Development of a Video-based Evaluation Tool in Rett Syndrome

Abstract

This paper describes the development of a video-based evaluation tool for use in Rett syndrome (RTT). Components include a parent-report checklist, and video filming and coding protocols that contain items on eating, drinking, communication, hand function and movements, personal care and mobility. Ninety-seven of the 169 families who initially agreed to participate returned a videotape within 8 months of the first request. Subjects whose videos were returned had a similar age profile to those who did not provide a video but were more likely to have classical than atypical RTT. Evidence of the content and social validity and inter-rater reliability on 11 videos is provided. Video may provide detailed, objective assessment of function and behaviour in RTT.

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Acknowledgments

The authors would like to acknowledge the National Medical and Health Research Council (NHMRC) for its funding of this particular project (303189) as well as the NIH for its current funding of the ARSD under NIH grant number R01 HD43100. HL is funded by NHMRC program grant 353514 and WEK by the NIH program grant P01 HD24448. We would like to express our gratitude to the families and in particular the Faydi family for their contribution to the filming and piloting of the video assessment tools; the Australian Paediatric Surveillance Unit and the Rett Syndrome Association of Australia who facilitated case ascertainment in Australia. We would also like to thank Bradley Martin, Andrew Leece, Sarah Ager, Zoran Gacik, Sarah Love, Alison Anderson, Christiane Cox and Cecile O’Connor for their contribution to the study. The parent-report checklist, filming and coding protocols are available on request from Dr Helen Leonard.

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Fyfe, S., Downs, J., McIlroy, O. et al. Development of a Video-based Evaluation Tool in Rett Syndrome. J Autism Dev Disord 37, 1636–1646 (2007). https://doi.org/10.1007/s10803-006-0293-9

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Keywords

  • Rett syndrome
  • Video analysis
  • Functional ability
  • Movement disorder
  • Behavioural phenotype
  • Participatory research