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
2010 Neurobiology of Disease  
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
more » ... luded 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 geneexpanded 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
doi:10.1016/j.nbd.2010.07.014 pmid:20688164 pmcid:PMC2955824 fatcat:2ho6dgavbbcdja3wpyc6yayl5m