Neurologists have today demanded urgent research to work out whether playing contact sports causes motor neurone disease (MND).
Their desperate plea comes after rugby league star Rob Burrow yesterday revealed he has the crippling condition, aged just 37.
Studies have long suggested head trauma may play a role in the disease, which kills most sufferers within 18 months of being diagnosed.
But leading doctors and scientists have now told MailOnline they are ‘frustrated’ the exact cause has not yet been pinpointed.
They said ‘more work needs to be done’ to provide concrete evidence so sportsmen and women are aware of the risk.
Leeds Rhinos stalwart Burrow announced his retirement from rugby yesterday, after becoming the latest player to be diagnosed with the illness.
He is the latest in a long string of professional athletes to be struck down by MND, which is also known as ALS (amyotrophic lateral sclerosis).
Doddie Weir, 49 – a Scottish former rugby union player – was given the devastating diagnosis three years ago and continues to battle the disease.
And South African rugby star Joost Van Der Westhuiven died from MND in 2017, six years after being given the fatal prognosis.
Leeds Rhinos legend Rob Burrow announced his retirement from rugby this week after being diagnosed with motor neurone disease and given years to live
Burrow pictured in 2017 with wife Lindsey (left) and friends James and Charlotte Broughton
As well as their two young daughters (pictured left and right), the Burrows also have a son called Jackson
Former Scottish rugby union player Doddie Weir was given the devastating diagnosis three years ago and continues to battle the disease
The condition, which strikes one in 50,000 people and also killed Professor Stephen Hawking, robs patients of movement and speech.
Professor Gareth Miles, a neuroscientist based at the University of St Andrews, told MailOnline: ‘The feeling is that data out there clearly suggests head trauma in contact sports is a risk factor.
‘But I think more that needs to be done to clarify that.
‘So far they [the studies] have looked at whether people playing these sports are more or less likely to get the disease.
‘We need more of them. But if it [contact sports] is popping up as a risk factor then we need to work out why, and what exactly is causing it.
Professor Miles said that because MND is relatively rare there was a lack of urgency to study it.
But he added: ‘Hopefully Rob Burrow’s case will spur on these studies.’
South African rugby star Joost Van Der Westhuiven died from MND in 2017, six years after being given the fatal prognosis
Professional athletes from other sports have also been plagued with the debilitating disorder. Former Rangers footballer Fernando Ricksen died in September after a long battle with MND
About 1,200 rugby players suffer head trauma while playing the sport each year from tough tackles, and studies have shown them to be eight times more likely to develop MND.
But professional athletes from other sports have also been plagued with the debilitating disorder.
Former Rangers footballer Fernando Ricksen died in September after a long battle with MND. And ex-Liverpool right-back Stephen Darby was diagnosed with the disease in 2018 and given just years to live.
It is thought that concussions from heading the ball accumulate damage in the brain and eventually trigger MND. But with no definitive evidence, football associations have been left in limbo.
Some have taken precautionary measures – for instance the Scottish Youth Football Association has issued advice that under-11s shouldn’t head the ball to protect against brain damage.
Former head of the Scottish FA Gordon Smith warned in October that youngsters should practice with soft balls until their brains fully develop.
Dr Nicholas Cole, head of research at The Motor Neurone Disease Association, said: ‘It is one of our frustrations as an Association that we haven’t yet pinpointed the causes of MND.
‘We are currently funding around £16 million of research which we hope will lead to answers.
‘We do know a combination of environmental and lifestyle factors act together with specific genes to predispose people to get MND.
There is currently no known cure for motor neurone disease. The cruel condition killed Professor Stephen Hawking, pictured
Glial cells under a microscope are now said to be ‘key players’ in motor neurone disease
‘What we don’t know is the exact recipe of these factors that triggers onset of the disease.’
Previous research has suggested the MND is caused by an accumulation of proteins staying in a jelly-like state and killing off brain cells.
Professor Miles told MailOnline more studies are needed to delve into this – including postmortems that investigate differences in the brains of sufferers.
