An Environmental Cause for ALS?

What does a map plotting patients with ALS look like? One such map shows that lakes with cyanobacteria, or blue-green algae, may be linked to  ALS.

Dr. Elijah Stommel and Dr. Tracie Caller of the Dartmouth-Hitchcock Medical Center found that people living around a freshwater lake that has frequent blooms of cyanobacteria are up 25 times more likely than the general population to develop ALS.

Cyanobacteria is a life form, closely related to regular bacteria, that lives in water.  The cyanobacteria can multiply under the right conditions and create a whole colony that can turn clear water into cloudy water with blue-green or even reddish-brown algae or scum.  The water may have a thin “oily” film similar to what a paint spill may look like.  Fortunately, a bloom with a high population of cyanobacteria cannot survive for very long.  They typically die after one or two weeks.  Another bloom can replace the first one if water conditions remain favorable for growth.

Some cyanobacterial blooms are not toxic, but some produce BMAA, a nerve toxin that can affect the brain.  A report in the Proceedings of the British Royal Society published on Jan 20, 2016 shows in animals that chronic exposure to the toxin BMAA from cyanobacteria causes “brain tangles,” which can lead to ALS related symptoms and other neurodegenerative illnesses.

Lead author of the study, Paul Cox, Ph.D. is the Director of the Institute for Ethnomedicine.  His current research is focused on neurodegenerative illness with the goal of discovering new therapies for ALS.  

The recent publication also shows that large doses of the dietary amino acid called L-serine can reduce the risk of BMAA exposure.  The L-serine works by “outcompeting” against low levels of BMAA in the body, preventing it from becoming incorporated into proteins.  The study was conducted on vervet monkeys, not humans, but offers reasonable proof of the BMAA link, which has been suspected before.

ALS is likely the result of a combination of several factors, that when they run together, create certain symptoms in the body.  It’s like creating the perfect storm.  This environmental neurotoxin BMAA seems to be one of the contributing factors.  Finding one causative piece of the ALS puzzle helps move forward to the cure.  
The scientific BMAA publication can be found here:Dietary exposure to an environmental toxin triggers neurofibrillary tangles and amyloid deposits in the brain

Promising ALS Copper-ATSM Treatments in Mice

New ALS research results from Oregon State University scientists may offer hope for a new kind of treatment for ALS.  The treatment uses a chemical compound called copper-ATSM.  The study recently published in the journal of Neurobiology of Disease shows significant results in treating laboratory mice with ALS.  The treatment has not been tested yet in humans.

The copper-ATSM treatments were able to extend the life of mice for 20 months, which is nearly a full lifespan for the genetically engineered ALS mice.  In contrast, the mice without treatment died within 8-13 days of birth.

The study also showed that progression of the motor neuron disease could be restarted and stopped by withholding or giving the copper-ATSM treatments.  The mice seemed to respond within hours of treatment.   

Joseph S Beckman, Ph.D with OSU’s Linus Pauling Institute has been studying ALS for 23 years.  He patterned this study after promising work being done at the University of Melbourne.  He knew that copper plays an important role in ALS, but it must be delivered  in the right way.  

Copper nutritional supplements cannot be used to treating ALS symptoms.  The published study states, “Copper supplements can damage the liver and are generally toxic at high dosages.”  But the copper-ATSM compound, used in this case, can prevent copper from being released in most tissues, and only be delivered to to hypoxic tissues, or areas with low oxygen supply.  The copper-ATSM can cross the blood-brain barrier and release the copper to cells with damaged mitochondria.

Although copper-ATSM has not been tried as a treatment in ALS patients, it is used as a PET-imaging agent for hypoxic tumors in humans.  The copper-ATSM has a low toxicity and can penetrate to the central nervous system within minutes.  

If results in humans are similar to the results seem in mice, this would be a huge breakthrough for the ALS community.  ALS is a condition that progressively degenerates nerve cells that control all muscle movement.  It is always fatal and most patients are told they have 3-5 years to live after diagnosis.  Each year 3,000-4,000 people are newly diagnosed with ALS in the U.S.

