Article Figures & Data
Figures
- Fig 1.
Case 1. Sample case of predominant striatal lesions in the early stage. Images were obtained at 3 (A and B) and 5 (C and D) months from the onset of symptoms.
A, FLAIR image shows changes, which are not as conspicuous as in B.
B, Striata appear hyperintense at diffusion-weighted imaging. Note that the anterior portion of the bilateral putamina (arrows) appears more hyperintense than does the posterior portion at diffusion-weighted imaging.
C and D, Severe atrophy is depicted in both cerebral cortices and the caudate nuclei heads at FLAIR imaging (C) and diffusion-weighted imaging (D). Note that the putamina are entirely involved in C as compared with their appearance in B. Hyperintensity in the heads of the caudate nuclei appears less prominent; this appearance is associated with their volume loss and the dilatation of the frontal horns.
- Fig 2.
Case 3. Sample case in which cerebral cortices were involved mainly in the early stage.
A and B, At 1 month from the onset of symptoms, FLAIR imaging (A) reveals only subtle changes, whereas diffusion-weighted imaging (B) reveals hyperintense lesions in the cerebral cortices with a faint left striatal lesion. Note that the left striatum is asymmetrically involved (the right striatum is spared), and the anterior portion of the left putamen appears hyperintense compared with the posterior portion (arrowhead in B).
C, At 3 months from onset of symptoms, diffusion-weighted imaging reveals not only cortical lesions but also diffuse bilateral striatal lesions (arrowheads). Note that the left putaminal lesion spread posteriorly and entirely, as compared with its appearance in B.
- Fig 3.
Case 13. Sample case in which a cortical lesion was apparent at diffusion-weighted imaging but not at FLAIR imaging. Images were obtained 2 months after onset of symptoms.
A, FLAIR image barely depicts the lesions shown in B.
B, Diffusion-weighted imaging reveals hyperintense cortical lesions in the medial aspects of bilateral frontal lobes.
- Fig 4.
Case 10. Sample case in which FLAIR imaging is of little diagnostic value because of severe motion artifacts. Images were obtained 2.5 months after the onset of symptoms.
A, Motion artifacts are so severe on the FLAIR image that it cannot provide diagnostic clues.
B, Diffusion-weighted imaging, however, clearly reveals hyperintense lesions (arrows) in right temporo-occipital cortices and cingulate gyrus.
- Fig 5.
Cases 3 (A and B) and 5 (C and D). Sample cases with chronologic changes in striatal lesions (arrow).
A, At 1 month after the onset of symptoms, diffusion-weighted imaging reveals left striatal lesions. Note that the anterior portion of the left putamina appears more hyperintense than the posterior portion.
B, At 3 months after the onset of symptoms, diffusion-weighted imaging reveals that the left putaminal lesions spread posteriorly and entirely, as compared with the findings in A.
C, At 3 months after the onset of symptoms, diffusion-weighted imaging shows the right striatal lesion. Note that the high-signal-intensity change in the putamen is restricted to its anterior portion.
D, At 5 months after the onset of symptoms, diffusion-weighted imaging shows that the putaminal lesion has spread in the posterior direction, as compared with the findings in C.
- Fig 6.
Case 2. Sample case with chronologic changes in ADC in a striatal lesion.
A and B, Diffusion-weighted imaging and corresponding ADC map, respectively. These images were obtained 7 months from the onset of symptoms. High signal intensity is present in the left striatum (A), and its lesional diffusivity is low (arrow in B).
C and D, Diffusion-weighted imaging and corresponding ADC map, respectively. These images were obtained 8 months from the onset of symptoms. The lesion still has high signal intensity (C), and its ADC is still low (arrow in D).
- Fig 7.
Case 2. Sample case with chronologic ADC changes in cerebral cortical lesions.
A and B, Diffusion-weighted imaging and corresponding ADC map, respectively. These images were obtained 7 months after the onset of symptoms. The cerebral cortices are hyperintense (A), and lesional ADCs are low (arrows in B).
C and D, Diffusion-weighted imaging and corresponding ADC map, respectively. These images were obtained 8 months after the onset of symptoms. The lesions are still hyperintense (C), and lesional ADCs are still low (arrows in D).
- Fig 8.
Short-TI inversion recovery images. Dotted arrows in the left striatum show the supposed pathway of lesional distribution along the putaminocaudate gray matter bridges and nucleus accumbens septi.
