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Table 1 Serial longitudinal neuropsychological assessment of E200G case

From: Genetic CJD with a novel E200G mutation in the prion protein gene and comparison with E200K mutation cases

 

Visit 1

Visit 2

Visit 3

25 months

26 months

28 months

Raw (Z-score)

Raw (Z-score)

Raw (Z-score)

Global cognition

   

MMSE

261

242

16

Memory

   

CVLT trial 5

4 (−4.00)

4 (−4.00)

0 (−5.00)

CVLT delay

1 (−4.00)

1 (−4.00)

0 (−4.00)

CVLT recog hits

15 (0.00)

15 (0.00)

13 (−1.00)

CVLT recog false pos

3 (−0.50)

3 (−0.50)

16 (−4.00)

Benson figure delay recall

3 (−4.17)

0 (−5.48)

N/D

Mean memory Z-score

−2.53

−2.80

−3.50

Executive function

   

Modified trails

59" (−3.00)

119" (−8.62)

120" (−8.71)

Design fluency

N/A

5 (−2.28)

0 (−4.07)

Digit backwards span

3 (−1.85)

3 (−1.85)

3 (−1.85)

Stroop inhibition

31 (−2.25)

19 (−3.50)

N/D

Mean executive Z-score

−2.37

−4.06

−4.88

Language

   

BNT-Abbrev

13 (−1.66)

15 (+0.55)

N/D

Visuospatial

   

Benson figure copy

13 (−1.27)

14 (−1.36)

N/D

  1. 1 Missed 2 points for orientation and 2 on recall. 2 Missed 3 points for orientation and 3 on recall. Abbreviations and Definitions: MMSE Mini-Mental State Examination, CVLT California Verbal Learning Test-II (16 word list), Recog Recognition, False Pos False Positive Total, Modified Trails Abbreviated trail-making task using numbers and days of the week, BNT-Abbrev Abbreviated (15-item) Boston Naming Test, N/D patient unable to perform, N/A not administered.