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.     

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.     

MR imaging of familial Creutzfeldt-Jakob disease: a blinded and controlled study

BACKGROUND AND PURPOSE: The E200K mutation of the PRNP (prion protein) gene is the most common cause of familial Creutzfeldt-Jakob disease (fCJD), which has imaging and clinical features that are similar to the sporadic form. The purpose of this study was to conduct a controlled and blinded evaluation of the sensitivity and specificity of MR imaging in this unique population.MATERIALS AND METHODS: We compared the MR imaging characteristics of 15 early stage familial CJD patients (age, 60 ± 7 years) with a group of 22 healthy subjects from the same families (age, 61 ± 8 years). MR imaging included diffusion-weighted imaging (DWI), T2-weighted fast spin-echo imaging, and a fluid-attenuated inversion recovery (FLAIR) sequence. The scans were rated for abnormalities by an experienced neuroradiologist blind to diagnosis, group assignment, age, and sex.RESULTS: Thirteen of 15 fCJD subjects had abnormal MR imaging. FLAIR signal intensity abnormality in the caudate or putamen nuclei demonstrated a sensitivity of 87% and specificity of 91%. DWI abnormality in the caudate nucleus showed a sensitivity of 73% and a specificity of 100%. Abnormalities in the thalamus (6 patients), cingulate gyrus (6 patients), frontal lobes (4 patients), and occipital lobes (3 patients) were best detected with DWI. No signal intensity abnormalities were demonstrated in the cerebellum. T2-weighted and T1-weighted sequences were uninformative.CONCLUSIONS: FLAIR and DWI abnormalities in the caudate nucleus and putamen offer the best sensitivity and specificity for diagnosing fCJD. Our findings support recent recommendations that MR imaging should be added to the diagnostic evaluation of CJD.

Creutzfeldt-Jakob disease (CJD) is the most common human prion disease. It is a rare neurodegenerative disorder that is progressive and invariably fatal, with nearly 90% of patients dying within 1 year of diagnosis.¹ CJD occurs in approximately 1 person per 1 million people per year worldwide.¹ The most common form is sporadic CJD (sCJD), which occurs randomly without a known risk factor and accounts for 85%-90% of cases. Familial or hereditary CJD (fCJD), seen in 5%-10% of cases, is caused by mutations in the gene that controls formation of the normal prion protein on chromosome 20.¹ The most common pathogenic mutation is the E200K mutation. The risk of fCJD is transmitted in an autosomal dominant inheritance pattern, with nearly 100% penetrance.²The imaging findings in sCJD typically consist of cortical atrophy and hyperintensities in the basal ganglia, thalamus, and cortex on fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted imaging (DWI).^3–13 fCJD has imaging findings and neuropathology that are, in general, similar to the most common forms of sCJD; however, most imaging studies on fCJD consist primarily of case reports or studies focused on sCJD that combine a few fCJD patients into a single CJD patient sample.^8,14–22 fCJD studies are limited not only by small sample sizes but also by nonstandardized imaging protocols and clinical and pathophysiologic heterogeneity. The complex interactions with disease duration and cognitive and neurologic severity have also made it difficult to interpret studies, especially because the sample sizes are small.²³To address the difficulties of clinical research in this area, we initiated a study of fCJD occurring among Libyan Jews living in Israel that is caused by familial transmission of the E200K mutation.^24–26 In a preliminary report, it was demonstrated that 4 patients with fCJD due to the E200K mutation had gray matter atrophy and decreased apparent diffusion coefficient (ADC) in the basal ganglia. Signal intensity hyperintensities were seen in the basal ganglia and thalamus with FLAIR and DWI.²⁷ The sample size in that study was insufficient to calculate sensitivity and specificity. Here we describe a rigorously blinded and controlled evaluation of MR imaging findings in a larger number of fCJD patients.     

Thalamic involvement in sporadic Creutzfeldt-Jakob disease: a diffusion-weighted MR imaging study

