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In vivo evidence of cerebellar atrophy and cerebral white matter loss in Huntington disease

From the Veterans Affairs San Diego Healthcare System (Drs. Fennema-Notestine, Jacobson, and Jernigan, and S.L. Archibald), CA; Laboratory of Cognitive Imaging, Department of Psychiatry (Drs. Fennema-Notestine, Jacobson, and Jernigan, and S.L. Archibald), Department of Neurosciences (Drs. Corey-Bloom, Peavy, Gamst, Hamilton, and Salmon), and Division of Biostatistics (Dr. Gamst), University of California, San Diego, La Jolla; and Departments of Psychiatry and Neurology (Dr. Paulsen), The University of Iowa, Iowa City.

Address correspondence and reprint requests to Dr. Christine Fennema Notestine, Laboratory of Cognitive Imaging (9151-B), University of California, San Diego, 9500 Gilman Drive MC 9151-B, La Jolla, CA 92093; e-mail: fennema{at}ucsd.edu

Objective: To investigate the regional pattern of white matter and cerebellar changes, as well as subcortical and cortical changes, in Huntington disease (HD) using morphometric analyses of structural MRI.

Methods: Fifteen individuals with HD and 22 controls were studied; groups were similar in age and education. Primary analyses defined six subcortical regions, the gray and white matter of primary cortical lobes and cerebellum, and abnormal signal in the cerebral white matter.

Results: As expected, basal ganglia and cerebral cortical gray matter volumes were significantly smaller in HD. The HD group also demonstrated significant cerebral white matter loss and an increase in the amount of abnormal signal in the white matter; occipital white matter appeared more affected than other cerebral white matter regions. Cortical gray and white matter measures were significantly related to caudate volume. Cerebellar gray and white matter volumes were both smaller in HD.

Conclusions: The cerebellum and the integrity of cerebral white matter may play a more significant role in the symptomatology of HD than previously thought. Furthermore, changes in cortical gray and cerebral white matter were related to caudate atrophy, supporting a similar mechanism of degeneration.

Received July 3, 2003. Accepted in final form May 14, 2004.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the September 28 issue to find the title link for this article.

Presented in part at the annual meeting of the American Academy of Neurology, April 2000.

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