Degenerative jargon aphasia: unusual progression of logopenic/phonological progressive aphasia?
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.
Department of Neuroscience, University of Parma, Parma, Italy. paolo.caffarra@unipr.it
Paolo
P
Caffarra
Simona
S
Gardini
Stefano
S
Cappa
Francesca
F
Dieci
Letizia
L
Concari
Federica
F
Barocco
Caterina
C
Ghetti
Livia
L
Ruffini
Guido Dalla Rosa
GD
Prati
D000368:Aged; D018888:Aphasia, Primary Progressive / Q000150:complications / Q000000981:diagnostic imaging / Q000523:psychology*; D001041:Aphasia, Wernicke / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000523:psychology*; D001921:Brain / Q000000981:diagnostic imaging / Q000503:physiopathology; D018450:Disease Progression; D005260:Female; D019788:Fluorodeoxyglucose F18; D059907:Functional Neuroimaging / Q000379:methods / Q000523:psychology*; D006801:Humans; D007807:Language Tests / Q000706:statistics & numerical data; D009483:Neuropsychological Tests / Q000706:statistics & numerical data; D049268:Positron-Emission Tomography / Q000379:methods / Q000523:psychology
D019788:Fluorodeoxyglucose F18