MND is a neurodegenerative disease that effects the muscles used for walking and talking. Patients have a life expectancy of about three to 10 years.
Half live for just 18 months, but the world-renowned physicist Hawking lived with the disease for more than five decades before his death in 2018.
The cause is not known, but scientific studies suggests that both genetics and environment play a role in the development.
It comes after Pete Frates, the former college baseball star whose battle with ALS inspired the Ice Bucket Challenge, died last week at the age of 34.
WHAT IS MOTOR NEURONE DISEASE (ALS)?
History
The NHS describes motor neurone disease (MND) as: ‘An uncommon condition that affects the brain and nerves. It causes weakness that gets worse over time.’
The weakness is caused by the deterioration of motor neurons, upper motor neurons that travel from the brain down the spinal cord, and lower motor neurons that spread out to the face, throat and limbs.
It was first discovered in 1865 by a French neurologist, Jean-Martin Charcot, hence why MND is sometimes known as Charcot’s disease.
In the UK, Amyotrophic Lateral Sclerosis (ALS) is referred to as Motor Neurone Disease, while in the US, ALS is referred to as a specific subset of MND, which is defined as a group of neurological disorders.
However, according to Oxford University Hospitals: ‘Nearly 90 per cent of patients with MND have the mixed ALS form of the disease, so that the terms MND and ALS are commonly used to mean the same thing.’
Symptoms
Weakness in the ankle or leg, which may manifest itself with trips or difficulty ascending stairs, and a weakness in the ability to grip things.
Slurred speech is an early symptom and may later worsen to include difficulty swallowing food.
Muscle cramps or twitches are also a symptom, as is weight loss due to leg and arm muscles growing thinner over time.
Diagnosis
MND is difficult to diagnose in its early stages because several conditions may cause similar symptoms. There is also no one test used to ascertain its presence.
However, the disease is usually diagnosed through a process of exclusion, whereby diseases that manifest similar symptoms to ALS are excluded.
Causes
The NHS says that MND is an ‘uncommon condition’ that predominantly affects older people. However, it caveats that it can affect adults of any age.
The NHS says that, as of yet, ‘it is not yet known why’ the disease happens. The ALS Association says that MND occurs throughout the world ‘with no racial, ethnic or socioeconomic boundaries and can affect anyone’.
It says that war veterans are twice as likely to develop ALS and that men are 20 per cent more likely to get it.
Treatment
There is no cure for MND and the disease is fatal, however the disease progresses at different speeds in patients.
People with MND are expected to live two to five years after the symptoms first manifest, although 10 per cent of sufferers live at least 10 years.
Occupational therapy, physiotherapy and medicines such as riluzole are used to palliate the effects of the the disease.
Lou Gehrig was a hugely popular baseball player, who played for the New York Yankees between 1923 and 1939. He was famous for his strength and was nicknamed ‘The Iron Horse’
Lou Gehrig’s Disease
As well as being known as ALS and Charcot’s disease, MND is frequently referred to as Lou Gehrig’s disease.
Lou Gehrig was a hugely popular baseball player, who played for the New York Yankees between 1923 and 1939.
He was famous for his strength and was nicknamed ‘The Iron Horse’.
His strength, popularity and fame transcended the sport of baseball and the condition adopted the name of the sportsman.
He died two years after his diagnosis.
Pete Frates, the former college baseball star whose battle with ALS inspired the Ice Bucket Challenge, has passed away at the age of 34, his family announced Monday
In 2014 he launched the ALS Bucket Challenge, a viral sensation in which people dumped buckets of ice on themselves, donated to ALS research, and nominated friends to do the same. The challenge raised a whopping $200million worldwide. Frates pictured above participating in the challenge surrounded by his family
Last week University of St Andrews scientists moved one step closer to finding a cure for the condition.
Researchers found that another type of brain cell – thought to be harmless – could play a role in the disease.
Tests of cells from skin samples of patients with MND showed glial cells can damage motor neurones.
