An exploratory study on emotion regulation strategy use in individuals with Williams syndrome, autism spectrum disorder and intellectual disability.

Background: Individuals with neurodevelopmental disorders often have atypical emotion profiles, but little is known about how they regulate their emotions. While several studies have examined emotion regulation strategy use in autism spectrum disorder (ASD), only a few have included individuals with intellectual disability (ID) or focused on specific syndromes such as Williams syndrome (WS). Methods: A parent-reported survey launched during the first months of the COVID-19 pandemic allowed to exploratorily study emotion regulation strategy use and its link to anxiety in individuals with ASD with (N=785) and without ID (N=596), WS (N=261), and Intellectual Disability not otherwise specified (N=649). Results: Using multilevel analyses, besides revealing specific group differences in emotion regulation strategy use, a variety of strategies (e.g., rumination, avoiding information, repetitive behaviors) were found to be linked to elevated levels of anxiety, while focusing on the positive was linked to lower anxiety levels in all groups. Moreover, only autistic people without ID used humor more frequently while experiencing lower anxiety levels. Conclusion: This study sheds light on an underexplored area of emotion regulation strategy use in different neurodevelopmental disorders. It also paves the way to further examine emotion regulation in more rigorous ways to better understand emotion regulation in different neurodevelopmental disorders as well as the impact on outcome measures such as anxiety. This exploratory study may help to develop and validate adequate measures to study a broad array of ER strategies used by individuals with neurodevelopmental disorders.

Psychotic Symptoms and Malignant Neuroleptic Syndrome in Williams Syndrome: A Case Report.

Background: Somatic and mental comorbidities are characteristic of individuals with Williams syndrome. The psychiatric profile of these patients mainly comprises affective disorders, while psychotic symptoms are rare. Methods: We present a case report of psychosis and malignant neuroleptic syndrome in a patient with Williams syndrome. We also conduct a review of recent works on the topic. Case Presentation: A 38-year-old Caucasian male with Williams syndrome presented with somatic delusions, previously experiencing severe anxiety and concerns about a headache. The patient was prescribed olanzapine, which did not, however, have any effect on the delusions. After switching to lurasidone, the patient presented with malignant neuroleptic syndrome (muscle rigidity, tremor, urinary retention, fluctuating level of consciousness). He was hospitalized and the antipsychotic medication was discontinued. After somatic recovery, the patient did not experience severe anxiety and the somatic delusions diminished notably. The patient was discharged from the hospital in a stable physical condition, albeit still with transient worries about his health condition. Conclusions: We present a case of the coincidence of Williams syndrome and psychosis. We hypothesize on the possible pathological relationships between the onset of the psychosis and severe anxiety in an individual with Williams syndrome. This case report duly contributes to the limited literature on psychiatric comorbidity in Williams syndrome.

Online and Face-to-Face Performance on Two Cognitive Tasks in Children With Williams Syndrome.

There has been an increase in cognitive assessment via the Internet, especially since the coronavirus disease 2019 surged the need for remote psychological assessment. This is the first study to investigate the appropriability of conducting cognitive assessments online with children with a neurodevelopmental condition and intellectual disability, namely, Williams syndrome. This study compared Raven's Colored Progressive Matrices (RCPM) and British Picture Vocabulary Scale (BPVS) scores from two different groups of children with WS age 10-11 years who were assessed online (n = 14) or face-to-face (RCPM n = 12; BPVS n = 24). Bayesian t-tests showed that children's RCPM scores were similar across testing conditions, but suggested BPVS scores were higher for participants assessed online. The differences between task protocols are discussed in line with these findings, as well as the implications for neurodevelopmental research.

Acute aortic dissection in a patient with Williams syndrome infected by COVID-19.

Cardiovascular involvement in COVID-19 has different features. Here we report the ominous fate of a neglected adolescent with Williams syndrome that was infected by SARS-CoV-2 and ended by acute aortic dissection.