It typically takes 10 years for a pharmaceutical drug to be developed and ready for patients. The process starts in laboratory test tubes, progresses to testing in animal models such as mice, then goes to human testing.  The next step for the copper-ATSM treatments would be to 1) optimize the copper-ATSM dosage in mice, and 2) verify treatment effectiveness in mice, through repeated studies using the same protocol.  After these steps the treatment could proceed to human clinical trials.

This copper-ATSM treatment has such promising results for halting the progression of ALS.  It will no doubt be watched closely and hopefully by ALS patients and families.  .


The full study seen in Neurobiology of Disease is titled: Copper delivery to the CNS by CuATSM effectively treats motor neuron disease in SOCG93A mice co-expressing the Copper-Chaperone-for-SOD. 

For ALS, Sharing Information is Critical

Just six weeks ago, 800 scientists and healthcare professionals attended the International Symposium on ALS/MND.  This was an opportunity to share information about what is being discovered in all areas of research, from basic lab research to clinical trials.  

Dr. Terry Heiman-Patterson of Drexel University College of Medicine recently summarized these professional meetings in an hour long webinar presentation for patients and families through NEALS. She spoke of the 120 NEALS sites around the world and the 21 active studies going on at multiple locations within that network.  

“I’ve been working in the field of ALS for almost 30 years and when I started we didn’t have a single clinical trial or clinical study going on.  Now, there is just an explosion and I’m very hopeful that we will really make major progress,” Heiman-Patterson explains.   

ALS research focuses on three areas: finding the causes of ALS, finding ways to treat the day to day symptoms, and finding ways to cure or reverse ALS.  All three are important areas that can give meaningful results.  Each area must be studied simultaneously, as breakthroughs in one area can be connected to progress in the other areas.  ALS is such a complex disease that no single group can research every aspect.  Integrating the results from each study in each area is critical.  

At this time ALS treatment strategies generally fall into five main approaches:

  1. Anti-glutamate treatments
  2. Neuroprotectant treatments
  3. Anti-inflammatory treatments
  4. Anti-oxidant treatments
  5. DNA modulation treatments

There are many other innovative ideas, immunosuppressive treatments, diet, and exercise, that are also being tried that don’t fit into the above categories.  

“I bring this up because there is really so much more activity,” says Heiman-Patterson.  It is a hopeful time in ALS research.  “We really are working, those of us who study ALS, those of us who are committed to making a difference.”  
Educational Webinars through NEALS are held monthly for ALS patients and families and can be viewed here.

Gleason ALS Film Premiers at the Sundance Film Festival

Steve Gleason and Seth Christensen, August 2015

Steve Gleason and Seth Christensen, August 2015

Steve Gleason was diagnosed with ALS five years ago, at age 34, and told he had two to five years to live. At the time he was a famous NFL player, a defensive back for the New Orleans Saints. A few weeks after his diagnosis, he learned his wife Michel was expecting their first child. As he lives with ALS now, he is sharing his story, not just with the sports world but everyone. He is truly inspiring others to live life to its fullest and to live intentionally.

A documentary titled Gleason, directed by Clay Tweel, will premiere at the Sundance Film Festival in Park City, Utah, on Saturday, Jan. 23, at 11:30 a.m. It will be shown seven times over the course of the Sundance Film Festival. It is one of 16 American documentaries that illuminate the ideas, people, and events that shape the present day.

The project began as a video journal for his unborn child, but expanded into a documentary that follows him living with ALS, and the emotional journey that affects all his relationships. It provides a real perspective of the daily struggles of people with ALS and their caregivers. Gleason uses his characteristic determination and even humor to deal with his complicated situation.

Steve Gleason founded Team Gleason, a non-profit foundation, that he hopes will inspire those living with the same diagnosis. His mission is to help provide technology software and equipment services to those with ALS, create global conversation about ALS to find solutions to end the disease, and raise public awareness of ALS.

