Abstract Details

Title Do Current Indices of Disease Burden Correlate with Clinical Assessments in Premanifest Huntington's Disease (preHD)?

Topic Movement Disorders

Presentation(s) P07 - (-)

Poster/Presentation
Number 209

Objective

Background BACKGROUND: Prior studies have suggested that the Penney Burden of Pathology (BOP) score, CAG/age product (CAP), and Aylward and Langbehn year-to-onset (YTO) estimates can be used as indices of disease severity and inclusion criteria for clinical trials of preHD. The present study examined the correlations between these measures and specific motor, functional, cognitive, and behavioral scales in preHD.

Design/Methods DESIGN/METHODS: Forty-six preHD subjects followed at one academic center were administered the UHDRS Functional Assessment (FA), Independence Scale (IS), and Total Functional Capacity (TFC); Mini-Mental State Examination, Montreal Cognitive Assessment, Stroop Color-Word Interference, Verbal Fluency, WAIS Digit Span, Symbol Digit Modalities Test; the depression scale on the UHDRS Behavioral Assessment; and the Symptom Checklist-90-Revised (SCL-90-R). Pearson r values were used to examine the correlations between BOP scores, CAP indices, Langbehn and Aylward YTO estimates, and each of the clinical assessments.

Results RESULTS: The mean age of the cohort was 41.0 years, mean education 15.5 years, and mean CAG repeat length 42.0. We found few significant correlations between disease burden measures and our clinical assessments. The Aylward YTO estimates showed moderate correlations (r=0.30-0.37, p=0.01-0.04) with the UHDRS FA, UHDRS TFC, and Symbol Digit Modalities Test. CAP index showed moderate correlations (0.32-0.34, p=0.02-0.03) with Stroop Color-Word Interference and an overall measure of symptom distress on the SCL-90-R only. There were no significant correlations with BOP scores or Langbehn YTO estimates.

Conclusions CONCLUSIONS: There is generally poor correlation between current indices of disease burden and clinical measures in preHD; therefore, caution should be used when selecting preHD subjects for clinical trials on the basis of these indices. Further studies are needed to confirm and extend these findings.

Authors/Disclosures
Shea Gluhm PRESENTER	No disclosure on file
	No disclosure on file
	No disclosure on file
Elizabeth Breen	No disclosure on file
Kimford J. Meador, MD, FAAN (Stanford University School of Medicine)	The institution of Dr. Meador has received research support from NIH. The institution of Dr. Meador has received research support from The Epilepsy Consortium.
Daniel S. Brown	No disclosure on file
Paul Gilbert, PhD (SDSU-UCSD)	Dr. Gilbert has nothing to disclose.
Jody Corey-Bloom, MD, PhD, FAAN (UCSD Neurosciences)	Dr. Corey-Bloom has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UniQure. Dr. Corey-Bloom has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Teva Pharmaceuticaks. Dr. Corey-Bloom has received personal compensation in the range of $10,000-$49,999 for serving as a Co-Director, HD-Net with Huntington Study Group.

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