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NINDS CDE Huntington's Disease Behavioral Psychology Subgroup ...

NINDS CDE Huntington's Disease Behavioral Psychology Subgroup ...

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Description of Frontal Systems Behavior Scale forHD Common Data ElementsRationale/Justification (includeany information onlanguage andcountries/ cultures/ethnic groups wheretested)Validity:Construct validity: Reviewed in manual and acceptable.Convergent validity with other behavioral measures was high (NPI, r=.64).Discriminant validity also good (Grace).Feasibility: Informants completing the Family Rating Form should have at leastweekly contact with the patient to ensure accurate behavioral observation.Patients must have cognitive capacity to read and complete the form.Factor structure: An exploratory principal component factor analysis using thefamily version with 324 neurological outpatients (mainly HD, PD and Alzheimer’sdisease patients) confirmed a f actor structure consistent with the subscalesoriginally proposed on theoretical grounds (Stout).Sensitivity to Change/ Ability to Detect Change (over time or in response toan intervention): This measure was designed in part to assess change overtime.Known Relationships to Other Variables: There have been no p ublishedreports of patients with manifest HD using the FrSBe, other than the factoranalysis referred to above (Stout). In the PREDICT-HD study, 745 mutationpositivesubjects, 163 mutation-negative control subjects and their companionscompleted subject and family versions respectively of the FrSBE (Duff). Mutationpositivesubjects reported more frontal behaviours than mutation-negativecontrols, even though most subjects were more than 10 y ears from predictedmotor onset. However, discrepancies between self-report and companion scoressuggested impaired insight in those closest to predicted disease onset. In non-HD studies, Apathy and Executive Dysfunction subscale scores are correlatedwith IADL’s (Grace), and the Disinhibition scale score is strongly related tocaregiver burden (Grace).Diagnostic Sensitivity and Specificity, if applicable: N/AStrengths: Assesses multiple domains of frontal lobe functioning and allows forcomparison of premorbid behavior with current status. Also allows for comparisonbetween patient and caregiver reports.Weaknesses: Large number of items may be a problem for more cognitivelyimpaired subjects. Scoring requires normative database and understanding of Tscores.Availability (copyright): Through Psychological Assessment Resources, Inc. Thismeasure is copyrighted and cannot be reproduced without permission.http://www4.parinc.com/Products/Product.aspx?ProductID=FRSBESpecial Requirements for administration: NoneAdministration Time: The scale takes 10 minutes to administer and 10-15minutes to score.HD Version 1.0 Page 2 of 3

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