Presentation
6-month history of cognitive decline, now severe.
Patient Data
Prominent restricted diffusion confined to the cortex throughout the parietal and occipital lobes and the anterior aspects of the frontal lobes bilaterally. The posterior aspects of the frontal lobes are relatively spared. The temporal lobes are also involved, allowing for artifact due to air within the middle ear cavities. The involved cortex shows subtle mild FLAIR hyperintensity, and is unremarkable on T2 weighted and proton density imaging. No involvement of the basal ganglia, brainstem, or cerebellum.
Scattered small T2 weighted hyperintense foci within the periventricular and deep white matter of the frontal and parietal lobes bilaterally. This is a nonspecific finding, favored to represent small vessel ischemic change. The brain parenchyma is otherwise normal.
CSF Analysis
- 14-3-3 protein: 41776 AU/ml. Normal <20000.
- Tau protein: 5154 pg/ml. Normal <976.
- EP-QuIC: Positive.
Case Discussion
The clinical history and MRI findings are consistent with CJD. Diagnosis confirmed by CSF analysis.