However, our patient's age of disease onset, typical EEG (PSWC), absent pulvinar sign
on MRI as well as lack of past medical/surgical and family history was not consistent with vCJD.
The pulvinar sign
on MRI refers to bilateral FLAIR hyperintensities in the pulvinar and thalamic nuclei and can be seen invariant and sporadic CJD [7,9,10,12].
MRI of the brain showed mild diffuse volume loss, T2 hyperintensity with subtle restricted diffusion in the pulvinar nuclei of bilateral thalami (pulvinar sign), and subtle restricted diffusion in the right frontal cortex (cortical ribbon sign) (arrows) (Figure 2).
The only positive finding of diagnostic value in vCJD from these tests is the pulvinar sign on MRI, which is not entirely specific for vCJD and can be absent in up to 9% of cases, even after multiple MRIs (14).
The pulvinar sign, that is, an increased signal on unenhanced Tl-weighted imaging involving the pulvinar, has been described in a portion (of especially male) Fabry patients (Figure 10) .
Konaka et al., "The "pulvinar sign" in a case of paraneoplastic limbic encephalitis associated with non-Hodgkin's lymphoma," Journal of Neurology, Neurosurgery and Psychiatry, vol.
The pulvinar sign was present on the T2-weighted images in 5 of the 10 cases.
The pulvinar sign has diagnostic value for vCJD, but studies have also reported it in sCJD cases where it was accompanied by signal changes in other regions of the brain (13,14).
One patient had florid plaques and a pulvinar sign
on magnetic resonance imaging, mimicking vCJD (5).
A characteristic high signal in the posterior thalamus on T2- and diffusion-weighted magnetic resonance imaging (the "pulvinar sign
") is demonstrated in >75% of vCJD patients, and in the appropriate clinical context, is highly indicative of a vCJD diagnosis (7).
Patients with vCJD can be distinguished from patients with the more common sporadic CJD by their younger median age at death (28 years and 68 years, respectively), predominantly psychiatric manifestations at illness onset, delayed appearance of frank neurologic signs, absence of a diagnostic electroencephalographic pattern, presence of the pulvinar sign
on MRI, and a longer median illness duration (<6 and 14 months, respectively) (3,8).
The pulvinar sign
on magnetic resonance imaging in variant Creutzfeldt-Jakob disease.