What is the Kids-JHD Study?

The Kids-JHD study is a brain imaging study run by researchers in the Department of Psychiatry at the University of Iowa Hospitals and Clinics.  The research focuses on young people who are diagnosed with Juvenile Huntington Disease (JHD).  This is a separate sister study to the Kids-HD study that has been designed to better address the additional needs and research questions that arise due to juvenile onset. 


The primary research interest of the Kids-JHD project is to evaluate the effects of the CAG repeat in the Huntingtin gene (HTT) on the developing human brain.  Although HD is a disease that typically manifests in adulthood, juvenile onset can occur in rare cases.  This study will try to identify and measure common symptoms and developmental patterns that might be happening in JHD, specifically the impact of higher CAG repeat expansion on early developmental processes as opposed to early degeneration.  


The Kids-JHD study is designed to evaluate brain structure (using Magnetic Brain Resonance Imaging or MRI) and brain function (cognitive, behavioral, and motor skills) in young people who are diagnosed with JHD.  Confirmed CAG repeat expansion is recorded for comparison to other groups of participants who are gene non-expanded or GNE (< 39 CAG repeats), gene-expanded or GE (> 40 CAG repeats), and healthy controls (HC) with no family history of HD.  All data collected is for research purposes only.


In addition, study participants and their families meet with three pediatric specialists:  a child

neurologist, a child psychiatrist, and a child neuropsychologist.  These doctors will perform their own evaluations and discuss current concerns and treatment ideas.  By request, they will consult and collaborate with other people involved in the participant’s care, including doctors, teachers, and counselors.   The evaluations and consultations are offered by the study as an additional resource for participants and their families regarding interventions, treatment and symptom management.  


What are the researchers trying to learn?

They hope that someday this study will help explain how HD can change the way a person’s brain grows. If scientists can find and measure the earliest changes in the brain and figure out when they usually happen, it should assist clinicians and scientists in their search to find new ways to tell if a person has HD and help identify the earliest biological pathways that are altered by mutant huntingtin.  These pathways could be ideal candidates for therapeutics to delay the onset of HD.


How do they study the brain?

They study brain structure (the different parts and how they’re put together) and function (how all those parts work).  To look at someone’s brain, they will use a special scan called Magnetic Resonance Imaging (MRI) to take a picture of it. An MRI can reveal structural readouts, such as the size and shape of different brain regions.  To get an idea of how someone’s brain works, they measure how they think and remember things, how they move and maintain balance, and what they think of themselves and other people. To do this, they make use of puzzles and brainteasers, quizzes, and activities that measure balance and coordination. 


Who can participate?

Young people ages 6 to 18 years old are eligible to participate in this study if they have a diagnosis of JHD.    



How to find out more…

1)      Try the website: www.kids-hd.org

2)      Visit them on Facebook: https://www.facebook.com/KidsHd

3)      Call their toll free number: 1-866-514-0858 or send them an e-mail: kids-hd@uiowa.edu

4)      There are also two people you can call and talk to about the study:

Sonia Slevinski, MS at (319) 353-8529

Joyce Goins, PhD at (319) 467-5519