Social knowledge and social reasoning abilities in a neurotypical population and in children with Down syndrome.

Social knowledge refers to the ability to analyze and reason about social situations in relation to social rules which are essential to the development of social skills and social behavior. The present research aimed to assess these abilities with the "Social resolution task" in a neurotypical population of 351 children (4 to 12 years) and 39 young adults, and in 20 participants (10 to 18 years) with Down syndrome. Results showed that young children aged 4 to 6 were well able to distinguish between appropriate and inappropriate social behavior but they had significantly more difficulties in judging and identifying social cues for the transgression of conventional rules than for moral ones. Between age 4 and 8, their social reasoning was mainly based on factual answers, while older children showed significantly more social awareness, making more reference to emotional and social consequences for the "victims". The representation of a more universal applicability of social rules seemed to develop later in childhood, as of age 8. In contrast, participants with Down syndrome exhibited significantly more difficulties in judging, identifying and reasoning about transgression of social rules without social awareness. In conclusion, the results have shown that social reasoning abilities develop throughout childhood. Social awareness seems to have a long developmental course, which includes a sensibility about welfare and intersubjectivity, critical for the development of prosocial behavior. The clinical population with difficulties in social interaction and socio-emotional behavior could benefit from an early assessment and from learning social reasoning abilities to improve social skills

Nusinersen Versus Sham Control In Infantile-Onset Spinal Muscular Atrophy

BACKGROUND & para;& para;Spinal muscular atrophy is an autosomal recessive neuromuscular disorder that is caused by an insufficient level of survival motor neuron (SMN) protein. Nusinersen is an antisense oligonucleotide drug that modifies pre-messenger RNA splicing of the SMN2 gene and thus promotes increased production of full-length SMN protein.& para;& para;METHODS & para;& para;We conducted a randomized, double-blind, sham-controlled, phase 3 efficacy and safety trial of nusinersen in infants with spinal muscular atrophy. The primary end points were a motor-milestone response (defined according to results on the Hammersmith Infant Neurological Examination) and event-free survival (time to death or the use of permanent assisted ventilation). Secondary end points included over all survival and subgroup analyses of event-free survival according to disease duration at screening. Only the first primary end point was tested in a prespecified interim analysis. To control the overall type I error rate at 0.05, a hierarchical testing strategy was used for the second primary end point and the secondary end points in the final analysis.& para;& para;RESULTS & para;& para;In the interim analysis, a significantly higher percentage of infants in the nusinersen group than in the control group had a motor-milestone response (21 of 51 infants [41 %] vs. 0 of 27 [0%], P<0.001), and this result prompted early termination of the trial. In the final analysis, a significantly higher percentage of infants in the nusinersen group than in the control group had a motor-milestone response (37 of 73 infants [51%] vs. 0 of 37 [0%]), and the likelihood of event-free survival was higher in the nusinersen group than in the control group (hazard ratio for death or the use of permanent assisted ventilation, 0.53; P=0.005). The likelihood of overall survival was higher in the nusinersen group than in the control group (hazard ratio for death, 0.37; P=0.004), and infants with a shorter disease duration at screening were more likely than those with a longer disease duration to benefit from nusinersen. The incidence and severity of adverse events were similar in the two groups.& para;& para;CONCLUSIONS & para;& para;Among infants with spinal muscular atrophy, those who received nusinersen were more likely to be alive and have improvements in motor function than those in the control group. Early treatment may be necessary to maximize the benefit of the drug.Wo