Table 2.

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

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

^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;

^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