Relationship between serum cortisol levels, stereotypies, and the presence of autism spectrum disorder in patients with severe intellectual disability.

Stereotypies are one of the diagnostic criteria for autism spectrum disorder (ASD) and are common to both ASD and intellectual disability (ID). Previous studies have been inconclusive, with some showing a positive correlation between stereotypies and cortisol, while others have shown a negative correlation. We hypothesised and investigated the presence of ASD as one of the variables involved in this discrepancy. We tested the following hypotheses on serum cortisol in a total of 84 hospitalised patients with severe ID and ASD with severe ID. Hypothesis (1) Higher levels of stereotypies are associated with higher levels of serum cortisol. Hypothesis (2) The presence of ASD will moderate the association between stereotypies and high serum cortisol levels. The results of the analysis supported hypotheses (1) and (2). We also found that in the population with ID, serum cortisol levels were significantly lower in the ASD group compared to the non-ASD group. The present findings that the association between stereotypies and serum cortisol levels in people with severe ID is moderated by the presence of ASD suggest that the stress response system may function differently in people with ID and ASD than in the general population.

Reliability and validity of the Japanese version of the Behavior Problem Inventory-Short Form.

BACKGROUND: The Behavior Problems Inventory-Short Form (BPI-S), which assesses behavior problems in individuals with intellectual disabilities (ID), is a shorter version of the BPI-01. This study investigated the reliability and validity of the BPI-S Japanese version (BPI-S-J) for adolescents/adults with ID and behavior problems. METHODS: The test-retest reliability included participants with ID and behavioral problems who were enrolled in welfare services. For test-retest reliability, 42 caregivers independently responded to the BPI-S-J every two weeks. Inter-rater reliability was independently assessed using the BPI-S-J by two caregivers who were familiar with the 42 participants. The participants of the validity assessment were 227 students from special needs schools or patients with ID admitted to medical institutions. The total frequency total score was compared based on the degree of ID. To examine the criterion-related validity, we analyzed the total frequency score, the total score of the Criteria for Determining Severe Problem Behavior (CDSPB) and the total score of the Aberrant Behavior Checklist-Japanese version (ABC-J). RESULTS: The BPI-S-J of test-retest reliability was satisfactory (intra-class correlation; ICC) = 0.954), and the total score significantly (ICC = 0.721) represented good inter-rater reliability. For the validity, the BPI-S-J score of participants who had severe and profound ID was significantly higher than those who had mild and moderate ID. Significant correlations were observed between the BPI-S-J score and CDSPB score (r = 0.499), and the ABC-J score (r = 0.699), indicating adequate criterion-related validity. CONCLUSION: This study showed the utility of the BPI-S-J to assess behavior problems in the Japanese ID population.

Oxytocin levels and sex differences in autism spectrum disorder with severe intellectual disabilities.

There were few reports of oxytocin (OXT) concentrations of autism spectrum disorder (ASD) patients with severe intellectual disabilities. We measured serum OXT concentrations in 79 hospitalized patients with severe intellectual disabilities (16-60 years old, 50 males and 29 females, 54 ASD patients) and investigated the associations between serum OXT concentration, symptom scores, sex differences, and autism spectrum disorder. There were no significant effects of diagnosis, severity of intellectual disabilities, and total score of the Japanese version of the Aberrant Behavior Checklist (ABC-J), the Childhood Autism Rating Scale-Tokyo Version (CARS-TV), and the Japanese version of the Repetitive Behavior Scale-Revised (RBS-R). However, there were sex differences in the correlations between OXT concentrations and subscale scores in the ASD group. The male ASD group (n = 39) showed negative correlations between RBS-R Self-injurious and Sameness subscale scores and serum OXT concentrations. In the female ASD group(n = 15), CARS-TV Nonverbal communication subscale scores and RBS-R Compulsive subscale scores were seen to positively correlate with serum OXT concentrations. These findings suggest that OXT functions differ in males and females with severe intellectual disabilities and that OXT partly affects autism and related to some of the repetitive behaviors and nonverbal communication, in ASD patients with severe intellectual disabilities.