BACKGROUND AND PURPOSE: Recent neuropathologic research suggests thalamic involvement in sporadic Creutzfeldt-Jakob disease (sCJD), which has been disregarded in imaging studies. Diffusion-weighted (DW) MR imaging has the highest sensitivity for the detection of signal intensity (SI) abnormalities in CJD. We hypothesized that pathologic changes in the thalamus in sCJD can be detected by using a subtle analysis of DW MR imaging. METHODS: Six sCJD patients and nine healthy controls were examined with a 1.5-T system by using DW single-shot spin-echo echo planar (b = 0, 1000 s/mm(2)), T2-weighted turbo spin-echo, and fluid-attenuated inversion recovery sequences. One patient was examined serially (3, 4, and 8 months after onset of symptoms). MR images were reviewed for SI changes in the striatum, hippocampus, mediodorsal thalamic nucleus (MD), and pulvinar thalami. Apparent diffusion coefficients (ADCs) were measured in these areas. RESULTS: All sCJD patients showed increased SI on DW images in the striatum bilaterally. ADCs in these areas were significantly reduced. Four of six sCJD patients showed increased SI on DW images in the pulvinar thalami, whereas ADCs were significantly reduced in all patients (mean ADC +/- SEM: in patients with SI changes, 701 +/- 38; in patients without SI changes, 684 +/- 37; in controls, 853 +/- 15 [P <.0001]). No patient showed SI changes in the MD on DW images, whereas ADCs were significantly reduced in all (664 +/- 28 as compared with 800 +/- 24 in controls [P =.0011]). Serial measurements in one sCJD patient showed ADC reduction in the pulvinar thalami preceding the SI changes on DW images. CONCLUSION: A quantitative analysis of DW images with ADC measurements shows slight MR imaging changes in the thalamus in sCJD when abnormal SI may not be present.     

Creutzfeldt-Jakob disease: focal symmetrical cortical involvement demonstrated by MR imaging.

The authors present two biopsy-proved cases of Creutzfeldt-Jakob disease. MR appears to be more sensitive than CT in detecting pathologic changes; signal abnormalities, when found, are predominantly within gray matter and may involve only peripheral cortex.     

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。     

White matter lesions in panencephalopathic type of Creutzfeldt-Jakob disease: MR imaging and pathologic correlations

BACKGROUND AND PURPOSE: A panencephalopathic type of Creutzfeldt-Jakob disease (pCJD) is characterized by the extensive involvement of the cerebral white matter as well as the cerebral gray matter. It has been a point of controversy, however, whether the white matter changes represent primary or secondary degeneration. The aim of this study was to elucidate, by using MR images and histologic examinations, whether the white matter lesions in pCJD are primary or secondary degeneration. METHODS: Serial changes of T2 hyperintensities and histologic findings of six autopsy-proved cases of pCJD were retrospectively analyzed. RESULTS: Serial MR images of brains affected by pCJD revealed that T2 hyperintensities appeared in the cerebral gray matter 2-5 months after onset and in the cerebral white matter around the lateral ventricles approximately 5 months after onset. They rapidly extended to deep and subcortical white matter during the next several months and then to the entire cerebral white matter 10 months after onset. Histologic examination of the white matter lesions revealed spongy changes or tissue rarefaction associated with gemistocytic astrocytosis, which indicates primary involvement of the white matter. At the terminal stages of cases with a longer clinical course, MR images showed T2 hyperintensities in the corticospinal tracts in the internal capsule and brain stem, which histologically disclosed loss of myelin and axons accompanied by fibrillary gliosis that indicates secondary degeneration. CONCLUSION: Cerebral white matter lesions in pCJD were considered to be primary changes of the disease, but the lesions of the corticospinal tracts were secondary to cortical or cerebral or both white matter lesions.     

MR breast imaging : a comparative analysis of conventional and parallel imaging acquisition

PURPOSE: The objective of this study was to compare conventional breast magnetic resonance imaging (MRI) with breast MRI acquired with the sensitivity-encoding (SENSE) technique on a 1.5-T MRI scanner in the same patient, on the basis of image quality and kinetics analysis. MATERIALS AND METHODS: Thirty-one patients with suspicious mammography and US findings were included in the study. Conventional breast MRI consisted of the following sequences: T1 (matrix, 288 x 512); T2 (matrix 225 x 512); short tau inversion recovery (STIR) (matrix 320 x 224) and dynamic T1 [2D fast-field echo (FFE)] (matrix 256 x 512; temporal resolution相似文献   

Creutzfeldt-Jakob disease: magnetic resonance imaging findings

Rapidly progressive dementia in an adult with findings of bilateral, symmetric high signal intensity on T2-weighted sequences and normal findings on T1-weighted sequences predominantly in the deep grey matter is suggestive of Creutzfeldt-Jakob disease (CJD). The peripheral cortex may be involved, as it was in the present case. The absence of subcortical periventricular white matter high signal intensity suggests that symmetric high signal intensities within the basal ganglia and cortical grey matter are more likely to be due to a degenerative process rather than due to ischaemia, infection or tumour.     

MR imaging of Parkinson disease with spin-echo and gradient-echo sequences

High-field MR with both spin-echo and gradient-echo sequences was performed in 21 patients with (idiopathic, drug-responsive) Parkinson disease. The use of gradient echoes allowed more sensitive detection than did spin echoes of susceptibility changes in the putamina and substantia nigra. No statistically significant difference in putaminal hypointensity on long TR/long TE spin-echo sequences or on T2*-weighted images using gradient-echo sequences was observed between Parkinson patients and controls. There was also no statistically significant difference in the frequency of restoration of the signal intensity of the substantia nigra between the two groups of patients. The width of the pars compacta of the substantia nigra in patients with Parkinson disease was 2.12 + 0.82 mm (mean +/- SD). This value in age- and gender-matched controls was 2.67 +/- 0.5. Comparing these two groups with an unpaired t test resulted in a p value less than or equal to .005. Our MR study with spin-echo and gradient-echo images in Parkinson and control patients was able to substantiate and elaborate on previously described MR features of Parkinson disease.     

