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Objective Previous research has suggested that neurodevelopmental conditions may be associated with distinctive cognitive profiles on the Wechsler intelligence tests (of which the most recent editions are the WAIS-IV and WISC-V). However, the extent to which a cognitive profile can be reliably identified for individuals meeting criteria for autism or ADHD remains unclear. The present review investigated this issue. Method A search was conducted in PsycInfo, Embase, and Medline in October 2022 for papers reporting the performance of children or adults diagnosed with autism or ADHD on the WAIS-IV or the WISC-V. Test scores were aggregated using meta-analysis. Results Scores were analyzed from over 1,800 neurodivergent people reported across 18 data sources. Autistic children and adults performed in the typical range for verbal and nonverbal reasoning, but scored ~1 SD below the mean for processing speed and had slightly reduced scores on working memory. This provides evidence for a "spiky" cognitive profile in autism. Performance of children and adults with ADHD was mostly at age-expected levels, with slightly reduced scores for working memory. Conclusion Although the pattern of performance on the Wechsler tests is not sufficiently sensitive or specific to use for diagnostic purposes, autism appears to be associated with a cognitive profile of relative strengths in verbal and nonverbal reasoning and a weakness in processing speed. Attention deficit hyperactivity disorder appears less associated with a particular cognitive profile. Autistic individuals may especially benefit from a cognitive assessment to identify and support with their strengths and difficulties. [ABSTRACT FROM AUTHOR]

Executive functions (EF) have been shown to be important for the understanding of Autism Spectrum Disorder (ASD), but dysfunctions of EF are not autism-specific. The specific role of EF in ASD, its relationship to core autism characteristics, such as mentalizing, needs to be explored. Medline- and PsychINFO databases were searched for studies published between 1990 and 2020 that included measures of EF in ASD and typically developing control persons (TD) in combination with either Theory of Mind (ToM) or Weak Central Coherence (WCC) tasks. A pre-registered meta-analysis and cross-study regression was performed including a total of 42 studies (ASD n = 1,546, TD n = 1,206). Results were reported according to PRISMA guidelines. In all cognitive domains, the ASD group showed significantly reduced performance. Importantly, EF subdomains and ToM were not significantly correlated. This finding rules out a significant association between EF subdomains and ToM and questions the relevance of EF dysfunctions for the autism-specific feature of reduced mentalizing. [ABSTRACT FROM AUTHOR]

Background: White-Sutton (WHSUS) is a recently recognized syndrome caused by mutations of the POGZ gene. Approximately 70 patients have been reported to date. Intellectual disability, hypotonia, behavioral abnormalities, autism, and typical facial dysmorphisms are recognized as WHSUS features; however, still few patients receive a comprehensive psychometric, behavioral and neurological examination. In this report, we describe the pediatric, dysmorphological, neurological, psychometric and behavioral phenotype in a new WHSUS patient due to a novel heterozygous POGZ mutation, highlighting the distinctive epileptic phenotype and the cognitive pattern. Case presentation: The patient, an 8 years-old girl, presented history of hypotonia, motor and speech delay, and distinctive facial features. The diagnosis of WHSUS followed the identification of the de novo variant p.Asp828GlyfsTer36 (c.2482dupG) in the POGZ gene. The patient showed a distinctive neurological phenotype with the occurrence of both paroxysmal not-epileptic events in the first 6 months of age and EEG abnormalities without evidence of clinical seizures after the first year of age. Psychological and behavioral testing highlighted moderate intellectual and communication deficit, mild autism spectrum and visual-motor integration deficit. Conclusions: This is the first described case of WHSUS with a co-existence of paroxysmal not-epileptic events and abnormal EEG without seizures in the same patient. Together with the available literature data, this observation suggests that paroxysmal not-epileptic events could be more frequent than expected and that this feature belongs to the WHSUS phenotypic spectrum. Autism is a known comorbidity of WHSUS but is still poorly investigated. Specific clinical testing could help detect also mild autistic phenotypes and better define autism prevalence in POGZ-related syndrome. Special attention should be given to symptoms such as stereotypies, social withdrawal, and hyperactivity that, when present, should be considered as possible signs of autism symptoms. The dissection of the neurological and behavioral phenotype is crucial for individualized therapies tailored to patient's needs. [ABSTRACT FROM AUTHOR]