This personal story of Steve Gleason will likely share the emotions of thousands of others of people living with ALS in the United States. According to the ALS Association, approximately 20,000 Americans have the disease at any given time. Gleason’s legacy on the football field may be overshadowed by his inspirational approach to life: to live as fully as possible.

New Mom with ALS Uses State-of-the-Art Technology to Communicate


As seen on Shoreline Times

By Sarah Page Kyrcz

MADISON >> Amanda Bernier works very hard, on a daily basis, to keep in touch with her family, friends and acquaintances. For the 31-year-old new mom this is no small feat.

Two weeks after finding out she was pregnant, Amanda Bernier was diagnosed with ALS. She delivered her daughter, Arabella Grace, Nov. 4, 2014, at Yale New Haven Hospital.

She has been chronically her nearly yearlong journey, living with ALS, through Facebook and videos. Many know her through her work as a Madison firefighter.

ALS is a progressive neurodegenerative disease that causes the deterioration of motor neurons. Amanda Bernier has the most aggressive form of ALS.

At present, her eyes and a small part of her jaw are the only parts of her body that move.

“What a lot of people don’t realize is she can fully hear everyone and she can understand everything they’re saying, she can feel everything,” explains her husband, Chris Bernier.

Amanda Bernier is able to communicate thanks to an eye tracker program donated by Team Gleason ( that works with her Windows tablet.

doc55ef44776ff465714381501Team Gleason is an organization that “is partnering with leading technology companies to purchase eye tracking portable computers in bulk.”

“A year ago today was the last time I spoke. It was the last time I smelt (smelled). It was the last time I took a breath on my own,” she writes on Aug.21. “A year ago I got my trach and went on a ventilator.”

In a 51-second video, posted on Amanda’s Angels ALS Facebook page, Amanda Bernier shows how she uses the eye tracker technology.

“The speaker is bluetooth as well so I can place it on my chest so the words sound like they are coming from me. The computer tracks my pupils. To select something I just pause for 0.3 seconds,” she explains.

Every hour, either Chris Bernier, a caregiver or a nurse, must move Amanda. The tablet and eye tracker device, mounted on a mobile frame, are moved, also.

“Whatever direction she’s facing that eye tracker device can be positioned and moved to whatever side she’s facing,” says Chris Bernier.

Amanda Bernier explains that this technology is vital in her daily life.

“It’s how I tell my caregivers what I need or if something is wrong with my trach, communicate with my loved ones, use the internet and write letters to Arabella,” she writes on Facebook.

Chris Bernier explains that his wife has become very proficient in the use of the eye tracker.

“She’s pretty quick at it,” says Chris Bernier. “In a sense, it’s like texting, you know, because you’re selecting each word and as that word populates you select it to do it automatically. The only difference is that she’s typing with her eyes.”

While it may take her a little longer for Amanda Bernier to type out a message, she is grateful to have this technology.

“My eyes dont tire. They get blurry since I cant close my eyes all the way and then it cant track my eyes well,” she writes in a message to the ShoreLine Times.

“I correct that by wiping my eyes and eye drops. I can type at about the same speed as typing with one finger since I can only type one letter at a time.

Chris Bernier appreciates being able to communicate with his wife, and says that without the eye tracker device it would be impossible to know what she is thinking or feeling.

“There’s no doubt that without having this piece of technology we would not know the level or the depth of her thoughts, feelings and needs and wants,” says Chris Bernier.

Amanda Bernier agrees.

“I can’t even imagine what life would be like if I had no way to communicate.”

Answer ALS: A Revolutionary Project to Cure ALS

Screen Shot 2015-09-29 at 8.56.40 PMAA-logo

As seen on ALS News Today

The launch of Answer ALS, the largest comprehensive research project to cure Amyotrophic Lateral Sclerosis (ALS) was announced in a press release by the Johns Hopkins University’s Robert Packard Center for ALS Research in Baltimore, MD, the Cedars-Sinai Medical Center’s Regenerative Medicine Institute in Los Angeles, CA, and the Massachusetts General Hospital Neurological Clinical Research Institute, Boston, MA.

