Smaller intracranial volume in prodromal Huntington's disease: evidence for abnormal neurodevelopment (original) (raw)

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11 Department of Psychiatry, The University of Iowa Roy and Lucille Carver College of Medicine, Iowa, IA 52242, USA

22 Department of Pediatrics, The University of Iowa Roy and Lucille Carver College of Medicine, Iowa City, IA 52242, USA

33 Department of Neurology, The University of Iowa Roy and Lucille Carver College of Medicine, Iowa City, IA 52242, USA

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44 Seattle Children's Research Institute, Seattle, Washington, USA

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55 Division of Neurology, Pharmacology and Neuroscience, Johns Hopkins University, Baltimore, MD, USA

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11 Department of Psychiatry, The University of Iowa Roy and Lucille Carver College of Medicine, Iowa, IA 52242, USA

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11 Department of Psychiatry, The University of Iowa Roy and Lucille Carver College of Medicine, Iowa, IA 52242, USA

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11 Department of Psychiatry, The University of Iowa Roy and Lucille Carver College of Medicine, Iowa, IA 52242, USA

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11 Department of Psychiatry, The University of Iowa Roy and Lucille Carver College of Medicine, Iowa, IA 52242, USA

66 Department of Radiology, The University of Iowa Roy and Lucille Carver College of Medicine, Iowa City, IA 52242, USA

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11 Department of Psychiatry, The University of Iowa Roy and Lucille Carver College of Medicine, Iowa, IA 52242, USA

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11 Department of Psychiatry, The University of Iowa Roy and Lucille Carver College of Medicine, Iowa, IA 52242, USA

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77 Hennepin County Medical Center, Department of Neurosciences, Saint Louis Park, MN, USA

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Revision received:

12 July 2010

Published:

04 October 2010

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Peggy C. Nopoulos, Elizabeth H. Aylward, Christopher A. Ross, James A. Mills, Douglas R. Langbehn, Hans J. Johnson, Vincent A. Magnotta, Ronald K. Pierson, Leigh J. Beglinger, Martha A. Nance, Roger A. Barker, Jane S. Paulsen, the PREDICT-HD Investigators and Coordinators of the Huntington Study Group, Smaller intracranial volume in prodromal Huntington's disease: evidence for abnormal neurodevelopment, Brain, Volume 134, Issue 1, January 2011, Pages 137–142, https://doi.org/10.1093/brain/awq280
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Abstract

Huntington’s disease is an autosomal dominant brain disease. Although conceptualized as a neurodegenerative disease of the striatum, a growing number of studies challenge this classic concept of Huntington’s disease aetiology. Intracranial volume is the tissue and fluid within the calvarium and is a representation of the maximal brain growth obtained during development. The current study reports intracranial volume obtained from an magnetic resonance imaging brain scan in a sample of subjects (n = 707) who have undergone presymptomatic gene testing. Participants who are gene-expanded but not yet manifesting the disease (prodromal Huntington’s disease) are compared with subjects who are non-gene expanded. The prodromal males had significantly smaller intracranial volume measures with a mean volume that was 4% lower compared with controls. Although the prodromal females had smaller intracranial volume measures compared with their controls, this was not significant. The current findings suggest that mutant huntingtin can cause abnormal development, which may contribute to the pathogenesis of Huntington’s disease.

© The Author (2010). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Topic:

• magnetic resonance imaging
• congenital abnormality
• huntington's disease
• genes
• neurodegenerative disorders
• brain
• nervous system development
• causality

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