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Observation | November 5, 2012 ONLINE FIRST

Sporadic Jakob-Creutzfeldt Disease Presenting as Primary Progressive Aphasia ONLINE FIRST

David Y. Johnson, MD; Diana L. Dunkelberger, MA; Maya Henry, PhD; Aissatou Haman, MD; Michael D. Greicius, MD, PhD; Katherine Wong, BA; Stephen J. DeArmond, MD, PhD; Bruce L. Miller, MD; Maria Luisa Gorno-Tempini, MD, PhD; Michael D. Geschwind, MD, PhD

Arch Neurol. 2012;():1-4. doi:10.1001/jamaneurol.2013.139.

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ABSTRACT

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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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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.

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Johnson DY, Dunkelberger DL, Henry M, et al. Sporadic Jakob-Creutzfeldt Disease Presenting as Primary Progressive Aphasia. Arch Neurol. 2012;():1-4. doi:10.1001/jamaneurol.2013.139.

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Sporadic Jakob-Creutzfeldt Disease Presenting as Primary Progressive Aphasia ONLINE FIRST

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