Matthew Kiernan first Australian to receive Sheila Essey Award

The American Academy of Neurology, The ALS Association and the American Brain Foundation have awarded the 2022 Sheila Essey Prize to highly cited University of Sydney neurologist Professor Matthew Kiernan.

Professor Kiernan, from the Faculty of Medicine and Health and the Brain and Mind Centre is recognised as a world-leading expert on amyotrophic lateral sclerosis (ALS), having dedicated his career to research that improves the lives of people affected by this progressive neurodegenerative disease.

ALS, also known as Motor Neuron Disease (MND) in Australia, currently has no cure. The average lifespan after diagnosis is two to five years, and it can take up to two years to diagnose the disease effectively.

Professor Kiernan’s applied research, undertaken with ALS patients and their families, includes discoveries of the mechanisms behind the disease and its progression and translation into clinical care, earlier diagnosis and better treatments.

His team has identified new disease biomarkers – the early signs of disease occurring in the brain before physical symptoms emerge – and established guidelines for diagnosis.

Professor Kiernan is a strong advocate for access to clinical trials, with key support from the federal government, Health Minister Greg Hunt and philanthropic supporters, particularly AFL personality Neale Daniher and FightMND. In a collaboration with the University of Queensland, Monash University, Queensland University of Technology and in partnership with MND Australia, his team released a patient and clinical trial registry to empower patients and families with access to research and clinical trial opportunities.

Accepting the award, Professor Kiernan said discoveries made in the progression and diagnosis for the disease have brought researchers ever – closer to better treatments and potential for a cure.
“I had a personal interest in ALS because it was a disease which seemed impossible to treat. It was first described 150 years ago and even at the start of my career was presented to me as a neuromuscular condition, with muscle wasting. We have come a long way.
“In recent years we have come to understand ALS primarily as a brain disease – as we continue to uncover the underlying mechanisms of ALS, it is increasingly possible to come up with effective therapies.
“I want to be part of a generation that unravels the mysteries of ALS and discovers effective treatments for patients suffering from this fatal disease,” he said.
“We need to improve outcomes for patients living with the disease today, while also providing insights to future research.”

His research team found that ALS may begin in the motor cortex, the part of the brain where muscular activity is initiated. They discovered hyperexcitability in this area of the brain before symptoms of muscle wasting appear. This discovery has led to new treatment options.

Professor Kiernan notes by identifying brain abnormalities, treatments for ALS may be started earlier and have a greater chance of rescuing diseased nerve cells.

Vice-Chancellor and Principal Professor Mark Scott said, “This focus on research translation and collaboration is a hallmark for research at the University of Sydney.

“Congratulations to Professor Kiernan for his dedication and achievement – this recognition is a testament to his important work improving outcomes for patients today, while laying down insights for future discovery.”
The Sheila Essey Award for ALS Research is presented to individuals who make significant contributions in the search for effective treatments and cures for ALS. The award is presented with a $50,000 prize to further recipients’ research. Professor Kiernan said it was an honour to be the first Australian to receive this award.

“This Award is shared with ALS patients and their families who have attended our research clinic and driven my research. I have been the beneficiary of support from generous teachers, family and friends throughout this journey,” he said.
“Thanks to the AAN, the ALS Association and the Essey family for supporting ALS research over many years.”
Professor Kiernan will present the ALS and Motor Neuron Disorders session at the American Academy of Neurology Annual Meeting in Seattle on Monday 4 April, and at the American Brain Foundation’s Commitment to Cures event on Wednesday 6 April.

Professor Kiernan is the Bushell Chair of Neurology, Editor in Chief for the prestigious Journal of Neurology, Neurosurgery and Psychiatry, President of the Brain Foundation and a member of the American Academy of Neurology.