Females with high-functioning ASD are known to camouflage their autistic symptoms better than their male counterparts, making them prone to being under-ascertained and delayed in diagnostic assessment. Thus far the underlying cognitive processes that enable such successful socio-communicative adaptation are not well understood. The current results show sex-related differences in the cognitive profile of ASD individuals, which were diagnosed late in life exclusively. Higher verbal abilities were found in males (n = 69) as opposed to higher processing speed and better executive functions in females with ASD (n = 38). Since both sexes remained unidentified during childhood and adolescence, these results are suggestive for sex-distinctive cognitive strategies as an alternative to typically-developed reciprocal social behavior and social mimicry in high functioning ASD. [ABSTRACT FROM AUTHOR]

A meta-analysis was performed to examine differences in IQ profiles between individuals with Asperger's disorder (AspD) and high-functioning autism (HFA). Fifty-two studies were included for this study. The results showed that (a) individuals with AspD had significantly higher full-scale IQ, verbal IQ (VIQ), and performance IQ (PIQ) than did individuals with HFA; (b) individuals with AspD had significantly higher VIQ than PIQ; and (c) VIQ was similar to PIQ in individuals with HFA. These findings seem to suggest that AspD and HFA are two different subtypes of Autism. The implications of the present findings to DSM-5 Autism Spectrum Disorder are discussed. [ABSTRACT FROM AUTHOR]

The aim of the present study was to assess the cognitive profiles of children with autistic spectrum disorder and of their healthy siblings (Siblings). With the term cognitive profile, we indicate the relationship extant among the values of verbal and performance subtests of the Wechsler Intelligence Scale. The conducted statistical analyses indicated that, although siblings showed a normal intelligent quotient and did not differ in this aspect from typically developing group, their cognitive profile was amazingly similar to that of their relatives affected by autism. A k-means clustering analysis on the values of single subtests further confirmed this result, showing a clear separation between typically developing children on the one side, and autistics and their siblings on the other. We suggest that the common cognitive profile observed in autistic children and their siblings could represent a marker of liability to autism and, thus, a possible intermediate phenotype of this syndrome. [ABSTRACT FROM AUTHOR]

This case study seeks to extend our knowledge of the phenotype associated with excellent word reading in autism spectrum disorder by a detailed examination of psy-cholinguistic, neuropsychological/cognitive, and classroom/academic functioning of a Swedish-speaking 7-year-old boy (called "Jonas"). On age-referenced word reading-decoding assessment, Jonas scored among the top 7%. Reading comprehension status varied as a function of information source. Jonas's phonological processing and phono-logical memory performance was found to be normal to strong. In contrast, he scored poorly on tests of oral semantic, syntactical, and narrative language. On the WISC-III, Jonas performed highly uneven across subtests from impaired to above average levels. On the Kaufman factors, Jonas had a peak in perceptual organization (IQ score 119), thus reflecting very strong visual attention skills, which together with normal to strong phonological abilities might underlie his excellent word reading. Finally, both his par-ents and teacher reported on other classroom-learning skills and difficulties. [ABSTRACT FROM AUTHOR]

Crespi and Badcock's (Behaviour Brain Sci 31: 241-261, ) novel theory, which presents autism and positive schizophrenia as diametrical opposites on a cognitive continuum, has received mixed support in the literature to date. The current study aimed to further assess the validity of this theory by investigating predictions in relation to empathizing and systemizing. Specifically, it is predicted by Crespi and Badcock that while mild autistic traits should be associated with a cognitive profile of superior mechanistic cognition (which overlaps with systemizing) but reduced mentalistic cognition (which overlaps with empathizing), positive schizotypy traits should be associated with the opposite profile of superior mentalistic but reduced mechanistic cognition. These predictions were tested in a student sample using a battery of self-report and behavioural measures. The pattern of results obtained provides no support for Crespi and Badcock's theory. [ABSTRACT FROM AUTHOR]

