Infant’s engagement and emotion as predictors of autism or intellectual disability in West syndrome

Abstract

West syndrome (WS) is a rare epileptic encephalopathy with early onset and a high risk of autistic outcome. The PréAut grid assesses this risk following WS onset by taking into account synchrony and emotion in interactions and by evaluating the baby's active desire to engage in pleasant interactions (especially the infant's early active behaviors that encourage being gazed at or kissed by the mother or to share joy with her). We followed a sample of 25 WS patients prospectively from disease onset and assessed whether the PréAut grid before 9 months, and the checklist for autism in toddlers (CHAT) at 18 and 24 months predicted autism or intellectual disability (ID) outcomes at 4 years. We found that the PréAut grid at 9 months (sensitivity = 0.83; specificity = 1) had similar prediction parameters as the CHAT at 18 months (sensitivity = 0.90; specificity = 0.83) and 24 months (sensitivity = 0.92; specificity = 1). WS patients with a positive PréAut screening at 9 months had a risk of having autism or ID at 4 years, which is 38 times that of children with a negative PréAut grid [OR = 38.6 (95 % CI 2.2-2961); p = 0.006]. We conclude that the PréAut grid could be a useful tool for the early detection of autism or ID risk in the context of WS. Further research is needed to assess the PréAut grid in other contexts (e.g. infants at high-risk for non-syndromic autism).

Tracking Social Motivation Systems Deficits: The Affective Neuroscience View of Autism

“…Interestingly, social play is one of the dimensions assessed by the Preaut scale, which also includes items looking at the presence of early active behaviors that encourage being gazed at or kissed by the mother or sharing joy with her. Interestingly, a recent study has shown that infants diagnosed with a West syndrome were 38 times more likely to receive an ASD diagnosis later in life if they had scored high on the Preaut scale at 9 months (Ouss et al 2013).…”

Early detection of autism spectrum disorders: From retrospective home video studies to prospective ‘high risk’ sibling studies

“…In particular children with specific forms of epilepsy, such as infantile spasms or West syndrome (a rare epileptic encephalopathy with early onset, which is often secondary to tuberous sclerosis complex) present higher prevalence of ASD comorbidity (Saemundsen et al, 2008;Berg et al, 2011;Ouss et al, 2014). Although it may be useful Q7 to screen these high risk samples for ASD symptoms, and to refine screening instruments and clinical tools for these specific disorders (Gipson et al, 2014), the insight this research may provide into the etiology and early manifestations of non-syndromic ASD is controversial, as ASD in genetic syndromes (i.e.…”

Autism spectrum disorders: An historical synthesis and a multidimensional assessment toward a tailored therapeutic program

“…E.g. in the case of Tuberous Sclerosis, it has been shown despite common genetic causal involvement that clinical phenotype was bimodal: on one hand a severe phenotype including ID, ASD and epileptic encephalopathies; on another hand, a mild phenotype with normal or borderline intelligence and ASD (Ouss et al, 2014). (2) The targeted impairment must not impact other dimensions, meaning that modularity in cognitive development is always verified.…”