title: Application of quantitative DTI metrics in sporadic CJD.
abstract: Diffusion Weighted Imaging is extremely important for the diagnosis of probable sporadic Jakob-Creutzfeldt disease, the most common human prion disease. Although visual assessment of DWI MRI is critical diagnostically, a more objective, quantifiable approach might more precisely identify the precise pattern of brain involvement. Furthermore, a quantitative, systematic tracking of MRI changes occurring over time might provide insights regarding the underlying histopathological mechanisms of human prion disease and provide information useful for clinical trials. The purposes of this study were: 1) to describe quantitatively the average cross-sectional pattern of reduced mean diffusivity, fractional anisotropy, atrophy and T1 relaxation in the gray matter (GM) in sporadic Jakob-Creutzfeldt disease, 2) to study changes in mean diffusivity and atrophy over time and 3) to explore their relationship with clinical scales. Twenty-six sporadic Jakob-Creutzfeldt disease and nine control subjects had MRIs on the same scanner; seven sCJD subjects had a second scan after approximately two months. Cortical and subcortical gray matter regions were parcellated with Freesurfer. Average cortical thickness (or subcortical volume), T1-relaxiation and mean diffusivity from co-registered diffusion maps were calculated in each region for each subject. Quantitatively on cross-sectional analysis, certain brain regions were preferentially affected by reduced mean diffusivity (parietal, temporal lobes, posterior cingulate, thalamus and deep nuclei), but with relative sparing of the frontal and occipital lobes. Serial imaging, surprisingly showed that mean diffusivity did not have a linear or unidirectional reduction over time, but tended to decrease initially and then reverse and increase towards normalization. Furthermore, there was a strong correlation between worsening of patient clinical function (based on modified Barthel score) and increasing mean diffusivity.
authors.affiliation: Department of Neurology, University of California, San Francisco (UCSF), San Francisco, CA, USA ; Department of Neuroradiology, C. Mondino National Neurological Institute, Pavia. University of Pavia, Italy.
authors.forename: E
authors.initials: E
authors.lastname: Caverzasi
authors.affiliation: Department of Neurology, University of California, San Francisco (UCSF), San Francisco, CA, USA ; Graduate Group in Bioengineering, UCSF, San Francisco, CA, USA ; Department of Radiology and Biomedical Imaging, UCSF, San Francisco, CA, USA.
authors.forename: R G
authors.initials: RG
authors.lastname: Henry
authors.affiliation: Brain MRI 3T Mondino Research Center C. Mondino National Neurological Institute, Pavia, Italy.
authors.forename: P
authors.initials: P
authors.lastname: Vitali
authors.affiliation: Department of Neurology, University of California, San Francisco (UCSF), San Francisco, CA, USA.
authors.forename: I V
authors.initials: IV
authors.lastname: Lobach
authors.affiliation: Department of Epidemiology and Biostatistics, UCSF, San Francisco, CA, USA.
authors.forename: J
authors.initials: J
authors.lastname: Kornak
authors.affiliation: Department of Neuroradiology, C. Mondino National Neurological Institute, Pavia. University of Pavia, Italy.
authors.forename: S
authors.initials: S
authors.lastname: Bastianello
authors.affiliation: Institute for Neurodegenerative Diseases, University of California, San Francisco (UCSF), USA ; Department of Pathology, University of California, San Francisco (UCSF), USA.
authors.forename: S J
authors.initials: SJ
authors.lastname: Dearmond
authors.affiliation: Memory and Aging Center, Department of Neurology, University of California, San Francisco, (UCSF), USA.
authors.forename: B L
authors.initials: BL
authors.lastname: Miller
authors.affiliation: Memory and Aging Center, Department of Neurology, University of California, San Francisco, (UCSF), USA.
authors.forename: H J
authors.initials: HJ
authors.lastname: Rosen
authors.affiliation: Memory and Aging Center, Department of Neurology, University of California, San Francisco, (UCSF), USA.
authors.forename: M L
authors.initials: ML
authors.lastname: Mandelli
authors.affiliation: Memory and Aging Center, Department of Neurology, University of California, San Francisco, (UCSF), USA.
authors.forename: M D
authors.initials: MD
authors.lastname: Geschwind
mesh_terms: D000375:Aging / Q000473:pathology*; D000465:Algorithms; D001921:Brain / Q000473:pathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis / Q000473:pathology*; D056324:Diffusion Tensor Imaging / Q000379:methods*; D005260:Female; D066128:Gray Matter / Q000473:pathology*; D006801:Humans; D007090:Image Interpretation, Computer-Assisted / Q000379:methods*; D008297:Male; D008875:Middle Aged; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity
keywords: Atrophy; Creutzfeldt–Jakob disease; DWI; MD; Prion disease; sCJD