Yorkshire scientists in new front line of the battle against a cruel disease

She first came across MND as a trainee neurologist more than 20 years ago, since when she has dedicated her research to trying to develop new, effective treatments and therapies to combat this dreadful condition. The new 12m Sheffield Institute for Translational Neuroscience (SITraN), which the Queen officially opens today and which Professor Shaw will lead, is a major step towards that goal, bringing together some of the world's top neurological scientists and researchers. For the 6,000 people in the UK suffering from the disease, as well as those yet to be diagnosed, it offers the hope that one day a cure can be found.

MND is a relentless disease that grinds down patients as it attacks the body's motor neurones, causing muscles to waste. There is no known cure and more than half of all sufferers will die, immobile and unable to communicate, less than two years after diagnosis. It has affected David Niven, Don Revie and Stephen Hawking, who has defied statistics by living with the disease for more than 35 years.

It is estimated that about 85 per cent of sufferers will not live longer than five years with MND. Shaun Keane, a happily married father-of-three from Hull, was 37 years old when he was diagnosed with MND in 2007. His father, Stuart, says the nightmare started when his son Peter gave Shaun a lift one day and noticed something was wrong. "He said Shaun was slurring his speech and dragging his foot and he thought he was having a stroke, so he took him to A and E." He was kept in hospital for two weeks while he underwent a series of tests before being told by doctors he had Motor Neurone disease.

"As a family we couldn't believe it and when we went online and found out there was no known cure and read about the horrendous forecast for sufferers, it devastated us," says Stuart.

In a desperate hope that there had been a mistake Shaun went for a second opinion at the Royal Hallamshire Hospital, in Sheffield. But further tests confirmed the dreadful diagnosis. "After that Shaun went for bimonthly check ups and as a family we're so grateful for the care and humanity that Professor Shaw showed."

In most cases MND occurs as a one-off incident in a family and Stuart says there was nothing to indicate his son, who ran his own ice cream business, might develop the disease.

"He always played sport as a kid, he was very fit and he didn't drink or smoke." However, his condition deteriorated quickly. "The first thing to go was his walking, he was forced to use a zimmer frame and by the end of the year he was unable to drive. Then his arms started to go and his hands."

Throughout 2008 the disease whittled away Shaun's quality of life. He struggled to get out of a chair and basic activities like eating and using the toilet became increasingly difficult. Despite the grim outlook, his father says he fought the illness stoically. "He was always very positive even though he had one of the most severe forms of the disease. He had an incredible attitude and his favourite saying was, 'you're not properly dressed until you're wearing a smile.' He never once asked 'why me?' He was always full of encouragement to other sufferers. He would say to them, 'concentrate on what you can do and don't dwell on what you can't.'"

Despite this courage his condition worsened. "It affected his ability to speak and the only way he could communicate was through a machine by tapping a switch with his left foot that was connected to a computer because that was the only movement he had," his father says.

"He had to rely on everybody else, although his wife Amanda and his three daughters were absolutely fantastic. But it's a terrible disease, you become a virtual prisoner in your own body."

Shaun was 40 when he finally succumbed to the disease earlier this year.

"He went very quickly in the end. I drove over to Hull to see him and he was still conscious and after half an hour he died. He kept fighting it but it was a relief that he went quickly." Stuart believes the new institute, based at Sheffield University, has a crucial role to play in combating MND.

"Shaun knew anything that might prolong the life of sufferers, or even a cure, would come too late for him. But what this does is it gives people hope where there was no hope."

Irene Beard, a patron of the Sheffield Institute Foundation for Motor Neurone Disease, has lived with MND for more than six years. "In December 2003, I was running to catch a flight at Heathrow and I noticed I couldn't run like I used to, which I thought was odd," she says. However, it wasn't until March the following year that she realised something was seriously wrong. "I was driving my car and suddenly I was unable to brake, my foot wouldn't work. I got into a panic and managed to pull over and I called my husband and he came to pick me up."

Initially, she went to see a physiotherapist but was referred to a neurological expert who diagnosed MND. "He was honest with me, he said it was a difficult disease, that my life would never be the same again and I probably had two years left to live."

Such devastating news can break some people's spirit, but after the initial shock Irene was determined to remain positive. "I said to myself, 'everybody has their journey through life and I've had a wonderful life and this is just part of it.' I thought if I only have two years I'm not going to worry about it, I'm going to enjoy whatever time I have left."

The institute has been funded by Sheffield University and a group of patrons and Irene, who has been one of the key fundraisers, is delighted to see it finally come to fruition. "I was lucky to have a slowly progressing form of MND and, seven years on, I have cheated that original forecast by five years, so it is with joy that I see the opening of SITraN within my lifetime which will now give hope to today's sufferers of MND and those who are yet to be diagnosed."

The new research centre is the first dedicated institute for Motor Neurone Disease in Europe and its focus will be to solve what Prof Shaw calls a "complex disease." The number of researchers will grow from 90 to 150 over the next few years allowing more extensive and faster research, with teams working on four core, inter-linking programmes.

"One has to be cautious in making any confident predictions," says Prof Shaw, "but the tools of science are there to make real progress in understanding the causes of Motor Neurone Disease. It will probably be step-by-step progress rather than a Eureka moment, but I think we will make significant progress over the next five to 10 years."

To find out more about the institute, MND, or to make a donation to the ongoing support of SITraN log on to www.sifoundation.com

Motor neurone disease facts

More than 6,000 people in the UK suffer from Motor Neurone Disease (MND).

It is an incurable disease which destroys the cells that control movement causing progressive disability.

Sufferers may lose their ability to walk, talk, eat and breathe.

1,200 new patients are diagnosed with MND each year.

It is most common between the ages of 50 and 70.

Only 10-15 per cent of people survive for longer than five years with MND.

The exact cause remains unknown but about 95 per cent of cases occur as a one-off condition within a family.

Early symptoms include clumsiness, a weak grip, difficulty walking and slurred speech.