Degenerative Jargon Aphasia: Unusual Progression of Logopenic/Phonological Progressive Aphasia?

Abstract

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.

Progression of logopenic variant primary progressive aphasia to apraxia and semantic memory deficits

“…As a case series study, Gorno-Tempini et al 2008 [3] reported that patients with lvPPA showed relative sparing of their functional status 5 years after the onset of symptoms. Caffarra et al 2013 [4] presented a patient with lvPPA who developed jargon aphasia as a late feature. Rogalski et al 2011 [5] suggested that the unique linguistic features of lvPPA in the early to middle stage may lose their distinctiveness from other types of PPA as the degeneration worsens.…”

Jargonaphasia: A Systematic Overview and Characterization in Primary Progressive Aphasia

“…We identified 3 case reports of jargonaphasic PPA patients [9,26,27] and several cases of jargon within 2 studies on PPA cohorts [28,29]. Twenty five cases of jargonaphasia were identified and one was excluded because the patient presented pure jargonagraphia [30].…”

“…Twenty five cases of jargonaphasia were identified and one was excluded because the patient presented pure jargonagraphia [30]. Regarding the case reports, the study of Caffarra et al [26] reported one lv-PPA patient with phonological jargon and the investigation of Rohrer et al [9] reported 2 patients with an initial diagnosis of mixed lv-PPA/sv-PPA. In both patients, disease evolution led to phonological jargon with neologisms.…”

Particularités du variant logopénique au sein des aphasies progressives primaires

“…Des é volutions cliniques diverses ont é té rapporté es comprenant un cas de jargonaphasie associé e à des troubles sé vè res de la compré hension à dix ans d'é volution [109], trois cas d'apraxie et de troubles sé mantiques [110], un cas aprè s 4 ans d'é volution é voquant une probable maladie à corps de Lewy, un DAT scan pathologique et SPECT et une hypoperfusion posté rieure occipitale [60], 2 cas qui é voluent vers des syndromes corticobasaux cliniques et radiologiques [111] [112][113][114][115]. Enfin, d'autres syndromes parkinsoniens « plus » comme notamment la maladie à corps de Lewy peuvent ê tre à l'origine du syndrome logopé nique [60,99].…”