Abstract: EV is a child with a talent for learning language combined with Asperger syndrome. EV’s talent is evident in the unusual circumstances of her acquisition of both her first (Bulgarian) and second (German) languages and the unique patterns of both receptive and expressive language (in both the L1 and L2), in which she shows subtle dissociations in competence and performance consistent with an uneven cognitive profile of skills and abilities. We argue that this case provides support for theories of language learning and usage that require more general underlying cognitive mechanisms and skills. One such account, the Weak Central Coherence (WCC) hypothesis of autism, provides a plausible framework for the interpretation of the simultaneous co-occurrence of EV’s particular pattern of cognitive strengths and weaknesses. Furthermore, we show that specific features of the uneven cognitive profile of Asperger syndrome can help explain the observed language talent displayed by EV. Thus, rather than demonstrating a case where language learning takes place despite the presence of deficits, EV’s case illustrates how a pattern of strengths within this profile can specifically promote language learning. [Copyright &y& Elsevier]

As part of an ongoing clinical service programme for pre-school children with developmental delay in an Asian developing country, we analysed the effect of three assessment tests, that is, Bayley Scale of Infant Development-II, Leiter International Performance Scale - Revised and Wechsler Preschool and Primary Scale of Intelligence - Revised - Chinese, on the stability of intelligence quotient (IQ) of children from pre-school through early childhood. The participants were 313 Taiwanese pre-school children with uneven or delayed cognitive profile and they were followed through early childhood. IQ stability was explored by different tests and among children of different clinical diagnosis: 168 children with non-autistic intellectual disability, 73 children with autism spectrum disorder, 58 children with mixed receptive-expressive language disorder and 14 children of other heterogeneous diagnoses. Stability of scores was evaluated using the r-squared for Pearson's coefficients to see the correlation between initial IQ (IQ1) and follow-up IQ (IQ2). Multiple linear regressions were also applied to see whether IQ1 had predictive ability for IQ2 and test-test difference in the total 313 children and each diagnostic subgroup. Results revealed that mean IQ1 was 65.8 ± 15.4 while mean IQ2 was 73.2 ± 17.9 for the total 313 children. The IQs were stable across an average follow-up duration of 38.6 ± 22.1 month from pre-school into early childhood. Patterns of positive correlations between IQ1 and IQ2 were noted by all the tests ( r-squared = 0.43-0.5, all P < 0.001) and in the majority of diagnostic subgroups. Multiple regressions analysis also revealed that IQ1 could predict IQ2 significantly in all the tests (all P < 0.001). After careful choice of appropriate initial test, stability of IQ in children with developmental delay was noted from pre-school through early childhood. In addition, the translated version of cognitive assessment was valid for the required context of an Asian developing country. With the current emphasis on early identification and intervention for pre-school children with developmental delay, this information bears merit in clinical practice. [ABSTRACT FROM AUTHOR]

Studies of inner speech use in ASD have produced conflicting results. Lidstone et al., J Autism Dev Disord () hypothesised that Cognitive Profile (i.e., discrepancy between non-verbal and verbal abilities) is a predictor of inner speech use amongst children with ASD. They suggested other, contradictory results might be explained in terms of the different composition of ASD samples (in terms of Cognitive Profile) in each study. To test this, we conducted a new analysis of Williams et al.’s, J Child Psychol Psychiatry 48(1): 51–58 () data on inner speech use in ASD. This revealed verbal ability predicted inner speech use on a short-term memory task over and above Cognitive Profile, but not vice versa. This suggests multiple factors determine whether children with ASD employ verbal mediation. [ABSTRACT FROM AUTHOR]

