Johdanto

Question 1

PPA?

Answer 1

Progressive aphasia

Question 2

AD?

Answer 2

Alzheimer’s disease

Question 3

nfvPPA

Answer 3

Non-fluent/agrammatic variant PPA

Question 4

SD?

Answer 4

Semantic dementia

Question 5

lvPPA?

Answer 5

Logopenic variant PPA

Question 6

Current diagnostic recommendations for primary progressive aphasia (PPA) include three main subtypes: ?

Answer 6

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

Question 7

non-fluent/agrammatic (nfvPPA), characterized by?

Answer 7

agrammatism and/or apraxia of speech (AOS)

Question 8

(lvPPA) characterized by?

Answer 8

logopenic (lvPPA), characterized by impaired repetition and word finding difficulties

Question 9

(svPPA) characterized by?

Answer 9

semantic (svPPA), characterized by anomia, impaired word comprehension, and impaired object recognition

Question 10

the term SD acknowl- edges the multimodal nature of patients’ semantic loss and the fact that the earliest presenting symptom may be in the ?

Answer 10

visual rather than verbal domain.

Question 11

The three PPA subtypes are associated?

Answer 11

with different distributions of atrophy

Question 12

different distributions of atrophy: nfvPPA with?

Answer 12

left posterior fronto-insular atrophy

Question 13

lvPPA with?

Answer 13

left posterior perisylvian or parietal atrophy

Question 14

svPPA with?

Answer 14

anterior temporal lobe atrophy

Question 15

Most patients with nfvPPA and svPPA/SD have?

Answer 15

frontotemporal lobar degeneration (FTLD) spectrum pathologies

Question 16

most lvPPA patients have ?

Answer 16

Alzheimer’s disease (AD) pathology, although this is not invariably the case

Question 17

Neuropsychological studies suggest that current classifications?

Answer 17

Do not encapsulate the full range of PPA syndromes observed, and patients may fulfil the criteria for more than one PPA variant

Question 18

while each PPA subtype is associated with characteristic deficits

Answer 18

a degree of overlap exists between the subtypes

the core features of lvPPA also occur frequently in nfvPPA

Question 19

In current classifications agrammatism is identified?

Answer 19

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

Question 20

What would be important to determine?

Answer 20

optimum measures for detecting characteristic language deficits prospectively in a cohort of PPA patients.

Question 21

Refinement of diagnosis may depend ?

Answer 21

not only on identification of optimal language measures but also on the recognition of associated deficits. One such area worthy of consideration is praxis.

Question 22

AOS is a core feature of ?

Answer 22

nfvPPA yet deficits may extend beyond the realm of speech to orofacial and/or limb apraxia

Question 23

Gestural apraxia might occur ?

Answer 23

secondary to a central disorder of communication

Question 24

Apraxia might be predicted to be ?

Answer 24

more integral component of nfvPPA than of other forms of PPA.

Question 25

working memory is ?

Answer 25

Another domain of potential diagnostic importance.

Question 26

Phonolog- ical working memory has been ?

Answer 26

suggested to play a pivotal role in the logopenic syndrome leading to patients’ difficulty in sentence repetition.

Question 27

Yet, the degree to which standard tests of working memory are able to differentiate lvPPA from nfvPPA

Answer 27

Is unclear

Question 28

NfvPPA patients might be expected to perform poorly on ?

Answer 28

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.

Question 29

The examination of performance on a variety of verbal and visual working memory tasks would ?

Answer 29

help to identify optimal measures to aid differentiation.

Question 30

In addition to the associated cognitive deficits,

Answer 30

accompanying behavioural changes might be of diagnostic relevance.

Question 31

NfvPPA and svPPA/SD are pathologically linked to ?

Answer 31

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.

Question 32

Language and behavioural symptoms can co-occur in disorders ?

Answer 32

caused by FTLD pathology

Question 33

Patients with SD have been reported to ?

Answer 33

exhibit behavioural change, particularly stereotyped and repetitive behaviours

Question 34

In nfvPPA can also occur?

Answer 34

behavioural change

Question 35

In lvPPA has been described with?

Answer 35

Increased apathy, anxiety, agitation, and depression have been described

Question 36

However, since lvPPA is typically not associated with ? but rather with ? these patients might be expected to have ?

Answer 36

not with FTLD but with AD pathology differing behavioural change when compared to patients with nfvPPA and svPPA/SD.

Question 37

The presence of certain behavioural characteristics might therefore ?

Answer 37

aid (support, help) differentiation of PPA subtypes and warrants (taata, määrätä) further investigation.

Question 38

The link between lvPPA and AD pathology raises the question ?

Answer 38

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

Question 39

Reports have thus far been mixed with some authors describing?

Answer 39

logopenic-type symptoms in non-focal AD patients and other authors describing a differing language profile in patients with AD

Question 40

In AD?

Answer 40

long-term and working memory may breakdown separately

Question 41

Working memory has been associated with

Answer 41

language functioning but spatial span may also be impaired

Question 42

A comparative study of lvPPA and AD showed?

Answer 42

poorer performance on tests of verbal working memory in lvPPA but similar performance in the two groups on tests of visuospatial working memory

Question 43

What remains to be established?

Answer 43

Whether this finding generalizes to other lvPPA and AD cohorts, and whether working memory profile distinguishes lvPPA from nfvPPA

Question 44

First aim of this study?

Answer 44

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.

Question 45

Second aim of this study?

Answer 45

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.

Question 46

Third aim of this study?

Answer 46

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.