Issues in Estimating Developmental Level and Cognitive Function in Rett Syndrome

Research output: Chapter in Book/Report/Conference proceedingChapter

4 Scopus citations


Severe deficits in communication and cognitive ability are among the necessary diagnostic criteria for Rett syndrome (RTT), a progressive neurodevelopmental disorder that affects almost exclusively females. Traditional assessments, which rely on motor and verbal responding to assess comprehension and cognition, may not accurately reflect functioning in this population. To date, however, there are no comprehensive, systematic reviews of the literature regarding estimates of developmental and cognitive functioning in RTT. The purpose of this paper is to review the literature regarding functioning in comprehension, cognition, and adaptive behavior with a specific focus on the limitations of existing assessment approaches. Suggestions for future research are also discussed.

Original languageEnglish (US)
Title of host publicationInternational Review of Research in Developmental Disabilities
PublisherAcademic Press Inc.
Number of pages39
StatePublished - 2012

Publication series

NameInternational Review of Research in Developmental Disabilities
ISSN (Print)2211-6095

Bibliographical note

Funding Information:
This work was supported, in part, by Eunice Kennedy Shriver NICHD Grant No. 44763 and 47201. We are grateful for the support of the Rett Syndrome Clinic at Gillette Children’s Specialty Healthcare and the Minnesota Rett Syndrome Research Association.


  • Adaptive behavior
  • Assessment
  • Cognitive functioning
  • Comprehension
  • Motor impairment
  • Rett syndrome


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