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Growth and Development of the Striatum in Huntington's Disease (Kids-HD)

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ClinicalTrials.gov Identifier: NCT01860339
Recruitment Status : Enrolling by invitation
First Posted : May 22, 2013
Last Update Posted : June 20, 2018
Sponsor:
Collaborators:
CHDI Foundation, Inc.
National Institute of Neurological Disorders and Stroke (NINDS)
Information provided by (Responsible Party):
Peggy C Nopoulos, University of Iowa

Tracking Information
First Submitted Date May 16, 2013
First Posted Date May 22, 2013
Last Update Posted Date June 20, 2018
Study Start Date July 2005
Estimated Primary Completion Date June 30, 2019   (Final data collection date for primary outcome measure)
Current Primary Outcome Measures
 (submitted: May 24, 2013)
Volume of brain structures as measured by Magnetic Resonance Imaging (MRI) [ Time Frame: 6-7 hour testing day ]
Magnetic Resonance Imaging (MRI) and Diffusion Tensor Imaging (DTI) data will be analyzed to assess brain structure based upon variables including global volume, total cerebral spinal fluid, subregion volumes, cortical surface anatomy including cortical depth, surface area and gyral shape, and symmetry between brain hemispheres, all in consideration of age, gender, and height. Results will be evaluated for comparative differences between the GE group, the GNE group, and the healthy control group. In addition, these measures of brain structure will be paired with corresponding measures of brain function to evaluate brain development based upon growth and performance.
Original Primary Outcome Measures
 (submitted: May 20, 2013)
Volume of brain structures as measured by Magnetic Resonance Imaging (MRI) [ Time Frame: Participant age of 6-18 years ]
Assessments of brain structure and function during developmental years will be evaluated for comparative differences between the GE group, the GNE group, and the healthy control group.
Change History Complete list of historical versions of study NCT01860339 on ClinicalTrials.gov Archive Site
Current Secondary Outcome Measures
 (submitted: May 24, 2013)
Quantitative assessment of cognitive skills and motor skills [ Time Frame: 6-7 hour testing day ]
Participants undergo a cognitive battery which will quantify skills such as attention, learning, memory. In addition, motor skill (both fine and gross) will be assessed and quantified. Results will be analyzed for comparative differences between the GE group, the GNE group, and the healthy control group. In addition, these measures of brain function will be paired with appropriate measures of brain structure to evaluate brain development based upon growth and performance.
Original Secondary Outcome Measures
 (submitted: May 20, 2013)
Quantitative assessment of cognitive skills and motor skills [ Time Frame: Participant age 6-18 years ]
Subject undergo a cognitive battery which will quantify skills such as attention, learning, memory. In addition, motor skill (both fine and gross) will be assessed and quantified.
Current Other Pre-specified Outcome Measures Not Provided
Original Other Pre-specified Outcome Measures Not Provided
 
Descriptive Information
Brief Title Growth and Development of the Striatum in Huntington's Disease
Official Title Growth and Development of the Striatum in Huntington's Disease
Brief Summary

Huntington's Disease (HD) is an autosomal dominant disease manifested in a triad of cognitive, psychiatric, and motor signs and symptoms. HD is caused by a triplet repeat (CAG)expansion in the gene Huntingtin (HTT). This disease has classically been conceptualized as a neurodegenerative disease. However, recent evidence suggests that abnormal brain development may play an important role in the etiology of HD. Huntingtin (HTT)is expressed during development and through life. In animal studies, the HTT gene has been shown to be vital for brain development. This suggests that a mutant form of HTT (gene-expanded or CAG repeats of 40 and above) would affect normal brain development. In addition, studies in adults who are gene-expanded for HD, but have not yet manifested the illness, (pre-HD subjects) have significant changes in the structure of their brain, even up to 20 years before onset of clinical diagnosis. How far back these changes are evident is unknown. One possibility is that these brain changes are present throughout life, due to changes in brain development,though initially associated with only subtle functional abnormalities.

