Journal of Autism and Developmental Disorders

, Volume 49, Issue 12, pp 5063–5072 | Cite as

Brief Report: Modest but Clinically Meaningful Effects of Early Behavioral Intervention in Twins with Rett Syndrome—A Case Study

  • Kristine Berg TitlestadEmail author
  • Sigmund Eldevik
Brief Report


A growing evidence base supports early intensive behavioral intervention (EIBI) for young children with autism spectrum disorder. We have found only one study exploring the outcome of EIBI for children with Rett syndrome, which reported little effect. It suggested that future studies should employ more fine-grained outcome measures. We provided EIBI for twin 3-year-old girls with Rett syndrome for a period of 3 years. We analyzed raw scores and standard scores from a measure of adaptive behavior and a detailed assessment of skills across 25 areas. We detected moderate but clinically meaningful gains in areas such as communication and self-help. Gains are discussed from a quality of life perspective and whether the moderate effects can justify the resources required in EIBI.


Rett syndrome Early intensive behavioral intervention Case study Clinical significance 



We thank the girls, the girls’ parents, the staff in the pre-school and the psychologist at the local pedagogical-psychological service that were involved in the behavioral intervention program. A special thanks to our colleges at Department of Autism.

Author Contributions

KBT and SE have made substantial contributions to conception and design, acquisition of data, analysis and interpretation of data. They both have been involved in drafting the manuscript and have given final approval of the version to be published.


This study was not funded. In Norway, where this study took place, the approach Early Behavioral Intervention for Children is publicly funded. All children are enrolled in community-based kindergartens and supervision is delivered by community agencies.

Compliance with Ethical Standards

Conflict of interest

The authors declare that they have no conflict of interest.


