Matthew Kiernan is an internationally renowned expert in neurodegenerative diseases, particularly motor neuron disease and frontotemporal dementia. He devised and introduced neurophysiological techniques that enable ion channel function to be studied in vivo in human subjects. His discoveries have delineated normal function and the mechanisms of disease and have identified therapeutic targets. He has defined diagnostic criteria for disease and established consensus guidelines, adopted globally by the World Federation of Neurology. Kiernan is implementing novel therapies based on these discoveries and using his techniques to measure responses to treatment, these are now being trialled in centres worldwide.