Role of magnetic resonance imaging, cerebrospinal fluid, and electroencephalogram in diagnosis of sporadic Creutzfeldt-J
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Role of magnetic resonance imaging, cerebrospinal fluid, and electroencephalogram in diagnosis of sporadic Creutzfeldt-Jakob disease Leo H. Wang • Robert C. Bucelli • Erica Patrick • Dhanashree Rajderkar • Enrique Alvarez III Miranda M. Lim • Gabriela DeBruin • Victoria Sharma • Sonika Dahiya • Robert E. Schmidt • Tammie S. Benzinger • Beth A. Ward • Beau M. Ances
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Received: 16 April 2012 / Revised: 25 July 2012 / Accepted: 25 August 2012 / Published online: 12 September 2012 Ó Springer-Verlag 2012
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
L. H. Wang, R. C. Bucelli and E. Patrick contributed equally to the manuscript.
Electronic supplementary material The online version of this article (doi:10.1007/s00415-012-6664-6) contains supplementary material, which is available to authorized users. L. H. Wang R. C. Bucelli E. Patrick E. Alvarez III M. M. Lim G. DeBruin V. Sharma B. A. Ward B. M. Ances (&) Department of Neurology, Washington University School of Medicine, Box 8111, 660 South Euclid Avenue, Saint Louis, MO 63110, USA e-mail: [email protected] L. H. Wang Department of Neurology, University of Washington School of Medicine, Seattle, WA, USA E. Patrick Department of Neurology, University of Rochester, Rochester, NY, USA
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 diffusionweighted 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.
D. Rajderkar T. S. Benzinger Department of Radiology, Washington University School of Medicine, Saint
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