Evidence for more widespread cerebral pathology in early HD: an MRI-based morphometric analysis.
BACKGROUND
Most clinical symptoms of Huntington disease (HD) have been attributed to striatal degeneration, but extrastriatal degeneration may play an important role in the clinical symptoms because postmortem studies demonstrate that almost all brain structures atrophy.


OBJECTIVE
To fully characterize the morphometric changes that occur in vivo in HD.


METHODS
High-resolution 1.5 mm T1-weighted coronal scans were acquired from 18 individuals in early to mid-stages of HD and 18 healthy age-matched controls. Cortical and subcortical gray and white matter were segmented using a semiautomated intensity contour-mapping algorithm. General linear models for correlated data of the volumes of brain regions were used to compare groups, controlling for age, education, handedness, sex, and total brain volumes.


RESULTS
Subjects with HD had significant volume reductions in almost all brain structures, including total cerebrum, total white matter, cerebral cortex, caudate, putamen, globus pallidus, amygdala, hippocampus, brainstem, and cerebellum.


CONCLUSIONS
Widespread degeneration occurs in early to mid-stages of HD, may explain some of the clinical heterogeneity, and may impact future clinical trials.
Rosas
H D
HD
Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, USA. rosas@helix.mgh.harvard.edu
Koroshetz
W J
WJ
Chen
Y I
YI
Skeuse
C
C
Vangel
M
M
Cudkowicz
M E
ME
Caplan
K
K
Marek
K
K
Seidman
L J
LJ
Makris
N
N
Jenkins
B G
BG
Goldstein
J M
JM
D000328:Adult; D001284:Atrophy; D001921:Brain / Q000473:pathology*; D016022:Case-Control Studies; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009410:Nerve Degeneration; D013997:Time Factors