An Open-Label Uncontrolled Trial of the Efficacy and Safety of a Hug with Singing and Rocking for Promotion of Relaxation in Pediatric Patients with Severe Motor and Intellectual Disabilities: Study Protocol.

Children with severe motor and intellectual disabilities (SMID) are continually exposed to stress due to their need to receive daily care. In particular, chronic physical and acute mental stress derived from daily medical care due to unstable health status are issues specific to SMID children. Therefore, it is important to approach these issues for the maintenance of their lives and quality of life. Seventeen children with a SMIDmedical care dependent group (SMID-MCDG) score of 25 or more will be enrolled in this study. Intervention by a hug while singing and rocking will be performed once a week for 24 weeks. The practitioner will sing, and slowly rock the child back and forth. Primary endpoint is high-frequency component of heart rate variability by frequency analysis. Secondary endpoints are low-frequency/high-frequency components of heart rate variability by frequency analysis, activity of salivary amylase, the incidence of adverse events, and changes in appearance. Frequency analysis of heart beat changes and salivary amylase activity are used as physiological indexes for assessing response to being held while singing and rocking. In this study, we will examine the efficacy and safety of hugging while singing and rocking as a practice of Ryouiku to promote relaxation in SMID-MCDG children.

[Clinical characteristics and pharmacotherapy of extremely disruptive behavior disorders in people with mental retardation].

People with mental retardation often have behavioral problems. Psychotropic medications are used for the treatment of extremely disruptive behavior disorders in many cases. We surveyed the clinical characteristics and the current status of the pharmacotherapy regimens for inpatients with severe intellectual disabilities and behavior disorders at 8 national psychiatric hospitals. Many of them were men between 20 and 40 years and more than half of them had both extremely mental retardation and high motor ability. Antipsychotic drugs and anticonvulsants were frequently used. As they had more severe behavior disorder, they were taken the more psychotropic drugs. Autism was significantly associated and rough behavior was apparently associated with higher doses. A very weak correlation between the total score of extremely disruptive behavior disorders and the number of psychotropic drugs being administered per patient was seen. Use of new medicines, such as atypical antipsychotic drugs, was found about fifteen percent of the cases. In order to establish useful pharmacotherapy regimens for people with severe mental retardation and behavior disorders, it is necessary to perform routine judgment and evaluation of the pharmacotherapy focusing on targeted symptoms. We should also be attention to the quality of life issue. The aim of medical treatment should not be care workers' convenience, but be the improvement of quality of life of patients.

Synergistic effect of valproate coadministration and hypoalbuminemia on the serum-free phenytoin concentration in patients with severe motor and intellectual disabilities.

We investigated whether a combination of risk factors affects the free phenytoin (PHT) fraction by multiple regression analyses in 30 patients with severe motor and intellectual disabilities (SMID) with epilepsy. The risk factors analyzed were gender, age, total PHT concentration, albumin concentration, aspartate aminotransferase, alanin aminotransferase, serum creatinine, blood urea nitrogen, and antiepileptic drug concentrations. Serum levels of total and free PHT were measured by fluorescence polarization immunoassay. Free PHT fractions were between 7.2% and 17.3% (average 10.9%). Two factors, hypoalbuminemia and valproate (VPA) coadministratation with PHT, increased free PHT fraction, and a combination of these two markedly increased free PHT fraction. Patients with these double risk factors have a high risk of exceeding the therapeutic range of serum-free PHT concentration even if their total PHT concentration does not. Therefore, we should monitor free PHT concentration, especially in SMID patients with epilepsy, because they may have hypoalbuminemia and are treated with antiepileptic drug polytherapy and, moreover, cannot report adverse effects of the drugs.