Smaller intracranial volume in prodromal Huntington's disease: evidence for abnormal neurodevelopment

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; PREDICT-HD Investigators and Coordinators of the Huntington Study Group

doi:10.1093/brain/awq280

Back

Smaller intracranial volume in prodromal Huntington's disease: evidence for abnormal neurodevelopment

Journal article

Open access

Peer reviewed

Smaller intracranial volume in prodromal Huntington's disease: evidence for abnormal neurodevelopment

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, …

Brain (London, England : 1878), Vol.134(Pt 1), pp.137-142

01/2011

DOI: 10.1093/brain/awq280

PMCID: PMC3025719

PMID: 20923788

Files and links (1)

url

https://doi.org/10.1093/brain/awq280View

Published (Version of record) Open Access

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.

Humans

Huntington Disease - pathology

Middle Aged

Organ Size

Male

Trinucleotide Repeats - genetics

Magnetic Resonance Imaging

Analysis of Variance

Brain - pathology

Huntington Disease - genetics

Adult

Female

Aged

Details

Title: Subtitle: Smaller intracranial volume in prodromal Huntington's disease: evidence for abnormal neurodevelopment
Creators: Peggy C Nopoulos - Department of Psychiatry, The University of Iowa Roy and Lucille Carver College of Medicine, Iowa, IA 52242, USA
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
PREDICT-HD Investigators and Coordinators of the Huntington Study Group
Resource Type: Journal article
Publication Details: Brain (London, England : 1878), Vol.134(Pt 1), pp.137-142
DOI: 10.1093/brain/awq280
PMID: 20923788
PMCID: PMC3025719
NLM abbreviation: Brain
ISSN: 0006-8950
eISSN: 1460-2156
Publisher: England
Grant note: G0800784 / Medical Research Council R01 NS040068 / NINDS NIH HHS Howard Hughes Medical Institute NS016375 / NINDS NIH HHS NS054893 / NINDS NIH HHS NS050568 / NINDS NIH HHS R01 NS050568 / NINDS NIH HHS NS40068 / NINDS NIH HHS RR023392 / NCRR NIH HHS NS055733 / NINDS NIH HHS UL1 TR000442 / NCATS NIH HHS
Language: English
Date published: 01/2011
Academic Unit: Roy J. Carver Department of Biomedical Engineering; Neurology; Radiology; Electrical and Computer Engineering; Psychiatry; Stead Family Department of Pediatrics; Psychological and Brain Sciences; Iowa Neuroscience Institute
Record Identifier: 9984003410202771

Metrics

17 Record Views

119 Times Cited - Web of Science