Amyotrophic lateral sclerosis (ALS) / Motor Neuron Disease (MND) is a progressive neurodegenerative condition characterised by upper and lower motor neuron degeneration, typically manifested as progressive muscle weakness affecting arms, legs, neck and diaphragm. In addition to the to the impairments that lead to muscle weakness, ALS also affects sensory and cognitive function in the brain. The cognitive impairment is a huge burden for both patients and caregivers of ALS, and therefore is important to characterise and quantify for treatment. This cognitive impairment can be monitored and analysed using brain scanning methods, such as electroencephalogram (EEG), magnetoencephalography (MEG) and MRI. EEG/MEG measure the functional activity in the brain network, and despite MRI can record the direct electrical activity of the brain. EEG is sensitive and can reflect cognitive network dysfunction in the absence of visible cognitive symptoms, and lead us to identifying quantifiable cognitive biomarkers. Quantitative biomarkers allow accurate measurements for clinical diagnosis and for testing and finding effective drugs. In this research we aim to distinguish and dissociate the sensory and cognitive impairments observed in ALS, using a cognitive EEG paradigm called mismatch negativity (MMN) and advanced analyses, and to find separate quantitative biomarkers for non-motor cognitive and sensory processing. The development of quantifiable EEG biomarkers of network disruption, can then be harnessed for measuring cognitive and sensory decline in clinical settings and in trials of new drugs. It can therefore lead to improved remedies for cognitive dysfunction in ALS and eventually improve the quality of life.
