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Brought to you by the Resident and Fellow Section of Neurology®.
June 26, 2012
Creutzfeldt–Jakob Disease (CJD) presents as a rapidly progressive dementia, typically with additional features including myoclonus, pyramidal, extrapyramidal, or cerebellar signs, or other cortical findings such as aphasia, apraxia, acalculia, or neglect. The utility of MRI in the evaluation of CJD is increasingly recognized. Typical findings include T2–weighted hyperintensity and restricted diffusion of at least two regions of the cortex (temporal, parietal, occipital, or frontal), or caudate, putamen, and thalamus. In CJD, hyperintensity on diffusion weighted imaging (DWI) is greater than that on FLAIR, and abnormalities on DWI are often bright very early in the disease course when T2–weighted images are not.
1. Zerr I, Kallenberg K, Summers DM, et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt–Jakob disease. Brain 2009; 132: 2659–2668.
2. Vitali P, Maccagnano E, Caverzasi E, et al. Diffusion–weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias. Neurology 2011; 76: 1711–1719. 3. Geschwind MD, Shu H, Haman A, et al. Rapidly progressive dementia. Ann Neurol 2008; 64: 97–108.
Submitted by: Christopher Newey, D.O., M.S.
Disclosure: Dr. Newey reports no disclosures.
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