Lou Gehrig's disease - Amyotrophic lateral sclerosis

[waltky]

Granny says eat yer fruits an' vegetables...

Eating Colorful Produce May Help Prevent ALS
February 04, 2013 - Amyotrophic lateral sclerosis, better known as ALS, is a rare but devastating neuromuscular condition with no known cure which is nearly always fatal.

However, a new study in the Annals of Neurology suggests preventing the paralyzing disease might be as simple as eating a diet of brightly-colored fruits and vegetables. ALS is believed to result, in part, from damage to motor neurons in the brain and spinal cord. For reasons not fully understood, corrosive oxygen molecules, called free radicals, overwhelm the body's cell maintenance and repair systems, inflicting irreversible damage on the nerve cells, causing progressive, and eventually total, paralysis.

Eating brightly-colored fruit and vegetables, such as red peppers, carrots and kale, which are rich in anti-oxidants, may head off or slow the development of ALS, according to Alberto Ascherio, professor of nutrition at Harvard University's School of Public Health in Boston, Massachusetts. Bright red, orange and green produce contains natural compounds known as carotinoids, in particular beta-carotene and lutein, which are known to counter the harmful effects of oxidative stress. “So, if it is true that carotinoids could reduce the risk, there is the possibility that it could also slow the disease process in people who have the disease; we don’t know that yet," Ascherio says. "But we are trying to find clues about things that may help people with the disease.”

Eating brightly-colored fruit and vegetables, which are rich in anti-oxidants, may help prevent or slow the development of ALS, according to a new study.

Ascherio and his colleagues analyzed data on one million people who took part in five large studies which tracked changes in their health as they aged. Information on the volunteers came from the National Institutes of Health’s Diet and Health study, the Cancer Prevention Study, the Multi-ethnic Cohort, the Health Professionals Follow-Up Study and the Nurses’ Health Study. About 1,000 of the participants eventually developed ALS. “We found that the group of people with the highest level of carotenoid intake had about a 25 percent lower risk of developing and dying of ALS,” Ascherio says.

Researchers also found that study participants who consumed the most carotenoid-rich foods tended to exercise more and eat foods containing a lot of vitamins C and E. However, investigators found that those vitamins did not reduce the risk of ALS. ALS is also known as Lou Gehrig's Disease, after the famed American baseball player who was diagnosed with the disease in 1939.

Source

 

[Politico]

Doctors are clueless.If you're going to get it you're going to get it.

 

[waltky]

ALS ice bucket challenge goes horribly wrong...

Firefighters still in hospital after ice stunt
August 22, 2014 - CAMPBELLSVILLE, Ky. (AP) — Two central Kentucky firefighters are still hospitalized a day after they were shocked by a power line while helping out with an "ice bucket challenge."

Campbellsville Fire Capt. Tony Grider was in critical condition in the University of Louisville Medical Center's burn unit Friday afternoon, a hospital spokesman said. Simon Quinn was in fair condition there. Both men were on the fire truck's ladder when it got too close to a power line after dumping water on Campbellsville University's marching band on Thursday in the charity stunt to raise awareness for the disease ALS. Campbellsville Fire Chief Kyle Smith told WDRB-TV (Accident that injured Campbellsville firefighters demonstrates p - WDRB 41 Louisville News on Friday that Grider suffered burns on 55 percent of his body and was sedated. Two other firefighters were hurt, but they are out of the hospital.

Campbellsville Police Chief Tim Hazlette said the power line was never touched Thursday morning, but it carried such a high voltage that it was able to energize the ladder truck, shocking the firefighters. Most of the students had already left the area and no students were hurt. University nursing student Julie Smith said she was nearby and spoke to a couple of friends who saw the firefighters being shocked and said "they are taking it really hard." "It's tragic, I feel for all the band members who were still there when it happened, that they had to see that," Smith said. Smith later led a prayer vigil with about 100 people on the campus Thursday night.

A Campbellsville Fire Department truck with the ladder extended remained at the scene where two firefighters were injured during an ice bucket challenge during a fundraiser for ALS on Thursday, Aug. 21, 2014, in Campbellsville, Ky. Officials say the ladder got too close to a power line and electricity traveled to the ladder, electrocuting the firefighters.

