Characterisation of “Positive” Behaviours in Primary Progressive Aphasias

Abstract

Background/Aims: Although some patients with primary progressive aphasia (PPA) exhibit novel or improved skills after the onset of dementia, these changes have yet to be quantified. Therefore, this study systematically explored and identified the emergence of positive behaviours after dementia onset. Methods: This study included 48 carers of patients with PPA: 12 nonfluent/agrammatic PPA (nfvPPA), 22 semantic variant PPA (svPPA), and 14 logopenic variant PPA (lvPPA). The presence and frequency of positive behaviour changes after dementia onset were established using the Hypersensory and Social/Emotional Scale (HSS). Results: Scores on Sensitivity to Details, Visuospatial Activities, and Music Activities differed significantly among the groups. More specifically, svPPA was associated with increased visuospatial activity, but only in the mild stage of the disease; nfvPPA was associated with increased visuospatial activity and decreased music activity, while lvPPA exhibited the reverse profile. Conclusions: The results demonstrate that subsets of PPA patients show novel or increased positive behaviours following dementia onset, and differences among subtypes may be helpful for improving diagnostic accuracy. Additionally, harnessing these skills may improve the quality of life of both patients and carers.

Music appreciation phenotypes in patients with frontotemporal dementia: A pilot study

“…Although singing and dancing are important musical activities, some patients in our cohort displayed increased singing or dancing without an increased interest in music. None of our PNFA patients showed musicophilia, which is in line with previous studies 12,28 . We further found that sound aversion was not always associated with music aversion and vice versa.…”

Primary progressive aphasia: a clinical approach

“…Symptoms such as absent or misplaced empathy, social disinhibition and faux pas, a more fatuous sense of humour and pathological sweet tooth are common in both svPPA and behavioural variant frontotemporal dementia [ 29 , 53 – 57 ]. Within this spectrum, certain behavioural features, such as food faddism, exaggerated reactions to pain and ambient temperature, behavioural rigidity with clock-watching and obsessional interest in numbers, puzzles (especially Sudoku and jigsaws) and music (‘musicophilia’) seem particularly linked to svPPA [ 30 , 53 , 58 – 60 ]. A unifying theme here may be impaired understanding of emotional and somatic signals due to both deficient and over-generalised responses to sensory information [ 41 , 42 , 55 , 56 , 61 ], analogous to recognition failures and ‘regularisation errors’ in other cognitive domains.…”

Faculty Opinions recommendation of Primary progressive aphasia: a clinical approach.

“…Symptoms such as absent or misplaced empathy, social disinhibition and faux pas, a more fatuous sense of humour and pathological sweet tooth are common in both svPPA and behavioural variant frontotemporal dementia [29,[53][54][55][56][57]. Within this spectrum, certain behavioural features, such as food faddism, exaggerated reactions to pain and ambient temperature, behavioural rigidity with clock-watching and obsessional interest in numbers, puzzles (especially Sudoku and jigsaws) and music ('musicophilia') seem particularly linked to svPPA [30,53,[58][59][60]. A unifying theme here may be impaired understanding of emotional and somatic signals due to both deficient and over-generalised responses to sensory information [41,42,55,56,61], analogous to recognition failures and 'regularisation errors' in other cognitive domains.…”