Whole-brain atrophy as a measure of progression in premanifest and early Huntington's disease.
Therapeutic trials in Huntington's disease (HD) are challenging as clinical progression is slow and variable and reliable biomarkers are lacking. We used magnetic resonance imaging and the brain boundary shift integral to quantify whole-brain atrophy rates over 1 year in early and premanifest HD subjects, and controls. Early HD subjects had statistically significantly (P = 0.007) increased (threefold higher) rates of whole-brain atrophy compared with controls. Higher atrophy rates were associated with longer CAG repeat length. MRI-based measures of whole-brain atrophy may have potential as a measure of progression in HD.
Henley
Susie M D
SM
Dementia Research Centre, Institute of Neurology, National Hospital for Neurology and Neurosurgery, University College London, Queen Square, London, United Kingdom. shenley@drc.ion.ucl.ac.uk
Wild
Edward J
EJ
Hobbs
Nicola Z
NZ
Frost
Chris
C
MacManus
David G
DG
Barker
Roger A
RA
Fox
Nick C
NC
Tabrizi
Sarah J
SJ
D000328:Adult; D001284:Atrophy / Q000209:etiology / Q000473:pathology; D001921:Brain / Q000473:pathology*; D001931:Brain Mapping; D018450:Disease Progression; D006801:Humans; D006816:Huntington Disease / Q000150:complications / Q000473:pathology*; D008279:Magnetic Resonance Imaging / Q000379:methods; D008875:Middle Aged; D012720:Severity of Illness Index