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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.