The Japanese journal of neuropsychology

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ArticleTitle	Caregiver questionnaire assessing behavioral symptoms in Prader-Willi syndrome: A validation study
Language	J
AuthorList	Mayumi Shinohara¹⁾⁵⁾, Kaeko Ogura²⁾, Toshikatu Fujii¹⁾, Kazumi Hirayama¹⁾³⁾, Kousaku Ohno⁴⁾, Etsuro Mori¹⁾
Affiliation	¹⁾Department of Behavioral Neurology and Cognitive Neuroscience, Tohoku University Graduate School of Medicine ²⁾Research Institute, National Rehabilitation Center for Persons with Disabilities ³⁾Department of Occupational Therapy, Yamagata Prefectural University of Health Sciences ⁴⁾Division of Child Neurology, Faculty of Medicine, Tottori University ⁵⁾Department of Nursing, Sendai Seiyo Gakuin College
Publication	Japanese Journal of Neuropsychology: 27 (1), 54-63, 2011
Received	Apr 24, 2009
Accepted	Mar 15, 2010
Abstract	Prader-Willi syndrome (PWS) is a genetically determined neurodevelopmental disorder presenting peculiar behavioral problems including hyperphagia, uncontrollable impulse, stereotype behavior and collecting behavior. The behavioral problems in individuals with PWS are similar to the characteristic symptoms of patients with pathologies affecting the orbitofrontal cortex (OFC). We developed a questionnaire designed for postal administration to caregivers, which was a modified version of three standardized instruments assessing behavioral derangement in patients with OFC dysfunction including frontotemporal dementia. This inventory was used for a study that was carried out as a nation-wide survey to examine the nature of such abnormal behaviors in individual with PWS, which has been reported elsewhere. In the present study, we examine the validity of the questionnaire by comparing it with the results of structured interviews. Thirty-four key caregivers of individuals with PWS were asked to fill out the mailed questionnaire. Then a pediatric behavioral neurologist interviewed them within 4-week interval with the structured-interview version of the questionnaire. There were no statistically significant differences in the symptom-domains between the results of the structured interview and those of the questionnaire in terms of prevalence, frequency, and severity. Frequency scores in all domains were highly correlated, and severity scores in all domains except for roaming were highly correlated between the two measures. The low correlation in the roaming domain was likely to attributable to the low prevalence of this symptom in PWS. Thus, the present caregiver questionnaire is equivalent to structured interview, and is a valid tool for assessing behavioral problems in PWS.
Keywords	Prader-Willi syndrome, postal questionnaire, structured interview, concurrent validity