Cortical neurodevelopment in pre-manifest Huntington's disease

• Verfasst von: Kubera, Katharina Maria [VerfasserIn]
• Verfasst von: Schmitgen, Mike [VerfasserIn]
• Verfasst von: Hirjak, Dusan [VerfasserIn]
• Verfasst von: Wolf, Robert Christian [VerfasserIn]
• Titel: Cortical neurodevelopment in pre-manifest Huntington's disease
• Verf.angabe: Katharina M. Kubera, Mike M. Schmitgen, Dusan Hirjak, Robert Christian Wolf, Michael Orth
• E-Jahr: 2019
• Jahr: 29 June 2019
• Umfang: 7 S.
• Fussnoten: Gesehen am 29.11.2019
• Titel Quelle: Enthalten in: NeuroImage: Clinical
• Ort Quelle: [Amsterdam u.a.] : Elsevier, 2012
• Jahr Quelle: 2019
• Band/Heft Quelle: 23(2019) Artikel-Nummer 101913, 7 Seiten
• ISSN Quelle: 2213-1582
• DOI: doi:10.1016/j.nicl.2019.101913
• Datenträger: Online-Ressource
• Sprache: eng
• Sach-SW: CAT12
• Sach-SW: Cortex
• Sach-SW: Huntington
• Sach-SW: MRI
• Sach-SW: Neurodevelopment
• Sach-SW: Striatum
• K10plus-PPN: 1683806646

Abstract

Background - The expression of the HTT CAG repeat expansion mutation causes neurodegeneration in Huntington's disease (HD). Objectives: In light of the - mainly in-vitro - evidence suggesting an additional role of huntingtin in neurodevelopment we used 3T MRI to test the hypothesis that in CAG-expanded individuals without clinical signs of HD (preHD) there is evidence for neurodevelopmental abnormalities. - Methods - We specifically investigated the complexity of cortical folding, a measure of cortical neurodevelopment, employing a novel method to quantify local fractal dimension (FD) measures that uses spherical harmonic reconstructions. - Results - The complexity of cortical folding differed at a group level between preHD (n=57) and healthy volunteers (n=57) in areas of the motor and visual system as well as temporal cortical areas. However, there was no association between the complexity of cortical folding and the loss in putamen volume that was clearly evident in preHD. - Conclusions - Our results suggest that HTT CAG repeat length may have an influence on cortical folding without evidence that this leads to developmental pathology or was clinically meaningful. This suggests that the HTT CAG-repeat expansion mutation may influence the processes governing cortical neurodevelopment; however, that influence seems independent of the events that lead to neurodegeneration.