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.     

Emerging patterns of diffusion-weighted MR imaging in Creutzfeldt-Jakob disease: case report and review of the literature

We report the use of diffusion-weighted MR imaging in the early diagnosis and monitoring of the progression of a histopathologically proved case of sporadic Creutzfeldt-Jakob disease. Ribbon-like areas of hyperintensity in the cerebral cortex on diffusion-weighted images corresponded to the localization of periodic sharp-wave complexes on the electroencephalogram.     

Moyamoya disease: MR imaging

Eleven patients with moyamoya disease were studied with magnetic resonance (MR) imaging performed with a high-field-strength (1.5-T) superconducting magnet. In all cases a fundamental pathologic change of moyamoya disease--occlusion or stenosis of the terminal portion of the internal carotid artery and the proximal portion of anterior and middle cerebral arteries--was clearly demonstrated. The characteristic collateral network from the suprasellar cistern to the basal ganglia, which corresponds to the "moyamoya vessels" on angiograms, was seen in several patients. Various ischemic changes, including infarction, brain atrophy, and ventricular dilatation, were also well demonstrated. MR imaging plays an important role in the diagnosis of moyamoya disease.     

Diagnosing variant Creutzfeldt-Jakob disease with the pulvinar sign: MR imaging findings in 86 neuropathologically confirmed cases

BACKGROUND AND PURPOSE: Variant Creutzfeldt-Jakob disease (vCJD) is a rare but important cause of dementia and death in young patients and is causally linked to bovine spongiform encephalopathy. Symmetrical hyperintensity in the pulvinar (posterior) nuclei of the thalamus (pulvinar sign) on brain MR images was described as a specific, noninvasive, diagnostic sign of vCJD in a previous small series. This purpose of this larger study was to evaluate this sign prospectively and further define the MR imaging characteristics of vCJD. METHODS: As part of the ongoing surveillance program in the United Kingdom, MR images of suspected cases of vCJD were collected during a 6-year period. All available images were assessed prospectively by one observer for the presence of the pulvinar sign. Images of neuropathologically confirmed cases were then assessed independently by two neuroradiologists for the degree of hyperintensity of the pulvinar on images of different MR sequences, and for the presence of abnormal hyperintensity in other areas of the brain. Discrepancies were reviewed jointly and a consensus opinion formed. RESULTS: Prospective analysis identified the pulvinar sign in 74 of 82 cases of vCJD. In the retrospective study, the pulvinar sign, as defined by hyperintensity of the pulvinar relative to the anterior putamen, was present on seven (9%) of 75 T1-weighted, 77 (71%) of 108 T2-weighted, 47 (81%) of 58 proton density-weighted, and 30 (100%) of 30 fluid-attenuated inversion-recovery (FLAIR) images. Diffusion-weighted images were available in two cases and were positive for the pulvinar sign in one. Other features were hyperintensity of the dorsomedial thalamic nuclei (93%), caudate head (40%), and periaqueductal gray matter (83%) on FLAIR images. CONCLUSION: In the appropriate clinical context, demonstration of the pulvinar sign on MR images is a highly accurate diagnostic sign for vCJD. FLAIR sequence is more sensitive than other sequences. Positive MR images may obviate more invasive diagnostic tests in most cases.     

MRI各序列诊断创伤性脑损伤的价值比较 MRI各序列诊断创伤性脑损伤的价值比较

目的：比较MRI各序列诊断创伤性脑损伤（traumatic brain injury,TBI）的价值.方法：对260例TBI患者行MRI序列组合扫描,包括FLASH、FLAIR、SE T1WI、TSE T2WI,分析不同类型TBI的影像特点,比较各序列对病灶的显示率.结果：260例中,FLASH显示244例（93.8％）,FLAIR显示249例（95.8％）,T2WI显示200例（76.9％）,T1WI显示199例（76.5％）,FLASH与FLAIR比较、T2WI与T1 WI比较,显示率差异均无统计学意义（P均＞0.05）;FLASH、FLAIR分别与T2WI、T1WI相比,显示率差异均有统计学意义（P均＜0.01）,FLASH、FLAIR对TBI病变的显示优于T2WI、T1WI.结论：MRI各序列显示TBI病灶总体敏感性由高至低依次为FLAIR、FLASH、T2WI、T1WI.FLAIR、FLASH应作为MRI诊断TBI的首选序列.