
doi: 10.5327/cbn241038
Case presentation: A.C.F.S, male, 58 years old, already diagnosed with unspecified dementia and using memantine 10mg/day for the last 3 years. His wife reports that 7 years ago he began to have progressive difficulties for communication, with hesitancy in the middle of sentences, which caused social isolation. He started to writ wrong, which was an issue since he works as a hospital receptionist and filled out forms. At time, he also had difficulty recalling recent events. Falls, signs of parkinsonism, behavioral problems, hallucinations and delusions weren´t present in the symptoms. In the first evaluation patient was completely dependent for daily activities, global cognition was moderately affected (CDR 2.0), despite the somatic neurological exam was normal. Neuropsychological evaluation exhibited marked difficulties in repeating verbal sentences, comprehension of long phrases and errors in writing and reading irregular words. Cerebrospinal analysis was normal, except for mild protein elevation (65mg/dl). Search for presenilin mutation (PSEN 1 and 2) was negative and the brain PET FDG-18F showed glycolytic hypometabolism in the posterior aspect of the cingulate gyrus as well as in the cortical topography of the bilateral temporal region, extending to the parahippocampal gyrus, inferior, middle and superior temporal gyri. Glycolytic hypometabolism was also present in the frontoparietal region, extending to the precuneus, more prominent on the left. Discussion: Logopenic variant of primary progressive aphasia (LV-PPA) is generally associated with atrophy of the posterosuperior temporal (perisylvian) and inferior parietal regions, which are areas involved in the auditory processing of speech and in the activation of phonological representations for language expression (phonological loop). The patient in this case presented hypometabolism in this region in the dominant hemisphere, which reinforces the diagnosis. Difficulty in lexical access during spontaneous speech results in hesitations and pauses in patients with LV-PPA. A more comprehensive assessment of language with specific tasks (comprehension of words and phrases; naming by visual confrontation; repetition of words and phrases with variable length; reading of regular and irregular words) may be necessary, as performance differs between the variants. Additionally, research into structural and molecular neuroimaging biomarkers may help, especially when demonstrating focal patterns of atrophy or hypometabolism. Final comments: The most common underlying neurodegenerative pathology in LV-PPA is Alzheimer’s disease, with the language alteration attributed primarily to a deficit in the phonological component of working memory, which explains the difficulty in repeating sentences, the preservation of understanding of isolated words (which helps to differentiate from the semantic variant of PPA) and the relative preservation of fluency when compared to the non-fluent variant.
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