Abstract Details

Title Restricted Diffusion in White Matter of Sporadic Jakob-Creutzfeldt Disease

Topic Aging and Dementia

Presentation(s) P06 - (-)

Poster/Presentation
Number 044

Objective

Background BACKGROUND: Sporadic Jakob-Creutzfeldt disease (sCJD) is considered primarily to affect gray matter, whereas evidence of white matter (WM) involvement is scarce.

Design/Methods DESIGN/METHODS: Twenty-six patients with sCJD and nine healthy controls (HCs) had a brain MRI with T1-weighted 3D FSPGR volume and DTI. A parcellation of the subcortical WM into 34 volumes of interest for each hemisphere was performed with Freesurfer. The DTI maps were co-registered to T1 space and the averages of each DTI metric were calculated in the whole brain WM and in the single volumes of interest (VOI). Tract-based spatial statistics analysis (TBSS) was also performed to investigate the deep WM for all the DTI metrics.

Results RESULTS: Cross-sectional analysis showed a significant reduction of mean (P=0.002), of axial (P=0.0003) and radial (P=0.0134) diffusivities, in whole brain WM in sCJD compared to HCs. Mean Diffusivity (MD) was significantly reduced in a symmetric pattern (P< 0.05 with multicomparison correction) in most of the WM VOIs in sCJD compared to HCs. TBSS showed a trend towards reduced MD in the majority of the WM regions, but mainly showed significant restriction of mean diffusivity in the deep nuclei white matter of the left hemisphere. Preliminary review of the autopsy findings shows in the white matter a diffuse gliosis, rarely with concomitant subcortical microvacuolation only in a subgroup of patients, and even rarer prion deposition.

Conclusions CONCLUSIONS: We show for the first time a significant global involvement, with reduced mean diffusivity, in the white matter in sCJD. We hypothesize this WM abnormality is primary and not secondary to neuronal degeneration/loss.

Authors/Disclosures
PRESENTER	No disclosure on file
Roland G. Henry, PhD (University of California, San Francisco)	Dr. Henry has received personal compensation in the range of $500-$4,999 for serving as a Consultant for MEDDAY. Dr. Henry has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Henry has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Henry has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Sanofi/Genzyme.
	No disclosure on file
Kendra Bechtel	No disclosure on file
	No disclosure on file
Sven Forner	No disclosure on file
	No disclosure on file
	No disclosure on file
Michael D. Geschwind, MD, PhD, FAAN (UCSF)	Dr. Geschwind has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Brainstorm Cell Therapeutics, Inc.. Dr. Geschwind has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Walter Grubb. Dr. Geschwind has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Gerson Lehrman Group. Dr. Geschwind has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Reata Pharmaceuticals, Inc..

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