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Introduction Wolfram Syndrome (WFS) is a rare genetic disease associated with a variety of progressive metabolic and neurologic impairments. Previous research has focused on WFS-related impairments and biomarkers for disease progression; however, information about how WFS impacts participation in daily activities is lacking. Methods WFS (n=45; 20 children, 25 adults) participants completed an online questionnaire about activity participation. Thirty-six non-WFS comparison participants (11 children; 25 adults) completed a portion of the questionnaire. Symptom data from a subset of WFS participants (n=20) were also examined in relation to participation data. Results WFS children and adults had lower participation than non-WFS children and adults in almost all activity domains, and social and exercise-related activities were the most problematic. In the subset of WFS adults with symptom data, poorer vision, balance, gait, hearing, and overall symptom severity related to lower participation. Conclusions WFS appears to negatively impact participation in a variety of activities, and this effect may increase as people age and/or WFS progresses. The most functionally-pertinent WFS symptoms are those associated with neurodegeneration especially vision loss and walking and balance problems. This study revealed symptoms and activity domains that are most relevant for people with WFS and, thus, can inform current practice and treatment development research.

Background: The course on cognitive assessment is mandatory for all school psychology programs. However, there have been no published studies that have explored learning objectives for this course. Objective: The purpose of this study is to examine content covered and complexity of learning objectives in the cognitive assessment course. Method: Five raters coded 90 syllabi from school psychology cognitive assessment courses across the United States on a sample of six American Psychological Association assessment-related standards and on complexity using the six Bloom’s Taxonomy levels. Results: The majority of objectives were addressing lower levels of complexity as measured by Bloom’s Taxonomy with few syllabi including learning objectives addressing a wide range of levels. Based on the analysis of objectives by the sample of Standards, learning objectives most frequently addressed administration accuracy and test selection, whereas integrating results and linking to recommendations were often missing. Conclusion: The learning objectives included in syllabi ranged in quantity, complexity, and content but focused on lower complexity skills. Learning objectives in cognitive assessment courses do not reflect the diverse assessment-related practice demands of school psychologists. Teaching Implications: To align training with future practice, instructors are advised to review learning objectives with attention to complexity and content standards.

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Date Presented 4/21/2018 Wolfram syndrome (WFS) is a rare progressive genetic disease with metabolic and neurological impairments. We found that WFS negatively affected activity participation and that this effect may increase as WFS progresses. The most salient WFS symptoms may be those associated with neurodegeneration. Primary Author and Speaker: Emily Bumpus Additional Authors and Speakers: Erin Foster Contributing Authors: Tamara Hershey, Tasha Doty, Samantha Ranck, Meredith Gronski, Fumihko Urano