1. Radiology Department J. Broncano, M. García de Eulate, J. Zubieta, P. Dominguez, J. Arias, M. Arraiza, G. Viteri, M. Simón Aparent diffusion coefficient (ADC) in Prion Encephalopathies: The road to diagnosis

2. Introduction Matherials & methodsResults Limitations Conclusions 1.Neuronal death 2.Spongiosis 3.Perilesional gliosis PrP c (α helices)PrP sc (β folds) - Severe, rapidly progresive and uninvariable fatal neurodegeneration - Altered protein structure - Types: 1. sCJD (85%) 2. fCJD (15%) 3. vCJD (<1%)

3. Extent cortical hiperintensity FLAIR/DWI + striatal alteration ± thalamic afectation = CJD Introduction Matherials & methodsResults Limitations Conclusions Young S et al; AJNR Am J Neuroradiol 2005 26:1551-1562 DWI in Prion Encephalopaties: - High sensitivity and specificity - Restriction in hyperintense areas on T2WI - Respect of Rolandic Cortices

4. IntroductionMatherials & methods Results Limitations Conclusions 1.Diferences between patients with Prion Encephalopathies and controls in diffusion MRI examinations with quantitative ADC assessment 2. No differences between two radiologists, one experienced and other not, in the quantitative evaluation of ADC Study Hypothesis:

5. IntroductionMatherials & methods Results Limitations Conclusions Study protocol: MRI 1.5 Tesla and 3 Tesla T2WI: TSE; TR 4100; TE 140. FLAIR: GRE; TR 8150; TE 125; TI 2580 DWI: EPI (b0; b500; b1000); TR 3780; TE 105 Automatic ADC maps Diagnostic criteria: 1. Clinical findings 2. EEG 3. Protein 14-3-3 in CSF 4. Brain biopsy-autopsy Posible CJD: clinical presentation ± EEG and/or 14-3-3 CSF Probable CJD: 3 criteria Definitive CJD: 3 criteria + brain biopsy/autopsy Exclusion criteria: 1.Intracranial masses 2.Intracranial collections 3.Ischemic pathology 4.Surgical findings Statistical analysis: Normality test: Kolmogorof-Smirnoff & Shapiro-Wilk α=0,05 and extact signification U Mann-Withney Bland-Altman and ICC

6. LimitationsIntroductionMatherials & methods ResultsConclusions Figure 1: ADC values of basal ganglia in patients with CJD (green) and controls (blue). Significant diferences: Caudate, putamen, thalamus, left globus pallidus No significant diferences: Right globus pallidus

7. LimitationsIntroductionMatherials & methods ResultsConclusions Figure 2: ADC values in cortical areas in patients with CJD (green) and controls (blue). Significant diferences: Bilateral PMC, bilateral PSC, bilateral SFG, right SMG No significant diferences: Left SMG

8. LimitationsIntroductionMatherials & methods ResultsConclusions Table 1: ADC cortical quotients: diffuse cortical involvement, INCLUDING Rolandic cortices. The interobserver correlation was excellent (ICC> 0.80) in all cases Ratio U Mann-WithneyAsintotic sig.Exact sig. PSC/SMGr_left67,000,7300,756 PSC/SMGr_right45,000,1310,140 PMC/SFGr_left68,000,7700,796 PMC/SFGr_right52,000,2540,272

9. Introduction Matherials & methodsResults Limitations Conclusions

10. Introduction Matherials & methodsResults Limitations Conclusions 2. Allows automated evaluation in a simple way and reproducible for any radiologist, regardless of their experience. 1. Diffuse affection of cerebral cortex is confirmed. 3. Offers a proper diagnosis sensitive, reliable, reproducible and applicable in daily clinical practice.

11. Jordi Broncano Cabrero jbroncano@unav.es Radiology Department