Early changes in the hypothalamic region in prodromal Huntington disease revealed by MRI analysis

Abstract Huntington disease (HD) is a fatal neurodegenerative disorder caused by an expanded CAG repeat. Its length can be used to estimate the time of clinical diagnosis, which is defined by overt motor symptoms. Non-motor symptoms begin before motor onset, and involve changes in hypothalamus-regul...

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Bibliographic Details
Published in:Neurobiology of disease 2010-12, Vol.40 (3), p.531-543
Main Authors: Soneson, Charlotte, Fontes, Magnus, Zhou, Yongxia, Denisov, Vladimir, Paulsen, Jane S, Kirik, Deniz, Petersén, Åsa
Format: Article
Language:English
Subjects:
Adult
Atrophy
Basal ganglia
Basic Medicine
Early Diagnosis
Female
Heterozygote
Humans
Huntingtin
Huntington Disease - genetics
Huntington Disease - pathology
Hypothalamus
Hypothalamus - pathology
Imaging
Longitudinal Studies
Magnetic Resonance Imaging
Male
Medical and Health Sciences
Medicin och hälsovetenskap
Medicinska och farmaceutiska grundvetenskaper
Neuroendocrine
Neurology
Neurosciences
Neurovetenskaper
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Summary:Abstract Huntington disease (HD) is a fatal neurodegenerative disorder caused by an expanded CAG repeat. Its length can be used to estimate the time of clinical diagnosis, which is defined by overt motor symptoms. Non-motor symptoms begin before motor onset, and involve changes in hypothalamus-regulated functions such as sleep, emotion and metabolism. Therefore we hypothesized that hypothalamic changes occur already prior to the clinical diagnosis. We performed voxel-based morphometry and logistic regression analyses of cross-sectional MR images from 220 HD gene carriers and 75 controls in the Predict-HD study. We show that changes in the hypothalamic region are detectable before clinical diagnosis and that its grey matter contents alone are sufficient to distinguish HD gene carriers from control cases. In conclusion, our study shows, for the first time, that alterations in grey matter contents in the hypothalamic region occur at least a decade before clinical diagnosis in HD using MRI.
ISSN:0969-9961
1095-953X
DOI:10.1016/j.nbd.2010.07.013