Taylor County Judge-Executive Eddie Rogers said Friday that the community was still reeling from the accident. "We never know when something like this is going to happen," he said. "Everybody's heartbroken over it." Power was knocked out for about an hour to 4,500 customers, including the school, said Natasha Collins, a spokeswoman for Kentucky Utilities, which owns the line. The ice bucket challenge has been sweeping social media websites. The ALS Association said it has raised more than $41 million.

Firefighter Keith Bricken, who has worked with Grider for about three years, said he was a tough firefighter who trained colleagues. "He's real outgoing. He loves to help in any way he can," Bricken said. Grider, 41, is a 16-year veteran of the department. Quinn, 22, is a part-time firefighter. Also injured was Capt. Steve Marrs, 37, who has been with the department for 11 years, and Alex Johnson, 28, who has been there three years. Campbellsville University, a private college, is a Christian institution that has about 3,600 students, according to its website. It is about 65 miles south of Louisville.

Firefighters still in hospital after ice stunt CNS News

 

[Noomi]

We don't call it ALS over here. Its MND. Motor Neurone Disease.

 

[waltky]

Former Ranger soldiers on despite having ALS...

Despite Lou Gehrig's Disease, Former Ranger Helps Students Soldier On
Sep 20, 2015 | Several years ago, Timothy Spayd and his family were alarmed to notice he was suddenly losing weight.

The weight loss -- two dozen pounds in little more than a month -- was accompanied by severe back pain. After visiting multiple doctors and undergoing a battery of tests, the former U.S. Army sergeant received grim news. He had amyotrophic lateral sclerosis, or ALS, a progressive neurodegenerative disease also known as Lou Gehrig's disease. The condition attacks nerve cells in the brain and the spinal cord, eventually affecting a person's ability to speak, eat, move and breathe. The average life expectancy of someone with the disease is two to five years from diagnosis. "You go through it all," the 54-year-old said, referring to his emotions after he was diagnosed. "You go through the, 'Why me?' You go through super depression. You contemplate suicide. Everything goes through your brain. I think that's a normal reaction to something that's so traumatic and terminal." In 2013, his wife Karen was diagnosed with lung cancer and Spayd sank further into despair. He was most upset at how their daughter, Sarah, now 13, would cope with the changes.

BACK TO SCHOOL

Sensing Spayd could use an emotional boost, a longtime friend of his and fellow ex-Ranger contacted Army officials at Ranger School to see if Spayd could spend a day soldiering with the premiere infantry training program -- similar to what the Make-A-Wish Foundation does for children with life-threatening medical conditions. The Army was receptive to the idea and Spayd found himself wading into the Florida swamps with soldiers half his age. He recalls the temperature was particularly cold that first night, in the upper 20 degrees -- so cold that a scheduled boat exercise was canceled. "It impressed the guys that I hung in there in those conditions," he said. "They thought I was pretty hard core -- old school. I guess I earned their respect then."

Timothy Spayd, 54, a former Army Ranger who has amyotrophic lateral sclerosis (ALS), takes a photo with Capt. Kristen Griest, one of the first two women in history to complete Ranger School, while volunteering with the swamp phase in Florida

But the 1980 graduate of Ranger School didn't stop there. Spayd kept going back to volunteer with the course on a part-time basis. He even got a chance to spend some time with Capt. Kristen Griest, one of the first two women in history to complete the course. This month, he participated with his 21st consecutive class. "You have to find some happiness somewhere," he said. "It's like your joy was taken. I just always loved being a Ranger." To be sure, Spayd only joins the group for about three days out of the two-month-long program, which is a grueling affair designed to push soldiers to their mental and physical limits. But he deliberately returns for some of the most difficult days, including waterboard operations, to inspire the soldiers and "motivate them and toughen them up."

More DEGENERATIVE DISEASE

 

[waltky]

ALS gene discovery funded by Ice Bucket Challenge events...

Ice Bucket Challenge funds gene discovery in ALS (MND) research
Wed, 27 Jul 2016 - The Ice Bucket Challenge that went viral in 2014 has funded the discovery of an important gene in the neurodegenerative disease ALS, the ALS Association says.

Scientists have identified a new gene contributing to the disease, NEK1. The Ice Bucket Challenge has raised $115m (£87.7m) from people pouring cold water over themselves and posting the video on social media. It was criticised as a stunt, but has funded six research projects. Research by Project MinE, published in Nature Genetics, is the largest-ever study of inherited ALS, also known as motor neurone disease (MND).