A, Coronal image shows the anatomic connections between the caudate head and the putamen. Solid arrow and arrowhead in the right cerebral hemisphere indicate the putaminocaudate gray matter bridges and the nucleus accumbens septi, respectively.
B, Solid arrows in the left putamen on this axial image show the direction of lesional extension observed in our study.
Tables
Case No./Patient Sex/Age (y) Dementia Myoclonus PSD Prion Protein Gene CDJ Type Diagnostic Level Diffusion-weighted Imaging FLAIR Imaging ADC Analysis 1/F/55 Yes Yes Yes M232R F Definite E(1), L(2) E(1), L(2) E(1), L(2) 2/M/78 Yes No No V180I F Definite L(2) L(2) L(2) 3/M/70 No Yes Yes NA S? Probable E(4), L(1) E(1) NA 4/M/66 Yes Yes Yes Wild type S Probable E(1), L(3) E(1), L(3) E(1), L(3) 5/M/60 Yes No Yes Wild type S Probable E(1), L(1) E(1), L(1) E(1), L(1) 6/M/58 Yes Yes Yes E200K F Definite L(3) NA NA 7/F/75* Yes No No Wild type† S Definite L(1) L(1) L(1) 8/M/68 Yes Yes Yes Wild type S Probable E(2) E(1) NA 9/F/74 Yes Yes Yes Wild type S Probable L(1) L(1) L(1) 10/F/76 Yes Yes Yes NA S? Probable E(1) E(1) E(1) 11/M/78 Yes Yes Yes NA S? Probable E(1) E(1) E(1) 12/F/76 Yes Yes Yes NA S? Probable E(2) E(2) E(1) 13/F/82 Yes No No V180I F Definite E(1) E(1) E(1) Note.—E200K indicates the change of codon 200 from glutamate to lysine; M232R, change of codon 232 from methionine to arginine; V180I, change of codon 180 from valine to isoleucine; NA, not available; F, familial; S, sporadic; S?, possibly sporadic; E, the early-stage study; L, late-stage study. The number in the parenthesis indicates the number of MR studies performed. Total numbers of examinations were as follows: diffusion-weighted imaging, 14 early stage and 14 late stage; FLAIR imaging, 10 early stage and 10 late stage; ADC imaging, seven early stage and 10 late stage.
* The diagnosis of CJD was confirmed at histopathologic analysis.
† Wild-type prion protein gene with valine homozygosity at codon 129.
Examination Early Stage Late Stage Total Diffusion-weighted imaging 9 (14) 8 (14) 13 (28) Diffusion-weighted imaging with FLAIR imaging 9 (10) 6 (10) 12 (20) ADC imaging 7 (7) 6 (10) 10 (17) Note.—Data are the number of patients. Data in parentheses are the number of studies.
Site of Lesion Early Stage (n = 9) Late Stage (n = 8) Striatum 8 (89) 8 (100) Cortex 8 (89) 6 (75) Thalamus 0 (0) 1 (12) Globus pallidus 0 (0) 0 (0) Note.—Data are the number of patients. Data in parentheses are percentages.
- TABLE 4:
Comparison of lesional conspicuity at diffusion-weighted imaging and FLAIR imaging
Site of Lesion Category* Total A B C Striatum Early 0 12 10 22 (7) Late 2 12 17 31 (6) Total 2 24 27 53 (11) Cortex Early 0 3 11 14 (7) Late 0 5 9 14 (4) Total 0 8 20 28 (10) Note.—Data are the number of lesions. Data in parentheses are the number of patients.
* We subjectively assessed lesional conspicuity and categorized it as follows: A, diffusion-weighted imaging was inferior to FLAIR; B, diffusion-weighted imaging was equal to FLAIR; and C, diffusion-weighted imaging was superior to FLAIR.
A: Progression pattern of striatal lesions* Lesion Initial Diffusion-weighted Imaging Study Latest Diffusion-weighted Imaging Study Asymmetric 4 (50) 1 (12) Symmetric 4 (50) 7 (88) B: Relationship between lesions† Caudate Head Lesion Initial Diffusion-weighted Imaging Study Latest Diffusion-weighted Imaging Study Without ipsilateral putaminal lesion 4 0 With ipsilateral putaminal lesion 11 15 Note.—The evaluation of chronologic changes involved data from eight patients with striatal lesions who were examined with diffusion-weighted imaging more than twice.
* Data are the number of patients (n = 8). Data in parentheses are percentages.
† Data are the number of caudate head lesions with or without ipsilateral putaminal lesion.
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