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 #
3R01NS040068-01A1S1
Application #
6598765
Study Section
Special Emphasis Panel (ZNS1 (02))
Program Officer
Oliver, Eugene J
Project Start
2001-09-01
Project End
2004-08-31
Budget Start
2001-09-01
Budget End
2002-08-31
Support Year
1
Fiscal Year
2002
Total Cost
$38,191
Indirect Cost

  Institution

Name
University of Iowa
Department
Psychiatry
Type
Schools of Medicine
DUNS #
041294109
City
Iowa City
State
IA
Country
United States
Zip Code
52242

  Publications

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
Carlozzi, Noelle E; Boileau, Nicholas R; Perlmutter, Joel S et al. (2018) Agreement between clinician-rated versus patient-reported outcomes in Huntington disease. J Neurol 265:1443-1453
Fritz, Nora E; Boileau, Nicholas R; Stout, Julie C et al. (2018) Relationships Among Apathy, Health-Related Quality of Life, and Function in Huntington's Disease. J Neuropsychiatry Clin Neurosci 30:194-201
Hong, Sungmin; Fishbaugh, James; Gerig, Guido (2018) 4D CONTINUOUS MEDIAL REPRESENTATION BY GEODESIC SHAPE REGRESSION. Proc IEEE Int Symp Biomed Imaging 2018:1014-1017
Lai, Jin-Shei; Goodnight, Siera; Downing, Nancy R et al. (2018) Evaluating cognition in individuals with Huntington disease: Neuro-QoL cognitive functioning measures. Qual Life Res 27:811-822
Carlozzi, Noelle E; Hahn, E A; Frank, S A et al. (2018) A new measure for end of life planning, preparation, and preferences in Huntington disease: HDQLIFE end of life planning. J Neurol 265:98-107
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 :

Showing the most recent 10 out of 172 publications