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Table 3 Dementia screening and neurocognitive test parameter scores in patients with NP-C and patients with eAD

From: Distinguishing neurocognitive deficits in adult patients with NP-C from early onset Alzheimer’s dementia

Test

n

NP-C patients (n = 7)

n

eAD patients (n = 15)

Mean group difference

Cohen’s d effect-sizea

p-valueb

Mean ± SD

Mean ± SD

Dementia screenings

 MMSE

4

27.0 ± 2.16

13

21.9 ± 1.9

5.1

2.5

p = 0.010

 FAB

5

14.4 ± 3.3

5

11.8 ± 3.3

2.6

0.8

p = 0.245

Neurocognitive test parameters

 RAVLT1 (verbal span)

7

7.0 ± 3.1

15

3.3 ± 1.6

3.7

1.5

p = 0.018

 RAVLT5 (verbal learning)

7

12.9 ± 3.1

15

6.7 ± 2.7

5.6

1.9

p = 0.002

 RAVLT1–5 (total learning)

7

51.0 ± 14.6

15

28.6 ± 8.7

22.4

1.9

p = 0.006

 RAVLT6 (recall after interference)

7

10.1 ± 4.4

15

3.3 ± 2.5

6.9

1.9

p = 0.005

 RAVLT5–6 (forgetting after interference)

7

2.1 ± 1.8

15

3.5 ± 1.9

−1.3

− 0.7

p = 0.141

 RAVLT7 (delayed verbal recall)

7

10.7 ± 3.5

15

1.9 ± 2.1

8.8

3.0

p < 0.001

 RAVLT5–7 (forgetting after delay)

7

1.6 ± 1.1

15

4.8 ± 2.1

−3.2

−1.9

p < 0.001

 RAVLT8 (recognition)

7

13.7 ± 1.9

14

11.3 ± 3.9

2.4

0.5

p = 0.058†

 RCFT copy (visuoconstructional ability)

5

29.8 ± 4.1

13

20.7 ± 12.9

9.1

0.9

p = 0.038

 RCFT time to copy (seconds)

5

233.4 ± 95.9

8

306.5 ± 123.5

− 73.1

−0.7

p = 0.259

 RCFT recall (visual memory)

5

12.3 ± 8.0

13

4.8 ± 4.8

7.5

1.1

p = 0.107

 TMT-A (processing speed; seconds)

7

61.6 ± 40.7

14

93.1 ± 81.5

−31.5

−0.3

p = 0.252†

 TMT-B (set shifting; seconds)

7

119.0 ± 54.4

10

226.7 ± 101.9

− 107.7

−1.3

p = 0.017

 RWT word fluency (1 min animals)

7

17.3 ± 5.1

13

14.8 ± 5.4

2.4

0.5

p = 0.335

 RWT word fluency (1 min letter S)

7

8.4 ± 3.1

14

11.4 ± 7.8

−3.0

−0.5

p = 0.225

 WMS digit span (forwards)

6

7.0 ± 2.4

14

5.9 ± 1.4

1.1

0.6

p = 0.353

 WMS digit span (backwards)

6

5.7 ± 1.5

14

3.8 ± 1.9

1.9

1.1

p = 0.037

  1. aEffect sizes calculated according to methodology for Cohen’s d statistic for paired t-tests; bp-values specified based on the Welch t-test, unless otherwise specified; †p-values based on the Mann-Whitney test; For TMT-A, TMT-B, RCFT time to copy, RAVLT5–6, and RAVLT5–7 subscales, lower scores indicate better performance. Higher raw scores signify better performance for all other neurocognitive test parameters. For dementia screening scores, higher scores represent less impairment on the MMSE and the FAB. FAB Frontal Assessment Battery, MMSE Mini-Mental Status Evaluation, RAVLT Rey Auditory Verbal Learning Test, RCFT Rey Complex Figure Test and Recognition Trial, RWT Regensburger verbal fluency test, TMT Trail-Making Tests A and B, WMS Wechsler Memory Scales
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172