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New clues on motor neurone disease

Researchers from Neuroscience Research Australia, UNSW and the POW Hospital have found a quicker and more effective way to diagnose Motor Neurone Disease (MND).

MND, which recently claimed the life of artist Pro Hart, commonly affects people in their 50s. It is characterised by twitching of muscles, progressive muscle wasting and weakness, and ultimately complete paralysis. Around 1,400 people in Australia are estimated to have MND, with one Australian dying every day from the disease. Patients typically live for two to three years after diagnosis, although a small proportion lives for more than five years.

The diagnostic test, which was carried out at NeuRA, involves Transcranial Magnetic Stimulation (TMS), a painless technique that involves a magnetic coil being held to the patient’s head. The coil stimulates the underlying motor cortex, resulting in involuntary movement of the hand.

“At the moment it may take up to 14 months for a diagnosis, because there is no test,” said Dr Steve Vucic, a clinician undertaking PhD studies in the Faculty of Medicine at UNSW. “You have to exclude other medical disorders before you can conclusively say that someone has MND. Our work could change that.

“While no-one wants a diagnosis of MND, it is better to know earlier, so that the patient can be treated as soon as possible with the current recommended treatment, the drug Rilutek,” he said. “The data suggests that Rilutek can prolong life by as much as six to 12 months. At the moment, we can’t give it until there is a definitive diagnosis, but that can be too late.”

The research has just been published in the journal Muscle and Nerve.

“We are stimulating the nerve in the brain which then sends an impulse down to the muscle,” said Prof Matthew Kiernan, a neurologist at the POW Hospital.

“One of the things we found is that those with MND need less current to excite the muscle than those who don’t have the disease,” said Prof Kiernan. “That suggests the brain of the MND sufferer is primed to fire. The brain is more excited than it should be. These changes in the brain then appear to initiate the process of nerve death underlying the development of paralysis.”

“The technique we have described is painless, definitive and takes only a short amount of time,” he said. The co-authors on the paper are James Howells and Louise Trevillion, both from NeuRA of Clinical Neurosciences, University of Sydney and the Royal Prince Alfred Hospital. Motor Neurone Disease Research Institute of Australia funded the research.