ALS is a rapidly progressive and fatal disease characterized by a degeneration of motor neurons. As disease evolves, the brain loses its ability to control muscle movement, eventually leading to body paralysis. There is no effective treatment for this disease, which annually affects 2-4 people in every 100,000 in Europe and the US.

Answer ALS aims at: i) finding the causes of ALS; ii) develop human cell models to understand ALS and screen for drugs and therapies; iii) implement individual ALS therapies based on each patients’ own brain cell data; iv) establish therapies that could improve ALS symptoms and eventually cure them; and v) freely disseminate all the information collected to worldwide in order to advance therapy development.

To achieve these goals, the project will have an aggressive and comprehensive research policy – real-time 24/7 monitoring and thorough clinical and biological data collection of a large patient population (minimal 1,000 patients); generation of pluripotent cell lines from each patients’ brain cells to create novel cellular models; simultaneous analysis of all data gathered using comprehensive algorithms that include patients’ clinical, cellular, biochemical, genetic, steam cells and robotic images; and the creation of a collaborative consortium with world’s experts on clinical, cellular, biochemical, genetics, stem cells and big data analysis fields.

Dr. Jeffrey Rothstein, Director of the Brain Science Institute and the Robert Packard Center for ALS Research at Johns Hopkins University and the Executive Director of Answer ALS stated“This project will bring together world-renowned ALS research scientists to work against an aggressive timeline for understanding, treating and eventually finding a cure for this disease. The substantial initial funding from these generous supporters is a critical step forward in our effort to provide hope to those affected by ALS.”

The project is expected to generate the world’s largest comprehensive ALS data, encompassing all clinical and biological aspects of each patient enrolled in the project. Machine learning and big data technology will be used in data analysis to unveil ALS causes, subtypes, key pathways and drug targets. This information will help in the development of new clinical trials and in novel categorization methods for patients to identify specific ALS pathways, biomarkers and disease pathophysiology, which will aid in the early diagnosis of the disease and treatment efficacy.

The project is a result of the 2013 ALS Team Gleason Summit – an initiative of the former NFL player Steve Gleason, who lives with ALS – in which leading ALS researchers, patients, caregivers and advocates came together to decide what measures were necessary to overcome this devastating disease. At the moment, the project encompasses nearly two dozen US research institutes, being directed by Dr. Jeffrey Rothstein co-aided by Dr. Clive Svendsen, Director of the Regenerative Medicine Institute at Cedars-Sinai Medical Center and Dr. Merit Cudkowicz, Chief of Neurology and Co-Director of the Neurological Clinical Research Institute at Massachusetts General Hospital.

Answer ALS is funded by a Committed Team of Donors that include the ALS finding a cure project from the Leandro P. Rizzuto Foundation, the Robert Packard Center for ALS Research, the National Football League, PGA TOUR, Travelers, The Fishman Family and The Bari Lipp Foundation. Thus far, $20 Million have been contributed. A total of about $25 million is required for the first phase of the program. Funding will be coordinated through the Robert Packard Center for ALS Research and the ALS Finding a Cure Foundation.

ALS Association and Target ALS to Expand Tissue Core for ALS Research

As seen on PRNewswire


WASHINGTONSept. 24, 2015 /PRNewswire-USNewswire/ — The ALS Association and Target ALS are pleased to announce the launch of a new collaborative effort to expand the collection of biofluids from people with ALS, to be used for research to better understand the disease and ultimately develop new treatments.

The ALS Association will be investing $1,392,668 over the next 38 months. Funding from The ALS Association will allow pre-mortem biofluids (serum, plasma, and cerebrospinal fluid) to be collected and stored at the sites that comprise the Target ALS Postmortem Tissue Core at Barrow Neurological Institute/Saint Joseph’sHospital in PhoenixColumbia University in New YorkGeorgetown University in Washington D.C.Johns Hopkins University in Baltimore, and University of California at San Diego. Detailed genetic analysis will be performed on all these cases at The New York Genome Center, all the data will be linked, and all of the samples and data will be made available for researchers around the world.

