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Age-Related Cognitive Changes as a Function of CAG Repeat in Child and Adolescent Carriers of Mutant Huntingtin
Journal article   Peer reviewed

Age-Related Cognitive Changes as a Function of CAG Repeat in Child and Adolescent Carriers of Mutant Huntingtin

Jordan L Schultz, Ellen van der Plas, Douglas R Langbehn, Amy L Conrad and Peg C Nopoulos
Annals of neurology, Vol.89(5), pp.1036-1040
05/2021
DOI: 10.1002/ana.26039
PMCID: PMC8176779
PMID: 33521985

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Abstract

Limited data exists regarding the disease course of Huntington's Disease (HD) in children and young adults. Here, we evaluate the trajectory of various cognitive skill development as a function of cytosine-adenine-guanine (CAG) repeat length in children and adolescents that carry the mutation that causes HD. We discovered that the development of verbal skills seems to plateau earlier as CAG repeat length increases. These findings increase our understanding of the relationship between neurodegeneration and neurodevelopment and may have far-reaching implications for future gene-therapy treatment strategies. ANN NEUROL 2021;89:1036-1040.

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