The second questionnaire survey for Rett's Syndrome guardian:

use of Rett Syndrome Behaviour Questionnaire (RSBQ)

Abstract

Based on the Rett Syndrome Behaviour Questionnaire (RSBQ), the total score for all items and the total points for each area can be obtained for the 45 question items given to caregivers who responded either “existent," “occasionally" or “absent." The goal of this study was to clarify the characteristics of the results of RSBQ for Rett Syndrome children (persons) in Japan and to define the correlation between their age, intellectual development and motor functions. The subjects were a total of 194 families of Japan Rett Syndrome Association members and Rett Syndrome support organization members and a self-administered questionnaire was used. Information from 72 persons was collected and 70 or more persons responded to each of the question items in the RSBQ. 70% or more of the children (persons) responded either “existent" or “occasionally" to all of the 6 items under the “hand movement" area. There was found to be significant correlation between the total points in the “hand movement" area and intellectual development, accurately reflecting the symptoms and characteristics of Rett Syndrome. Thus, the potential for this method to be employed in the evaluation for rehabilitation and educational support as well as to be used as one indicator for efficacy determination was suggested. On the other hand, 60% or more of the children (persons) responded primarily “absent" to the “nighttime behavior" area, indicating that there are some items and areas requiring a review with regard to their connection with Rett Syndrome symptoms.