ALS is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Eventually, people with ALS lose the ability to initiate and control muscle movement, which often leads to total paralysis and death within two to five years of diagnosis. For unknown reasons, veterans are twice as likely to develop ALS as the general population. There is no cure, and only one drug approved by the U.S. Food and Drug Administration (FDA) modestly extends survival.

The Target ALS Postmortem Tissue Core is a unique resource in the field of ALS research, providing high-quality post-mortem tissue for academic and industry researchers throughout the world. Target ALS has invested $1.04M to date into the core. The addition of pre-mortem biofluids from the same individuals will greatly expand the potential utility of the samples, by allowing researchers to explore biomarkers in biofluids that correspond to changes in tissues.

“The studies that will be made possible by the addition of biofluids to the Tissue Core have the potential to deepen the understanding of the ALS disease process and, through discovery of new biomarkers linked to specific aspects of the disease, accelerate the search for new treatments,” said ALS Association Chief Scientist Lucie Bruijn, Ph.D., M.B.A. “We are very pleased to be partnering with Target ALS on this important initiative.”

“This partnership has created a first-of-its-kind resource to lower the barriers for, and accelerate the pace of translational research in ALS.  Target ALS is delighted to partner with The ALS Association on this much-needed initiative,” said Target ALS Executive Director Manish Raisinghani, M.D., Ph.D.

About The ALS Association
The ALS Association is the only national non-profit organization fighting Lou Gehrig’s Disease on every front.  By leading the way in global research, providing assistance for people with ALS through a nationwide network of chapters, coordinating multidisciplinary care through certified clinical care centers, and fostering government partnerships, The Association builds hope and enhances quality of life while aggressively searching for new treatments and a cure.  For more information about The ALS Association, visit our website at

Target ALS is a privately funded international consortium of researchers from academic and biotech/pharma laboratories entirely focused on finding a treatment for patients living with ALS. We fund Research Consortia that have accelerated identification and validation of new targets for treatment of ALS, Springboard Fellowships to support work of emerging research leaders, and core facilities that lower barriers for cutting edge ALS research worldwide. For more information visit our website at

SOURCE The ALS Association


Haircuts & Hot Rods–Salt Lake City Car Show to Benefit ALSCrowd

The 1st annual Blues Barbershop Car Show will take place on September 19at Holladay City Hall  (2300 E. 4650 S. in Salt Lake City) with all proceeds benefiting ALS Crowd. The event will include t-shirt sales, live music (including Ryan Innes from NBC’s The Voice), concessions, family activities, and new and classic cars. Sponsors are invited to co-brand merchandise, show corporate cars, have a booth, and are encouraged to provide fundraising/ matching challenges during the event. Contact Christi Wedig, Blues Barbershop, at: 435-773-7031




Paralyzed patients can control computers just by moving their eyes, thanks to this free software

businessinsider optikey-logo

As seen on Business Insider

By Dan Turkel

Julius Sweetland wasn’t happy with the software options available to people with severe physical and speech impairments, so he spent the past three and a half years building his own eye-controlled interface as a cheap alternative to the competition.

With a $100 eye-tracker and his free software, people with amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig’s disease) or other impairments can type, click, and even speak through a computer without the use of their hands or voice.

In a two-minute demo, Sweetland, a 32-year-old London-based developer, shows off the latest build of his software, OptiKey. A large keyboard fills the screen and Sweetland, shown holding his hands up on a small embedded webcam feed, types using his eyes alone. Next, with an eye-controlled “click,” the software reads back what he’s written.

A device similar to a webcam tracks Sweetland’s eye movements as he shifts his gaze from one letter to the next. Automatic suggestions pop up like they would on an iPhone, but he hardly seems to need them as OptiKey correctly determines the words he wants by analyzing the letters he’s looked at.

