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Abstract

Objective:

Using a multi-informant approach, the authors assessed the psychiatric symptoms of adolescents and young adults with or without the huntingtin gene expansion and examined the association of psychiatric symptoms with cumulative disease exposure, a measure taking into account age and genetic data.

Methods:

The sample included 110 participants with (N=71) or without (N=39) the gene expansion, along with 85 family members who provided collateral reports. Saliva samples were used for genetic testing. Participants reported psychiatric symptoms with the age- and informant-appropriate Achenbach System of Empirically Based Assessment measure.

Results:

Family member ratings indicated that young people (ages 10–39) with the gene expansion were more likely to exhibit depression symptoms, attention difficulties, and behavior problems compared with those without the gene expansion. Self-reports of these symptoms did not differ between the two groups and indicated elevated depression symptoms, attention difficulties, thought problems, and obsessive-compulsive symptoms in both groups. In family member reports, 25% and 15% of the individuals with the gene expansion exceeded the clinical cutoffs for internalizing and attention difficulties, respectively. Little support was found for an association between psychiatric symptoms and cumulative disease exposure.

Conclusions:

These findings suggest that young people from families affected by Huntington’s disease are at elevated risk for psychiatric symptoms regardless of gene status or cumulative disease exposure. However, findings differed depending on the informant type. These results emphasize a need to screen for and monitor the psychiatric symptoms of all young people from families affected by Huntington’s disease regardless of gene status.

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Information & Authors

Information

Published In

Go to The Journal of Neuropsychiatry and Clinical Neurosciences
Go to The Journal of Neuropsychiatry and Clinical Neurosciences
The Journal of Neuropsychiatry and Clinical Neurosciences

History

Received: 25 May 2024
Revision received: 5 November 2024
Revision received: 13 November 2024
Accepted: 13 November 2024
Published online: 31 January 2025

Keywords

  1. Adolescents
  2. Genetics
  3. Huntington-s Disease
  4. Mood Disorders
  5. Neuropsychiatric Symptoms
  6. Young Adults

Authors

Details

Kelly H. Watson, Ph.D. k.watson@vumc.org
Departments of Neurology (Watson, Pfalzer, Snow, Diehl, McDonell, Claassen) and Pathology, Microbiology, and Immunology (Vnencak-Jones), Vanderbilt University Medical Center, Nashville; Department of Psychology and Human Development (Ciriegio, Snow, Compas), Vanderbilt University, Nashville; Department of Psychiatry, University of Iowa, Iowa City (Long).
Abagail E. Ciriegio, M.S., M.Ed.
Departments of Neurology (Watson, Pfalzer, Snow, Diehl, McDonell, Claassen) and Pathology, Microbiology, and Immunology (Vnencak-Jones), Vanderbilt University Medical Center, Nashville; Department of Psychology and Human Development (Ciriegio, Snow, Compas), Vanderbilt University, Nashville; Department of Psychiatry, University of Iowa, Iowa City (Long).
Anna C. Pfalzer, Ph.D.
Departments of Neurology (Watson, Pfalzer, Snow, Diehl, McDonell, Claassen) and Pathology, Microbiology, and Immunology (Vnencak-Jones), Vanderbilt University Medical Center, Nashville; Department of Psychology and Human Development (Ciriegio, Snow, Compas), Vanderbilt University, Nashville; Department of Psychiatry, University of Iowa, Iowa City (Long).
Abigail L. B. Snow, B.A.
Departments of Neurology (Watson, Pfalzer, Snow, Diehl, McDonell, Claassen) and Pathology, Microbiology, and Immunology (Vnencak-Jones), Vanderbilt University Medical Center, Nashville; Department of Psychology and Human Development (Ciriegio, Snow, Compas), Vanderbilt University, Nashville; Department of Psychiatry, University of Iowa, Iowa City (Long).
Spencer Diehl, L.C.S.W.
Departments of Neurology (Watson, Pfalzer, Snow, Diehl, McDonell, Claassen) and Pathology, Microbiology, and Immunology (Vnencak-Jones), Vanderbilt University Medical Center, Nashville; Department of Psychology and Human Development (Ciriegio, Snow, Compas), Vanderbilt University, Nashville; Department of Psychiatry, University of Iowa, Iowa City (Long).
Katherine E. McDonell, M.D.
Departments of Neurology (Watson, Pfalzer, Snow, Diehl, McDonell, Claassen) and Pathology, Microbiology, and Immunology (Vnencak-Jones), Vanderbilt University Medical Center, Nashville; Department of Psychology and Human Development (Ciriegio, Snow, Compas), Vanderbilt University, Nashville; Department of Psychiatry, University of Iowa, Iowa City (Long).
Cindy L. Vnencak-Jones, Ph.D.
Departments of Neurology (Watson, Pfalzer, Snow, Diehl, McDonell, Claassen) and Pathology, Microbiology, and Immunology (Vnencak-Jones), Vanderbilt University Medical Center, Nashville; Department of Psychology and Human Development (Ciriegio, Snow, Compas), Vanderbilt University, Nashville; Department of Psychiatry, University of Iowa, Iowa City (Long).
Jeffrey D. Long, Ph.D.
Departments of Neurology (Watson, Pfalzer, Snow, Diehl, McDonell, Claassen) and Pathology, Microbiology, and Immunology (Vnencak-Jones), Vanderbilt University Medical Center, Nashville; Department of Psychology and Human Development (Ciriegio, Snow, Compas), Vanderbilt University, Nashville; Department of Psychiatry, University of Iowa, Iowa City (Long).
Bruce E. Compas, Ph.D.
Departments of Neurology (Watson, Pfalzer, Snow, Diehl, McDonell, Claassen) and Pathology, Microbiology, and Immunology (Vnencak-Jones), Vanderbilt University Medical Center, Nashville; Department of Psychology and Human Development (Ciriegio, Snow, Compas), Vanderbilt University, Nashville; Department of Psychiatry, University of Iowa, Iowa City (Long).
Daniel O. Claassen, M.D., M.S.
Departments of Neurology (Watson, Pfalzer, Snow, Diehl, McDonell, Claassen) and Pathology, Microbiology, and Immunology (Vnencak-Jones), Vanderbilt University Medical Center, Nashville; Department of Psychology and Human Development (Ciriegio, Snow, Compas), Vanderbilt University, Nashville; Department of Psychiatry, University of Iowa, Iowa City (Long).

Notes

Send correspondence to Dr. Watson (k.watson@vumc.org).

Competing Interests

Dr. Long reports serving as a consultant to Annexon, AskBio, Harness, Jazz, Latus Bio, LiefEDIT, Prilenia, PTC, Rgenta, Sage, Skyhawk, Spark, Teva, Trace Neuroscience, Vertex, and Wave; as a committee member for Alexion, Alynylam, Roche, and uniQure; and on the data safety monitoring board for NIMH’s research project Rapidly Acting Treatments for Treatment-Resistant Depression. The other authors report no financial relationships with commercial interests.

Funding Information

This study was supported in part by the CHDI Foundation, the Eunice Kennedy Shriver National Institute of Child Health and Human Development (grant R01 HD104188), the Griffin Foundation, the National Center for Advancing Translational Sciences (grants UL1TR002243 and KL2TR002245), and NIMH (grant T32 MH18921). Dr. McDonell received an NIH K23 award from the National Institute of Neurological Disorders and Stroke.

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