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Poole General Hospital Dorset, BH15 2JB, England
The most commonly recognized features of sporadic Creutzfeldt-Jakob disease as seen on MR images are bilateral high signal within the caudate nuclei and putamina on T2 and proton density sequences [1]. Occasionally cortical involvement accompanies such changes, and, rarely, they may be present in isolation [2, 3]. Standard MR sequences have been reported as being normal in up to 20% of patients with Creutzfeldt-Jakob disease [1]. Fluid-attenuated inversion recovery (FLAIR) is more sensitive than standard MR imaging, and it can produce heavily weighted T2 images without artifact or partial-volume effects from the cerebrospinal fluid. It is valuable in picking up subtle changes [4], particularly in the cerebral cortex and brain stem.
A 51-year-old man was admitted with a history of rapidly progressive dementia. Over a 6-month period, he developed increasing problems with memory and concentration. He had no remarkable medical history. At examination, he made occasional monosyllabic utterings and was unable to follow simple one-step commands. He exhibited generalized myoclonus.
His blood tests at admission showed normal values. The electroencephalogram showed generalized slowing with sharp waves and periodic complexes. The cerebrospinal fluid was acellular with normal protein. MR imaging of the brain showed normal appearances on spin echo T1-weighted axial images. On the T2-weighted sequences, subtle gyral high signal was thought to be present (Figs. 5A and 5C). FLAIR images were then obtained. These revealed much more extensive high signal within the cerebral cortex bilaterally (Figs. 5B and 5D). The white matter, basal ganglia, and thalami appeared normal. A clinical diagnosis of Creutzfeldt-Jakob disease was made. At autopsy, spongiform change was present within the cerebral cortex, consistent with the features seen on the MR image.
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The type of gyral high signal seen in this patient has been reported only rarely, and it may be missed on conventional MR sequences, particularly in the early stages of the disease. On review of the T2-weighted images, subtle changes are present, but these are much less extensive than on the FLAIR images. In cases where a strong clinical suspicion of Creutzfeldt-Jakob disease exists, FLAIR, with its greater sensitivity, is a valuable addition to standard sequences, and is more widely available than diffusion-weighted MR imaging.
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  M. Hori, K. Ishigame, S. Aoki, and T. Araki Creutzfeldt-Jacob Disease Shown by Line Scan Diffusion-Weighted Imaging Am. J. Roentgenol., May 1, 2003; 180(5): 1481 - 1482. [Full Text] [PDF]
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