Motor Neurone Disease researcher wins prestigious award

A Motor Neurone Disease researcher at NeuRA is the first Australian to win a prestigious award granted by the international neuroscience community for his work on the pathology of the disease.

Dr Steve Vucic, a member of the Kiernan Group, has been awarded the M.A.B. Brazier Young Investigator Award by the International Federation of Clinical Neurophysiology for his study, ‘Corticomotoneuronal function in asymptomatic SOD-1 mutation carriers’.

“We wanted to look at whether nerves were dying in people with Motor Neurone Disease before there was any evidence of clinical disease, such as weakness or muscle twitching,” says Dr Vucic. “If this is the case, then you would want to treat them as early as possible.”

Motor neurone disease (MND) is a fatal neurodegenerative disease that causes rapidly progressive muscle weakness. Specifically, the disease affects the nerves between the brain and spinal cord (motor neurons) that control the muscles that enable you to move, speak, breathe and swallow.

Vucic and colleagues looked at a group of people with a familial (genetic) form of MND. This group of people had a particular genetic mutation called SOD-1, the most common known mutation that causes MND, but had yet to develop clinical symptoms of the disease.

The team looked at the structure and function of the nerves that connect the brain to the spinal cord, called corticomotoneurons, which are affected by the disease.

They looked at both the anatomy of the nerves, using magnetic resonance imaging (MRI) to detect any nerve loss, as well as how well the nerves were working, using a technique developed at NeuRA called threshold tracking transcranial magnetic stimulation.

“This is the first study of its kind to use two techniques to look at the integrity of the corticomotoneurons,” says Dr Vucic.

What they found was that both the anatomical and functional integrity of the nerves in people who have yet to develop outward symptoms of the disease was normal.

“What this means is that people with familial MND should be closely followed, with testing every 6 to 12 months. As soon as there are any changes in the tests, then we should start giving neuroprotective treatments, but not before.”

Dr Vucic says the study also demonstrates the validity of using their novel technique for measuring nerve function as a diagnostic tool, both for familiar and sporadic MND.

The M.A.B. Brazier Young Investigator Award is awarded once every four years. Dr Vucic will receive an award of US$3,500 and has been invited to present his work in Kobe, Japan later this year at the International Congress of Clinical Neurophysiology.

‘Corticomotoneuronal function in asymptomatic SOD-1 mutation carriers’ is published in Clinical Neurophysiology.