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Arch Neurol. Sporadic Jakob-Creutzfeldt Disease Presenting as Primary Progressive Aphasia

Giuseppe

Emeritus
[Source: Archives of Neurology, full text: (LINK). Abstract, edited.]

Observation| 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.
Published online November 5, 2012



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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