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Table 2 Overview of tasks used to assess cognitive functioning in ET AwPKU across different cognitive domains

From: A systematic review of cognitive functioning in early treated adults with phenylketonuria

Cognitive domain Taska Sensitivityb
Attention and processing speed
 Attentional capacity California Verbal Learning Test (CVLT) – List A trial 1 [33] 1/1
Choice Reaction Time [11, 34] 1/2c
Conjoined Search [11] 1/1c
Detection with Distractors [11] 0/1
Digit Span Forward (WAIS-R) [33] 0/1
Feature Search [11] 1/1c
n-back (0-back and 1-back trials) [36] 1/1c
Stroop colour [11, 33] 2/2c
Stroop word [33] 0/1
Telephone Search Test (TEA) [45] 1/1
Video tracking [55] 1/1
  9/13
 Vigilance/focus Continuous Performance Test (CPT) – omission errors [33] 0/1
Rapid Visual Processing [11, 34] 2/2
Sustained Attention Dots (ANT) [40] Dot Pattern Exercise (SVAT) [37] 2/2
Telephone Search Test with Counting (TEA) [45] 1/1
Test d2 [52] 1/1d
  6/7
 Processing speed CPT – response rate [33] 0/1
Motor Screening Test – latency [48] 0/1
Saccadic Latency [38] 1/1e
Simple Detection [11] Simple Reaction Time (CANTAB) [34] Finger Motor Speed Exercise (SVAT) [37] 0/3
Stockings of Cambridge – initial thinking time [48] 0/1
Trail Making Test-A [1, 2, 33, 52] Attention Diagnostic Method [56] 2/3
WAIS-III (Processing Speed Index) [35] 1/1e
  4/11
Executive functions
 Complex executive functions Brixton Test [45] 0/1
Elithorn Perceptual Maze Test [49] 1/1
Object alternation learning [36] 0/1
Set Shifting Visual (ANT) [42] 1/1f
Six Elements Test [45] 0/1
Spatial Working Memory – Strategy [48] 1/1
Stockings of Cambridge [48] 1/1
Tower of Hanoi [11] Tower of London [49] 1/2
Trail Making Test (TMT) B-A [11, 33, 35] 0/3
WAIS-III (Perceptual Organisation Index) [35] 1/1e
Wisconsin Card Sorting Test (WCST) [11, 33, 49, 56] 3/4
  9/17
 Inhibitory control CPT [33] 1/1
Flanker [36, 42] 1/2c
Go-nogo [44] 0/1
Set Shifting Visual (ANT) [42] 0/1
Stop Signal Task [34] 1/1
Stroop (interference) [11, 33] 0/2
Sustained Attention Dots (ANT) [40] 1/1
  4/9
 Working memory Corsi Block Tapping Test [11] 0/1
CVLT – perseverative error [33] 1/1
Digit Span – backward [11, 33] 2/2
Feature Integration (ANT) [40] 1/1
Letter Pattern Exercise (SVAT) [37] 0/1
Memory Search 2 Dimensional (ANT) [40] 1/1
n-back (2-back trials) [36] 1/1c
Non-word Repetition [11] 1/1
Self-Ordered Pointing test [45] 1/1
Spatial Span [34] 1/1
Spatial Working Memory [34, 48] 2/2
Visuo-Spatial Sequencing (ANT) [40] 1/1
WAIS-III (Working Memory Index) [35] 0/1
  12/15
 Verbal fluency Animal naming [11, 33] 2/2
Controlled Oral Word Association Test [33, 35] 1/2
Letter fluency [11, 45] 1/2
  4/6
Language
 Basic language skills: semantic processing Boston Naming Test [33] Picture naming [11] 1/2
Blocked cyclic naming [47] 0/1
Emotional Prosody Discrimination test [47] 0/1
Hayling Sentence Completion Test – Part A [47] 0/1
Narrative production: Recalling the Cinderella story [47] 0/1
Non-emotional Prosody Discrimination test [47] 0/1
Peabody Picture Vocabulary Test – Revised (PPVT-R) [33] 0/1
Word reading [11] 1/1c
Word spelling [11] 0/1
  2/10
 Complex language skills Appreciation of Humour test [47] 0/1
Blocked cyclic naming – semantic Inference [47] 0/1
Comprehension of Inferred Meaning test [47] 1/1c
Conflicting prosody – attend to prosody test [47] 0/1
Hayling Sentence Completion Test – Part B [45, 47] 1/2
Metaphor Picture test [47] 1/1c
Naming: semantic inference [11] 0/1
Narrative production: Recalling the Cinderella story [47] 1/1
Non-word reading [11] 1/1c
Non-word spelling [11] 0/1
Phoneme deletion [11] 0/1
Similarities (WAIS-R/WASI) [11, 33] 1/2
Spoonerisms [11] 0/1
Vocabulary (WAIS-R/WASI) [11, 33] 0/2
WAIS-III (Verbal Comprehension Index) [35] 0/1
Perceptual judgement task [36] 0/1
  6/19
Memory and learning
 Immediate recall: verbal / visual CVLT – List A trial 5 and trials 1–5 [33] 1/1
Paired Associates Verbal Learning – trial 1–5 [11] 0/1
Paired Associates Visual Learning [11] 0/1
Rey Auditory Verbal Learning Test (RAVLT) – Trial A1-A5 [11] 0/1
ROCFT – Immediate recall [49] 0/1
WMS-III [35] 0/1
  1/6
 Delayed recall: verbal / visual CVLT – Sort delayed recall and Long delayed recall [33] 1/1
Paired Associates Verbal Learning – delayed [11] 0/1
RAVLT – Delayed recall [11, 45] and retention [11] 0/2
ROCFT – Delayed recall [33, 45] 0/2
WMS-III [35] 0/1
  1/7
 Recognition: verbal / visual CVLT – Recognition memory [33] 0/1
Delayed Matching to a Sample [11] 0/1
RAVLT – Recognition [45] 0/1
ROCFT – Recognition [45] 0/1
WMS-III [35] 0/1
  0/5
Motor skills Digit Symbol (Substitution) Task [11, 33] 1/2
Grooved Pegboard [11, 33] 1/2
MLS [55] 1/1
Motor Screening Test – errors [48] 1/1
Pursuit (ANT) [42] 1/1f
  5/6
Social-cognitive abilities Face Recognition (ANT) [41] 1/1g
Faux-Pas Recognition Test [41] 1/1h
Identification of Facial Emotions (ANT) [41] 1/1g
Reading the Mind in the Eyes Task [41] 1/1h
  4/4
Visual-spatial abilities Block design (WAIS-R) [33] 0/1
Picture arrangement (WAIS-R) [33] 0/1
Picture completion (WAIS-R) [33] 0/1
ROCFT – with copy/initial copy [33, 45, 49, 56] 2/4
  2/7
  1. a Number of observed impairments in task performance in ET AwPKU / frequency of us; b (−sub measure) if task relates to multiple cognitive domains); c Compared to controls, ET AwPKU differed in speed (slower), not accuracy; d Only for older (>32) ET AwPKU;
  2. e Only for off-diet ET AwPKU; f High-high ET AwPKU group worse performance than low-high PKU group (no controls included in analysis); g Effect disappeared after adding age as a covariate; h Effect disappeared after including IQ as a covariate