Examination of the cognitive profile of adults with Prader–Willi syndrome using the Dementia Rating Scale-2: A pilot study

Abstract

Background The goal of the current study was to determine if the Dementia Rating Scale-2 (DRS-2) is an appropriate tool for evaluating cognitive abilities in adults with Prader–Willi syndrome (PWS). The DRS-2 is a screening measure previously utilised in low-functioning populations with and without dementia, including individuals with intellectual disability.

Method Thirteen adults with PWS between 21 and 59 years old were administered the DRS-2.

Results Participants demonstrated impairment on all 5 subscales, with the largest impairments observed on the Initiation/Perseveration and Construction subscales. No significant floor or ceiling effects were observed. No correlations were observed between age and any subscale score.

Conclusion The DRS-2 may be a valid assessment tool for capturing the full spectrum of cognitive functioning in adults with PWS. The largest impairments were observed on items requiring verbal and motor fluency and visuo-spatial processing. Future directions for the use of the DRS-2 in this population are discussed.

Keywords:

• Prader–Willi syndrome
• neuropsychological testing
• cognitive abilities
• Dementia Rating Scale
• adults with intellectual disability

Acknowledgements

We would like to thank the staff of Hope Network who supported our work and the consumers of Hope Network who participated. We would also like to recognise the Prader–Willi Syndrome Association of Michigan for their support.

Funding

This research was supported by the Hope Institute for Research, US.

Notes

*This manuscript was accepted under the Editorship of Ian Dempsey.