Creutzfeldt‐Jakob disease

Abstract

In a patient with Creutzfeldt-Jakob Disease (CJD), MRI showed increased signal intensity in striatum, thalamus, and cerebral cortex in images obtained with TR 1,600 msec, and TE 35 and 70 msec. In postmortem examination, all affected areas showed the hallmarks of CJD, such as status spongiosus, gliosis, and nerve cell loss. MRI can help to differentiate CJD from other dementing processes.

Susceptibility of Domestic Cats to Chronic Wasting Disease

“…Cerebral MRI has been used to detect specific abnormalities between sporadic CJD (sCJD), vCJD, and nonprion rapidly progressive dementias (47)(48)(49)(50)(51)(52). In sCJD patients, T2 hyperintensities are observed in the cortex and deep gray matter, in particular the striatum, whereas in vCJD, T2 hyperintensities are most commonly seen in posterior pulvinar and medial thalamus (47,(52)(53)(54)(55)(56).…”

Pattern of Cortical Changes in Sporadic Creutzfeldt-Jakob Disease

“…Several studies have shown that the intensity of MR changes correlates with the neuropathologic findings (spongiosis, neuronal loss, and astrogliosis). 5,[25][26][27] In areas that are bright on MR imaging in FLAIR or DW images, neuropathologic changes are more severe than in areas with normal cortical signal intensity. 26,27 The hypothesis that MR signal intensity changes reflect pathologic conditions is additionally supported by clinicoradiologic comparison studies showing that cortical signal intensity changes correlate with clinical signs (eg, affection of the occipital cortex and visual disturbances 28 or unilateral cortical affection and contralateral motor signs).…”

“…5,[25][26][27] In areas that are bright on MR imaging in FLAIR or DW images, neuropathologic changes are more severe than in areas with normal cortical signal intensity. 26,27 The hypothesis that MR signal intensity changes reflect pathologic conditions is additionally supported by clinicoradiologic comparison studies showing that cortical signal intensity changes correlate with clinical signs (eg, affection of the occipital cortex and visual disturbances 28 or unilateral cortical affection and contralateral motor signs). 29 Concerning the cause of the distribution of lesions, we propose 2 hypotheses: 1) predominant manifestation of the disease in cortical areas with signal hyperintensity in MR imaging and 2) a sequence-inherent higher sensitivity in cortical areas bright on MR imaging as a result of intrinsic variations in the water content of different cortical regions in a diffuse cortical process.…”

Pre-Clinical Changes Observed by Magnetic Resonance Imaging in a Hamster Model of Transmissible Spongiform Encephalopathy: a Potential Biomarker of Prion Infection

“…Although the 263K strain of scrapie is very fast acting, it was remarkable to find significant differences as early as we did. Abnormalities on MR images have previously been reported for prion diseases in both humans [12,14,[20][21][22] and rodents [18,19,23], with variable results. In the case of the human studies, all MRI scans were carried out during the symptomatic stage of disease.…”