  1. Amir, R. E., Van den Veyver, I. B., Wan, M., Tran, C. Q., Francke, U., & Zoghbi, H. Y. (1999). Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2. Nature Genetics,23(2), 185–188. Scholar
  2. Bondy, A., & Frost, L. (2011). A picture’s worth: PECS and other visual communication strategies in autism. Bethesda: Routledge.Google Scholar
  3. Dunn, H. G. (2001). Importance of Rett syndrome in child neurology. Brain and Development,23(Supplement 1), S38–S43. Scholar
  4. Eikeseth, S. (2009). Outcome of comprehensive psycho-educational interventions for young children with autism. Research in Developmental Disabilities,30(1), 158–178. Scholar
  5. Eikeseth, S., Klintwall, L., Hayward, D., & Gale, C. (2015). Stress in parents of children with autism participating in early and intensive behavioral intervention. European Journal of Behavior Analysis,16(1), 112–120. Scholar
  6. Eikeseth, S., Klintwall, L., Jahr, E., & Karlsson, P. (2012). Outcome for children with autism receiving early and intensive behavioral intervention in mainstream preschool and kindergarten settings. Research in Autism Spectrum Disorders,6(2), 829–835. Scholar
  7. Eldevik, S., Jahr, E., Eikeseth, S., Hastings, R. P., & Hughes, C. J. (2010). Cognitive and adaptive behavior outcomes of behavioral intervention for young children with intellectual disability. Behavior Modification,34(1), 16–34. Scholar
  8. Ellaway, C. J., Peat, J., Williams, K., Leonard, H., & Christodoulou, J. (2001). Medium-term open label trial of l-carnitine in Rett syndrome. Brain and Development,23(Suppl 1), S85–S89. Scholar
  9. Fukumizu, M., Hayashi, M., Miyajima, T., Ishizaki, A., Tanaka, H., & Kohyama, J. (2015). A nationwide survey on the uses of melatonin and ramelteon in Japanese children. No to Hattatsu [Brain & Development],47(1), 23–27.Google Scholar
  10. Green, G. (2011). Early intensive behavior analytic intervention for autism spectrum disorders. In E. A. Mayville & J. A. Mulick (Eds.), Behavioral foundations of effective autism treatment (pp. 183–199). Cornwall-on-Hudson: Sloan Publishing.Google Scholar
  11. Hammett, T., Harris, A., Boreham, B., & Mehdian, S. M. (2014). Surgical correction of scoliosis in Rett syndrome: Cord monitoring and complications. European Spine Journal,23(Suppl 1), S72–S75. Scholar
  12. Kerr, A., & Witt Engerström, I. (2001). Rett disorder and the developing brain. Oxford: Oxford University Press.Google Scholar
  13. Kozinetz, C. A., Skender, M. L., Macnaughton, N., Almes, M. J., Schultz, R. J., Percy, A. K., et al. (1993). Epideminology of Rett syndrome—A population-based registry. Pediatrics,91(2), 445–450.PubMedGoogle Scholar
  14. Leonard, H., Bower, C., & English, D. (1997). The prevalence and incidence of Rett syndrome in Australia. European Child and Adolescent Psychiatry,6, 8–10.PubMedGoogle Scholar
  15. Lovaas, O. I. (1987). Behavioral treatment and normal educational and intellectual functioning in young autistic children. Journal of Consulting and Clinical Psychology,55(1), 3. Scholar
  16. Maffei, S., De Felice, C., Cannarile, P., Leoncini, S., Signorini, C., Pecorelli, A., et al. (2014). Effects of omega-3 PUFAs supplementation on myocardial function and oxidative stress markers in typical Rett syndrome. Mediators of Inflammation,2014, 983178. Scholar
  17. Makrygianni, M. K., Gena, A., Katoudi, S., & Galanis, P. (2018). The effectiveness of applied behavior analytic interventions for children with Autism Spectrum Disorder: A meta-analytic study. Research in Autism Spectrum Disorders,51, 18–31. Scholar
  18. Partington, J. (2008). The assessment of basic language and learning skills-revised (the ABLLS-R): An assessment, curriculum guide, and skills tracking system for children with autism or other developmental disabilities. Pleasant Hill: Behavior Analysts Inc.Google Scholar
  19. Reichow, B., Barton, E., Boyd, B., & Hume, K. (2014). Early intensive behavioral intervention (EIBI) for young children with autism spectrum disorders (ASD). Campbell Collaboration, 10. Retrieved from
  20. Reichow, B., Hume, K., Barton, E. E., & Boyd, B. A. (2018). Early intensive behavioral intervention (EIBI) for young children with autism spectrum disorders (ASD). Cochrane Database of Systematic Reviews. Scholar
  21. Schultz, R. J., Glaze, D. G., Patterson, M. C., Firth, H., & Dashe, J. F. (2018). Rett syndrome: Genetics, clinical features, and diagnosis. Retrieved from
  22. Sigafoos, J., Green, V. A., Schlosser, R., O’Eilly, M. F., Lancioni, G. E., Rispoli, M., et al. (2009). Communication intervention in Rett syndrome: A systematic review. Research in Autism Spectrum Disorders,3(2), 304–318. Scholar
  23. Sigafoos, J., Kagohara, D., van der Meer, L., Green, V. A., O’Reilly, M. F., Lancioni, G. E., et al. (2011). Communication assessment for individuals with Rett syndrome: A systematic review. Research in Autism Spectrum Disorders,5(2), 692–700. Scholar
  24. Simacek, J., Reichle, J., & McComas, J. J. (2016). Communication intervention to teach requesting through aided AAC for two learners with Rett syndrome. Journal of Developmental and Physical Disabilities,28(1), 59–81. Scholar
  25. Smith, T., Klevstrand, M., & Lovaas, O. I. (1995). Behavioral treatment of Rett’s disorder: Ineffectiveness in three cases. American Journal of Mental Retardation,100(3), 317–322.PubMedGoogle Scholar
  26. Sparrow, S. S., Cicchetti, D. V., & Balla, D. A. (2005). Vineland Adaptive Behavior Scales, (Vineland™-II) (2nd ed.). San Antonio: PEARSON.Google Scholar
  27. Stasolla, F., De Pace, C., Damiani, R., Di Leone, A., Albano, V., & Perilli, V. (2014). Comparing PECS and VOCA to promote communication opportunities and to reduce stereotyped behaviors by three girls with Rett syndrome. Research in Autism Spectrum Disorders,8(10), 1269–1278. Scholar
  28. Waters, C. F., Amerine Dickens, M., Thurston, S. W., Lu, X., & Smith, T. (2018). Sustainability of early intensive behavioral intervention for children with autism spectrum disorder in a community setting. Behavior Modification. Scholar
  29. Weissman, L., Bridgemohan, C., Augustyn, M., Patterson, M., & Torchia, M. (2018). Autism spectrum disorder in children and adolescents: Overview of management. UpToDate [Internet]. Version, 19.Google Scholar
  30. Wong, K., Leonard, H., Jacoby, P., Ellaway, C., & Downs, J. (2015). The trajectories of sleep disturbances in Rett syndrome. Journal of Sleep Research,24(2), 223–233. Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Welfare and ParticipationWestern Norway University of Applied SciencesBergenNorway
  2. 2.Department of Autism, Pedagogical-Psychological ServicesBergenNorway
  3. 3.Department of Behavioral ScienceOslo Metropolitan UniversityOsloNorway

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