MRI biomarkers in Huntington's disease

Abstract

Huntington's disease (HD) is a devastating neurodegenerative disease affecting the brain resulting in neuronal dysfunction and neuronal loss. Since the identification of the gene responsible for HD, genetic testing has become widely available, allowing for genetic status of persons at risk for HD to be determined. For the effective evaluation of future therapeutic trials a great need exists for sensitive biomarkers. In (premanifest) HD, MRI of the brain is one of the most logical candidates as a biomarker, as opposed to clinical measures, since brain neurons are the main target of the disease. These biomarkers can facilitate early detection of disease related changes, but are also needed to monitor disease progression from the premanifest phase of HD onwards. MRI derived parameters have this biomarker potential as they have been shown to identify brain abnormalities before symptom onset. In this review the available MRI techniques of conventional MRI, Diffusion Tensor Imaging, Magnetization Transfer Imaging, Magnetic Resonance Spectroscopy and Functional MRI will be discussed and the findings will be placed into context of different HD stages.