Article Figures & Data
Figures
- Fig 1.
A, Normal FLAIR image at the level of the basal ganglia shows the thalamus is normally isointense or slightly hypointense relative to the putamen. This appearance is depicted with most sequences, particularly the FLAIR sequence.
B, Pulvinar sign of vCJD. FLAIR image shows marked, symmetrical hyperintensity of the pulvinar (posterior) thalamic nuclei. In this case, the pulvinar signal intensity was scored as grade 4 by both observers.
C, “Hockey-stick” sign of vCJD. FLAIR image shows symmetrical pulvinar and dorsomedial thalamic nuclear hyperintensity. This combination gives a characteristic “hockey-stick” appearance and was present in 93% of cases with FLAIR imaging.
- Fig 2.
Grading of degree of pulvinar hyperintensity in vCJD. Because only hard-copy images were available in most cases of vCJD, a 5-point semiquantitative scoring system was used to grade the degree of hyperintensity of the cerebral structures, with white matter scored as 0, normal gray matter scored as 1, and pathologic hyperintensity scored from 2 to 4. Examples of FLAIR images are presented.
A, Normal FLAIR image for comparison.
B, Grade 2 hyperintensity of pulvinar.
C, Grade 3 hyperintensity of pulvinar.
D, Grade 4 hyperintensity of pulvinar
- Fig 3.
Effect of MR sequence on visibility of the pulvinar sign.
A, Nonenhanced T1-weighted image. High signal intensity was relatively rarely detected on T1-weighted images, which was thought to be secondary to the T1-shortening effect from marked prion protein deposition. Contrast enhancement was not seen in any case.
B–D, The pulvinar sign was more easily seen on T2-weighted (B) and PD-weighted (C) images, but was clearly visible on FLAIR (D) images.
E, DW image was also positive in one of two available cases.
- Fig 4.
Graph shows timing of MR imaging in relation to vCJD duration. Dark gray indicates negative MR images; light gray, positive MR images
- Fig 5.
Other MR imaging findings in vCJD.
A, Axial FLAIR image shows periaqueductal gray matter hyperintensity (arrow). Though not a specific sign, periaqueductal gray matter hyperintensity was seen in 83% of cases with FLAIR imaging.
B, FLAIR image shows abnormal hyperintensity in the centrum semiovale white matter (arrows), reflecting the diffuse involvement of the brain by the disease.
C, FLAIR image shows asymmetrical bilateral pulvinar hyperintensity. Asymmetrical hyperintensity was a rare finding in vCJD, seen in less than 5% of cases.
D, PD-weighted image shows widespread basal ganglia hyperintensity. Though hyperintensity in the basal ganglia is also seen in a number of other conditions, the signal intensity of the pulvinar remains the most hyperintense, helping to categorize this case correctly as probable vCJD.
E and F, Case of progressive cerebral atrophy in vCJD. Two images taken 3 months apart show subtle but definite global cerebral atrophy. Unlike in sCJD, cerebral atrophy is not a prominent feature of vCJD and is most easily seen in the parietooccipital region.
Tables
Class or Diagnosis Definition I A Progressive neuropsychiatric disorder. B Duration of illness > 6 months. C Routine investigations do not suggest alternative diagnosis. D No history of potential iatrogenic exposure. E No history of familial form of transmissible spongiform encephalopathy. II A Early psychiatric symptoms.* B Persistent painful sensory symptoms.† C Ataxia. D Myoclonus or chorea or dystonia. E Dementia. III A EEG does not show typical appearance of sCJD (or no EEG).‡ B Brain MR image shows bilateral symmetrical pulvinar high signal intensity.§ IV A Positive tonsil biopsy.‖ Definite Class IA and neuropathologic confirmation of vCJD.# Probable Class I and 4 of 5 of class II and classes IIIA and IIIB, or class I and class IVA. Possible Class I and 4 of 5 of class II and class IIIA. * Depression, anxiety, apathy, withdrawal, delusions.
† This includes both frank pain and/or dysesthesia.
‡ Generalized triphasic periodic complexes at approximately one per second.
§ Relative to the signal intensity of the other deep gray matter nuclei and cortical gray matter. (Modification of the case definition of the characteristic MR imaging features [IIIB] to “Brain MR image shows bilateral symmetrical pulvinar hyperintensity relative to the signal intensity of the anterior putamen” is recommended to improve the accuracy of the pulvinar sign in vCJD).
‖ Tonsil biopsy is not recommended routinely, nor in cases with an EEG appearance typical of sCJD, but may be helpful in suspect cases in which the clinical features are compatible with vCJD and in which the MR image does not show bilateral pulvinar high signal intensity.
# Spongiform change and extensive prion protein deposition with florid plaques throughout the cerebrum and cerebellum.
- TABLE 2:
Variation in Interobserver Agreement for the Presence of the Pulvinar Sign on MR Images in 86 Cases of vCJD
Sequence No. of Images Observer 1 Positive* Observer 2 Positive* Concordance Positive* κ Value PD 58 45 (78) 51 (88) 47 (81) 0.64 T2 108 71 (66) 83 (77) 76 (70) 0.73 FLAIR 30 30 (100) 30 (100) 30 (100) 1.0 All images 110 91 (83) 100 (91) 96 (87) 0.65 * Data are number of images. Numbers in parentheses are percentages.
Sequence No. of Images Consensus Positive for Pulvinar Hyperintensity* T1 75 7 (9) PD 58 47 (81) T2 108 77 (71) FLAIR 30 30 (100) All images 110 96 (87) * Data are number of images. Numbers in parentheses are percentages.
Structure T2 PD FLAIR Hyperintense Moderate or Marked Hyperintensity Hyperintense Moderate or Marked Hyperintensity Hyperintense Moderate or Marked Hyperintensity Dorsomedial thalamus 45.4 8.3 77.6 29.3 93.3 36.7 Caudate head 25.9 0 62.1 3.5 40 0 Putamen 22.2 0 55.2 1.7 23.3 0 Periaqueductal gray matter 50 4.6 62.1 19.0 83.3 16.7 Note.—Data are percentages.
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