Low threshold to Vestibular and Oral Sensory stimuli might affect quality of sleep among children with autism spectrum disorder.

AIMS: The current study aimed to validate the relationship between sensory characteristics and sleep dynamics among children with autism spectrum disorder (ASD) using an actigraph, which is an objective assessment device used for sleep monitoring. METHODS: A total of 40 children (age range, 3-6 years) participated in this study (n = 20, with ASD and n = 20, age-matched children with typical development [TD]). We examined sleep dynamics using actigraph for 7 consecutive days, and the relationship between sleep parameters and sensory characteristics was analyzed using the Japanese Version of Sensory Profile (SP-J). RESULTS: Significant differences were observed in terms of activities per minute during sleep (p = 0.02), sleep efficiency (SE) (p = 0.005), and wake after sleep onset (WASO) (p = 0.02) between the two groups. In the ASD group, significant positive correlations were observed between activities per minute during sleep and low thresholds for Vestibular Sensory stimuli (p = 0.046) and Oral Sensory stimuli (p = 0.006) using the SP-J. Based on a multiple regression analysis, the activities per minute during sleep were associated with low thresholds for Oral Sensory stimuli (ß = 0.51, t = 2.29, p = 0.03), but not with other factors, in the ASD group. CONCLUSIONS: The current study showed that atypical Vestibular and Oral Sensory modulation may be a risk indicator for high activities during sleep among preschool children with ASD. Thus, whether the interventions for these sensory characteristics are effective in improving sleep quality, daytime activities, behaviors, and cognitive functions in this group of children must be considered.

Long-Term Evaluation of Low-Dose Betamethasone for Ataxia Telangiectasia.

BACKGROUND: Ataxia telangiectasia is an autosomal recessive disorder characterized by cerebellar ataxia, telangiectases, immune defects, and a predisposition to malignancy. Quality of life is severely impaired by neurological symptoms. However, curative options for the neurological symptoms are limited. Recent studies have demonstrated short-term improvement in neurological symptoms with betamethasone therapy. However, the long-term and adverse effects of betamethasone are unclear. The aim of this study was to evaluate the long-term effects, benefits, and adverse effects of low-dose betamethasone in ataxia telangiectasia. METHODS: Six patients with ataxia telangiectasia received betamethasone at 0.02 mg/kg/day for two years. After cessation of betamethasone, the patients were observed for two additional years. Neurological assessments were performed, and adverse effects were monitored every three months throughout the four-year study period. RESULTS: Transient improvement of neurological symptom was observed in five of the six patients. However, after two years betamethasone treatment, only one of the six patients showed a slight improvement in the neurological score, one patient showed no change, and the neurological scores of the remaining four patients deteriorated. After the cessation of betamethasone treatment, neurological symptoms worsened in all patients. As an adverse effect of betamethasone, transient adrenal dysfunction was observed in all cases. CONCLUSIONS: Although these findings are in agreement with previous studies suggesting that short-term betamethasone treatment transiently benefits patients with ataxia telangiectasia, the long-term benefits and risks should be carefully considered.