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. 2022 Dec;37(12):2459-2460.
doi: 10.1002/mds.29227. Epub 2022 Sep 29.

Striatal Development in Early-Onset Huntington's Disease

Affiliations

Striatal Development in Early-Onset Huntington's Disease

Jordan L Schultz et al. Mov Disord. 2022 Dec.
No abstract available

Keywords: Huntington's disease; children; neurodevelopment.

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Figures

Figure 1:
Figure 1:. Assessment of Striatal Volume Amongst HD Children with Long CAG Repeats
A) The mean striatal volume of the pre-EOHD children was significantly elevated compared to matched GNE participants. The large circles represent the adjusted group means and the 95% confidence interval. B) A participant with a CAG of 73 who participated in the at-risk study from age 6–12 and their striatal volume is compared to a group of GNE participants comparable in age and matched for sex. At the age of 6, the participant was far from their predicted motor onset and had a striatal volume that was comparable to the GNE group.This participant’s striatal volume decreased quickly and they received a diagnosis of JOHD before the age of 12. Their trajectory suggests that this participant’s striatal volume was elevated prior to the age of 6 years. EOHD, Early-Onset HD; GNE, Gene-non-expanded; HD, Huntington Disease; ICV, Intracranial Volume; JOHD, Juvenile-Onset Huntington Disease

References

    1. Barnat M, Capizzi M, Aparicio E, et al. Huntington’s disease alters human neurodevelopment. Science 2020;369(6505):787–793. - PMC - PubMed
    1. van der Plas E, Langbehn DR, Conrad AL, et al. Abnormal brain development in child and adolescent carriers of mutant huntingtin. Neurology 2019;93(10):e1021–e1030. - PMC - PubMed
    1. Tereshchenko A, Magnotta V, Epping E, et al. Brain structure in juvenile-onset Huntington disease. Neurology 2019;92(17):e1939–e1947. - PMC - PubMed
    1. Zhang Y, Long JD, Mills JA, Warner JH, Lu W, Paulsen JS. Indexing disease progression at study entry with individuals at-risk for Huntington disease. American journal of medical genetics Part B, Neuropsychiatric genetics : the official publication of the International Society of Psychiatric Genetics 2011;156B(7):751–763. - PMC - PubMed
    1. Schultz JL, van der Plas E, Langbehn DR, Conrad AL, Nopoulos PC. Age-Related Cognitive Changes as a Function of CAG Repeat in Child and Adolescent Carriers of Mutant Huntingtin. Ann Neurol 2021;89(5):1036–1040. - PMC - PubMed

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