Inhibition, shifting and updating in relation to psychometric intelligence across ability groups in the psychiatric population.

BACKGROUND: Assessment of intelligence and executive function (EF) is common in complex neuropsychiatric practice. Although previous studies have shown that EF and intelligence are related, it is unknown whether these constructs relate to one another in a similar manner across different ability groups (mild intellectual disability, borderline intellectual disability and normal/high intelligence). This study therefore examines the relation between three EFs (inhibition, shifting and updating) and intelligence in a heterogeneous psychiatric sample. It is hypothesised that the strength of the relation between intelligence and the three EFs decreases when the level of intelligence increases, in accordance with Spearman's Law of Diminishing Returns. METHODS: In a cross-sectional, between and within subject design, one of the three intelligence tests (Kaufman Adolescent and Adult Intelligence Test and Wechsler Adult Intelligence Scale - third and fourth editions) and several EF tests (Stroop Colour-Word Test, Trail Making Test and Spatial Working Memory task) were administered to 250 neuropsychiatric inpatients and outpatients (Mage  = 39.8, standard deviation = 14.3, 52.8% male). Based upon their full-scale IQ score, patients were divided into three ability groups (mild intellectual disability, borderline intellectual disability or normal/high intelligence). The relation between EF and intelligence was assessed through analyses of the correlation pattern; groups were compared using analysis of covariance. RESULTS: Analyses showed significant correlations between the constructs of EF and intelligence. A significant interaction effect was found for shifting, with highest correlations in the normal to high intelligence group, but not for inhibition and updating. CONCLUSIONS: Results support a specific role for shifting in this EF-intelligence relation. The correlational pattern of updating and intelligence, as well as the differential relation of shifting and intelligence across ability groups, suggests that EF tasks may not measure distinct EFs in lower intellectual ability but rely on cognitive primitives such as processing speed. EF tasks can be considered less valid indicators of EF ability. Implications in terms of the need for development of specific tasks to measure cognition in low intellectual ability are discussed.

Exploring the cognitive phenotype of Kabuki (Niikawa-Kuroki) syndrome.

BACKGROUND: Kabuki syndrome (KS) is a Mendelian disorder, characterised by short stature, facial dysmorphisms and developmental delay and/or intellectual disability. Clarification of the neurocognitive profile in KS may provide directions for education and treatment interventions for KS. Previous studies on cognitive functioning in KS are scarce and have mainly focused on the general level of intelligence. The few more extensive studies suggested weaknesses in language skills, visuoconstruction, perceptual reasoning and speed of information processing. Other relevant domains such as memory, executive functioning and social cognition have not been studied yet. METHOD: This is the first study in which cognitive functioning within multiple domains is systematically explored in 29 participants with KS (age range: 5-48 years) and compared to both norm groups (healthy population) and an appropriate control group of 15 individuals with other genetic syndromes (age range: 6-28 years). RESULTS: Compared to the norm groups of the cognitive test manuals, as expected, participants with KS show a weaker performance on all cognitive tests. Comparison with the more appropriate genetic control group indicates weaknesses in visuoconstruction and visual memory and no weaknesses in planning, cognitive flexibility or social cognition. Verbal memory seems to be a relative strength. CONCLUSIONS: Individuals with KS suffer from specific weaknesses in visuoconstruction, in addition to their intellectual disability/developmental delay. These impairments in visuoconstruction plausibly result from problems in visual perceptual processing, which highlight the importance of the use of auditory cues instead of visual cues in targeted educational support and psychosocial interventions.