No abstract

Behavioral and emotional disturbance was assessed in 70 children and adolescents with Williams syndrome. They were compared with an epidemiological control population, which was statistically controlled for age, gender, and level of mental retardation. Those with Williams syndrome were more likely to be diagnosed as suffering psychiatric disorder. The disorder was characterized by anxiety, hyperactivity, preoccupations, and inappropriate interpersonal relating. Significantly increased rates of other individual symptoms were also found, including sleep disturbance and hyperacusis. These results, considered with earlier findings, suggest that there is a valid behavior phenotype of Williams syndrome. This is frequently associated with sufficient impairment to consider inclusion of the behavior phenotype in future official taxonomies of mental disorders.

PurposePeople with intellectual disability (ID) experience high rates of physical and mental health problems, while access to appropriate healthcare is often poor. This cohort was established to develop an epidemiological profile related to the health, health service use, disability services, mortality and corrective services records of people with ID.ParticipantsThe cohort contains 92 542 people with ID (40% females) with a median age of 23 years (IQR: 12–43 years) and 2 004 475 people with a neuropsychiatric or developmental disorder diagnosis (50% females) with a median age of 51 years (IQR: 29–73 years) from New South Wales, Australia. The whole sample contains records for 2 097 017 individuals with most data sets spanning financial years 1 July 2001 to 30 June 2016. A wide range of data from linked population data sets are included in the areas of disability, health, corrective services and targeted specialist support services in public schools, Public Guardian and Ombudsman services.Findings to dateThis study includes one of the largest cohorts of people with ID internationally. Our data have shown that the presence of ID is significantly associated with emergency department presentations and psychiatric readmissions after the first psychiatric admission based on a subcohort of people with a psychiatric admission. Adults with ID experience premature mortality and over-representation of potentially avoidable deaths compared with the general population.Future plansWithin the health service system, we will examine different components, that is, inpatient, emergency adult services, children and younger people services and costs associated with healthcare as well as mortality, cause and predictors of death. The neuropsychiatric and developmental disorders comparison cohort allows comparisons of the physical health, mental health and service use profiles of people with ID and those with other neuropsychiatric disorders.

Our findings indicate excess mortality of people with an intellectual disability when compared to the general population. They also indicate that females with an intellectual disability have a higher relative mortality compared to female same-aged general population peers, and also males with an intellectual disability, a finding replicated in other similar cohort studies. For international comparison using the WHO standard population, our intellectual disability cohort (ages 5-69 years) had a mortality of 4.04 deaths per 1000.