Affiliations: Unit of Neurology, Italian National Research Center on
Aging, Via della Montagnola, Ancona, Italy | Unit of Radiology, Italian National Research Center on
Aging, Ancona, Italy | Vita-Salute University and Division of Neuroscience,
San Raffaele Scientific Institute, Milan, Italy | Scientific Direction, Italian National Research Center
on Aging, Ancona, Italy
Note: [] Corresponding author: S. Paolini, Unit of Neurology, INRCA, Via
della Montagnola 81, 60125, Ancona, Italy. Tel.: +39 71 8003578; Fax: +39 71
8003530; E-mail: [email protected]
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.
