Behaviour disorders in children with an intellectual disability.

Behaviour disorders are frequent in children with an intellectual disability, regardless of the underlying etiology. They are often disabling, and can create problems in everyday life and can mask, or reveal, an organic or psychiatric illness. Such behaviours are often chronic and more than one may be present in the same individual. This is further complicated by the fact that parents often do not seek help for the problem, perhaps believing that it is due to the child's disability and cannot be treated. The present review describes some general concepts dealing with the management of behaviour disorders commonly seen in children and youth with an intellectual disability, and gives a high level overview of behaviours commonly problematic in this patient population including sleep disturbances, agitated and aggressive behaviours, and self-injury behaviour. In general, while pharmacological treatment is possible, behavioural intervention is a more effective and better tolerated form of treatment.

Les troubles de comportement sont fréquents chez les enfants ayant une déficience intellectuelle, quelle que soit leur étiologie sous-jacente. Ils sont souvent invalidants, peuvent créer des problèmes dans la vie quotidienne et masquer ou révéler une maladie organique ou psychiatrique. Ces comportements sont souvent chroniques et peuvent être multiples chez la même personne. Ils sont compliqués davantage par le fait que, souvent, les parents ne demandent pas d'aide, croyant peut-être qu'ils sont causés par la déficience de l'enfant et qu'ils ne peuvent pas être traités. La présente analyse décrit certains concepts généraux sur la prise en charge des troubles de comportement souvent observés chez les enfants et les adolescents ayant une déficience intellectuelle et donne un survol des troubles de comportement souvent problématiques au sein de cette population de patients, y compris les troubles du sommeil, les comportements agités et agressifs et l'automutilation. En général, le traitement pharmacologique est possible, mais l'intervention comportementale est plus efficace et mieux tolérée.

Impact of electronic health records on predefined safety outcomes in patients admitted to hospital: a scoping review.

OBJECTIVES: Review available evidence for impact of electronic health records (EHRs) on predefined patient safety outcomes in interventional studies to identify gaps in current knowledge and design interventions for future research. DESIGN: Scoping review to map existing evidence and identify gaps for future research. DATA SOURCES: PubMed, the Cochrane Library, EMBASE, Trial registers. STUDY SELECTION: Eligibility criteria: We conducted a scoping review of bibliographic databases and the grey literature of randomised and non-randomised trials describing interventions targeting a list of fourteen predefined areas of safety. The search was limited to manuscripts published between January 2008 and December 2018 of studies in adult inpatient settings and complemented by a targeted search for studies using a sample of EHR vendors. Studies were categorised according to methodology, intervention characteristics and safety outcome.Results from identified studies were grouped around common themes of safety measures. RESULTS: The search yielded 583 articles of which 24 articles were included. The identified studies were largely from US academic medical centres, heterogeneous in study conduct, definitions, treatment protocols and study outcome reporting. Of the 24 included studies effective safety themes included medication reconciliation, decision support for prescribing medications, communication between teams, infection prevention and measures of EHR-specific harm. Heterogeneity of the interventions and study characteristics precluded a systematic meta-analysis. Most studies reported process measures and not patient-level safety outcomes: We found no or limited evidence in 13 of 14 predefined safety areas, with good evidence limited to medication safety. CONCLUSIONS: Published evidence for EHR impact on safety outcomes from interventional studies is limited and does not permit firm conclusions regarding the full safety impact of EHRs or support recommendations about ideal design features. The review highlights the need for greater transparency in quality assurance of existing EHRs and further research into suitable metrics and study designs.

ODD irritability is associated with obsessive-compulsive behavior and not ADHD in chronic tic disorders.

Gilles de la Tourette syndrome (TS) and chronic tic disorder (CT) are often associated with a variety of behavioral comorbidities including attention-deficit hyperactivity disorder (ADHD), obsessive-compulsive behavior (OCB), oppositional-defiant disorder (ODD) and temper outbursts. ODD is often associated with ADHD but its links to other symptoms of TS/CT is not as clear. This study examined whether the various symptoms of ODD were differentially linked to the various comorbidities in TS. A clinical sample of 135 children diagnosed with TS was evaluated through parent questionnaires and semi-structured interviews. Regressions and structural equation modeling confirmed that ODD is multidimensional in a TS/CT sample and showed that OCB was associated with the irritability symptoms of ODD whereas ADHD was associated with the Headstrong symptoms of ODD. Results suggest that increased attention to the different facets of ODD may help improve our understanding of emotional symptoms in TS/CT.

Association of intronic variants of the BTBD9 gene with Tourette syndrome.

OBJECTIVE: To test the association between Tourette syndrome (TS) and genetic variants in genomic loci MEIS1, MAP2K5/LBXCOR1, and BTBD9, for which genome-wide association studies in restless legs syndrome and periodic limb movements during sleep revealed common risk variants. DESIGN: Case-control association study. SETTING: Movement disorder clinic in Montreal. Subjects We typed 14 single-nucleotide polymorphisms spanning the 3 genomic loci in 298 TS trios, 322 TS cases (including 298 probands from the cohort of TS trios), and 290 control subjects. MAIN OUTCOME MEASURES: Clinical diagnosis of TS, obsessive-compulsive disorder, and attention-deficit disorder. RESULTS: The study provided 3 single-nucleotide polymorphisms within BTBD9 associated with TS (chi(2) = 8.02 [P = .005] for rs9357271), with the risk alleles for restless legs syndrome and periodic limb movements during sleep overrepresented in the TS cohort. We stratified our group of patients with TS according to presence or absence of obsessive-compulsive disorder and/or attention-deficit disorder and found that variants in BTBD9 were strongly associated with TS without obsessive-compulsive disorder (chi(2) = 12.95 [P < .001] for rs9357271). Furthermore, allele frequency of rs9357271 inversely correlated with severity of obsessive-compulsive disorder as measured by the Yale-Brown Obsessive Compulsive Scale score. CONCLUSION: Variants in BTBD9 that predispose to restless legs syndrome and periodic limb movements during sleep are also associated with TS, particularly TS without obsessive-compulsive disorder.