Project - The distinct neural signatures of motor neuron disease
All Chief investigators and associate investigators
Supervisors (PI): Professor Matthew Kiernan (University of Sydney) and Professor Martin Turner (University of Oxford)
Research Project Abstract
Motor neuron disease (MND) is a progressive neurodegenerative disease without effective treatment, which significantly shortens life expectancy for the majority of sufferers. Marked variability of clinical disease patterns in MND have been major barriers to identifying successful disease-modifying therapies, which has highlighted an urgent need to better understand disease mechanisms and develop robust markers of disease activity.
Our aim is to characterise the evolution of MND from its earliest stages and understand patterns of disease spread using a multimodal, clinically translational approach across two world leading centres in ALS research. The initial two years of this 4-year National Health and Medical Research Council (NHMRC) funded project is currently being undertaken at the University of Oxford (United Kingdom), where we are exploring state-of-the-art advanced neuroimaging techniques to obtain a detailed analysis of ALS brain networks in vivo. This will be integrated with comprehensive neurophysiological and clinical assessments to define the key functional, structural, and metabolic neuronal signatures associated with distinct clinical subgroups of the disease, and to help identify unique biomarkers of disease and progression.
Disease area:
MND
Challenges within the field
The challenges in MND critically revolve around the lack of highly effective treatment options or standardised biomarkers of disease activity, which is testament to the complexity and heterogeneity of this syndrome.
Our research attempts to tackle these difficulties by using advanced multi-modal techniques to gain a greater understanding of disease mechanisms across the different subtypes of MND. This will help us identify the earliest changes that occur in the affected motor pathways and understand how the disease spreads, in order to help identify potential drug targets and disease markers.
Research Objectives
- Develop an advanced multimodal clinical pipeline to explore affected motor pathways in MND patients in vivo and gain detailed insight into disease mechanisms;
- Explore the earliest changes of disease and track longitudinal patterns of spread across MND subtypes along these distinct brain networks;
- Neuroanatomically evaluate clinical and prognostic risk factors and develop unique biomarkers of disease progression and prognosis.