title: Role of magnetic resonance imaging, cerebrospinal fluid, and electroencephalogram in diagnosis of sporadic Creutzfeldt-Jakob disease.
abstract: Sporadic Creutzfeldt-Jakob disease (sCJD) is a rapidly progressive dementia (RPD) that can be difficult to identify antemortem, with definitive diagnosis requiring tissue confirmation. We describe the clinical, magnetic resonance imaging (MRI), cerebrospinal fluid (CSF), and electroencephalogram (EEG) measures of a small cohort of 30 patients evaluated for RPD. Clinical and diagnostic measures were cross-sectionally obtained from 17 sCJD patients (15 definite, two probable), 13 non-prion rapidly progressive dementia patients (npRPD), and 18 unimpaired controls. In a subset of patients (nine sCJD and nine npRPD) diffusion tensor imaging (DTI) measures [fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD)] were also obtained for the caudate, corpus callosum, posterior limb of the internal capsule, pulvinar, precuneus, and frontal lobe. Differences among groups were assessed by an analysis of variance. Compared to npRPD individuals, sCJD patients had cerebellar dysfunction, significantly higher CSF tau, "positive" CSF 14-3-3, and hyperintensities on diffusion-weighted imaging (DWI) that met previously established imaging criteria for sCJD. EEG changes were similar for the two groups. In addition, sCJD patients had significant decreases in DTI measures (MD, AD, RD but not FA) within the caudate and pulvinar compared to either npRPD patients or unimpaired controls. Our results confirm that CSF abnormalities and MRI (especially DWI) can assist in distinguishing sCJD patients from npRPD patients. Future longitudinal studies using multiple measures (including CSF and MRI) are needed for evaluating pathological changes seen in sCJD patients.
authors.affiliation: Department of Neurology, Washington University School of Medicine, Box 8111, 660 South Euclid Avenue, Saint Louis, MO 63110, USA.
authors.forename: Leo H
authors.initials: LH
authors.lastname: Wang
authors.forename: Robert C
authors.initials: RC
authors.lastname: Bucelli
authors.forename: Erica
authors.initials: E
authors.lastname: Patrick
authors.forename: Dhanashree
authors.initials: D
authors.lastname: Rajderkar
authors.forename: Enrique
authors.initials: E
authors.lastname: Alvarez Iii
authors.forename: Miranda M
authors.initials: MM
authors.lastname: Lim
authors.forename: Gabriela
authors.initials: G
authors.lastname: Debruin
authors.forename: Victoria
authors.initials: V
authors.lastname: Sharma
authors.forename: Sonika
authors.initials: S
authors.lastname: Dahiya
authors.forename: Robert E
authors.initials: RE
authors.lastname: Schmidt
authors.forename: Tammie S
authors.initials: TS
authors.lastname: Benzinger
authors.forename: Beth A
authors.initials: BA
authors.lastname: Ward
authors.forename: Beau M
authors.initials: BM
authors.lastname: Ances
mesh_terms: D000368:Aged; D001921:Brain / Q000473:pathology; D015331:Cohort Studies; D007562:Creutzfeldt-Jakob Syndrome / Q000134:cerebrospinal fluid / Q000175:diagnosis / Q000503:physiopathology; D038524:Diffusion Magnetic Resonance Imaging; D004569:Electroencephalography; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies