Article Figures & Data
Figures
- Fig 1.
Location of the MRS voxels on axial FLAIR (A and C) and sagittal T1-weighted (B) sequences: lenticular nucleus (1), pulvinar (2), cerebellar vermis (3), and frontal cortex (4).
- Fig 2.
Typical images in cases of sCJD (A), vCJD (B), and gCJD (C). FLAIR, DWI, and ADC map, respectively, are shown. Areas of increased signal intensity, which involve the cortex and the striatum are more extensive and more clearly visible on diffusion images. On the basal ganglia, these changes are associated with a decreased ADC. There is widespread involvement of the cortex in the patient with sCJD. gCJD and vCJD both present with lesions of the thalamus and lenticular nuclei. However, in the variant case, as opposed to the genetic one, the areas of high signal intensity are more pronounced in the pulvinar than in the striatum as has been previously described in this phenotype.
- Fig 3.
Typical spectra recorded in a patient with sCJD (left column) and in a healthy volunteer (right column) in the vermis, pulvinar, right lenticular nucleus, and frontal gray matter. Metabolic anomalies are observed on the bifrontal voxel (decreased NAA and increased mIns), on the lenticular voxel (decreased NAA), and on the bipulvinar voxel (decreased NAA and increased mIns). Note that to get an accurate idea of NAA and mIns variations, one should compare their resonances with “stable” metabolites (eg, Cr).
- Fig 4.
ADC values in the lenticular (A) and caudate (B) nuclei and mIns/NAA ratios on the vermis (C), pulvinar (D), right lenticular nucleus (E), and frontal gray matter (F) in patients with prion disease (p), controls (c), and those with AltD. ADC is decreased and mIns/NAA is increased in patients with prion disease compared with both controls and AltDs. Neither ADC values nor the mIns/NAA ratios can discriminate between the 2 latter groups.
- Fig 5.
ADC values plotted against the NAA/S (A) and mIns/S (B) ratios measured on the same area of the pulvinar in patients with prion disease. There is an absence of correlation between these parameters (R2 = 0.003, P = .8 and R2 = 0.11, P = .08 respectively).
Tables
No. Form Age (yr) PRNP EEGa 14.3.3b Durationc MRI Delayedd 1 FFIe 54 MM D178N-129 mol/L NS – 6 5 2 gCJDe 69 MM E200K + + 4 6 3 gCJDe 58 MM E200K + NA 6 3 4 gCJDe 49 MV D178N-129V – – 10+ 4 5 gCJDe 70 MV V203I NS + 11 10 6 gCJDe 67 MM E200K NA NA 5 3 7 iCJD 18 MV NA – 16 6 8 iCJD 34 MV + + 23 8 9 iCJDe 25 MM – – 12 8 10 GSSe 47 MV P102 L – – 54 27 11 vCJD 43 MM + – 15 12 12 vCJD 52 MM NS – 8 7 13 sCJD 66 NA NA NA NA 3 14 sCJDe 54 MV + + 10 6 15 sCJDe 62 MM + + 19 7 16 sCJDe 66 VV + + 4 2 17 sCJDe 51 MM + + 4 2 18 sCJD 56 MV – + 13 8 19 sCJD 74 MM NA NA 3 2 20 sCJD 52 MM + + 3 2 21 sCJDe 81 MM + + 4 4 22 sCJD 53 MV NS + 21 1 23 sCJD 55 MV + – 60+ 23 24 sCJD 80 MV + – 9 8 25 sCJD 77 MV + – 24+ 10 26 sCJD 40 NA NA NA 10+ 9 27 sCJD 72 MM + + 3 2 28 sCJDe 64 MM + + 4 1 29 sCJD 55 NA – + 26 20 30 sCJD 84 NA NA NA 5 3 31 sCJD 80 NA NS – 2 1 Note:— – indicates not present.
a + Indicates periodic sharp wave complexes.
b Detection of 14.3.3 protein in the CSF.
c Duration of the disease in months.
d Time in months between first symptoms and MR imaging examination.
e The diagnosis was confirmed by postmortem examination and/or mutation was present in the PRNP. PRNP: genotype at codon 129 (MM, VV, MV) and mutation when present.
- Table 2:
Number of patients presenting with areas of significant (≥3 on the visual scale) increased signal/decreased ADC in the different brain structuresa
N = 31 FLAIR DWI Frontal 17 25 Parietal 15 18 Temporal 14 18 Occipital 11 13 Insula 11 15 Cortex, all 19 26 Lenticular 18 23 Caudate 16 23 Thalamus 6 11 Basal ganglia, all 20 25 Brain stem 1 2 Global 26 29 a The most frequently involved cerebral lobes are the frontal, the temporal, and the parietal. In the deep brain structures, the lenticular and the caudate nuclei are affected in a similar number of patients
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