Imaging of prion diseases

Abstract

Prion diseases are caused by self‐replicating proteins that induce lethal neurodegenerative disorders. In the last decade, the understanding of the different clinical, pathological, and neuroimaging phenotypes of this group of disorders has evolved paralleling the advances in prion molecular biology. From an imaging standpoint, the implementation of diffusion‐weighted imaging in routine practice has markedly facilitated the detection of prion diseases, especially Creutzfeldt‐Jakob. Less frequent prion‐related disorders, including genetic diseases, may also benefit from progresses in the field of quantitative diffusion‐weighted imaging, MR spectroscopy or molecular imaging. Herein, we present a review of the neuroimaging features of the prion disorders known to affect humans emphasizing the important contribution of MRI in the diagnosis of this group of disorders. J. Magn. Reson. Imaging 2012;35:998‐1012. © 2012 Wiley Periodicals, Inc.

Metabolic patterns in prion diseases: an FDG PET voxel-based analysis

“…In fact, the thalamus is clearly affected by severe gliosis in other prion diseases such as FFI and vCJD [15,16]. Moreover, thalamic hyperintensities have also been observed on brain MRI in some subjects with sCJD [1,6], complicating the differential diagnosis from vCJD. Unfortunately, in our study we do not have a direct comparison between histopathological and FDG PET findings.…”

“…Thus, probability criteria are used based on clinical symptoms and complementary examinations such as electroencephalogram (EEG), detection of 14-3-3 protein in the cerebrospinal fluid (CSF) or magnetic resonance imaging (MRI). Other imaging modalities such as single photon emission computed tomography (SPECT) and positron emission tomography (PET) imaging might have an important role in the diagnosis of prion diseases [6]. In fact, PET has already been demonstrated to highlight more extensive pathological findings than MRI in a series of patients [7] and to have a high sensitivity for early diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) [8].…”

Sporadic Creutzfeldt-Jakob disease with focal findings: caveats to current diagnostic criteria

“…31 In all of these sCJD subtypes, the damage occurs in the cerebral cortex, basal ganglia, and/or thalamus. However, certain molecular subtypes will correlate well with certain lesion patterns in the MRI.…”

Early cortical and late striatal diffusion restriction on 3T MRI in a long‐lived sporadic creutzfeldt–jakob disease case

“…Seven 3T MRI scans were acquired at 4,6,8,9,11,13 (with sedation), and 17 (with sedation) months with a 3T Siemens Skyra scanner (Erlangen, Germany). A high-resolution 3D T 1 -weighted scan (repetition time / echo time / inversion time [TR/TE/TI] = 2300/2.95/900 msec, flip angle = 9 , 176 sagittal slices, matrix = 256 × 256, voxel = 1.05 × 1.05 × 1.2 mm), a DTI spin-echo echo planar imaging (SE-EPI) sequence (TR/TE = 10,000/97 msec, 70 axial slices, matrix = 122 × 122, 2 mm isotropic voxel; 64 diffusion directions with b = 1200 s/mm 2 and 10 volumes with b 0 = 0 s/mm 2 ) and a 3D pulsed ASL gradient-and spin-echo (GRASE) sequence (TR/TE/TI = 3500/21.1/2020 msec, 20 axial slices, matrix = 128 × 128, voxel = 1.9 × 1.9 × 5.3 mm) were acquired.…”

“…exclusive cortical involvement, with deep nuclei preservation. 3,4 Due to the usual rapid progressive course, a few longitudinal studies have analyzed the pattern of MRI changes in sCJD, with only two studies analyzing quantitative diffusion changes. 5,6 Here we present a unique long-lived sCJD case longitudinally studied with seven 3T MRI scans, including quantitative assessment by diffusion tensor imaging (DTI) and arterial spin labeling (ASL).…”