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. 2011 Dec;7(4):227-30.
doi: 10.3988/jcn.2011.7.4.227. Epub 2011 Dec 29.

Pure word deafness in a patient with early-onset Alzheimer's disease: an unusual presentation

Sook Hui Kim  1 , Mee Kyung Suh, Sang Won Seo, Juhee Chin, Seol-Heui Han, Duk L Na
Affiliations

Pure word deafness in a patient with early-onset Alzheimer's disease: an unusual presentation

Sook Hui Kim et al. J Clin Neurol. 2011 Dec.

Abstract

Background and purpose: The occurrence of PWD in neurodegenerative disease is very rare, and this is the first report of it being related to early-onset AD. We describe a patient with early-onset Alzheimer's disease (AD) who presented with pure word deafness (PWD).

Case report: The patient had experienced PWD for 2 years, followed by other cognitive deficits suggestive of parietotemporal dysfunction. Brain imaging including (18)FDG-PET and [(11)C] PIB-PET supported the diagnosis of AD.

Conclusions: Our case highlights the clinical variability that characterizes early-onset AD.

Keywords: Alzheimer's disease; primary progressive aphasia; pure word deafness.

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Conflict of interest statement

The authors have no financial conflicts of interest.

Figures

Fig. 1
Fig. 1
Brain MRI of the patient 2 years after the onset of pure word deafness. (A) Mild left temporal atrophy was suspected on axial and coronal scans. (B) 18FDG-PET demonstrated bilateral temporoparietal hypometabolism with greater involvement on the left side, and (C) [11C] PIB-PET showed increased PIB uptake in diffuse cortical areas. (D) A cortical thickness analysis showed cortical thinning in the bilateral Heschl's gyri, planum temporale, and superior temporal sulcus.
See this image and copyright information in PMC

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