Motor Neurone Disease (MND) over one year. MND is a fatal neurodegenerative disease which affects motor neurones in the brain and spinal cord. People with MND usually first notice weakness in the arms, legs (limb onset) or swallowing muscles (bulbar onset), which gets worse and eventually affects most of the muscles in the body. Some people with MND also develop Frontotemporal Dementia (FTD) and almost half may have mild cognitive problems. Recognition of non-motor impairment has increasingly led to the view of MND as a syndrome or multisystem disorder rather than a purely motor disorder. Whilst it is accepted that MND involves the degeneration of motor neurons, there is currently a debate over whether there is a dying forward (disease process starts in the brain neurones) or dying back process (disease process starts in the neuronal endings in the muscle). Furthermore the involvement of non-motor brain areas and mechanism of how the disease spreads in the brain has also not been determined. Brain imaging is a non-invasive way to detect structural and functional changes. Structural and functional brain imaging studies in MND have both shown abnormalities in clinically definite or clinically probable ALS (Turner et al., 2012). Brain imaging can help examine which brain changes are present before or after the limb weakness starts and track its progression. The aim of this project is to use a combination of brain imaging techniques to examine patients who have been characterised for motor, cognitive and behavioural symptoms in the early stages of the disease and follow the sequence of degeneration in the brain. Information from the Australian Motor Neurone Disease Registry (AMNDR) will also be obtained for registered patients. This study will address the issue of whether central changes are present early on in the brain in those with limb onset ALS and how these changes evolve over time, especially in the contralateral brain and non-motor regions. The design of the project will be a longitudinal study involving 20 patients with MND beginning in one limb scanned three times at 6 monthly intervals to track the onset progression of the disease in the brain. The methods of the project include using a combination of combination of structural (T1 weighted scans), diffusion tensor imaging (DTI), quantitative susceptibility mapping (QSM), magnetic resonance spectroscopy (MRS) and functional (resting-state fMRI (rsfMRI)) brain imaging methods and motor, behavioural and cognitive assessments in participants with limb onset ALS over one year.
Motor neurone disease, neuroimaging
School of Clinical Sciences at Monash Health / Hudson Institute of Medical Research
Monash Health Translation Precinct (Monash Medical Centre)