[Frontiers in Bioscience E4, 1910-1925, January 1, 2012]

MRI biomarkers in Huntington's disease

Simon van den Bogaard1, Eve Dumas1, Jeroen van der Grond2, Mark van Buchem2, Raymund Roos1

1Department of Neurology, Leiden University Medical Centre, Albinusdreef 2, 2300 RC Leiden, The Netherlands, 2Department of Radiology, Leiden University Medical Centre, Albinusdreef 2, 2300 RC Leiden, The Netherlands

TABLE OF CONTENTS

1. Abstract
2. Introduction
3. Biomarkers
4. Methods
5. Volumetric MRI
6. Diffusion tensor imaging
7. Magnetization transfer imaging
8. Magnetic resonance spectroscopy
9. Functional MRI
10. Discussion
11. Conclusion
12. References

1. 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.