Abstract

The logopenic variant of Primary Progressive Aphasia, or logopenic aphasia, is a the most recently described variant of Primary Progressive Aphasia and also the least well defined. This variant can present clinical findings that are also common to Alzheimer's disease, given they both share the same cytopathologic findings. This article reports the clinical case of a patient for whom it proved difficult to define a clinical diagnosis, being split between the logopenic variant and Alzheimer's disease at different phases of the disease. Using this case as an example and drawing on the latest evidence from the literature on the logopenic variant, we postulate the hypothesis that this variant may present as an initial symptom of Alzheimer's disease in some atypical cases.

Highlights

  • Primary Progressive Aphasia (PPA) is a term used to describe a group of neurodegenerative diseases that predominantly affect language.[1,2]

  • The main findings in this case study were the progressive aspect of the symptoms, predominantly languagerelated complaint, deficits on cognitive screening tests of the Mini-Mental State Exam (MMSE), Clinical Dementia Rating (CDR), verbal and non-verbal assessment of the CERAD, WAIS forward and backward digit span, the BNT, Clock Drawing Test (CDT), Montreal-Toulouse Language Assessment Battery (MTL) and verbal fluency tests concomitant with relative sparing on the Activities of Daily Living Questionnaire (ADLQ) and the oral comprehension test

  • Besides the linguistic aspects differentiating logopenic variant of PPA (lvPPA) from typical Alzheimer’s disease (AD), the neuropsychological profiles of these patient groups differs, with a dissociation in performance of verbal and visual memory between the two conditions, where verbal memory is poorer in patients with lvPPA.[27]. These findings suggest that lvPPA has a different phenotype to AD.[25,27]

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Summary

Introduction

Primary Progressive Aphasia (PPA) is a term used to describe a group of neurodegenerative diseases that predominantly affect language.[1,2] The term encompasses three different variants, each with a specific language profile: semantic, agrammatic/non-fluent and logopenic. The diagnosis of PPAs has long been restricted to the non-fluent and semantic variants, where logopenic aphasia has only recently been defined, based on the diagnostic criteria of Gorno-Tempini et al.[2] The logopenic variant of PPA (lvPPA) is characterized by difficulties in single-word retrieval, repetition of sentences/phrases, presence of phonologic errors, left posterior perisylvian or parietal atrophy and typical association with the pathological finding of Alzheimer’s disease (AD). The objective of this article is to report a clinical case for which it proved difficult to define a clinical diagnosis, being split between lvPPA and AD at different phases of the disease

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