Amyloid- and FDG-PET in sporadic Creutzfeldt-Jakob disease: Correlation with pathological prion protein in neuropathology

Figures & data

FIGURE 1. MRI and FDG-PET. In upper row, diffusion-weighted imaging with no alterations. In middle row, fluid attenuated inversion recovery (FLAIR) sequence showing global atrophy. In lower row, FDG-PET imaging showed a left frontal-parietal, bilateral thalamus and cerebellar hypometabolism.

FIGURE 2. ¹⁸F-florbetaben PET imaging. Uptake tracer in white matter is generally higher than in gray matter. In left parietal and right superior temporal lobes, tracer uptake is similar in white and gray matter.

FIGURE 3. Frontal cortex showing no deposition of β-amyloid.

FIGURE 4. Pathological prion protein expression in several brain regions. Immunoreactivity of pathological prion protein in 6 regions (inferior temporal, caudate, anterior cingulate, calcarine, supplementary motor area, and precuneus) is shown. Each region is observed at 1.5x and 20x magnification. Different levels of expression (low, medium, and high) may be observed.

TABLE 1. Studies of prion diseases using amyloid PET tracers.

	Number of cases	Type of prion disease	Tracer	Main results
Boxer et al. 2007	2	Two brother with hereditary CJD (PRNP mutation	PiB in one case, FDDNP in the other	PiB uptake similar to controls; FDDNP higher uptake than controls
Villemagne et al. 2009	2	Sporadic CJD	PiB	PiB uptake similar to controls
Kepe et al. 2010	6	Gerstmann-Sträussler-Scheinker disease	FDDNP	Increased FDDNP uptake. Tracer uptake correlates with symptoms.
Hyare et al. 2012	5	1 variant CJD, 2 patients with P102L mutation, and 2 carriers of PRNP mutation	PiB	PiB uptake similar to controls
Present study	1	Sporadic CJD	Florbetaben	Florbetaben uptake correlates to prion protein deposition