Article Figures & Data
Figures
- Fig 1.
Patient 5. DW MR images (b = 1000 s/mm2) (A and B) and corresponding ADC maps (C and D) show the level of the basal ganglia (A and C) and the hippocampus (B and D). The marked areas show the region-of-interest measurements in the head of the caudate nucleus, putamen, pulvinar thalami, and MD (A and C) and in the hippocampus (B and D).
- Fig 2.
Patient 1, second examination. DW image (A), T2-weighted TSE (B), and FLAIR (C) images. Hyperintense SI changes in the striatum and the pulvinar thalami are discernable on the DW (A) and FLAIR (C) images bilaterally, whereas on the T2-weighted TSE image, increased SI is discernable only in the striatum bilaterally.
- Fig 3.
Mean ADCs (± SEM) of sCJD patients (right dark columns) and controls (left bright columns). ADCs in sCJD patients are significantly reduced in the caudate nucleus, putamen, MD, and pulvinar thalami as compared with controls. In the hippocampus (last pair of columns on the right), ADCs are comparable.
- Fig 4.
Patient 1. Serial DW images (A–C) and corresponding ADC maps (D–F) acquired 3 (A and D), 4 (B and E), and 8 (C and F) months after onset of symptoms. The DW image acquired 3 months after onset of symptoms (A) shows bilateral high SI in the caudate nucleus and the rostral part of the putamen. Four months after onset of symptoms, the DW image (B) shows high SI also in the dorsal part of the putamen and additionally in the pulvinar thalami. Eight months after onset of symptoms, the DW image (C) shows severe brain atrophy with widening of the ventricles and of the sulci. The head of the caudate nucleus on the left side totally disappeared; the right caudate nucleus and the putamen on both sides show severe atrophy. There is high SI in the right caudate nucleus and putamen bilaterally and in the frontal, parietal, and cingulate cortex. The ADC maps (D–F) show low SI in the hyperintense areas on DW images corresponding to restricted diffusion.
- Fig 5.
Patient 1. Time courses of the ADC values in serial images. Measurements were performed in five anatomic regions: the head of the caudate nucleus (empty rectangle), the putamen (open rhomboid), the pulvinar thalami (filled triangle), the MD (filled circle), and the hippocampus (star). In all areas except for the hippocampus, ADCs were lowest in the first examination and highest in the last examination. In the caudate nucleus, the putamen, and the MD, there was a gradual increase in the ADCs during the course of the disease. In the pulvinar thalami, ADCs were lowest in the second examination; this was paralleled by an increased SI on DW images in the second examination (shown in Fig 4B, E).
Tables
Patient 1 2 3 4 5 6 Age at onset 56.2 years 58.9 years 71.9 years 62.9 years 63.6 years 61 years Gender Male Male Male Female Male Female Symptoms/signs Dementia, ataxia, myoclonus Dementia, vertigo, ataxia Dementia, ataxia, myoclonus Dementia, vertigo, gait disturbances Dementia, ataxia, signs of pyramidal tract Dementia, vertigo, ataxia EEG Not typical for CJD Not typical for CJD PSWCsa PSWCsa PSWCs Not typical for CJD Protein 14-3-3 Positive Positive Positive Positive Positive Positive Diagnosis of CJDb Probable Probable Probable Probable Definite Probable a PSWCs are periodic sharp wave complexes (4).
b WHO 1998 (1).
Pulvinar MDa Putamen Caudate Nucleus Hippocampus Other Patient 1:b DW images — — ↑ SI bilat. ↑ SI bilat. — — T2w images — — ↑ SI bilat. ↑ SI l. > r. — — FLAIR images — — ↑ SI bilat. ↑ SI bilat. Patient 2: DW images (↑ SI bilat.) — ↑ SI bilat. ↑ SI bilat. — — T2w images — — ↑ SI l. ↑ SI l. — — FLAIR images (↑ SI bilat.) — ↑ SI bilat. ↑ SI bilat. — Patient 3: DW images — — (↑ SI bilat.) (↑ SI bilat.) — — T2w images — — (↑ SI bilat.) — — FLAIR images — — (↑ SI bilat.) (↑ SI bilat.) — Patient 4: DW images ↑ SI bilat. — ↑ SI bilat. ↑ SI bilat. — — T2w images — — — — — — FLAIR images (coronal) —d —d ↑ SI bilat. ↑ SI bilat. — — Patient 5: DW images ↑ SI bilat. — ↑ SI bilat. ↑ SI bilat. — ↑ SI Cerebellar cortex b0 images of DW imagingc — — ↑ SI r. ↑ SI r. — — FLAIR images — — ↑ SI bilat. ↑ SI bilat. — ↑ SI Cerebellar cortex Patient 6: DW images ↑ SI bilat. — ↑ SI bilat. ↑ SI bilat. — — T2w images — — ↑ SI bilat. ↑ SI bilat. — — FLAIR images ↑ SI bilat. — ↑ SI bilat. ↑ SI bilat. — — Note.—Parentheses indicate slight change. An upward-pointing arrow (↑) indicates that a value has increased. A dash (—) indicates no signal change; bilat., bilateral; l., left; r., right; SI, signal intensity.
a Mediodorsal thalamic nucleus.
b The first of the three consecutive measurements of patient 1 is considered.
c b0 images of the DW imaging scan were used, because no T2-weighted TSE images were acquired.
d Disturbed by motion artifacts.
Area Patientsa (mean ± SEM) Controls (mean ± SEM) Pb (unpaired two-tailed Student t test) Caudate nucleus 548 ± 27 767 ± 35 <0.0001 Putamen 585 ± 28 754 ± 22 <0.0001 Mediodorsal thalamic nucleus 664 ± 28 800 ± 24 0.0011 Pulvinar: 1. All patients 695 ± 27 853 ± 15 <0.0001 2. Patients with SI changes 701 ± 38 c 3. Patients without SI changes 684 ± 37 Hippocampus 853 ± 22 871 ± 21 0.56 a In patient 1 in Table 1, the first of the three consecutive measurements was considered.
b P < .05 is considered significant.
c P = .78 (not significant) comparing sCJD patients with and those without SI changes in the thalamus.
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