Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access
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Case Report

Volume 4, Number 11, November 2013, pages 765-768


Pulvinar Sign in Sporadic Creutzfeldt-Jakob Disease: Exploring the Diagnostic Modalities of Prion Diseases

Figure

Figure 1.
Figure 1. Clear findings for bilateral hyperintensities of the posterior thalamus relative to the signal intensity of the anterior putamen (pulvinar sign).

Table

Table 1. WHO’s Diagnostic Criteria for Creutzfeldt-Jakob Disease, 2010
 
Probable sCJDCaseSuspected vCJD
Other shared criteria include: alternative diagnosis ruled out by routine investigations. No history of iatrogenic exposure. No evidence of familial form of TSE. Duration of the disease greater than 6 months but less than 2 years.
Age-71< 55 years old (current age or age of death)
Clinical featuresRapidly progressive dementia & (2/4): 1: myoclonus, 2: visual or cerebellar signs, 3: pyramidal/extrapyramidal signs, 4: akinetic mutismRapidly progressive dementia, visual and cerebellar signs, pyramidal signs, akinetic mutism, early psychiatric symptoms, dysesthesia changes, gait ataxiaProgressive neuropsychiatric disorder & (4/5): 1: early psychiatric symptoms, 2: persistent painful sensory symptoms, 3: ataxia, 4: myoclonus/chorea/dystonia, 5: dementia
Laboratory testing(1/3): 1: typical sCJD EEG abnormality: periodic sharp bursts, 2: +14-3-3 CSF assay, 3: typical sCJD MRI abnormalities: signal hyperintensity in the basal ganglia and cortical ribboning (DWI & FLAIR)EEG not diagnostic of sCJD
MRI: pulvinar sign: symmetrical high diffusion signal in bilateral pulvinar thalami
+14-3-3 CSF assay
EEG not diagnostic of sCJD
MRI: pulvinar sign: symmetrical high diffusion signal in bilateral pulvinar thalami. Posterior thalami involvement (FLAIR, DWI & T2)