A Multi-Modal Quantitative Analysis of Neurodegeneration in Spinocerebellar Ataxia Type 1 using Magnetic Resonance Imaging and Spectroscopy

Abstract

Spinocerebellar Ataxia Type 1 (SCA1) is a hereditary neurodegenerative disorder that is predominantly characterized by degeneration of the cerebellum and the brainstem. Symptoms of SCA1 include worsening gait and a progressive loss of motor coordination, as well as a host of other symptoms. Magnetic resonance imaging (MRI) and spectroscopy (MRS) offer a means to measure the structural and chemical changes that occur in the brain in a reliable, quantitative, and non-invasive manner. This thesis aims to use multiple modalities of magnetic resonance, including MRS, structural MRI, and diffusion MRI, in order to quantitatively understand how the chemical, macrostructural, and microstructural characteristics of the brain are affected in SCA1. The information obtained from each of these modalities will then be used to outline a model of SCA1 that aims to describe the process of neurodegeneration from the cellular level, to the structural level, and finally to the symptomatic level. The results from this thesis may prove useful in understanding the process of neurodegeneration in SCA1, as well as offering a means of evaluating brain pathology for future clinical trials.