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Sanjay Mittal, MD; Peter Farmer, MD; Peter Kalina, MD; Peter B. Kingsley, PhD; John Halperin, MD

Arch Neurol. 2002;59:128-134.

Background  Although the diagnosis of Creutzfeld-Jakob disease (CJD) is straightforward in fully developed cases, a definitive diagnosis can be difficult early in the course of the illness. T2-weighted magnetic resonance imaging (MRI) signal abnormalities, and recently, diffusion-weighted MRI abnormalities, have been described in patients with CJD, suggesting the utility of MRI in the early recognition of CJD.

Objective  To correlate diffusion-weighted MRI signal abnormalities with neuropathologic changes in CJD.

Materials and Methods  Diffusion-weighted MRI and neuropathologic changes of 2 patients with autopsy-proven CJD were examined in a blinded fashion by a neuroradiologist and a neuropathologist.

Results  Areas of bright signal on diffusion-weighted MRI correlated with a higher degree of spongiform changes.

Conclusion  Diffusion-weighted MRI in CJD demonstrates specific-signal abnormalities that correlate well with areas of the most severe and characteristic neuropathologic changes.

From the Departments of Neurology (Drs Mittal and Halperin), Pathology (Dr Farmer), and Radiology (Drs Kalina and Kingsley), North Shore University Hospital, Manhasset, NY; and New York University School of Medicine, New York.

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