Radiological assessment of Creutzfeldt-Jakob disease

Abstract

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.

MR Imaging of Familial Creutzfeldt-Jakob Disease: A Blinded and Controlled Study

“…This area of the brain is usually abnormal more than any other region, though the distribution of signal intensity changes can vary across cases and can differ within an individual over time. 8,11,19,35 That FLAIR and DWI are the best sequences to use in evaluation of fCJD supports previous studies of sCJD. 21,22,36,37 In one study of sCJD patients with an average symptom duration of 12 months at the time of MR imaging, DWI and FLAIR were 91% sensitive, 95% specific, and 94% accurate in diagnosing sCJD.…”

“…8,11 Furthermore, when using cortical DWI and FLAIR hyperintensity for diagnostic purposes, it must be kept in mind that the insula and cingulate can normally have an increased signal intensity that is probably due to the cytoarchitecture of these regions. 22,35,40,41 Thalamic hyperintensity is often associated with variant CJD (vCJD), but it is not unique to it. Signal intensity abnormality in the thalamus was seen in 40% of fCJD cases in our study and is also seen in sCJD.…”

Pattern of Cortical Changes in Sporadic Creutzfeldt-Jakob Disease

“…4,5,8,11 To our knowledge, this is the first systematic study on the extent and distribution of cortical signal intensity changes in a large group of patients with sCJD. We found extensive cortical involvement, predominantly in the insula, the cingulate gyrus, the frontal gyri, and cortical areas situated near the midline.…”

“…1 Typical findings on MR imaging in sCJD are signal hyperintensity abnormalities in the cortical and deep gray matter. [2][3][4][5] The first reports, relying mainly on T2-and proton attenuation-weighted sequences, focused on hyperintense and often symmetrical high signal intensity in the head of the caudate nucleus and the putamen. 2,6,7 The sensitivity of striatal MR imaging changes in the diagnosis of sCJD is reported in 79% 6 and 39% 7 of patients.…”

High-b-Value Diffusion MR Imaging and Basal Nuclei Apparent Diffusion Coefficient Measurements in Variant and Sporadic Creutzfeldt-Jakob Disease

“…[5][6][7] With the routine clinical use of DWI, MR imaging has gained increasing importance in the diagnosis of human prion diseases. 8 MR imaging is now included in the diagnostic criteria for vCJD, and DWI has enabled earlier and more accurate diagnosis of sCJD.…”