Abstract: Many studies have shown that children with autism spectrum disorders (ASD) and attention-deficit/hyperactivity disorder (ADHD) have had early indicators of language delay. The aim of the present study was to examine the cognitive profile of school age children referred to a specialist clinic for ASD, ADHD, or both, and relate this profile specifically to the age at which these children were first flagged up (or not) as suspected from language delay during the preschool years. Forty clinic children with ASD, ADHD, or the combination of the two (without clinical suspicion of learning disability) were assessed cognitively and as regards language development and language function at a mean age of 7.3 years. They were contrasted with a group of 21 children from the community who had been flagged at 2.5 years as suspected of language delay, and who had been followed up neuropsyhiatrically/neuropsychologically and in respect of language at a mean age of 7.9 years. Mean WISC-III full scale IQ was lower than population norms (in spite of the exclusion in both samples of cases with obvious learning disability) and similar across diagnostic groups (ASD and ADHD), and across settings (clinic and community). WISC-III Kaufman factor profiles separated the diagnostic groups as regards Perceptual Organisation. Early concern about language delay was a strong predictor of lower IQ and of distinguishing between “pure” cases of ASD and ADHD. School age clinic children who present with ASD and ADHD have a similar cognitive and early language development profile as do those children from the community, followed prospectively, who present with a suspicion of early preschool language delay and are shown at school age to suffer from ASD or ADHD. Concern about early language delay in the preschool age should prompt assessments (psychiatric and cognitively) for ASD and ADHD in a multidisciplinary setting much more often than is currently the case. In many cases early language delay, even in the absence of clear learning disability should be taken as a signal that – regardless of specific diagnosis – intellectual functioning might be in the low average range. [Copyright &y& Elsevier]

We present a new analysis of Whitehouse, Maybery, and Durkin’s (2006, Experiment 3) data on inner speech in children with autism (CWA). Because inner speech development is thought to depend on linguistically mediated social interaction, we hypothesized that children with both autism and a nonverbal > verbal (NV > V) skills profile would show the greatest inner speech impairment. CWA and typically developing controls ( n = 23 in each group) undertook a timed mathematical task-switching test, known to benefit from inner speech use. Participants completed the task with and without articulatory suppression (AS), which disrupts inner speech. The hypothesis was supported: AS interference varied with cognitive profile among CWA but not among controls. Only the NV > V autism group showed no AS interference, indicating an inner speech impairment. [ABSTRACT FROM AUTHOR]

The Fifth Edition of the Stanford–Binet Intelligence Scales (SB5; Roid, G. H. (). Stanford Binet intelligence scales (5th ed.). Itasca, IL: Riverside Publishing) is relatively new, with minimal published research on general populations and none with special populations. The present study provides information on the cognitive profiles of children with ASD ( N = 63) and on the whether the abbreviated battery is representative of the full scale. A high percentage of the children had significantly stronger nonverbal (vs. verbal) skills. This pattern was not related to Full Scale IQ, age or diagnostic subgroup. IQs derived from the abbreviated battery accounted for a large proportion of the variance in FSIQ relative to comparable abbreviated batteries. However, caution is warranted when using the abbreviated battery, as it misrepresents actual ability in a small percentage of cases. [ABSTRACT FROM AUTHOR]

