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AJNR - American Journal of Neuroradiology

Published ahead of print on July 17, 2008
doi: 10.3174/ajnr.A1217

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MR Imaging of Familial Creutzfeldt-Jakob Disease: A Blinded and Controlled Study

R.K. Fulbright^a, C. Hoffmann^b, H. Lee^d, A. Pozamantir^d, J. Chapman^c,f and I. Prohovnik^a,d,e

^a Department of Radiology, Yale University School of Medicine, New Haven, Conn
^b Department of Radiology, Chaim Sheba Medical Center, Ramat Gan, Israel
^c Department of Neurology, Chaim Sheba Medical Center, Ramat Gan, Israel
^d Department of Psychiatry, Mount Sinai School of Medicine, New York, NY
^e Department of Radiology, Mount Sinai School of Medicine, New York, NY
^f Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel

View larger version (123K): [in this window] [in a new window]   Fig 1. Representative imaging findings on FLAIR, DWI, and ADC sequences in 3 different subjects with familial CJD. In A (fCJD 1), signal intensity abnormality involves the caudate nucleus, putamen, and thalamus, bilaterally. B (fCJD 2) demonstrates abnormal signal intensity primarily in the left caudate nucleus. In C (fCJD 3), there is involvement of both caudate nuclei, right greater than left, right cingulate gyrus, and right frontal lobe.

View larger version (91K): [in this window] [in a new window]   Fig 2. This subject (fCJD 4, case 2012) was rated as normal by FLAIR and DWI and was considered to be a false-negative by MR criteria (see Discussion). The ADC images (not shown) appeared normal.