In an effort to better understand the developmental aspects of this brain disease, the current study proposes to evaluate brain structure and function in children, adolescents, and young adults (ages 6-25) who are at risk for developing HD - those who have a parent or grandparent with HD. Brain structure will be evaluating using Magnetic Resonance Imaging (MRI) with quantitative measures of the entire brain, cerebral cortex, as well as white matter integrity via Diffusion Tensor Imaging. Brain function will be assessed by cognitive tests, physical and neurologic evaluation, behavioral assessment, and quantitative craniofacial structure assessment. Subjects that are gene-expanded (GE) will be compared to subjects who are gene non-expanded (GNE) and as well to matched healthy controls. Changes in brain structure and/or function in the GE group, compared to both GNE and control groups, would lend support to the notion that this disease has an important developmental component.

Detailed Description Not Provided
Study Type Observational
Study Design Observational Model: Case-Control
Time Perspective: Cross-Sectional
Target Follow-Up Duration Not Provided
Biospecimen Retention:   Samples With DNA
Description:

Bio-specimens are collected and retained for both case and control participants for genetic testing purposes. Specimens are either a single sample of 1-2 teaspoons of blood drawn from the arm or 1 teaspoon of saliva via collection device.

Biospecimens will be tested for the number of CAG repeats in the Huntingtin Gene. Each sample obtained will be coded with a randomly assigned number and never linked with personal identifiers. The results from the genetic analysis will be sent directly to the data manager on the project, who is the ONLY research member with access to this data. This person has no direct contact with study participants.

Sampling Method Non-Probability Sample
Study Population Young people ages 6-25 years old who have a parent or grandparent that has been diagnosed with Huntington's Disease
Condition Huntington's Disease
Intervention Not Provided
Study Groups/Cohorts
  • Gene-expanded (GE)
    Children at risk for HD who have a CAG repeat length of 40 and above.
  • Gene Non-Expanded (GNE)
    Children at risk for HD who have a CAG repeat length of 39 or less
  • Healthy Controls (HC)
    Healthy children with no medical or psychiatric disorder and no history of HD in their family
  • Case Parent
    Parent of participant at risk for Huntington Disease
  • Control Parent
    Parent of healthy control participant
Publications * Not Provided

*   Includes publications given by the data provider as well as publications identified by ClinicalTrials.gov Identifier (NCT Number) in Medline.
 
Recruitment Information
Recruitment Status Enrolling by invitation
Estimated Enrollment
 (submitted: June 18, 2018)
700
Original Estimated Enrollment
 (submitted: May 20, 2013)
400
Estimated Study Completion Date June 30, 2019
Estimated Primary Completion Date June 30, 2019   (Final data collection date for primary outcome measure)
Eligibility Criteria

Inclusion Criteria:

  • Family history of Huntington's Disease
  • Age 6-25 years
  • Age-appropriate knowledge of HD and personal risk

Exclusion Criteria:

  • Metal in body, including braces
  • History of head trauma, brain tumor, seizures, epilepsy
  • History of major surgery and/or significant ongoing medical issue(s)
Sex/Gender
Sexes Eligible for Study: All
Ages 6 Years to 25 Years   (Child, Adult)
Accepts Healthy Volunteers Yes
Contacts Contact information is only displayed when the study is recruiting subjects
Listed Location Countries United States
Removed Location Countries  
 
Administrative Information
NCT Number NCT01860339
Other Study ID Numbers 200507759
4R01NS055903-08 ( U.S. NIH Grant/Contract )
Has Data Monitoring Committee No
U.S. FDA-regulated Product Not Provided
IPD Sharing Statement
Plan to Share IPD: No
Plan Description: Aggregate, de-identified data may be shared with approved collaborators, but individual participant data will not be shared.
Responsible Party Peggy C Nopoulos, University of Iowa
Study Sponsor Peggy C Nopoulos
Collaborators
  • CHDI Foundation, Inc.
  • National Institute of Neurological Disorders and Stroke (NINDS)
Investigators
Principal Investigator: Peggy C Nopoulos, MD University of Iowa
PRS Account University of Iowa
Verification Date June 2018