Literature DB >> 33521985

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

Jordan L Schultz1,2,3, Ellen van der Plas1, Douglas R Langbehn1, Amy L Conrad4, Peg C Nopoulos1,2,4.   

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.
© 2021 American Neurological Association.

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Year:  2021        PMID: 33521985      PMCID: PMC8176779          DOI: 10.1002/ana.26039

Source DB:  PubMed          Journal:  Ann Neurol        ISSN: 0364-5134            Impact factor:   10.422


  16 in total

1.  The Bender-Gestalt II.

Authors:  Gary G Brannigan; Scott L Decker
Journal:  Am J Orthopsychiatry       Date:  2006-01

2.  Prediction of manifest Huntington's disease with clinical and imaging measures: a prospective observational study.

Authors:  Jane S Paulsen; Jeffrey D Long; Christopher A Ross; Deborah L Harrington; Cheryl J Erwin; Janet K Williams; Holly James Westervelt; Hans J Johnson; Elizabeth H Aylward; Ying Zhang; H Jeremy Bockholt; Roger A Barker
Journal:  Lancet Neurol       Date:  2014-11-03       Impact factor: 44.182

3.  Abnormal brain development in child and adolescent carriers of mutant huntingtin.

Authors:  Ellen van der Plas; Douglas R Langbehn; Amy L Conrad; Timothy R Koscik; Alexander Tereshchenko; Eric A Epping; Vincent A Magnotta; Peggy C Nopoulos
Journal:  Neurology       Date:  2019-08-01       Impact factor: 9.910

4.  Abnormal development of cerebellar-striatal circuitry in Huntington disease.

Authors:  Alexander V Tereshchenko; Jordan L Schultz; Joel E Bruss; Vincent A Magnotta; Eric A Epping; Peg C Nopoulos
Journal:  Neurology       Date:  2020-04-07       Impact factor: 9.910

5.  Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington's disease in the TRACK-HD study: analysis of 36-month observational data.

Authors:  Sarah J Tabrizi; Rachael I Scahill; Gail Owen; Alexandra Durr; Blair R Leavitt; Raymund A Roos; Beth Borowsky; Bernhard Landwehrmeyer; Chris Frost; Hans Johnson; David Craufurd; Ralf Reilmann; Julie C Stout; Douglas R Langbehn
Journal:  Lancet Neurol       Date:  2013-05-09       Impact factor: 44.182

6.  A new model for prediction of the age of onset and penetrance for Huntington's disease based on CAG length.

Authors:  D R Langbehn; R R Brinkman; D Falush; J S Paulsen; M R Hayden
Journal:  Clin Genet       Date:  2004-04       Impact factor: 4.438

7.  Neurofilament light protein in blood as a potential biomarker of neurodegeneration in Huntington's disease: a retrospective cohort analysis.

Authors:  Lauren M Byrne; Filipe B Rodrigues; Kaj Blennow; Alexandra Durr; Blair R Leavitt; Raymund A C Roos; Rachael I Scahill; Sarah J Tabrizi; Henrik Zetterberg; Douglas Langbehn; Edward J Wild
Journal:  Lancet Neurol       Date:  2017-06-07       Impact factor: 44.182

Review 8.  Structural brain development: A review of methodological approaches and best practices.

Authors:  Nandita Vijayakumar; Kathryn L Mills; Aaron Alexander-Bloch; Christian K Tamnes; Sarah Whittle
Journal:  Dev Cogn Neurosci       Date:  2017-11-22       Impact factor: 6.464

9.  Biological and clinical characteristics of gene carriers far from predicted onset in the Huntington's disease Young Adult Study (HD-YAS): a cross-sectional analysis.

Authors:  Rachael I Scahill; Paul Zeun; Katherine Osborne-Crowley; Eileanoir B Johnson; Sarah Gregory; Christopher Parker; Jessica Lowe; Akshay Nair; Claire O'Callaghan; Christelle Langley; Marina Papoutsi; Peter McColgan; Carlos Estevez-Fraga; Kate Fayer; Henny Wellington; Filipe B Rodrigues; Lauren M Byrne; Amanda Heselgrave; Harpreet Hyare; Cristina Sampaio; Henrik Zetterberg; Hui Zhang; Edward J Wild; Geraint Rees; Trevor W Robbins; Barbara J Sahakian; Douglas Langbehn; Sarah J Tabrizi
Journal:  Lancet Neurol       Date:  2020-05-26       Impact factor: 44.182

10.  The HTT CAG-Expansion Mutation Determines Age at Death but Not Disease Duration in Huntington Disease.

Authors:  Jae Whan Keum; Aram Shin; Tammy Gillis; Jayalakshmi Srinidhi Mysore; Kawther Abu Elneel; Diane Lucente; Tiffany Hadzi; Peter Holmans; Lesley Jones; Michael Orth; Seung Kwak; Marcy E MacDonald; James F Gusella; Jong-Min Lee
Journal:  Am J Hum Genet       Date:  2016-02-04       Impact factor: 11.025

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  3 in total

Review 1.  Effects of Exercise on Skeletal Muscle Pathophysiology in Huntington's Disease.

Authors:  Bruno Trovato; Benedetta Magrì; Alessandro Castorina; Grazia Maugeri; Velia D'Agata; Giuseppe Musumeci
Journal:  J Funct Morphol Kinesiol       Date:  2022-05-11

Review 2.  Genotoxic Damage During Brain Development Presages Prototypical Neurodegenerative Disease.

Authors:  Glen E Kisby; Peter S Spencer
Journal:  Front Neurosci       Date:  2021-12-02       Impact factor: 4.677

3.  Behavioral features in child and adolescent huntingtin gene-mutation carriers.

Authors:  Erin E Reasoner; Ellen van der Plas; Hend M Al-Kaylani; Douglas R Langbehn; Amy L Conrad; Jordan L Schultz; Eric A Epping; Vincent A Magnotta; Peggy C Nopoulos
Journal:  Brain Behav       Date:  2022-05-23       Impact factor: 3.405

  3 in total

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