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Journal of Huntington's disease
2022;11 (2): 173-178.

Cortical Features in Child and Adolescent Carriers of Mutant Huntingtin (mHTT).

Erin E Reasoner, Ellen van der Plas, Douglas R Langbehn, Amy L Conrad, Timothy R Koscik, Eric A Epping, Vincent A Magnotta, Peggy C Nopoulos
Author Information
  1. Erin E Reasoner: Department of Psychiatry, University of Iowa Hospital and Clinics, Iowa City, IA, USA.
  2. Ellen van der Plas: Department of Psychiatry, University of Iowa Hospital and Clinics, Iowa City, IA, USA.
  3. Douglas R Langbehn: Department of Psychiatry, University of Iowa Hospital and Clinics, Iowa City, IA, USA.
  4. Amy L Conrad: Stead Family Department of Pediatrics, University of Iowa Hospital and Clinics, Iowa City, IA, USA.
  5. Timothy R Koscik: Department of Psychiatry, University of Iowa Hospital and Clinics, Iowa City, IA, USA.
  6. Eric A Epping: Department of Psychiatry, University of Iowa Hospital and Clinics, Iowa City, IA, USA.
  7. Vincent A Magnotta: Department of Psychiatry, University of Iowa Hospital and Clinics, Iowa City, IA, USA.
  8. Peggy C Nopoulos: Department of Psychiatry, University of Iowa Hospital and Clinics, Iowa City, IA, USA.
PMID: 35275555 DOI: 10.3233/JHD-210512

Abstract

BACKGROUND: Molecular studies provide evidence that mutant huntingtin (mHTT) affects early cortical development; however, cortical development has not been evaluated in child and adolescent carriers of mHTT.
OBJECTIVE: To evaluate the impact of mHTT on the developmental trajectories of cortical thickness and surface area.
METHODS: Children and adolescents (6-18 years) participated in the KidsHD study. mHTT carrier status was determined for research purposes only to classify participants as gene expanded (GE) and gene non-expanded (GNE). Cortical features were extracted from 3T neuroimaging using FreeSurfer. Nonlinear mixed effects models were conducted to determine if age, group, and CAG repeat were associated with cortical morphometry.
RESULTS: Age-related changes in cortical morphometry were similar across groups. Expanded CAG repeat was not significantly associated with cortical features.
CONCLUSION: While striatal development is markedly different in GE and GNE, developmental change of the cortex appears grossly normal among child and adolescent carrier of mHTT.

Keywords

Huntington’s disease children at risk for HD cortical development magnetic resonance imaging trinucleotide repeat disorder

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Grants

  1. S10 OD025025/NIH HHS
  2. R01 NS055903/NINDS NIH HHS
  3. P50 HD103556/NICHD NIH HHS
  4. /Wellcome Trust
  5. U01 NS055903/NINDS NIH HHS

MeSH Term

Adolescent
Child
Humans
Huntingtin Protein
Huntington Disease

Chemicals

HTT protein, human
Huntingtin Protein
Journal Article Research Support, Non-U.S. Gov't Research Support, N.I.H., Extramural

Word Cloud

Created with Highcharts 10.0.0corticalmHTTdevelopmentrepeatchildadolescentdevelopmentalcarriergeneGEGNECorticalfeaturesCAGassociatedmorphometryBACKGROUND:MolecularstudiesprovideevidencemutanthuntingtinaffectsearlyhoweverevaluatedcarriersOBJECTIVE:evaluateimpacttrajectoriesthicknesssurfaceareaMETHODS:Childrenadolescents6-18yearsparticipatedKidsHDstudystatusdeterminedresearchpurposesclassifyparticipantsexpandednon-expandedextracted3TneuroimagingusingFreeSurferNonlinearmixedeffectsmodelsconducteddetermineagegroupRESULTS:Age-relatedchangessimilaracrossgroupsExpandedsignificantlyCONCLUSION:striatalmarkedlydifferentchangecortexappearsgrosslynormalamongFeaturesChildAdolescentCarriersMutantHuntingtinHuntington’sdiseasechildrenriskHDmagneticresonanceimagingtrinucleotidedisorder

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