Methods

Question 1

Participants consist of?

Answer 1

47 patients with a clinical diagnosis of nfvPPA (12), lvPPA (13), SD (8), and multi-domain AD (14) who attended a specialist early-onset dementia clinic and agreed to take part in the study. These participants included new referrals and follow-up patients. Most patients were seen within a year of first presenting to the clinic

Question 2

Which participants were excluded?

Answer 2

Participants with a history of alcohol abuse or head injury

Question 3

Patient were diagnosed?

Answer 3

By experienced neurologists based on detailed clinical history, neuroimaging results, and neurological examination, using guidelines published by Snowden et al. (2011), which have been shown, in clinico-pathological correlation studies, to yield high levels of diagnostic accuracy.

Question 4

All SD patients showed?

Answer 4

the language characteristics of svPPA

Question 5

However, as three patients showed early face and object recognition problems, in addition to their language disorder, and had greater right than left temporal lobe atrophy, the term ?

Answer 5

SD is used to designate the group.

Question 6

Diagnoses were supported by neuropsychological examination using ?

Answer 6

the Manchester Neuropsychological Profile

Question 7

How many age-matced controls were recruited?

Answer 7

19, nineteen

Question 8

There was a significant association between

Answer 8

Gender and group

Question 9

The lvPPA group included a higher proportion of?

Answer 9

males than the control group and AD group

Question 10

There was a significant group difference in ?

Answer 10

Age

Question 11

Which group was significantly younger than the other groups?

Answer 11

The AD group

Question 12

The youthful age of the AD patients, in part, reflects a ?

Answer 12

referral (lähete) bias of younger patients to the neurology clinic but also an inherent (luontainen/synnynnäinen) bias towards early onset in AD presenting with multi-domain impairment.

Question 13

All participants provided?

Answer 13

written informed consent to take part in the study. The study was approved by North West NRES committee

Question 14

Participants underwent a

Answer 14

neuropsychological test battery with a particular focus on language and working memory.

Question 15

Language assessment included tests of

Answer 15

naming, single word comprehension, sentence

processing, narrative production, reading, and spelling.

Question 16

Naming included?

Answer 16

Object and action naming,
Manchester picture naming. This test involves naming forty line drawings from the
Snodgrass and Vanderwart pictures

Question 17

Single word comprehension included?

Answer 17

Manchester comprehension. This involves matching a printed word with one of four pictures (the same 40 target items are used as in the Manchester naming test).

Question 18

Sentence processing tasks?

Answer 18

Modified PALPA 55 subtest Auditory sentence comprehension test and PALPA subtest 58 Auditory comprehension of locative relations

Question 19

Manchester sentence ordering?

Answer 19

A locally constructed task, which requires participants to order five individually printed words to make a sentence.

Question 20

Manchester tense (aikamuoto) production?

Answer 20

In this locally constructed task, participants are shown action pictures (e.g., a boy kicking a ball) accompanied by the written word ‘yesterday’ or ‘tomorrow’. Participants are asked to generate a descriptive sentence beginning with the word provided (e.g., Yesterday, the boy kicked the ball).

Question 21

Measures of narrative production?

Cookie theft picture?

Answer 21

Participants were recorded describing the picture. The number of phonetic errors, phonemic errors, fluency disruptions, length of utterance, number of dependent clauses (lause) per utterance=comment, and percentage of well- formed sentences were calculated.

Question 22

Reading test involves?

Answer 22

• Manchester reading. This test involves reading aloud the printed words used in the Manchester word-picture matching test.
• Modified PALPA subtest 36 Non-word reading.
• Modified PALPA test 31 Imageability/frequency reading.
• Spelling/sound regularity(säännöllisyys) reading

Question 23

Spelling

Written spelling

Answer 23

Participants are requested to write 20 dictated (saneltu) words, drawn from the Manchester naming test.

Question 24

Other tests evaluated?

Answer 24

working memory, praxis, calculation, and behaviour.

Question 25

Working memory?

Answer 25

The tasks addressed both verbal (phonological) and visuospatial working memory. Verbal tasks encompassed (ympäröidä, käsittää, sisällyttää) those with written as well as spoken presentation and tasks requiring pointing as well as spoken responses.

Question 26

Verbal (phonological) tasks ?

Answer 26

• Digits: Forwards and reverse Digit span
• Single words: Manchester word repetition. This test involves repetition of items from the Manchester naming/reading/comprehension tests.

Question 27

Verbal (phonological) tasks jatkuu?
Word sequences?
Sentences?

Answer 27

Word Sequences: Manchester immediate and delayed word repetition. This is a locally constructed test, which involves repeating one or two words with variable delays of zero and five seconds.

• Sentences: PALPA test 12 Sentence repetition
• PALPA test 60 Pointing span for noun-verb sequences

Question 28

Modified Brown-Peterson task of verbal working memory?

Answer 28

Modified Brown–Peterson task of verbal working memory.
In this test, participants are presented visually with three words. They are asked to repeat the words either immediately, following a five second delay or following a five second delay during which the participant reads aloud numbers.

Question 29

Visuospatial tasks?

Answer 29

Visual patterns span, involving copying from memory grids of black and white squares to blank grids.

Question 30

Visuospatial tasks? More

Visual array comparison test?

Answer 30

Visual array comparison test constructed and modified from and programmed in E-Prime 2.0
There are two subtests: colour and location. In both conditions, participants are briefly presented with one square and asked whether a second square is the same colour or in the same location as the first square. The arrays (taulukko, järjestys) increase in size to a maximum of 10 squares.

Question 31

Praxis?