They found glial cells from MND patients caused motor neurones of healthy people to stop producing the electrical signals needed to control muscles.
Aaron Hernandez had the most severe CTE case ever seen: Scientists found 27-year-old’s brain tissue showed extreme damage to memory and impulse control
by Mia De Graaf, Health Editor
The neuroscientist who analyzed Aaron Hernandez’s brain confirmed that he suffered the worst case ever seen in someone so young, with severe damage to regions that affect memory, impulse control and behavior.
The 27-year-old former New England Patriots player killed himself in April 2017 while serving life in prison for murder.
In September, Dr Ann McKee of the CTE Center at Boston University posthumously diagnosed Hernandez with chronic traumatic encephalopathy, a football-linked disease that causes dementia and aggression.
Aaron Hernandez was a star tight end for the New England Patriots on a $41 million contract when he was arrested for double murder. in June 2013. Four years later, while serving life in prison, he took his own life. He was posthumously diagnosed with the worst case of CTE ever seen in a football player
She formally presented her findings a month later, and confirmed that she had never encountered such extreme degradation in a young brain, pointing out areas of severe tissue damage and microbleeds likely caused by blows to the head.
They also found a variant of the APOE gene, which has been linked to increased risk of Alzheimer’s, but the scientists emphasized that no gene could inflict the same damage as years of heavy impact from tackling.
Dr McKee says she could not say for certain that Hernandez’s criminal and suicidal acts were a result of his severe case of CTE, nor whether other 27-year-old players could plausibly have the same pathology. But she says Hernandez suffered substantial damage to several important regions, including the frontal lobe.
‘In this age group, he’s clearly at the severe end of the spectrum,’ McKee said.
‘There is a concern that we’re seeing accelerated disease in young athletes. Whether or not that’s because they’re playing more aggressively or if they’re starting at younger ages, we don’t know. But we are seeing ravages of this disease, in this specific example, of a young person.’
Hernandez was diagnosed with stage three out of four, with four being the most severe.
This picture shows a scan of Hernandez’s brain compared to that of a normal 27-year-old. It shows severe decay in the center of his brain from CTE
His brain scans reveal huge clumps of tau protein in Hernandez’s frontal lobes, and in the nerve cells around small blood vessels, a unique feature of CTE.
These proteins, also seen in dementia, disrupts the normal functioning of the brain, triggering aggressiveness, explosiveness, impulsivity, depression, memory loss and other cognitive changes.
Dr McKee cautioned that she has not received many brains of players so young who played to such a high level as Aaron Hernandez, who started playing before the age of eight and was regarded as one of the NFL’s top tight ends.
The tests showed Hernandez had early brain atrophy and large perforations in the septum pellucidum, a central membrane which is essential to control behavior.
Hernandez’s brain scans revealed he had a variant of the APOE gene, which has been linked to neurodegenerative diseases like Alzheimer’s, a similar disease to CTE. However, CTE uniquely affects certain nerve cells which Alzheimer’s appears not to.
Hernandez was indicted in August 2013 (pictured). He was convicted of first-degree murder, and sentenced to life in prison in April 2015
The disgraced star had a $41 million NFL contract when he was arrested at his home in June 2013 and charged with the murder of a semi-pro football player Odin Lloyd.
Lloyd was the boyfriend of Hernandez’s fiancee’s sister. He was found dead in an industrial park on June 17, 2013, riddled with bullets. Surveillance footage showed Hernandez at the scene an hour before, then arriving at home minutes after gunshots were fired.
In April 2015, Hernandez was convicted of first-degree murder, and sentenced to life in prison.
While in prison, Hernandez was charged with another killing – a double murder committed by a drive-by shooting. But in April 2017, he was acquitted of both charges.
The next day, he took his own life.
His family has since filed a lawsuit against the NFL and the Patriots on behalf of Hernandez’s four-year-old daughter Avielle, claiming the club and the league knew about the connections between football and CTE long before Hernandez was drafted.
