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Table 2 Neuropsychological evaluation in NPC patients

From: Cognitive impairment profile in adult patients with Niemann pick type C disease

Functions

Tests

n

Mean

SD

Median

IQR

Normsa

Global cognitive efficiency

Impaired (Y/N)

17/4

     

MMSE (/30)

20

20.6

6.8

22

17.2–25

≥ 27

Mattis (/140)

12

103.6

28.6

117

110–125

≥ 123

Executive functions

Impaired (Y/N)

17/3

     

FAB (/18)

19

11.6

4.1

12

11–14

≥ 16

Wisc. (criteria) (/6)

9

3.6

1.9

3

2–6

≥ 5.6

Lexical fluency

12

6.5

4.4

5.5

3–9.7

≥ 17–19

Semantic fluency

11

13.3

5.8

11

10–15

≥ 26–29

Attention

Impaired (Y/N)

11/1

     

Direct verbal spans

11

4.7

1.3

5

4–6

7 ± 2

Indirect verbal spans

11

2.6

1.7

3

2–3

5 ± 2

Direct visual spans

9

4.1

1.5

4

3–5

7 ± 2

Indirect visual spans

9

3.2

1.8

3

2–4.5

5 ± 2

Memory (FCSRT)

Impaired (Y/N)

6/7

     

Immediate Recall (/16)

8

13.6

1.5

14

12.5–14

≥ 14

Free recall (/48)

8

22.5

5.7

22.5

17.5–28.2

≥ 17

Total recall (/48)

9

43.4

5.6

45

40.5–47

≥ 40

Cued free recall (/16)

8

8

3.6

8.5

6.2–10.7

≥ 10

Cued total recall (/16)

8

14.5

2.5

15.5

13.5–16

≥ 15

Language

Impaired (Y/N)

8/3

     

BNT 34 (/34)

4

21.3

8.7

19.5

14–30.2

≥ 27

Praxis

Impaired (Y/N)

10/3

     

Visuo-contructive functions

Impaired (Y/N)

5/5

     

Rey figure (score)

6

28.3

8.1

31.5

25.5–32.2

≥ 29

Rey figure (second)

6

319.2

149.9

341

179.5–459.7

≤ 360

  1. This table presents results of the first neuropsychological examination. This test took place an average of 14.4 years after the onset of the disease and 16.6 months after the diagnosis
  2. BNT Boston naming test, FAB frontal assessment battery, FCSRT free and cued selective reminding test, IQR interquartile range, MMSE mini-mental state examination, N number of available tests, SD standard deviation, Y/: yes/no.
  3. aThese are approximated norms for patients between 30 and 40 years old to give an approximate cut-off of expected results