Measures of growth in children at risk for Huntington disease

Jessica K Lee; Kathy Mathews; Bradley Schlaggar; Joel Perlmutter; Jane S Paulsen; Eric Epping; Leon Burmeister; Peg Nopoulos

doi:10.1212/WNL.0b013e3182648b65

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Measures of growth in children at risk for Huntington disease

Journal article

Open access

Peer reviewed

Measures of growth in children at risk for Huntington disease

Jessica K Lee, Kathy Mathews, Bradley Schlaggar, Joel Perlmutter, Jane S Paulsen, Eric Epping, Leon Burmeister and Peg Nopoulos

Neurology, Vol.79(7), pp.668-674

08/14/2012

DOI: 10.1212/WNL.0b013e3182648b65

PMCID: PMC3414667

PMID: 22815549

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Abstract

The effect of mHTT on human development was examined by evaluating measures of growth in children at risk for Huntington disease (HD). Children at risk for HD with no manifest symptoms (no juvenile HD included) were enrolled and tested for gene expansion for research purposes only. Measurements of growth (height, weight, body mass index [BMI], and head circumference) in children tested as gene-expanded (n = 20, 7-18 years of age, CAG repeats ≥39) were compared to those of a large database of healthy children (n = 152, 7-18 years of age). Gene-expanded children had significantly lower measures of head circumference, weight, and BMI. Head circumference was abnormally low even after correcting for height, suggesting a specific deficit in brain growth, rather than a global growth abnormality. These results indicate that, compared to a control population, children who were estimated to be decades from HD diagnosis have significant differences in growth. Further, they suggest that mHTT may play a role in atypical somatic, and in particular, brain development.

Body Height - physiology

Body Mass Index

Humans

Male

Risk

Cephalometry

Adolescent Development - physiology

Adolescent

Female

Child Development - physiology

Body Weight - physiology

Child

Huntington Disease - physiopathology

Details

Title: Subtitle: Measures of growth in children at risk for Huntington disease
Creators: Jessica K Lee - Department of Psychiatry, University of Iowa, Carver College of Medicine, Iowa City, USA. jessica-k-lee@uiowa.edu
Kathy Mathews
Bradley Schlaggar
Joel Perlmutter
Jane S Paulsen
Eric Epping
Leon Burmeister
Peg Nopoulos
Resource Type: Journal article
Publication Details: Neurology, Vol.79(7), pp.668-674
DOI: 10.1212/WNL.0b013e3182648b65
PMID: 22815549
PMCID: PMC3414667
NLM abbreviation: Neurology
ISSN: 0028-3878
eISSN: 1526-632X
Publisher: United States
Grant note: R01 DE014399-05 / NIDCR NIH HHS R01 DE014399 / NIDCR NIH HHS UL1 TR000448 / NCATS NIH HHS R01 NS055903 / NINDS NIH HHS 5R01NS055903-03 / NINDS NIH HHS
Language: English
Date published: 08/14/2012
Academic Unit: Neurology; Psychiatry; Stead Family Department of Pediatrics; Psychological and Brain Sciences; Iowa Neuroscience Institute; Neurology (Pediatrics)
Record Identifier: 9984003972502771

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