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.
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.
Authors: Núria Martín-Flores; Joan Romaní-Aumedes; Laura Rué; Mercè Canal; Phil Sanders; Marco Straccia; Nicholas D Allen; Jordi Alberch; Josep M Canals; Esther Pérez-Navarro; Cristina Malagelada Journal: Mol Neurobiol Date: 2015-04-16 Impact factor: 5.590
Authors: Jorge Jovicich; Silvester Czanner; Xiao Han; David Salat; Andre van der Kouwe; Brian Quinn; Jenni Pacheco; Marilyn Albert; Ronald Killiany; Deborah Blacker; Paul Maguire; Diana Rosas; Nikos Makris; Randy Gollub; Anders Dale; Bradford C Dickerson; Bruce Fischl Journal: Neuroimage Date: 2009-02-20 Impact factor: 6.556
Authors: Christopher W Hess; Edward Ofori; Umer Akbar; Michael S Okun; David E Vaillancourt Journal: Curr Neurol Neurosci Rep Date: 2013-11 Impact factor: 5.081