Functional disability in primary progressive aphasia

O’Connor, Claire; Ahmed, Samrah; Mioshi, Eneida

doi:10.1080/02687038.2014.910588

Cited by 16 publications

(17 citation statements)

References 59 publications

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“…Compared to the nfvPPA group, svPPA patients showed heightened Sensitivity to Details (e.g., “Is sensitive to misaligned objects such as paintings” and “Is rigid and particular on how items must be ordered”) and increased Hypersensitivity. The presence of these behaviours aligns well with studies of other rigid behaviours that have been reported in this group, such as sticking to fixed timetables or always requesting the same foods [12,29]. Group differences were also observed in the social domain, such that lvPPA patients showed an increase in Music Activities compared to nfvPPA patients.…”

Section: Discussionsupporting

confidence: 71%

“…Previous studies of the clinical characteristics of PPA subtypes reported changes beyond language skills, including other cognitive domains [6,7], psychiatric symptoms [8,10], social cognition [9,28], and functional abilities [12]. The present study is the first to demonstrate and quantify the presence of various “positive” behaviours by PPA subtype.…”

Section: Discussionmentioning

confidence: 69%

“…Additionally, each PPA subtype is associated with a specific and predominant abnormal deposition of protein in the brain: tau (>70%) in nfvPPA, TDP-43 (>90%) in svPPA, and Alzheimer disease pathology in lvPPA [5]. Recent evidence has shown that the clinical phenomenology of PPA extends beyond the language domain and includes a range of cognitive (e.g., [6,7]) and behavioural deficits (e.g., [8,9,10,11]) as well as changes in functional abilities [12]. …”

Section: Introductionmentioning

confidence: 99%

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Characterisation of “Positive” Behaviours in Primary Progressive Aphasias

Midorikawa¹,

Kumfor²,

Leyton³

et al. 2017

Dement Geriatr Cogn Disord

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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.

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Section: Discussionsupporting

confidence: 71%

Section: Discussionmentioning

confidence: 69%

Section: Introductionmentioning

confidence: 99%

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Characterisation of “Positive” Behaviours in Primary Progressive Aphasias

Midorikawa¹,

Kumfor²,

Leyton³

et al. 2017

Dement Geriatr Cogn Disord

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“…Behavioural changes are often evident at presentation in patients with svPPA, resembling behavioural variant FTD, but these behavioural changes do not seem to be relevant in predicting functional decline in svPPA. While marked behavioural changes are present in svPPA, they seem to somehow allow for continued participation in activities, albeit in a very rigid manner, instead of hindering their performance [22,23,24]. While there was a trend for global cognition to contribute to around a third of the variance in functional decline, the remaining factors which contribute to functional decline in svPPA remain unclear.…”

Section: Discussionmentioning

confidence: 99%

The Relationship between Behavioural Changes, Cognitive Symptoms, and Functional Disability in Primary Progressive Aphasia: A Longitudinal Study

O'Connor

Clemson

Flanagan

et al. 2016

Dement Geriatr Cogn Disord

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Background: The contribution of behavioural changes to functional decline is yet to be explored in primary progressive aphasia (PPA). Objectives: (1) investigate functional changes in two PPA variants [semantic (svPPA) and non-fluent (nfvPPA)], at baseline and after 12 months; (2) investigate baseline differences in behavioural changes between groups, and (3) explore predictors of functional decline after a 12-month period. Methods: A longitudinal study involving 29 people with PPA (18 svPPA; 11 nfvPPA) seen annually in Sydney/Australia was conducted. A total of 114 functional and behavioural assessments were included for within-group (repeated-measures ANOVA; annual rate of change; multiple regression analyses) and between-group analyses (pairwise comparisons). Results: Functional profiles in svPPA and nfvPPA were similar in people with up to 5 years of disease duration. Behavioural changes were marked in svPPA patients (stereotypical behaviour and apathy) but did not predict annual rate of change of functional abilities; global cognitive scores at baseline did. Despite mild behavioural changes in nfvPPA (disinhibition, apathy), these were significant predictors of annual rate of functional change. Conclusions: The presentation and interplay of behavioural changes and functional disability differ in svPPA and nfvPPA. These varying factors should be taken into account when considering prognosis, disease management, and selection of outcome measures for interventions.

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“…While these ADLS are often impaired initially, the typically slower rate of progression means that svPPA patients have preservation of basic ADLs for much longer when compared with bvFTD and nfvPPA patients [84,85]. Driving skills may remain intact until more moderate stages of the disease [86], and language-mediated activities may remain intact, despite a patient's inability to name items or communicate their intentions [86]. …”

Section: Semantic Variant Primary Progressive Aphasiamentioning

confidence: 99%

Frontotemporal Dementia: diagnosis, Deficits and Management

Bott

Radke

Stephens

et al. 2014

Neurodegener. Dis. Manag.

101

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Summary Frontotemporal dementia (FTD) is a progressive neurologic syndrome with diverse clinical presentations and attendant underlying pathologies. Psychiatric prodrome, neuropsychiatric symptoms and language difficulties are common in FTD, but the diversity of presentation raises unique diagnostic challenges that can significantly impact patient care and counsel for caregivers regarding clinical status and prognosis. While neuropsychiatric symptom measures are helpful, more sensitive assessments delineating the specific behavioral and linguistic deficits accompanying FTD are needed. Comprehensive clinical assessment in combination with evaluation of language, socio-emotional functioning, cognition and neuroimaging aid in accurate and early diagnosis and treatment planning. In what follows, we review each of the FTD syndromes, highlight current research investigating the cognitive, behavioral and socio-emotional deficits observed with this disease, address common diagnostic challenges and summarize best practices associated with management of FTD.

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Functional disability in primary progressive aphasia

Cited by 16 publications

References 59 publications

Characterisation of “Positive” Behaviours in Primary Progressive Aphasias

Characterisation of “Positive” Behaviours in Primary Progressive Aphasias

The Relationship between Behavioural Changes, Cognitive Symptoms, and Functional Disability in Primary Progressive Aphasia: A Longitudinal Study

Frontotemporal Dementia: diagnosis, Deficits and Management

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