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From the Department of Neurology (Drs. Rosas, Koroshetz, and Cudkowicz, and Ms. Skeuse) and Center for Morphometric Analyses (Dr. Makris), Massachusetts General Hospital and Harvard Medical School, Boston; MGH-NMR Center (Drs. Rosas, Chen, Vangel, Makris, and Jenkins), Department of Radiology, Massachusetts General Hospital and Harvard Medical School, Charlestown; Institute for Neurogenetic Disorders (Dr. Marek, and Ms. Caplan), New Haven, CT; and Department of Psychiatry (Drs. Seidman and Goldstein), Massachusetts Mental Health Center and Massachusetts General Hospital, Institute for Psychiatric Epidemiology and Genetics, Harvard Medical School, Boston.
Address correspondence and reprint requests to Dr. H. Diana Rosas, Massachusetts General Hospital, Center for Genetics, Aging and Neurodegeneration, 114 16th Street, Suite 2000, Charlestown, MA 02114; e-mail: rosas{at}helix.mgh.harvard.edu
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.
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