More than 80 researchers in 11 countries searched for ALS risk genes in families affected by the disease. "The sophisticated gene analysis that led to this finding was only possible because of the large number of ALS samples available," Lucie Bruijn of the ALS Association says. The identification of gene NEK1 means scientists can now develop a gene therapy treating it. Although only 10% of ALS patients have the inherited form, researchers believe that genetics contribute to a much larger percentage of cases.

Massachusetts Governor Charlie Baker and his deputy Karyn Polito participate in the Ice Bucket Challenge on 10 August 2015 in Boston​

Social media was awash with videos of people pouring cold water over their heads to raise money for ALS in the summer of 2014. More than 17 million people uploaded videos to Facebook, including many celebrities who rose to the challenge, which were then watched by 440 million people worldwide.

What is amyotrophic lateral sclerosis (ALS), also known as motor neurone disease (MND)?

* fatal, rapidly progressive disease that affects the brain and spinal cord
* attacks nerves that control movement so muscles refuse to work (sensory nerves are not usually affected)
* can leave people locked in a failing body, unable to move, talk and eventually, breathe
* affects people from all communities
* scientist Stephen Hawking is best-known person with the disease
* kills around a third of people within a year of diagnosis and more than half within two years
* there is no cure

Ice Bucket Challenge funds gene discovery in ALS (MND) research - BBC News

See also:

Ice Bucket Challenge mum praises ALS 'breakthrough'
Wed, 27 Jul 2016 - Nancy Frates, whose son Pete has ALS, welcomes a research breakthrough, but says more donations are needed to find a "cure".

The woman who is credited with starting the Ice Bucket Challenge said she hopes a scientific breakthrough in research into the disease will lead to a cure. The ALS Association has said that the campaign, which went viral in 2014, has funded an important scientific gene discovery in the progressive neurodegenerative disease.

Ice Bucket Challenge mum praises 'breakthrough'​

Nancy Frates, whose son Pete was diagnosed with ALS (amyotrophic lateral sclerosis), known as Motor Neurone Disease in the UK, told BBC 5 live: "We are hoping that it leads to the treatment and the cure, and it will, but it's going to take donations to get us there."

Nancy also defended the campaign against critics who said it was just a "publicity stunt", telling Afternoon Edition that anyone who thought that should "come and live in my house for one day" to see the "horrific" reality of living with the disease.

Ice Bucket Challenge mum praises ALS 'breakthrough' - BBC News

 

[waltky]

Hope for Stephen Hawking?

Completely 'locked-in' patients can communicate
Wed, 01 Feb 2017 | Patients with no control over their body answer questions as a computer interprets brain signals.

A brain-computer interface was used to read the thoughts of patients to answer basic yes-or-no questions. One man was able to repeatedly refuse permission for his daughter to get married. The study on four patients in Switzerland - published in PLOS Biology - also showed they were happy despite the effects of being "locked-in".

The patients all had advanced forms of amyotrophic lateral sclerosis, in which the brain loses the ability to control muscles. It eventually traps people in their own body - they are able to think, but incapable of moving or talking. When they become "locked in", it can still be possible to develop ways of communication using eye movements. But all the patients in the study, at the Wyss Center in Switzerland, were "completely locked in" and could not even move their eyes.

Brain signals

The activity of brain cells can change oxygen levels in the blood, which in turn changes the colour of the blood. And scientists were able to peer inside the brain using light to detect the blood's colour, through a technique called near-infrared spectroscopy. They then asked the patients yes-or-no questions such as: "Your husband's name is Joachim?" to train a computer to interpret the brain signals. The system achieved an accuracy of about 75%.

Completely locked in patient​

It means questions need to be asked repeatedly in order to be certain of a patient's answer. Prof Ujwal Chaudhary, one of the researchers, told BBC News: "It makes a great difference to their quality of life. "Imagine if you had no means of communicating and then you could say yes or no - it makes a huge impact." In one case a daughter wanted the blessing of her completely locked-in father before marrying her boyfriend.

'Remarkable'

But eight times out of 10 the answer came back no. "We don't know why he said no," said Prof Chaudhary. "But they got married… nothing can come between love." The form of communication is being used for more practical day-to-day means such as finding out if patients are in pain or want a family visit.