Background: A community-representative sample of screened and clinically examined children with language delay at 2.5 years of age was followed up at school age when their language development was again examined and the occurrence of neuropsychiatric/neurodevelopmental disorder (attention deficit/hyperactivity disorder (ADHD) and/or autism spectrum disorder (ASD)) was documented. Aims: (1) To determine whether these 7-8-year-old children with a history of language delay have deficits in narrative skills compared with the age norms of standardized tests; and (2) to analyse if there is a relationship between narrative outcome, cognitive profile, and neuropsychiatric diagnosis. Methods & Procedures: Twenty-one children recruited from a community sample and with a history of language delay underwent an in-depth multidisciplinary examination at 7-8 years of age. Their narrative and cognitive skills were examined using the Bus Story Test, the Narrative Memory Subtest from the Developmental Neuropsychological Assessment (NEPSY), and The Wechsler Intelligence Scale for Children III (WISC-III). Outcomes & Results: The three measures of the Bus Story Test (information, sentence length, and subordinate clauses) were below age norms for all 21 children, of whom 13 also had a neuropsychiatric diagnosis, i.e. ADHD and/or ASD. Half of the children with language delay had problems on Bus Story Test Information and on the Narrative Memory subtest independently of co-occurrence of neuropsychiatric disorder. The only difference across the children with language delay pure and those who had language delay plus ADHD or language delay plus ASD was on Freedom from Distractibility, where children with ADHD and ASD scored low. In addition, children with ASD had a much lower overall cognitive level (FSIQ) and poorer results on Processing Speed. Conclusions: Swedish children with late developing language at 2.5 years of age have persisting difficulties with oral narrative skills at age 7-8 years. However, almost none of the children with language delay had problems when responding to story-related questions - irrespective of whether or not they had an additional diagnosis of ADHD or ASD. Thus, asking story-related questions may be a good intervention strategy when working with these children. Because narrative difficulties are a reflection of linguistic, cognitive and/or pragmatic/social difficulties, it is important for clinicians of different specialities to work in close collaboration in order to establish a reliable measure that can be used in clinical assessment. Poor results on the WISC-III Kaufman Freedom from Distractibility factor had a strong relationship with a neuropsychiatric diagnosis (not just ADHD), whereas poor results on Bus Story Test Information or NEPSY Narrative Memory (measured as Free Recall) did not. Narrative problems were present among the language delay children even in the presence of adequate speech and verbal comprehension. Thus, narrative assessment may be a useful tool for identifying children with more persistent subtle language and pragmatic problems who are at risk for academic failure. [ABSTRACT FROM AUTHOR]

Background Prader–Willi syndrome (PWS) is associated with a characteristic behavioural phenotype whose main features are, alongside compulsive hyperphagia, deficits in social behaviour: social withdrawal, temper tantrums, perseverative speech and behaviour, mental rigidity, stereotyped behaviour, impulsiveness, etc. Similar symptoms may also be found in autistic spectrum disorders and lesional pathologies of the frontal lobe. In both cases, such symptoms have been related to dysfunctions in frontal cognitive processes such as attention, working memory and executive functions. This study uses standardized neuropsychological instruments to analyse the degree to which these processes are affected in PWS. Methods The sample comprised 16 individuals with a genetically confirmed PWS diagnosis. Subjects’ IQ (Wechsler Adult Intelligence Scale), academic level, laterality and body mass index (BMI) were calculated. Attention, memory and executive functions were analysed using standard, widely employed neuropsychological tests. We compared the results of the sample group with the general population. Correlation analyses were carried out with IQ, academic level and BMI. Results In all the neuropsychological measures focusing on attention, executive functions and visuoperceptual organization, the study sample scored significantly lower than the normative reference population. The scores of the tests used for measuring immediate memory were also significantly lower when trials required sequential processing, although not when they required simultaneous processing. In the memorization of a list of words, subjects showed an initial deficit which disappeared with repetition, enabling them to obtain scores similar to the reference population. No significant correlations were found with BMI, and a higher IQ or academic level did not improve scores in the majority of tests. Conclusions The study shows a deficit in elementary frontal cognitive processes in PWS patients. This deficit may be involved in the social behaviour disorders that characterize such patients, as described in other development or frontal syndrome pathologies. However, we cannot affirm that the deficits found are specific to PWS; they could also occur in other causes of intellectual disability. Although in the study sample IQ did not correlate with frontal deficits, further research is needed to establish whether the neuropsychological alterations described form part of a cognitive phenotype for PWS. We believe that our understanding of the social behaviours typical of PWS may be improved by taking into consideration the cognitive functioning models of the prefrontal lobe, particularly those applied to pervasive developmental disorders. [ABSTRACT FROM AUTHOR]