Degenerative jargon aphasia: Unusual progression of logopenic/phonological progressive aphasia?

Issue title: Primary Progressive Aphasia and Post-Stroke Aphasia: Some Complementary Insights into Brain-Behavior Relationships/Hemispatial Neglect and Related Disorders

Article type: Research Article

Authors: Caffarra, Paolo^; | Gardini, Simona | Cappa, Stefano | Dieci, Francesca | Concari, Letizia | Barocco, Federica | Ghetti, Caterina | Ruffini, Livia | Prati, Guido Dalla Rosa

Affiliations: Department of Neuroscience, University of Parma, Parma, Italy | Outpatient Clinic for the Diagnosis and Therapy of Cognitive Disorders, AUSL, Parma, Italy | Department of Clinical Neuroscience, Vita-Salute San Raffaele Hospital, Milan, Italy | Medical Physic Department, Azienda Ospedaliero-Universitaria, Parma, Italy | Department of Nuclear Medicine, Azienda Ospedaliero-Universitaria, Parma, Italy | Poliambulatorio Dalla Rosa Prati, Centro Diagnostico Europeo, Parma, Italy

Note: [] Address for correspondence: Paolo Caffarra M.D., Department of Neuroscience, University of Parma, Via Gramsci, 14, 43100 Parma, Italy. Tel./Fax: +39 0521 704116; E-mail: paolo.caffarra@unipr.it

Abstract: Primary progressive aphasia (PPA) corresponds to the gradual degeneration of language which can occur as nonfluent/agrammatic PPA, semantic variant PPA or logopenic variant PPA. We describe the clinical evolution of a patient with PPA presenting jargon aphasia as a late feature. At the onset of the disease (ten years ago) the patient showed anomia and executive deficits, followed later on by phonemic paraphasias and neologisms, deficits in verbal short-term memory, naming, verbal and semantic fluency. At recent follow-up the patient developed an unintelligible jargon with both semantic and neologistic errors, as well as with severe deficit of comprehension which precluded any further neuropsychological assessment. Compared to healthy controls, FDG-PET showed a hypometabolism in the left angular and middle temporal gyri, precuneus, caudate, posterior cingulate, middle frontal gyrus, and bilaterally in the superior temporal and inferior frontal gyri. The clinical and neuroimaging profile seems to support the hypothesis that the patient developed a late feature of logopenic variant PPA characterized by jargonaphasia and associated with superior temporal and parietal dysfunction.

Keywords: Primary progressive aphasia, logopenic aphasia, language, jargon

DOI: 10.3233/BEN-2012-110218

Journal: Behavioural Neurology, vol. 26, no. 1-2, pp. 89-93, 2013