Sporadic Jakob-Creutzfeldt disease presenting as primary progressive aphasia

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

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.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural

MeSH terms

  • Aphasia, Primary Progressive / complications*
  • Aphasia, Primary Progressive / diagnosis*
  • Aphasia, Primary Progressive / psychology
  • Creutzfeldt-Jakob Syndrome / complications*
  • Creutzfeldt-Jakob Syndrome / diagnosis*
  • Creutzfeldt-Jakob Syndrome / psychology
  • Diagnosis, Differential
  • Humans
  • Male
  • Middle Aged
  • Neuropsychological Tests