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Behavioural Neurology
Volume 27 (2013), Issue 2, Pages 221-227
http://dx.doi.org/10.3233/BEN-120299
Case Report

Change of Accent as an Atypical Onset of non Fluent Primary Progressive Aphasia

Susy Paolini,1 Lucia Paciaroni,1 Antonio Manca,2 Roberto Rossi,2 Daniela Fornarelli,2 Stefano F. Cappa,3 Angela M. Abbatecola,4 and Osvaldo Scarpino1

1Unit of Neurology, Italian National Research Center on Aging, Via della Montagnola, Ancona, Italy
2Unit of Radiology, Italian National Research Center on Aging, Ancona, Italy
3Vita-Salute University and Division of Neuroscience, San Raffaele Scientific Institute, Milan, Italy
4Scientific Direction, Italian National Research Center on Aging, Ancona, Italy

Received 1 February 2013; Accepted 1 February 2013

Copyright © 2013 Hindawi Publishing Corporation and the authors. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Language disorders can be the first symptom of many neurodegenerative diseases, including Alzheimer's disease (AD) and primary progressive aphasia (PPA). The main variants of PPA are: the non-fluent/agrammatic variant, the semantic variant and the logopenic variant.

Several additional variants of PPA, however, have been described and are considered as atypical presentations.

We describe the case of a woman presenting a progressive isolated language disturbance, characterized by an early dysprosodia, phonological and semantic paraphasias, agrammatism, impairment in repetition, writing of non-words and sentence comprehension. This clinical picture pointed to an atypical presentation of the non-fluent variety. The frequent symptom overlap between the different variants of PPA, most likely reflecting differences in the topography of the pathological changes, needs to be considered in the definition of diagnostic criteria.