Language processing and executive functions in early treated adults with phenylketonuria (PKU)


We provide an in-depth analysis of language functions in early-treated adults with phenylketonuria (AwPKUs, N = 15–33), as compared to age- and education-matched controls (N = 24–32; N varying across tasks), through: a. narrative production (the Cinderella story), b. language pragmatics comprehension (humour, metaphors, inferred meaning), c. prosody discrimination d. lexical inhibitory control and planning (Blocked Cyclic Naming; Hayling Sentence Completion Test, Burgess & Shallice, 1997). AwPKUs exhibited intact basic language processing (lexical retrieval, phonology/articulation, sentence construction). Instead, deficits emerged in planning and reasoning abilities. Compared to controls, AwPKUs were: less informative in narrative production (lower rate of Correct Information Units); slower in metaphorical understanding and inferred meaning; less accurate in focused lexical-search (Hayling test). These results suggest that i) executive deficits in PKU cannot be explained by an accumulation of lower-order deficits and/or general speed impairments, ii) executive functions engage dedicated neurophysiological resources, rather than simply being an emergent property of lower-level systems.

Publication DOI:
Divisions: College of Health & Life Sciences > School of Psychology
College of Health & Life Sciences > Clinical and Systems Neuroscience
College of Health & Life Sciences
College of Health & Life Sciences > School of Optometry > Vision, Hearing and Language
Additional Information: This is an Accepted Manuscript of an article published by Taylor & Francis Group in Cognitive Neuropsychology on 28 Feb 2018, available online at:
Publication ISSN: 1464-0627
Full Text Link:
Related URLs: https://www.tan ... 94.2017.1422709 (Publisher URL)
PURE Output Type: Article
Published Date: 2018-05-19
Published Online Date: 2018-02-28
Accepted Date: 2017-12-21
Authors: De Felice, Sara
Romani, Cristina (ORCID Profile 0000-0002-5693-4131)
Geberhiwot, Tarekegn
Macdonald, Anita
Palermo, Liana (ORCID Profile 0000-0001-8047-7201)



Version: Accepted Version

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