OptiKey handless demoYoutube/Julius Sweetland Developer Julius Sweetland shows off typing without the use of his hands in the first OptiKey demo video.

As shocking as it may seem to the average user, OptiKey is not groundbreaking technology. In fact, the study of eye-tracking dates back to the 19th century and its use for computer control began in the 1980s.

However, many eye-tracking interfaces are prohibitively expensive, making computer use difficult or even impossible for people — like those with ALS — who are unable to use a keyboard, mouse, or voice controls. Speaking with Business Insider, Sweetland said that this isn’t fair. “There are a lot of ‘off-the-shelf’ solutions out there … [but]you’re looking at thousands of dollars for something like this.”

It’s true. On the lower end of the spectrum, the PCEye Go from Tobii will cost you $1,995. And commentors in a Reddit thread discussing OptiKeyreference alternatives priced as high as $14,000 or $17,000, which Sweetland calls “absurd.”

Cheaper eye-tracking hardware has come to the market, but there isn’t always accessibility software to use with it. The Eye Tribe offers a $99 eye-tracker but, since it’s currently aimed at developers, it comes with little software of its own. That makes it a perfect match for the OptiKey, and it comes as no surprise that the two projects endorse each other.

Sweetland has been programming since he was 5, and his day job has him coding trading software for the financial sector. It was the experience of an aunt who died of motor neuron disease (a blanket term for a family of diseases including ALS) that inspired him to get involved in “augmentative and alternative communication,” a fancy term for technology that helps people with physical or speech impairments communicate.

Sweetland initially tested OptiKey himself, along with a small group of users around the globe. Recently, however, he has been working with the Royal Hospital for Neuro-disability in Putney, London, to get better feedback from a patient there.

“Users never do things how you’d expect them to,” remarks Sweetland. “I came away with a lot of things to change. It took me over a month to get them all into version 2.”

As for the patient, he enjoyed his OptiKey experience. “He’s an ex-software developer also, so we geeked out a bit. I’m going to go and see him again soon to show him the changes he helped shape,” Sweetland said.

All of the code for OptiKey is available on the collaborative code-sharing site GitHub, and Sweetland pledges that it will remain free and open-source forever. “I couldn’t see a way to charge people for this that I felt comfortable with. Lots of people told me I’d be crazy to open source it, but this has always been a project close to my heart and it didn’t feel right to profiteer.”

Since it is a spare-time passion project for him, he hopes that developers will contribute to the project. “I’d love to see it expand beyond my original plans.”

Intel releases Stephen Hawking’s speech software online for free for ALS Patients

As seen on FirstPost

London: A software by Intel that lets physicist Stephen Hawking communicate via a computer has been published online by the company in the hopes that it will be used by researchers developing new interfaces for sufferers of diseases like amyotrophic lateral sclerosis (ALS).

The programme interprets visual signals and translates them into words, which are then “spoken” by a machine.

Intel originally developed the technology specially for Hawking but it has been used by other sufferers of motor neurone disease (MND).

Hawking, 73, suffers from a rare early-onset slow-progressing form of ALS that has gradually paralysed him over the decades.

Photo: The Guardian

Photo: The Guardian

The Assistive Context-Aware Toolkit (ACAT) helps Hawking communicate by interpreting sensor data capturing movements in his cheek muscles but other parts of the body may be used.

Anyone can now download and experiment with the system.

Intel hopes that ACAT, which runs on Microsoft Windows 7 or higher, will be used by researchers developing new interfaces for sufferers of diseases like ALS.

The programme and full source code have been published on code-sharing site GitHub.

Intel told the BBC that the software can carry out various functions besides sending text to a speech synthesiser.

“We have contextual menus to access all different parts of your computer,” said Lama Nachman, principal engineer.

“If you want to use Word, surf the web or speak you can use ACAT for that,” Nachman said.

She added that the team had already experimented with a variety of different sensors, and they are hoping developers will try out other options suited to each patient’s needs and abilities.