Abstract

The proposal is a longitudinal study of potential neurobiological and neurobehavioral markers of disease onset and progression in pre-symptomatic individuals who have the CAG expansion in the HD gene. A total of 500 subjects will be enrolled. Study subjects will be 30 to 55 years old and have a parental history of Huntington's disease. The study will enroll 425 cases with >39 CAG repeats (affected), and 75 controls with <30 CAG repeats (wildtype). The patients will be recruited from 20 research sites in the United States and Canada. Subjects will be evaluated every 12 months for up to 5 years. At baseline, blood will be drawn for centralized confirmatory gene testing. At every visit, subjects will undergo standardized assessments of motor, cognitive, functional, and psychiatric signs of HD using the Unified Huntington's Disease Rating Scale (UHDRS). More comprehensive evaluations will be done at baseline, and 24 and 48 months of follow-up. These more comprehensive evaluations will include a videotaped examination, volumetric magnetic resonance imaging, detailed neuropsychometric testing and behavioral ratings.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Institute of Neurological Disorders and Stroke (NINDS)
Type
Research Project (R01)
Project #
5R01NS040068-03
Application #
6647605
Study Section
Special Emphasis Panel (ZNS1-SRB-K (02))
Program Officer
Oliver, Eugene J
Project Start
2001-09-01
Project End
2005-08-31
Budget Start
2003-09-01
Budget End
2004-08-31
Support Year
3
Fiscal Year
2003
Total Cost
$1,739,116
Indirect Cost

  Institution

Name
University of Iowa
Department
Psychiatry
Type
Schools of Medicine
DUNS #
062761671
City
Iowa City
State
IA
Country
United States
Zip Code
52242

  Publications

Carlozzi, N E; Schilling, S; Kratz, A L et al. (2018) Understanding patient-reported outcome measures in Huntington disease: at what point is cognitive impairment related to poor measurement reliability? Qual Life Res :
Ghayoor, Ali; Vaidya, Jatin G; Johnson, Hans J (2018) Robust automated constellation-based landmark detection in human brain imaging. Neuroimage 170:471-481
Long, Jeffrey D; Lee, Jong-Min; Aylward, Elizabeth H et al. (2018) Genetic Modification of Huntington Disease Acts Early in the Prediagnosis Phase. Am J Hum Genet 103:349-357
Hahn, Elizabeth A; Downing, Nancy R; Stout, Julie C et al. (2018) Understanding the need for assistance with survey completion in people with Huntington disease. Qual Life Res 27:801-810
Downing, Nancy R; Goodnight, Siera; Chae, Sena et al. (2018) Factors Associated With End-of-Life Planning in Huntington Disease. Am J Hosp Palliat Care 35:440-447
Liu, Jingyu; Ciarochi, Jennifer; Calhoun, Vince D et al. (2018) Genetics Modulate Gray Matter Variation Beyond Disease Burden in Prodromal Huntington's Disease. Front Neurol 9:190
Espinoza, Flor A; Vergara, Victor M; Reyes, Daisy et al. (2018) Aberrant functional network connectivity in psychopathy from a large (N = 985) forensic sample. Hum Brain Mapp 39:2624-2634
Carlozzi, Noelle E; Hahn, Elizabeth A; Goodnight, Siera M et al. (2018) Patient-reported outcome measures in Huntington disease: Quality of life in neurological disorders (Neuro-QoL) social functioning measures. Psychol Assess 30:450-458
Wesson, Melissa; Boileau, Nicholas R; Perlmutter, Joel S et al. (2018) Suicidal Ideation Assessment in Individuals with Premanifest and Manifest Huntington Disease. J Huntingtons Dis 7:239-249
Hong, Yi; O'Donnell, Lauren J; Savadjiev, Peter et al. (2018) Genetic load determines atrophy in hand cortico-striatal pathways in presymptomatic Huntington's disease. Hum Brain Mapp 39:3871-3883

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