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

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

doi:10.1155/2013/965782

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.

Highlights

Progressive language disorders without generalized dementia are defined primary progressive aphasia (PPA) [1]
Nonfluent speech output or nonfluent/agrammatic PPA may include or not apraxia of speech, while fluent output corresponds to semantic variant PPA or logopenic variant PPA [3]
The language disorder profile of the patient at onset was characterised by anomic pauses, and progressed within ten years into a severe form of fluent, unintelligible speech including a severe deficit in repetition, corresponding to the characteristics of mixed, semantic and neologistic jargonaphasia, associated with severe comprehension deficits

Summary

Introduction

Progressive language disorders without generalized dementia are defined primary progressive aphasia (PPA) [1]. Logopenic variant PPA shows phonemic paraphasia, word-finding hesitation, slow speech and decreased verbal output with long word-finding pauses and preserved grammar and articulation, corresponding to left posterior temporal and parietal lobules degeneration. LEA was a patient with severely reduced speech production and defective articulation, affected by what seems to be a severe, rapidly progressive form of fronto-temporal degeneration [6], who produced fluent, neologistic writte jargon. Two patients with fluent jargonaphasia were present among the pathological series reported by Deramecourt and colleagues [8] Both showed left temporo-parietal hypoperfusion on SPECT, and had a pathological diagnosis of Alzheimer’s disease. The present single case study aimed to describe the clinical evolution of a similar patient who developed neologistic jargon in the course of PPA, reporting both cognitive and neuroimaging correlates of this unusual clinical picture

Case study

Findings

Discussion