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

Research output: Contribution to journalArticlepeer-review

54 Scopus citations

Abstract

Objective: The effect of mHTT on human development was examined by evaluating measures of growth in children at risk for Huntington disease (HD). Methods: 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 geneexpanded (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). Results: 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. Conclusions: 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.

Original languageEnglish
Pages (from-to)668-674
Number of pages7
JournalNeurology
Volume79
Issue number7
DOIs
StatePublished - Aug 14 2012

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