Cerebral cortex structure in prodromal Huntington disease

Peggy C. Nopoulos, Elizabeth H. Aylward, Christopher A Ross, Hans J. Johnson, Vincent A. Magnotta, Andrew R. Juhl, Ronald K. Pierson, James Mills, Douglas R. Langbehn, Jane S. Paulsen

Research output: Contribution to journalArticle

Abstract

Neuroimaging studies of subjects who are gene-expanded for Huntington Disease, but not yet diagnosed (termed prodromal HD), report that the cortex is "spared," despite the decrement in striatal and cerebral white-matter volume. Measurement of whole-cortex volume can mask more subtle, but potentially clinically relevant regional changes in volume, thinning, or surface area. The current study addressed this limitation by evaluating cortical morphology of 523 prodromal HD subjects. Participants included 693 individuals enrolled in the PREDICT-HD protocol. Of these participants, 523 carried the HD gene mutation (prodromal HD group); the remaining 170 were non gene-expanded and served as the comparison group. Based on age and CAG repeat length, gene-expanded subjects were categorized as "Far from onset," "Midway to onset," "Near onset," and "already diagnosed." MRI scans were processed using FreeSurfer. Cortical volume, thickness, and surface area were not significantly different between the Far from onset group and controls. However, beginning in the Midway to onset group, the cortex showed significant volume decrement, affecting most the posterior and superior cerebral regions. This pattern progressed when evaluating the groups further into the disease process. Areas that remained mostly unaffected included ventral and medial regions of the frontal and temporal cortex. Morphologic changes were mostly in thinning as surface area did not substantially change in most regions. Early in the course of HD, the cortex shows changes that are manifest as cortical thinning and are most robust in the posterior and superior regions of the cerebrum.

Original languageEnglish (US)
Pages (from-to)544-554
Number of pages11
JournalNeurobiology of Disease
Volume40
Issue number3
DOIs
StatePublished - Dec 2010

Fingerprint

Huntington Disease
Cerebral Cortex
Genes
Corpus Striatum
Cerebrum
Frontal Lobe
Temporal Lobe
Masks
Neuroimaging
Magnetic Resonance Imaging
Control Groups
Mutation

Keywords

  • Cerebral cortex
  • Cortical thickness
  • Huntington disease
  • MRI
  • Surface area

ASJC Scopus subject areas

  • Neurology

Cite this

Nopoulos, P. C., Aylward, E. H., Ross, C. A., Johnson, H. J., Magnotta, V. A., Juhl, A. R., ... Paulsen, J. S. (2010). Cerebral cortex structure in prodromal Huntington disease. Neurobiology of Disease, 40(3), 544-554. https://doi.org/10.1016/j.nbd.2010.07.014

Cerebral cortex structure in prodromal Huntington disease. / Nopoulos, Peggy C.; Aylward, Elizabeth H.; Ross, Christopher A; Johnson, Hans J.; Magnotta, Vincent A.; Juhl, Andrew R.; Pierson, Ronald K.; Mills, James; Langbehn, Douglas R.; Paulsen, Jane S.

In: Neurobiology of Disease, Vol. 40, No. 3, 12.2010, p. 544-554.

Research output: Contribution to journalArticle

Nopoulos, PC, Aylward, EH, Ross, CA, Johnson, HJ, Magnotta, VA, Juhl, AR, Pierson, RK, Mills, J, Langbehn, DR & Paulsen, JS 2010, 'Cerebral cortex structure in prodromal Huntington disease', Neurobiology of Disease, vol. 40, no. 3, pp. 544-554. https://doi.org/10.1016/j.nbd.2010.07.014
Nopoulos, Peggy C. ; Aylward, Elizabeth H. ; Ross, Christopher A ; Johnson, Hans J. ; Magnotta, Vincent A. ; Juhl, Andrew R. ; Pierson, Ronald K. ; Mills, James ; Langbehn, Douglas R. ; Paulsen, Jane S. / Cerebral cortex structure in prodromal Huntington disease. In: Neurobiology of Disease. 2010 ; Vol. 40, No. 3. pp. 544-554.
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