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AJNR - American Journal of Neuroradiology

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Emerging Patterns of Diffusion-Weighted MR Imaging in Creutzfeldt-Jakob Disease: Case Report and Review of the Literature

Yang Mao-Draayer^a, Steven P. Braff^b, Keith J. Nagle^a, William Pendlebury^c, Paul L. Penar^d and Robert E. Shapiro^a

^a Department of Neurology, College of Medicine, University of Vermont, Burlington
^b Department of Radiology, College of Medicine, University of Vermont, Burlington
^c Department of Pathology, College of Medicine, University of Vermont, Burlington
^d Department of Neurosurgery, College of Medicine, University of Vermont, Burlington

View larger version (375K): [in a new window]   FIG 1. Serial MR images demonstrate the evolution of ribbon-like cortical signal intensity abnormalities. MR images are presented at each of three brain axial levels (1–3), at each of three time points from the onset of symptoms (left columns, 4 months; middle columns, 5.5 months; right columns, 6 months) for FLAIR (top row), DW imaging (middle row), and ADC (bottom row) studies. At 4 months from onset of symptoms, DW images demonstrate gyriform increased signal intensity predominantly in the right temporal cortex (DWI1D) with decreased ADC signal intensity consistent with restricted diffusion (ADC1G). At 5.5 months from onset, the hyperintense signals on DW images involve more cortical gyri, extending into the left temporoparietal cortex (DWI3E). At 6 months from onset, DW images (DWI1F, DWI2F, and DWI3F) show ribbon-like areas of hyperintensity involving the right temporoparietooccipital cortex, as well as the left frontotempoparietal cortex, extending into the parafalcine occipital region. Images on this page were obtained at level 1. (continued) Images on this page were obtained at level 2. (continued) Images on this page were obtained at level 3.

View larger version (65K): [in a new window]   FIG 2. A 21-channel EEG with the patient awake demonstrates periodic sharp-wave complex discharges, which are of greater amplitude over the left frontotemporal region and are superimposed on diffusely disorganized and slow background.

View larger version (167K): [in a new window]   FIG 3. Photomicrograph of brain biopsy specimen obtained from the cortex of the left frontal lobe demonstrates prominent neuronal loss. Astrocytosis is not conspicuous. There are characteristic spongiform changes (**) that tend to be cell-associated (hematoxylin-eosin stain, original magnification x400).