Johdanto Flashcards
PPA?
Progressive aphasia
AD?
Alzheimer’s disease
nfvPPA
Non-fluent/agrammatic variant PPA
SD?
Semantic dementia
lvPPA?
Logopenic variant PPA
Current diagnostic recommendations for primary progressive aphasia (PPA) include three main subtypes: ?
non-fluent/agrammatic (nfvPPA), characterized by agrammatism and/or apraxia of speech (AOS); logopenic (lvPPA), characterized by impaired repetition and word finding difficulties; and semantic (svPPA), characterized by anomia(patient is unable to recall the names of everyday objects), impaired word comprehension, and impaired object recognition
non-fluent/agrammatic (nfvPPA), characterized by?
agrammatism and/or apraxia of speech (AOS)
(lvPPA) characterized by?
logopenic (lvPPA), characterized by impaired repetition and word finding difficulties
(svPPA) characterized by?
semantic (svPPA), characterized by anomia, impaired word comprehension, and impaired object recognition
the term SD acknowl- edges the multimodal nature of patients’ semantic loss and the fact that the earliest presenting symptom may be in the ?
visual rather than verbal domain.
The three PPA subtypes are associated?
with different distributions of atrophy
different distributions of atrophy: nfvPPA with?
left posterior fronto-insular atrophy
lvPPA with?
left posterior perisylvian or parietal atrophy
svPPA with?
anterior temporal lobe atrophy
Most patients with nfvPPA and svPPA/SD have?
frontotemporal lobar degeneration (FTLD) spectrum pathologies
most lvPPA patients have ?
Alzheimer’s disease (AD) pathology, although this is not invariably the case
Neuropsychological studies suggest that current classifications?
Do not encapsulate the full range of PPA syndromes observed, and patients may fulfil the criteria for more than one PPA variant
while each PPA subtype is associated with characteristic deficits
a degree of overlap exists between the subtypes
the core features of lvPPA also occur frequently in nfvPPA
In current classifications agrammatism is identified?
Through tasks involving sentence production yet different tasks may not have equal sensitivity or specificity
recent review suggests that evaluation of agrammatism should include the assessment of comprehension and production of grammatical morphology, functional categories, verbs, and complex syntactic structures
What would be important to determine?
optimum measures for detecting characteristic language deficits prospectively in a cohort of PPA patients.
Refinement of diagnosis may depend ?
not only on identification of optimal language measures but also on the recognition of associated deficits. One such area worthy of consideration is praxis.
AOS is a core feature of ?
nfvPPA yet deficits may extend beyond the realm of speech to orofacial and/or limb apraxia
Gestural apraxia might occur ?
secondary to a central disorder of communication
Apraxia might be predicted to be ?
more integral component of nfvPPA than of other forms of PPA.
working memory is ?
Another domain of potential diagnostic importance.
Phonolog- ical working memory has been ?
suggested to play a pivotal role in the logopenic syndrome leading to patients’ difficulty in sentence repetition.
Yet, the degree to which standard tests of working memory are able to differentiate lvPPA from nfvPPA
Is unclear
NfvPPA patients might be expected to perform poorly on ?
verbal working memory tasks because of their marked (huomattava) speech production problems, and indeed there is compelling evidence of phonological processing impairments in nfvPPA to suggest problems in verbal/ phonological working memory.
On the other hand, they might reasonably do well on working memory tasks that do not make phonological demands.
The examination of performance on a variety of verbal and visual working memory tasks would ?
help to identify optimal measures to aid differentiation.
In addition to the associated cognitive deficits,
accompanying behavioural changes might be of diagnostic relevance.
NfvPPA and svPPA/SD are pathologically linked to ?
behavioural variant frontotemporal dementia (bvFTD) which is the commonest clinical disorder associated with FTLD (frontotemporal lobar degeneration) spectrum pathology and is characterized by changes in behaviour and personality.
Language and behavioural symptoms can co-occur in disorders ?
caused by FTLD pathology
Patients with SD have been reported to ?
exhibit behavioural change, particularly stereotyped and repetitive behaviours
In nfvPPA can also occur?
behavioural change
In lvPPA has been described with?
Increased apathy, anxiety, agitation, and depression have been described
However, since lvPPA is typically not associated with ? but rather with ? these patients might be expected to have ?
not with FTLD but with AD pathology
differing behavioural change when compared to patients with nfvPPA and svPPA/SD.
The presence of certain behavioural characteristics might therefore ?
aid (support, help) differentiation of PPA subtypes and warrants (taata, määrätä) further investigation.
The link between lvPPA and AD pathology raises the question ?
whether the language characteristics of lvPPA mirror those seen in early-onset AD, in which language problems typically constitute (asettaa, perustaa) one component of a multi-domain disorder that includes also deficits in episodic memory, working memory, visual perception, and spatial (avaruudellinen) function
Reports have thus far been mixed with some authors describing?
logopenic-type symptoms in non-focal AD patients and other authors describing a differing language profile in patients with AD
In AD?
long-term and working memory may breakdown separately
Working memory has been associated with
language functioning but spatial span may also be impaired
A comparative study of lvPPA and AD showed?
poorer performance on tests of verbal working memory in lvPPA but similar performance in the two groups on tests of visuospatial working memory
What remains to be established?
Whether this finding generalizes to other lvPPA and AD cohorts, and whether working memory profile distinguishes lvPPA from nfvPPA
First aim of this study?
First, we aimed to identify optimum language measures for differentiating SD, nfvPPA, and lvPPA, focussing on tests of naming, single word comprehension, sentence processing, narrative production, reading, spelling, and repetition that tap core features of PPA conditions.
Second aim of this study?
Second, we examined the potential diagnostic contribution of non-language measures, specifically in the domains of praxis, working memory, and behaviour. We included as a comparison group, patients with multidomain AD. We expected greater commonalities in language and non-language performance with lvPPA than other forms of PPA, but it was an open question whether distinct characteristics would be identified between lvPPA and AD.
Third aim of this study?
Third, we aimed to determine, through principal component analysis of performance measures, the degree to which core factors can be identified that have predictive value in distinguishing forms of PPA and to delineate (describe, set forth, set out) optimal combinations of discriminating features.