Orofacial and limb praxis were evaluated with the ?

Answer 31

Manchester praxis screen, a locally constructed instrument taken from the Manchester neuropsychological profile, which is described previously. Participants are asked to carry out the actions described below. If participants are unable to carry out the action to command, they are shown the action to copy.

Orofacial praxis tasks consist of five actions, including tongue protrusion and coughing, three speech sounds (alternating repetitive vowel sounds, consonant sounds, and maintaining an extended phoneme),
three emotional gestures (surprise, anger, and happiness),
and three pantomimes (sniffing, sucking, and blowing).
Upper limb praxis tasks included four gestures and four pantomimes. Each action is performed with both left and right upper limbs.

Question 32

Calculation?

Answer 32

The Manchester calculation test involves 12 calculations; 6 administered verbally and 6 in writing.

Question 33

Behaviour?

Answer 33

A locally constructed behavioural questionnaire was given to relatives/carers to complete. The questionnaire includes questions aiming to evaluate changes in areas of behaviour, which are known to be affected in bvFTD and are described below. Each question is rated on the basis of three options: increase, decrease, or no change.

Question 34

The behavioural domains have been found sensitive in distinguishing (tunnusomainen) ?

Answer 34

bvFTD (behavioural variant frontotemporal dementia) from other forms of dementia and within subtypes of FTD and are incorporated within contemporary diagnostic criteria for bvFTD

Question 35

Behaviour questions?

Answer 35

• Social behaviour and interactions with others: interest and enjoyment being around others, social greetings and courtesies, amount of eye contact and frequency of personal questions, and comments of a complimentary or critical nature.
• Social emotions: happiness, sadness, fear, anger, guilt, surprise, disgust, embarrass- ment, empathy, laughter, and sense of humour.
• Response to environment: awareness of danger and response to painful and neutral stimuli.
• Eating and drinking: quantity and range of food eaten.
• Self-care:frequencyofwashing/bathing,changingclothes,andcareaboutappearance.
• Hobbies, order, and routine: frequency of doing puzzles, TV quizzes, clockwatching, carrying out simple or complex repetitive behaviour, and hoarding(hamstraus).

Question 36

Statistical analyses were conducted with ? And p?

Answer 36

SPSS version 22. A p value of

Question 37

Group comparisons of demographic data were carried out with

Answer 37

ANOVAs and non- parametric equivalents as appropriate

Question 38

Group comparisons on neuropsychological measures were analysed with?

Answer 38

Kruskal–Wallis tests.

Question 39

Due to the variation in ages and duration of symptoms across the cohort, group comparisons among the patient groups were also carried out with ?

Answer 39

Quade’s non-parametric rank analysis of covariance (ANCOVA), with age and duration of illness entered as nuisance covariates.

Question 40

Post-hoc

Fisher’s LSD tests were performed. A p value of ?

Answer 40

P value of <0.05 was adopted.

Question 41

Multiple post-hoc comparisons were corrected for ?

Answer 41

using Bonferroni corrections (i.e.,

for comparisons of all groups, p < .005 was adopted and for comparisons of the patient groups p < .0083 was adopted).

Question 42

Sensitivity, specificity

Answer 42

positive and negative predictive values of individual tests were calculated.

Question 43

The tests and groups were chosen on the basis of ?

Answer 43

Group comparisons.

Question 44

Any test where a patient group scored significantly worse relative to the other patient groups was ?

Answer 44

included in the sensitivity and specificity analyses.

Question 45

Receiver operating characteristics curves were fitted to the data and Louden’s index was used to

Answer 45

Determine cut-offs.

Question 46

a principal components analysis (PCA) was carried out to ?

Answer 46

delineate (describe) core factors underlying (primary, fundamental) language, working memory, and behavioural deficits in these conditions.

Question 47

Which variable ratio has yielded to good results for PCA?

Answer 47

A participant to variable ratio of 1.2

Question 48

In order to have a reasonable participant to variable ratio, only scores from tests with both sensitivities and specificities greater than ?

Answer 48

50 % were included in the analysis.

Question 49

For variables in which both ‘total’ and ‘subtests’ reached the threshold, for example, buccofacial praxis, only ?

Answer 49

subtest variables were entered into the PCA?

Question 50

In total how many variables were included in the PCA?

Answer 50

25 variables

Question 51

Once missing data are taken into account, this study gives a 1.32 ratio. Factors above the inflection point on a scree plot were retained. In order to interpret factor loadings, a variance maximizing (varimax) rotation was performed. Factor loadings were used to gain individual participants’ factor scores, which provide information about the participants’ performance on the cognitive tests that cluster on said factor.

SUOMEKSI?

Answer 51

ABOUT SINNE PÄIN:

Kun puuttuvat tiedot on otettu huomioon, tämä tutkimus antaa 1,32-suhteen. Leikkauspisteen taivutuspisteen yläpuolella olevat tekijät säilytettiin. Tekijäkuormitusten tulkitsemiseksi suoritettiin varianssin maksimoiva (varimax) kierto. Faktorikuormituksia käytettiin yksittäisten osallistujien tekijäpisteiden saamiseksi, jotka antavat tietoa osallistujien suorituskyvystä kognitiivisissa testeissä, jotka keskittyvät kyseiseen tekijään.

Question 52

LDA?

Answer 52

Linear canonical discriminant analysis

Question 53

Individual participants’ factor scores were used for a stepwise linear canonical discriminant analysis (LDA) to ?

Answer 53

to determine the strongest neuropsychological discriminators between the four groups (nfvPPA, lvPPA, SD, and multi-domain AD).