Behavioral Intervention Improves Social Communication Skills in Infants with Tuberous Sclerosis Complex

Saturday, May 14, 2016: 11:30 AM-1:30 PM
Hall A (Baltimore Convention Center)
A. Gulsrud1, S. S. Jeste2, N. Tu3, S. Huberty4, C. K. McCracken5 and C. Kasari3, (1)UCLA Semel Institute for Neuroscience & Human Behavior, Los Angeles, CA, (2)Semel Institute for Neuroscience and Human Behavior, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, (3)University of California Los Angeles, Los Angeles, CA, (4)Psychiatry and Biobehavioral Sciences, UCLA Center for Autism Research and Treatment, Los Angeles, CA, (5)University of California, Los Angeles, El Segundo, CA
Background: Tuberous Sclerosis Complex (TSC) is an autosomal dominant disorder strongly associated with cognitive impairment, behavioral disturbances, and autism spectrum disorders (ASD), with rates of ASD ranging from 30-60% (Jeste, 2008). Young children with TSC demonstrate specific delays in nonverbal cognition, visual attention and social communication, making these important targets for intervention (Jeste, 2014). Despite this developmental profile, no studies have investigated the efficacy of behavioral interventions in this population. The current pilot study integrates infants and toddlers with TSC ages 12-36 months into an existing early intervention study of the Joint Attention, Symbolic Play, Engagement and Regulation (JASPER), which targets social communication function in infants and toddlers at high risk for ASD (NIMH 2P50HD055784-06 PI: Kasari). The JASPER intervention (a) targets the foundations of social-communication (joint attention, imitation, play), (b) uses naturalistic strategies to increase the rate and complexity of social-communication and (c) includes parents as implementers of the intervention to promote generalization across settings and activities and to ensure maintenance over time.

Objectives: To examine the effects of the experimental intervention (JASPER) on the primary outcome of joint engagement and secondary outcome of parental strategy use in TSC infant-toddlers compared to IQ matched non-TSC infant-toddlers at risk for ASD.

Methods: Participants included five infant-toddlers with TSC and five IQ matched non-TSC infant-toddlers at-risk for ASD. Infant-toddlers were approximately 19 months old, with the TSC group consisting of slightly older children. Average IQ was 75.4 (sd=24.1) and 70.2 (sd=17.1), respectively for TSC and non-TSC children; t(8)=-.39, p=.70. All children received the experimental JASPER intervention. Parents and children came to UCLA twice per week for 2.5 hours per session across the 8 weeks. A 10 minute parent- child play interaction was videotaped prior to and immediately following an 8 week intervention. The total time in mutually shared play routines and parent use of JASPER strategies were coded.

Results: A paired samples t-test revealed that the TSC group increased in total time spent in jointly engaged play routines (t(4)=-3.5, p=.03) from pre to post treatment. Similarly parents improved in their implementation of the JASPER intervention by increasing their use of joint attention gesturing (t(4)=-3.6, p=.02) and the quality of their play routines (t(4)=-4.3, p=.01) across the intervention period. The non-TSC group showed similar findings in increased time in play routines (t(4)=-2.6, p=.06), and parents use of joint attention (t(4)=-5.0, p=.01), and overall quality of play routine (t(4)=-9.5, p=.001).  The pre to post intervention change was in the same order magnitude for the TSC group compared to the non-TSC group at-risk for ASD.

Conclusions: This study provides preliminary evidence for the effectiveness of a targeted social-communication intervention for young children with TSC. Similar to children at-risk for ASD, children with TSC increased their time spent jointly engaged in play with their parents and parents were able to improve their use of social communication strategies. Replication with a larger sample sizes is underway that investigates both behavioral and biological markers of treatment outcome.