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

    Authors

    M. A. Sugarman; D. R. Jacobs; D. Paulson; M. M. Tomlinson;P. A. Lichtenberg

    Comments

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    Abbreviated Journal Title

    J. Intellect. Dev. Dis.

    Keywords

    Prader-Willi syndrome; neuropsychological testing; cognitive abilities; Dementia Rating Scale; adults with intellectual disability; ALZHEIMERS-DISEASE; MENTAL-RETARDATION; SCHIZOPHRENIA; INDIVIDUALS; DECLINE; Education, Special; Rehabilitation

    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.

    Journal Title

    Journal of Intellectual & Developmental Disability

    Volume

    39

    Issue/Number

    1

    Publication Date

    1-1-2014

    Document Type

    Article

    Language

    English

    First Page

    102

    Last Page

    107

    WOS Identifier

    WOS:000331057400011

    ISSN

    1366-8250

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