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Table 2 Pooled results of the studies included in the quantitative meta-analysis. MMSE: mini-mental state examination; MOCA: MOntreal Cognitive Assessment; TMT: trail making test; DSF: digital span forward; DSB: digital span backward; WAIS Wechler adult intelligence scale, III: third version, R: revised; CVLT: California verbal learning test; SPART: 10/36 Spatial recall test; RAVLT, Rey Auditory Verbal Learning Test; SLD: segment length discrimination task; *: Test corrected by patients’ PATA rate test and nine hole pegboard test score using the methods described in Sacca et al. [35] d : Cohen’s d; CI: confidence interval

From: The cognitive profile of Friedreich ataxia: a systematic review and meta-analysis

Neuropsychological tests

Pooled test results

p

Pooled effect size (d,CI)

Number of studies

Number of patients

SCREENING

MMSE

28,6±1,4 vs 29,3±0,98

0.0005

1.4 (1-1.8)

3

71

MOCA

23.9±3.4 vs 26.9±2

<0.0001

1.2 (0.8/1.5)

3

66

VERBAL FLUENCIES

Phonemic Fluency

10,3±4,1 vs 14,4±4,3

<0.0001

1 (0.6-1.4)

2

49

Semantic verbal fluency

19,6±5,3 vs 24,2±4,8

<0.0001

1.13(0.8-1.4)

4

99

Semantic fluency, corrected*

18,9±5,8 vs 23,7±5

0.0001

1(0.6-1.4)

2

44

F-A-S

26,7±9,8 vs 41,9±5,6

<0.0001

2.2 (1.7-2.6)

2

60

F-A-S, corrected*

28,2±11,5 vs 41,5±9,1

<0.0001

1.3 (0.8-1.7)

2

44

Action verbal fluency

12,8±5 vs 18,8±5,8

<0.0001

1.3 (0.9-1.7)

2

56

LANGAGE

Hayling sentence completion task

5,9±1,1 vs 6,4±0,7

0.00026

1.4 (1.1-1.8)

2

64

ATTENTION/EXECUTIVE

Stroop interference score

54±9,4 vs 50,2 6,9

0.0003

0.5(0.3/0.7)

6

130

TMT (B-A)

44,4±30,8 vs 26,4±15

<0.0001

0.8 (0.4-1.1)

3

68

TMT A

137±143 vs 31,5±12

<0.0001

1.3 (0.8-1.7)

2

37

TMT B

213 ±162 vs 78 ± 31

<0.0001

1.4 (1-1.8)

2

37

Simon incongruent reaction time

861±219 vs 585 ± 131

<0.0001

1.6 (0.9-2.2)

2

25

TMT(B-A), corrected*

56,2±19,6 vs 33,9±10,6

<0.0001

1.4 (0.9-1.9)

2

41

Attentive Matrice test

44,9±10,2 vs 55,4±4,1

<0.0001

1.4(0.9-2)

2

37

ATTENTION AND WORKING MEMORY

DSFWAIS-III

7,5±2,6 vs 8,3±0,9

0.0067

0.7 (0.4-1)

3

89

DSBWAIS-III

5,3 ±0,9 vs 6,4 ±1

<0.0001

1.4 (1-1.8)

2

59

DSFWAIS-R

6,9±1,3 vs 7,6±1,3

0.015

1(0.5-1.5)

2

42

DSBWAIS-R

5,5±1,2 vs 5,5±1,6

1

0 (-0.4-0.4)

2

42

MEMORY AND LEARNING

CVLT

12,4±0,9 vs 12,9±0,6

0.2

0

2

48

SPART

18,9±6,2 vs 23,2 ± 3,5

<0.0001

1 (0.7-1.4)

3

64

VISUO-SPATIAL

RAVLT

41,6±12,9 vs 47,6±9,3

0.014

0.5(0.2-0.9)

2

44

SLD

26,8±2 vs 28,7±2 1,6

<0.0001

1.3(0.8-1.7)

2

44