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Patient code
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Age (years)
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Education (years)
|
ACE (93.7 ± 4.3)
|
MMSE (28.8 ± 1.3)
|
LST
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BDST
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Verbal fluency
|
Semantic fluency
|
RCFT copying
|
RCFT recall
|
|---|
|
SYNE1 patients
|
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AT-04
|
35
|
14
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89
|
29
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2 (3.38 ± 0.79)a
|
4 (5.88 ± 1.1)a
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10.5 (17.61 ± 5.42)a
|
14 (17.25 ± 3.96)
|
NA
|
NA
|
|
AT-05
|
43
|
14
|
93
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29
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2.33 (3.38 ± 0.79)a
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3 (5.88 ± 1.1)b
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14.5 (17.61 ± 5.42)
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17 (17.25 ± 3.96)
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35 (31.1 ± 3.6)
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15 (23.7 ± 5.2)a
|
|
AT-06
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37
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12
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89
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29
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2 (3.38 ± 0.79)a
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2 (5.88 ± 1.1)c
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16 (17.61 ± 5.42)
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14 (17.25 ± 3.96)
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36 (31.1 ± 3.6)
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21.5 (23.7 ± 5.2)
|
|
Friedreich ataxia patients
|
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AT-08
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23
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12.5
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93
|
30
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3 (3.45 ± 0.89)
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4 (5.88 ± 1.1)a
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14.5 (16.13 ± 5.65)
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11 (15.84 ± 4.51)
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36 (31.1 ± 3.6)
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24 (23.7 ± 5.2)
|
|
AT-11
|
57
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16
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87a
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27
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2.33 (3.11 ± 0.61)a
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3 (5.34 ± 0.96)b
|
12 (11.02 ± 4.98)
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16 (13.77 ± 4.05)
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36 (29.2 ± 4.2)
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26.5 (15.5 ± 5.5)
|
|
AT-12
|
60
|
17
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95
|
29
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3 (3.11 ± 0.61)
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2 (5.34 ± 0.96)c
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15.5 (11.02 ± 4.98)
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20 (13.77 ± 4.05)
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34 (29.2 ± 4.2)
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27 (15.5 ± 5.5)
|
|
AT-20
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16
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10
|
88
|
26a
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2.66 (3.33 ± 0.59)
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5 (5.88 ± 0.96)
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13.5 (13.83 ± 4.31)
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14 (13.44 ± 3.52)
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36 (31.1 ± 3.6)
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26 (23.7 ± 5.2)
|
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AT-21
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59
|
14
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94
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30
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3 (3.11 ± 0.61)
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5 (5.34 ± 0.96)
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15 (11.02 ± 4.98)
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14 (13.77 ± 4.05)
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32 (29.2 ± 4.2)
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17.5 (15.5 ± 5.5)
|
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AT-22
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34
|
15
|
96
|
30
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3.3 (3.38 ± 0.79)
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4 (5.88 ± 1.1)a
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16.5 (16.13 ± 5.65)
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21 (15.84 ± 4.51)
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34 (31.1 ± 3.6)
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21 (23.7 ± 5.2)
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- In ACE and MMSE the lower normal threshold values of the normal population are in the brackets
- In LST, BDST, verbal fluency, semantic fluency, RCFT copying and RCFT recall the age- and education-matched lower threshold values of the normal population are in the brackets
- ACE Addenbrooke’s Cognitive Examination, BDST Backward Digit Span Task, LST Listening Span Task, MMSE Mini-Mental State Examination, NA not available, RCFT Rey Complex Figure Test
- aMild deficit, bmoderate deficit, csevere deficit (Age- and education-matched standards, and standard deviations of the literature are delineated [23,24,25]. Mild, moderate and severe deficits mean that the cognitive impairment of the subject is more pronounced than one, two and three standard deviations of the normal standards, respectively)
