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Lee, Song, Lee, Yu, and Choi: Diffusion-Weighted MRI in Creutzfeldt-Jakob Disease: Focus on the Cerebral Cortex and Chronologic Change
Neuroradiology
Original Article

Journal of the Korean Society of Radiology 2010; 62(4): 327-333.

Published online: 30 April 2010

DOI: https://doi.org/10.3348/jksr.2010.62.4.327

Diffusion-Weighted MRI in Creutzfeldt-Jakob Disease: Focus on the Cerebral Cortex and Chronologic Change

Jeong Eun Lee, M.D., Chang Joon Song, M.D., In Ho Lee, M.D., In Kyu Yu, M.D.2, See Sung Choi, M.D.3

1Department of Radiology, College of Medicine, Chungnam National University, Korea.

2Department of Radiology, Eulji University Hospital, Korea.

3Department of Radiology, Wonkwang University Hospital, Korea.

Address reprint requests to: Chang Joon Song, M.D., Department of Radiology, College of Medicine, Chungnam National University, 640 Daesa-dong, Jung-gu, Daejeon 301-721, Korea. Tel. 82-42-280-7834, Fax. 82-42-253-0061, cjsong@cnu.ac.kr

Received 28 August 2009       Accepted 12 December 2009

Copyright © 2010 The Korean Society of Radiology

Abstract

Purpose

To evaluate high cortical signal intensity and chronologic changes for diffusion-weighted MR imaging (DWI) in sporadic Creutzfeldt-Jakob disease.

Materials and Methods

We retrospectively analyzed the DWI results of 16 patients with probable CJD (according to WHO criteria) and evaluated the distribution, extent and bilaterality of the lesions in the cortex, basal ganglia and thalamus. We also reviewed the chronologic changes of the lesions by evaluating the follow-up MR examination results in 8 of 16 patients.

Results

Cortical abnormalities were present in 15 (94%) of 16 patients. Isolated cortical involvement was present in 6 patients (40%), while the combined involvement of the cortex and basal ganglia was present in 9 patients (60%). The distribution of the lesions was bilateral in 12 patients and predominantly on the right side in 8 patients. Upon follow-up MR imaging, the cortical lesions showed progress in terms of extent and signal intensity. Basal ganglia abnormalities were present in 9 of 15 patients. Moreover, 4 of 6 patients who had no abnormal signal intensity in the basal ganglia on the initial MR imaging results, showed abnormally high signal intensity upon follow-up MR imaging.

Conclusion

The characteristically high cortical signal intensities on DWI in an elderly patient with rapidly progressive dementia should point to the diagnosis of early phase CJD and might be useful for the differential diagnosis.

Keywords: Creutzfeldt - Jakob disease, Prion, Diffusion Magnetic Resonance Imaging, Brain, Cerebral Cortex

Figures and Tables

Fig. 1

Isolated cortical high signal intensity lesions in CJD.

A, B. Axial diffusion-weighted images of a 67-year-old woman show abnormal high signal intensities in bilateral parietal and occipital lobes, and right insula (arrows).
C, D. Axial diffusion-weighted images of a 41-year-old man show abnormal high signal intensities in bilateral frontal and parietal lobes (arrows).
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Fig. 2

Combined cortex and basal ganglia high signal intensities in CJD.

A, B. Axial diffusion-weighted images of a 53-year-old man show abnormal high signal intensities in right striatum, right frontal lobe, left medial frontal lobe, and right medial parietal lobe (arrows).
C. Diffusion-weighted image of a 78-year-old woman shows cortical high signal intensities in right cerebral hemisphere, and focally left frontal and occipital lobes (arrows).
D. FLAIR image of the same patient shows subtle hyperintensity of the lesions (arrows).
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Fig. 3

The chronologic changes of a 59-year-old man with CJD. Diffusion-weighted images were obtained at 30 days (A), 80 days (B), 95 days (C), and 165 days after onset of symptoms.

A. Diffusion-weighted image shows cortical high signal intensities in bilateral parietal lobes.
B. Diffusion-weighted image shows high signal intensities in bilateral cerebral hemisphere and bilateral striatum. The signal intensity of the lesions is more prominent in right than left cerebral hemisphere.
C. Diffusion-weighted image shows more increased extent and signal intensity of the high signal intensity lesions in left cerebral hemisphere and striatum.
D. The last follow-up diffusion-weighted image shows more decreased high signal intensities in the cerebral cortex and striatum, and more progressed cerebral atrophy than C.
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Table 1

Summary of Clinical Findings, MR Imaging Results

jksr-62-327-i001

Note.- 14-3-3: Increased 14-3-3 protein, Onset I.M: interval from onset to initial MR, Interval M - D: interval from initial MR to death, B: bilateral, R: right, L: left, F: frontal lobe, P: parietal lobe, T: temporal lobe, O: occipital lobe, NA: not available

Table 2

Summary of Serial MR Findings

jksr-62-327-i002

Note.- B: bilateral, R: right, L: left, F: frontal lobe, P: parietal lobe, T: temporal lobe, O: occipital lobe

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