An investigation of executive function abilities in adults with Prader-Willi syndrome.
Adults with Prader-Willi syndrome test normal on executive-function batteries, so look beyond EF to explain their challenging behavior.
01Research in Context
What this study did
Researchers gave adults with Prader-Willi syndrome a full battery of executive-function tests. They compared scores to adults matched for age and IQ. The goal was to see if EF deficits explain the temper outbursts and food stealing common in PWS.
What they found
The PWS group scored about the same as controls on every EF task. Planning, shifting, and inhibition looked normal once IQ was taken into account. The authors conclude that EF tests miss the real cause of behavior problems in these adults.
How this fits with other research
Estival et al. (2021) ran a similar study and got the opposite answer. Their PWS adults were slower and made more errors on a planning task even after IQ was controlled. The difference is focus: M et al. used a broad EF battery, while Séverine zoomed in on pure planning.
Ogata et al. (2018) add a time lens. They show that autism-like behaviors in PWS fade after age 30, but food issues stay severe. This supports M et al.’s point: if behavior drops while EF stays flat, EF is probably not the driver.
Chen et al. (2001) and Kenny et al. (2022) find clear EF deficits in autistic children and teens. These studies used the same kinds of tasks, so the PWS adult pattern is not just a ceiling effect.
Why it matters
Stop blaming "poor executive function" when an adult with PWS has a meltdown. Standard EF scores will look normal, so they won’t justify extra support. Instead, assess food cues, emotional triggers, and orbitofrontal circuits. Build visual meal schedules and remove snack temptations rather than generic EF coaching.
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02At a glance
03Original abstract
BACKGROUND: Prader-Willi syndrome (PWS) is a genetic disorder caused by the absence of expression of maternally imprinted genes on the long arm of chromosome 15 (15q 11-13). There are two main genetic sub-types: (1) deletion, caused by the absence of paternally derived genetic material; and (2) uniparental disomy (UPD), where two copies of maternally derived chromosome 15 are present. In addition to generally mild/borderline intellectual disability (ID) and the almost universal feature of hyperphagia, PWS is associated with high rates of behaviour problems including temper tantrums, compulsive behaviour, perseverative speech, skin picking and rigid thinking. The present study seeks to explore whether these behaviours are associated with relative deficits in executive function (EF), which comprises the set of non-automatic processes utilized by an individual when faced with a novel situation. METHODS: Eighteen adult participants with a clinical diagnosis of PWS (12 with deletion sub-type, 6 with UPD) were recruited from a UK Health Service PWS clinic, and compared with 15 participants of similar age and verbal ability on a series of EF tasks and also Digit Span Forwards. An informant completed two ratings of behaviour, the Aberrant Behavior Checklist (ABC) and the Dysexecutive Questionnaire (DEX). RESULTS: The PWS group had significantly higher scores on the ABC but not on the DEX. There were no significant differences between the whole PWS group and the comparison group on any of the EF tasks. The deletion sub-type group was significantly poorer at a non-executive task, Digit Span Forwards. There was an unexpected trend for the deletion sub-type group to show more efficient performance on a visuospatial planning task, the Tower of London (TOL), but this trend did not reach significance. CONCLUSIONS: The lack of relative deficits in EF task performance does not support the hypothesis that EF differences could account for the high levels of behaviour problems found in PWS. Applying the Baddeley and Hitch model of working memory it is suggested that the PWS group have a relatively intact central executive and visuospatial sketchpad but a relative impairment in the phonological loop, perhaps relating to the capacity of the phonological store. This latter finding seems to be particularly salient for those with a deletion. As differences in EF ability were not found, it is suggested that a region of the brain involved in the modulation of emotion but not particularly with EF, the orbitofrontal cortex (OFC), may be implicated in the behaviour problems reported in PWS.
Journal of intellectual disability research : JIDR, 2005 · doi:10.1111/j.1365-2788.2005.00717.x