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Case Reports
. 2013 Feb;70(2):254-7.
doi: 10.1001/2013.jamaneurol.139.

Sporadic Jakob-Creutzfeldt disease presenting as primary progressive aphasia

David Y Johnson  1 , Diana L Dunkelberger, Maya Henry, Aissatou Haman, Michael D Greicius, Katherine Wong, Stephen J DeArmond, Bruce L Miller, Maria Luisa Gorno-Tempini, Michael D Geschwind
Affiliations
Case Reports

Sporadic Jakob-Creutzfeldt disease presenting as primary progressive aphasia

David Y Johnson et al. JAMA Neurol. 2013 Feb.

Abstract

Objective: To report the clinical, neuropsychological, linguistic, imaging, and neuropathological features of a unique case of sporadic Jakob-Creutzfeldt disease in which the patient presented with a logopenic variant of primary progressive aphasia.

Design: Case report.

Setting: Large referral center for atypical memory and aging disorders, particularly Jakob-Creutzfeldt disease.

Patient: Patient presenting with logopenic variant primary progressive aphasia initially thought to be due to Alzheimer disease.

Results: Despite the long, slow 3.5-year course, the patient was shown to have pathology-proven sporadic Jakob-Creutzfeldt disease.

Conclusions: These findings expand the differential of primary progressive aphasia to include prion disease.

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Conflict of interest statement

Conflict of Interest Disclosures: Dr Miller reports that he was a consultant for Tau Rx, Allon Therapeutics, Bristol-Myers Squibb, and Siemens, that he received grants from the National Institutes of Health/National Institute on Aging and Novartis, and that he received royalties of Cambridge University Press. Dr Geschwind reports that he has done consulting for Neurophage, Gerson Lehrman Group, and MEDACorp and has received grants from the National Institutes of Health/National Institute on Aging.

Figures

Figure
Figure
Axial fluid-attenuated inversion recovery (FLAIR), diffusion weighted imaging (DWI)/magnetic resonance imaging scans at 22, 24, and 31 months after onset showing extensive left greater than right cortical ribboning (hyperintensity) in the insula and peri-insular cortex (dotted arrow) and the cingulate gyrus (dashed arrow) and left greater than right cortical ribboning in the parietal-temporal cortex (angular gyrus; solid arrow). There is also a slight bilateral caudate hyperintensity. The apparent diffusion coefficient map confirmed restricted diffusion (not shown). Images are per radiological convention; thus, right is left and left is right.
See this image and copyright information in PMC

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