Prof John Donoghue, the director of the Wyss Center, told the BBC: "If a person who is totally locked-in is able to communicate, you're freeing the mind to interact with the world around them. "That is remarkable."

Completely 'locked-in' patients can communicate - BBC News

 

[waltky]

Artificial Intelligence Robots Aiding in Battle Against ALS...

Artificial Intelligence Robots Aiding in Battle Against Crippling Nerve Disease
August 10, 2017 — Artificial intelligence robots are turbocharging the race to find new drugs for the crippling nerve disorder ALS, commonly called Lou Gehrig's disease. The condition attacks and kills nerve cells controlling muscles, leading to weakness, paralysis and, ultimately, respiratory failure.

There are only two drugs approved by the U.S. Food and Drug Administration to slow the progression of ALS (amyotrophic lateral sclerosis), one available since 1995 and the other approved just this year. About 140,000 new cases are diagnosed a year globally, and there is no cure. "Many doctors call it the worst disease in medicine, and the unmet need is huge," said Richard Mead of the Sheffield Institute of Translational Neuroscience, who has found artificial intelligence (AI) is already speeding up his work. Such robots — complex software run through powerful computers — work as tireless and unbiased super-researchers. They analyze huge chemical, biological and medical databases, alongside reams of scientific papers, far quicker than humanly possible, throwing up new biological targets and potential drugs.

Cell deaths prevented

One candidate proposed by AI machines recently produced promising results in preventing the death of motor neurone cells and delaying disease onset in preclinical tests in Sheffield. Mead, who aims to present the work at a medical meeting in December, is now assessing plans for clinical trials. He and his team in northern England are not the only ones waking up to the ability of AI to elucidate the complexities of ALS. In Arizona, the Barrow Neurological Institute last December found five new genes linked to ALS by using IBM's Watson supercomputer. Without the machine, researchers estimate the discovery would have taken years rather than only a few months. Mead believes ALS is ripe for AI and machine-learning because of the rapid expansion in genetic information about the condition and the fact there are good test-tube and animal models with which to evaluate drug candidates.

Former Tennessee Titans linebacker Tim Shaw, second from left, leaves the field with Titans general manager Jon Robinson, left, and head coach Mike Mularkey, right, after a team activity at its training facility in Nashville, Tenn., June 15, 2017. Shaw, who was diagnosed with ALS in 2014, has worked with the special teams staff and players.​

That is good news for ALS patients seeking better treatment options. Famous sufferers include Gehrig, the 1923-39 New York Yankees baseball player; actor and playwright Sam Shepard, who died last month; and cosmologist Stephen Hawking, a rare example of someone living for decades with the condition. If the research goes on to deliver new medicines, it would mark a notable victory for AI in drug discovery, bolstering the prospects of a growing batch of startup companies focused on the technology. Those firms are based on the premise that while AI robots won't replace scientists and clinicians, they should save time and money by finding drug leads several times faster than conventional processes.

British 'unicorn'

Mead from Sheffield is working with BenevolentAI, one of a handful of British "unicorns" — private companies with a market value above $1 billion, in this case $1.7 billion — which is rapidly expanding operations at its offices in central London. Others in the field include Scotland's Exscientia and U.S.-based firms Berg, Numerate, twoXAR, Atomwise and InSilico Medicine — the last of which recently launched a drug discovery platform geared specifically to ALS. "What we are trying to do is find relationships that will give us new targets in disease," said Jackie Hunter, a former drug hunter at GlaxoSmithKline (GSK) who now heads Benevolent's pharma business. "We can do things so much more dynamically and be really responsive to what essentially the information is telling us."

A view of BenevolentAI's home page.​

Unlike humans, who may have pet theories, AI scans through data and generates hypotheses in an unbiased way. Conventional drug discovery remains a hit-and-miss affair, and Hunter believes the 50 percent failure rates seen for experimental compounds in mid- and late-stage clinical trials due to lack of efficacy is unsustainable, forcing a shift to AI.[ A key test will come with a study by Benevolent to assess a previously unsuccessful compound from Johnson & Johnson in a new disease area — this time for treating Parkinson's disease patients with excessive daytime sleepiness.[ Big pharmaceutical companies like GSK, Sanofi and Merck are now exploring the potential of AI through deals with startups.

Being careful