The relationship between challenging behaviours, mood and interest/pleasure in adults with severe and profound intellectual disabilities.

BACKGROUND: We investigated whether current mood and interest/pleasure ratings in adults with moderate to profound intellectual disabilities were predictive of challenging behaviour [self-injurious behaviour (SIB), aggressive/destructive behaviour and stereotypic behaviour] and vice versa. METHOD: In this combined cross-sectional and longitudinal study, staff members of a Hungarian residential facility completed translated versions of the Behaviour Problems Inventory-Short Form (BPI-S), the Challenging Behaviour Interview (CBI) and the Mood, Interest and Pleasure Questionnaire-Short Form (MIPQ-S) for 50 participants at two time points, approximately 4 to 5 months apart. RESULTS: Bivariate correlations from data concurrently assessed at Time-1 showed significant linear relationships between the SIB (both frequency and severity scores) and Interest/Pleasure sub-scales, and the Aggressive/Destructive Behaviour (severity scores) and the MIPQ-S Mood sub-scales (unadjusted for multiple correlations). All of these effects were found with the BPI-S data, but not with the CBI. Multiple regression analyses revealed that (1) low interest/pleasure assessed at Time-1 predicted high SIB (frequency and severity) at Time-2. (2) Interest/pleasure was not predictive of aggressive or stereotypic behaviour. (3) Mood at Time-1 did not predict any of the three types of behaviour problems at Time-2. (4) In reverse, high SIB (frequency and severity) at Time-1 predicted low interest/pleasure ratings at Time-2. (5) Surprisingly, frequent aggressive/destructive behaviour predicted high interest/pleasure. (6) Stereotypic behaviour scores at Time-1 did not predict interest/pleasure ratings at Time-2. Again, all of these effects were only found with the BPI-S data, but not with the CBI. Internal consistency, test-retest reliability and concurrent validity of the Hungarian versions of all three questionnaires had generally satisfactory outcomes. DISCUSSION: The fact that increasingly frequent and severe SIB was predicted by declining measures of interest/pleasure is consistent with previous studies. Contrary to those earlier studies, however, we found that SIB was not predicted by mood and that aggressive/destructive behaviour actually predicted future elevated mood. Implications for future research regarding the directional relationship between affective states such as mood and interest and pleasure, on the one hand, and challenging behaviour, on the other, were discussed.

Neurodevelopmental outcomes in children after the fontan operation.

BACKGROUND: Previous studies of patients after the Fontan operation have reported IQ scores lower than population norms. In the past decade, changes have occurred both in surgical methods used and in the patient population undergoing Fontan palliation. The present study examined the impact of these changes on neurodevelopmental outcomes after Fontan. METHODS AND RESULTS: Neuropsychological tests were administered to 27 five-year-old children after Fontan. Mean age at repair was 2 years 4 months. The present sample was compared with an earlier Fontan group (EFG) of 133 patients who underwent surgery in the 1970s and 1980s. Mean age at repair for the EFG was 7 years 3 months. Compared with EFG, the present study sample was younger at Fontan (P=0.0001) and more likely to have undergone a Norwood procedure (P=0.02), a pre-Fontan bidirectional cavopulmonary anastomosis (P<0.001), and Fontan fenestration (P=0.001). Although mean full-scale, verbal, and performance IQ scores were within 1 SD (15 points) of the population mean of 100 (93+/-16, 95+/-15, and 91+/-17, respectively), mean full-scale and performance IQ scores were significantly lower than this population mean (P=0.03 and P=0.01, respectively). CONCLUSIONS: Compared with a historical cohort of Fontan patients from this institution, a staged approach to Fontan earlier in life is not detrimental to neurodevelopmental outcome. Neurodevelopmental outcomes in children after Fontan are in the normal range, but performance remains lower than the general population.

Developmental outcome after surgical versus interventional closure of secundum atrial septal defect in children.

BACKGROUND: The assessment of the impact of cardiopulmonary bypass (CPB) on developmental outcomes in children who undergo open heart surgery is hampered by the absence of a suitable comparison group. The development of interventional catheterization techniques for the repair of certain types of congenital heart lesions provides the opportunity to study children who have not been exposed to CPB. METHODS AND RESULTS: We performed standardized neuropsychological testing on children after closure of a secundum atrial septal defect through the use of surgery (n=26) or a transcatheter device (n=19). Device patients, compared with surgical patients, were similar in age at defect closure (mean, 6 years) but older at follow-up testing (12.3 versus 10.6 years). The mean weight percentile at closure was greater and the defect size was smaller in the device patients. Families of device patients tended to have a higher parent IQ, higher level of maternal education, and higher level of maternal occupation. In general, however, children's IQ and achievement scores were in the normal range for both groups. In regression analyses with adjustment for age at testing and parent IQ, surgical repair was associated with a 9.5-point deficit in Full-Scale IQ (P=0. 03) and a 9.7-point deficit in Performance IQ (P=0.05). Block Design was the IQ subtest on which treatment groups differed the most (P=0. 01). Surgical patients achieved significantly better scores on errors of commission (P=0.05) and attentiveness index (P=0.03) on a continuous performance test of attention. Scores on tests of achievement and other neuropsychological domains did not differ significantly between the groups. Regression analyses within the surgical group failed to identify significant CPB-related risk factors. CONCLUSIONS: A prospective randomized trial or a study that includes prerepair and postrepair assessments is necessary to establish whether the observed advantages of device closure in neuropsychological outcome represent deleterious effects of CPB or a methodological artifact.