Journal
BIOLOGICAL PSYCHIATRY
Volume 59, Issue 1, Pages 57-63Publisher
ELSEVIER SCIENCE INC
DOI: 10.1016/j.biopsych.2005.06.003
Keywords
HD; imaging; neurodevelopment; neurodegeneration
Categories
Funding
- NATIONAL INSTITUTE OF MENTAL HEALTH [R01MH040856, R37MH031593, R01MH060990, R01MH031593] Funding Source: NIH RePORTER
- NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE [R01NS040068] Funding Source: NIH RePORTER
- NIMH NIH HHS [MH60990, MH031593, MH01579, MH40856] Funding Source: Medline
- NINDS NIH HHS [NS40068] Funding Source: Medline
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Huntington's disease (HD) is traditionally conceptualized as a degenerative disease of the striatum. Recent scientific advances, however, have suggested neurodevelopmental contributions and extrastriatal brain abnormalities. This study was designed to assess the morphology of the brain in participants who had previously undergone elective DNA analyses for the HD mutation who did not currently have a clincil diagnosis of HD (preclinical HD subjects). Methods: Twenty-four preclinical participants with the gene expansion for HD underwent brain magnetic resonance imaging and were compared wit ha group of 25 healthy control subjects, matched by gender and age. Results: Huntington's disease preclinical participants had substantial morphologic differences from controls throughout the cerebrum. Volume of the cerebral cortex was significantly increased in preclinical HD, whereas the basal ganglia and cerebral white matter volume were substantially decreased. Conclusion: In individuals with HD gene mutation who were considered healthy (Preclinical for manifest disease), the morphology of the brain is substantially altered compared with matched control subjects. Although decreased volumes of the striatum and cerebral white matter could represent early degenerative changes, the novel finding of enlarged cortex suggests that developmental pathology occurs in HD.
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