Neuroimaging in human prion disease: Searching in the mist

Figure 1 MRI sequences of a patient with sporadic CJD (A-C), inherited CJD (D, E) and variant CJD (F-J). A: Axial T2 weighted image shows a mild signal increase in the left putamen only; B: Axial diffusion weighted images (b = 1000) shows increased signals in both putamina and caudate nuclei (more on left than right),along with cortical hyper-intensity (“ribbons”) in both insulae as well as in the frontal, temporal and occipital cortex; C: Apparent diffusion coefficient map demonstrates reduced signals in the areas which were hyperintense on the DWI confirming restricted water diffusion in these regions; D: The coronal T1 MRI shows supratentorial cortical and cerebellar atrophy in an inherited CJD case; E: MRI shows marked supratentorial atrophy with enlarged lateral ventricles accompanying cerebellar atrophy with prominent enlargement of the fourth ventricle in another case with inherited CJD; F: Axial fluid-attenuated inversion recovery image reveals the “hockey stick” sign, with hyper-intensity in the dorsomedial thalamic nuclei and pulvinar bilaterally in a patient with vCJD; G: Axial DWI (b = 1000) also shows increased signals which are less marked than on FLAIR image; H: DWI acquired after 1 mo demonstrates progression of the signal change in the thalami; I: Limited diagnostic value of axial T2 image is degraded by movement in a patient with vCJD; J: DWI (b = 1000) shows a high signal in the dorsomedial thalamic nuclei and pulvinar in the same case as above (All the above images have been reproduced and adapted by permission from BMJ Publishing Group Ltd. Macfarlane RG, Wroe SJ, Collinge J, Yousry TA, Jäger HR. Neuroimaging findings in human prion disease. J Neurol Neurosurg Psychiatry 2007; 78: 664-670).