pontine tegmentum of four, and the lower pontine tegmentum of two, but in no patient with PD or SND. The inferior olivary nuclei gave high signal intensity on T2-weighted images in one patient with PSP These signal intensity changes were consistent with the pathological findings. One patient with PSP showed abnormal signal intensity in the upper pontine tegmentum without atrophy of the midbrain. Midbrain atrophy and diffuse high-signal lesions on T2-weighted images in the tegmentum and tectum of the brain stem are characteristic of PSP     

Radiologic diagnosis of spondylodiscitis: role of magnetic resonance

PURPOSE: To report the Magnetic Resonance Imaging (MRI) features of acute and chronic spontaneous spondylodiscitis as well as any typical patterns which can be useful for the differential diagnosis between pyogenic and tuberculous forms. MATERIAL AND METHODS: Eleven patients affected with spontaneous spondylodiscitis were selected for the study; they were 7 men and 4 women ranging in age 33-87 years (mean: 64). We excluded the patients with iatrogenic spondylodiscitis. MR images were acquired with a superconductive magnet at 1.5, with the following sequences: sagittal PD and T2-weighted TSE, sagittal T1-weighted SE, axial PD and T2-weighted TSE for the lumbar spine, axial T2-weighted GRE for the cervical and dorsal spine and axial and sagittal T1-weighted SE after contrast agent (gadolinium DTPA) injection. MR images were reviewed by three experienced radiologists and morphological and signal intensity changes of vertebral body and disk were recorded on a standard form. In 9 patients it was possible to compare MR to CT findings. RESULTS: At the time of our observation all patients reported pain at the spine level, associated with fever and weight loss in 50% of cases and with increased values of the inflammatory markers. Three patients had infectious diseases in other organs and 2 were diabetics. Biopsy was performed in two cases only and demonstrated Staphylococcus aureus in one and Mycobacterium tuberculosis in the other patient. MRI allowed the correct diagnosis to be made in all cases, demonstrating the pathological involvement of the paravertebral structures and into the spinal canal earlier and more accurately than CT. A common finding in pyogenic and tuberculous spondylodiscitis was the low signal of the subcortical bone marrow on T1-weighted sagittal images, which enhanced after Gd-DTPA administration and became intermediate or high on T2-weighted images. Moreover, the steady high signal intensity of the disk on T2-weighted images and its contrast enhancement on T1-weighted images is typical for an acute inflammatory process. CONCLUSIONS: Based on our personal experience and literature data, we believe MRI to be the most sensitive technique for the diagnosis of spondylodiscitis in the acute phase, whereas it is comparable to CT in the chronic stage of the disease. At present MRI does not allow to differentiate pyogenic from tuberculous forms.     

X-ray computed tomography and MRI in Krabbe's disease

Krabbe's disease whose CT appearance is well known should benefit of the development of MRI as this method is more accurate. MRI permits early diagnosis of leucodystrophy. Yet it must be emphasized that abnormal white matter patterns are not sufficient to permit the diagnosis of Krabbe disease. In the case reported atrophy of the brain, the cerebellum and the brain-stem, demyelination of the white matter and necrosis of corpus callosum were observed. The abnormalities of the brain on MRI pictures help in the diagnosis as they may alert clinicians to the possibility of Krabbe disease in infants with progressive encephalopathy. A definitive diagnosis can be established thanks to the laboratory tests.