Impact d’un entraînement de la mémoire à court terme verbale sur le langage d’enfants ayant une déficience intellectuelle

La mémoire à court terme verbale (MCTV) agit en interaction avec d’autres fonctions cognitives (langage, raisonnement, lecture, etc.) Or, les personnes ayant une déficience intellectuelle (DI) présentent un trouble de ce système mnésique. L’objectif de cette étude est d’évaluer l’impact d’un entraînement de la MCTV sur le langage oral d’enfants présentant une DI. Nous avons proposé un programme de rééducation de la MCTV à sept enfants qui ont un tel diagnostic et une rééducation de la catégorisation à un groupe contrôle de six enfants ayant une DI. Une augmentation de l’empan à court terme a ainsi été démontrée, mais également une amélioration de certains aspects du langage. Un entraînement de la MCTV serait nécessaire chez ces enfants afin d’aider au développement de leur langage oral.The Verbal short term memory (STM) acts together with others functions such as language, reasoning and reading...People with a mental deficiency are STM-impaired, in particularly in the verbal modality. The aim of the present study is to estimate the impact of verbal STM training on the language of children with a mental deficiency. A first group of seven children was trained with verbal STM training program and a second group (N=6) followed categorization training exercises. Results demonstrated an improvement in STM and some language domains like vocabulary, syntax comprehension or phonology, for the first group only. In conclusion, verbal STM training seems to improve the language of mental deficient children

Autism spectrum disorder associated with 49,XYYYY: case report and review of the literature

International audienceBACKGROUND:Sex chromosome aneuploidies occur in approximately one in 420 live births. The most frequent abnormalities are 45,X (Turner syndrome), 47,XXX (triple X), 47,XXY (Klinefelter syndrome), and 47,XYY. The prevalence of males with more than one extra sex chromosome (e.g. 48,XXYY or 48,XXXY) is less common. However, the literature provides little information about the cognitive and behavioural phenotype and the natural history of the disease. We report the clinical, neurocognitive, social cognitive and psychiatric characterization of a patient with 49,XYYYY syndrome.CASE PRESENTATION:The patient presented with a complex phenotype including a particular cognitive profile with intellectual deficiency and autism spectrum disorder (ASD) with limited interests. Moreover, social anxiety disorder with selective mutism and separation anxiety disorder were observed (DSM-5 criteria, MINI Assessment).CONCLUSION:It is now admitted that 49,XYYYY has unique medical, neurodevelopmental and behavioural characteristics. Interestingly, ASD is more common in groups with Y chromosome aneuploidy. This clinical report suggests that understanding the cognitive and social functioning of these patients may provide new insights into possible therapeutic strategies, as cognitive remediation or social cognitive training

Facial emotion perception by intensity in children and adolescents with 22q11.2 deletion syndrome

International audienceDifficulties in the recognition of emotions in expressive faces have been reported in people with 22q11.2 deletion syndrome (22q11.2DS). However, while low-intensity expressive faces are frequent in everyday life, nothing is known about their ability to perceive facial emotions depending on the intensity of expression. Through a visual matching task, children and adolescents with 22q11.2DS as well as gender- and age-matched healthy participants were asked to categorise the emotion of a target face among six possible expressions. Static pictures of morphs between neutrality and expressions were used to parametrically manipulate the intensity of the target face. In comparison to healthy controls, results showed higher perception thresholds (i.e. a more intense expression is needed to perceive the emotion) and lower accuracy for the most expressive faces indicating reduced categorisation abilities in the 22q11.2DS group. The number of intrusions (i.e. each time an emotion is perceived as another one) and a more gradual perception performance indicated smooth boundaries between emotional categories. Correlational analyses with neuropsychological and clinical measures suggested that reduced visual skills may be associated with impaired categorisation of facial emotions. Overall, the present study indicates greater difficulties for children and adolescents with 22q11.2DS to perceive an emotion in low-intensity expressive faces. This disability is subtended by emotional categories that are not sharply organised. It also suggests that these difficulties may be associated with impaired visual cognition, a hallmark of the cognitive deficits observed in the syndrome. These data yield promising tracks for future experimental and clinical investigations

Social cognition in Wilson’s disease: A new phenotype?

<div><p>Studies focusing on neuropsychological impairments in Wilson’s disease (WD) have highlighted that patients showing neurological signs present significant deficits in a wide range of cognitive domains. Attentional and executive impairments have also been described in people with hepatic WD. However, social cognition abilities, <i>i</i>.<i>e</i>. cognitive processes required to perceive the emotions, intentions and dispositions of other people, have not been clearly investigated in WD. In this study we examined the social cognitive functioning in 19 patients with WD depending on their clinical status–Neurological versus Non-Neurological (“hepatic”) forms–compared to 20 healthy controls. For the very first time, results highlighted that patients with WD had significant impairments in the three major components of social cognition: emotion recognition, Theory of Mind and attributional style. However, these deficits differ depending on the form of the disease: patients with neurological signs showed a wide range of deficits in the three components that were assessed–results notably revealed impairments in recognizing “fear”, “anger”, and “disgust”, a significant Theory of Mind deficit and an “aggression bias”–whereas Non-Neurological patients only showed deficits on test assessing attributional bias, with a trend to react more “aggressively” to ambiguous social situations than healthy controls, as observed in Neurological WD patients, and a specific impairment in “anger” recognition. Our findings are discussed in the light of both neurocognitive impairments and brain damages, and especially those affecting the basal ganglia, as observed in people with WD.</p></div

Characteristics of Neurological WD patients, Non-Neurological WD patients and healthy controls.

<p>Characteristics of Neurological WD patients, Non-Neurological WD patients and healthy controls.</p

Total score and error type on the MASC test assessing ToM.

<p>Total score and error type on the MASC test assessing ToM.</p

Results on the AIHQ assessing three social cognitive biases.

<p>Results on the AIHQ assessing three social cognitive biases.</p

Scores on the TREF assessing emotion recognition.

<p>Scores on the TREF assessing emotion recognition.</p