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

De Felice, Sara, Romani, Cristina, Geberhiwot, Tarekegn, Macdonald, Anita and Palermo, Liana (2018). Language processing and executive functions in early treated adults with phenylketonuria (PKU). Cognitive Neuropsychology ,


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: Life & Health Sciences > Psychology
Life & Health Sciences > Clinical and Systems Neuroscience
Life & Health Sciences
Life & Health Sciences > 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:
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Related URLs: https://www.tan ... 94.2017.1422709 (Publisher URL)
Published Date: 2018-02-28
Published Online Date: 2018-02-28
Authors: De Felice, Sara
Romani, Cristina
Geberhiwot, Tarekegn
Macdonald, Anita
Palermo, Liana ( 0000-0001-8047-7201)



Version: Accepted Version

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