Combating Locked-In Syndrome: New Methods of Communication for ALS Patients

SerendipUpdate's picture

Biology 202
2006 First Web Paper
On Serendip

Combating Locked-In Syndrome: New Methods of Communication for ALS Patients

Andrea Goldstein

Imagine having a fully-functional brain trapped within a non-functioning body. The brain would be conscious and aware of its surroundings; it could think and process stimuli, but it would be unable to translate thought into action. Locked-In Syndrome is a rare disorder that results in just that; all of the body's voluntary muscles, with the exception of those that control eye movement, become completely paralyzed. There are no treatments available, nor is there a cure. (1) While Locked-In Syndrome is most commonly caused by damage to the pons (2), numerous other events or disorders can lead to this tragic locked-in state. Perhaps the most well-known disease that causes Locked-In Syndrome is Amyotrophic Lateral Sclerosis (ALS), known colloquially as Lou Gehrig's disease.

ALS is a progressive neurodegenerative disorder that causes the death of motor neurons. With no motor neurons to receive signals from the brain and cause movement, patients become completely paralyzed. After a period of no input from motor neurons, the muscles themselves atrophy and become weak. Finally, the brain loses the ability to start signals that cause voluntary movement altogether. Many ALS patients die from respiratory failure when their diaphragms and chest wall muscles fail to contract any longer. (3) The majority of ALS patients die within 3-5 years of diagnosis, but about 10% survive for ten years or more past their diagnosis, and 5% survive for twenty years after being diagnosed (4).

People are living longer with ALS due to several therapies. The newest treatment is a drug called riluzole, which reduces motor neuron damage by decreasing the release of glutamate, a neurotransmitter which may cause the degeneration of motor neurons in the first place. Riluzole has been clinically proven to prolong life for at least several months. Other treatments include speech and physical therapy and drugs that reduce symptoms that accompany ALS, such as fatigue, muscle spasms, and excess salivation. (3)

Because ALS destroys motor neurons, its effects are irreversible. Unlike the proportion of people with Locked-In Syndrome that eventually recover some motor function (2), ALS patients can never be freed from the prison of their minds. Despite the promising research being done to find more effective treatments and a cure, the more immediately pertinent research deals with how best to combat the imprisonment of the mind that comes with ALS. Most communication methods use eye movement to convey information, while some more experimental technologies use electrical impulses directly from the brain (5).

The simplest method of communication for ALS patients who are no longer able to move anything but their eyes involves no sophisticated technology at all. With the help of an assistant, patients can use eye blinks to signify which letters or syllables they would like to select from a chart, slowly and methodically putting together words and sentences. Eye response technologies are a more complicated method of communication that allows ALS patients to communicate significantly faster and independent of others. Eye-gaze Response Interface Computer Aid (ERICA), for example, uses a camera and infrared light to detect the position of a person's gaze on a computer screen. In this way, a person can essentially type with his or her eyes, performing computer tasks normally. In addition, the software that ERICA uses can produce a computerized voice, allowing the patient to "talk" to others. (6) This technology can provide a highly effective means of communication for as long as voluntary muscles in the eye remain intact.

In most cases, control of the eye muscles is retained throughout the span of ALS, but researchers are currently looking into devices that will allow ALS patients in advanced stages of the disease to communicate without using the eyes. In one method, an electrode is implanted directly into the brain. The electrode picks up electrical impulses and translates them into instructions for the control of a computer cursor, which in turn allows the patient to communicate via computer. Another method involves electrodes on the scalp that yield much the same result as the electrode in the brain itself. These devices take a relatively long amount of time to process information and create words (about 30 seconds per letter), but for patients whose eye movements are not under voluntary control, it is the only option. (5) Other experimental devices translate EEG activity to translate signals into words through biofeedback (7).

These new technologies give people with ALS hope for more normal lives. With more efficient communication technology, patients with ALS would no longer suffer from the most devastating mental effects of Locked-In Syndrome. Despite the fact that the motor neuron degeneration cannot currently be reversed, patients no longer have to remain imprisoned in their own minds. Eye response technologies and new experimental devices in the future can allow ALS patients to communicate effectively, freeing them from their personal jails. Perhaps this new technology that uses computers translating signals into speech will shed light on what "thoughts" really are and what produces speech. Further research can only lead to new ideas and more information about ALS and the Locked-In Syndrome that it produces.

Resources:
1)Locked-In Syndrome Information Page, from the National Institute of Neurological Disorders and Stroke website
2)A clinical review of Locked-In Syndrome by Eimear Smith and Mark Delargy
3)Amyotrophic Lateral Sclerosis Fact Sheet, from the National Institute of Neurological Disorders and Stroke website
4) Facts You Should Know About ALS, from the ALS Association website
5) Unlocking Locked-In Syndrome, from the Society for Neuroscience website
6)Eye-gaze technology, from the University of Minnesota Duluth website
7)Biofeedback and Locked-In Syndrome in ALS, from the Futurehealth Inc. website

 

 

Comments made prior to 2007
My husband age 60 has ALS, vent dependent, only limited movement of right index. Is the electrode implantation still an option--any suggestion? ... Mary Baker, 1 November 2007

Comments

Ruby's picture

Locked in Syndrome Communication System

Our son, Billy, has locked-in syndrome from a brain stem stroke he had two and a half years ago. He was only 28 years old with a good job , a wife, and a two year old daughter. His stroke killed much of his brain stem, but the rest of his brain was left unscathed. Billy knows everything he did prior to the stroke, he just can't move or speak.

After his stroke, he went close to 5 months without "talking" with anyone... He only answered yes and no questions that we thought to ask him. Finally, one of his physical therapists at a rehab facility taught us (and Billy) a way of communication. Over time, it has evolved into this super-easy system:

---

When Billy wants to say something he looks up several times. When we see him doing this, we ask him if he wants to say something. He will look up for "yes."

The actual system consists of Billy spelling out what he wants to say. We have divided up the alphabet into 5 sections to get through the alphabet fairly quickly. The divisions are: A-E, F-J, K-O, P-T, and U-Z. Billy looks up when we get to the section that includes the letter he needs. Because some of the things that need to be said get quite lengthy, we use a small dry-erase board to record what Billy spells out. (NOTE: In the past, we have used a spiral notebook to write things down, but we eventually began using a dry-erase board so that Billy had the ability to say something to someone that would not later be read by all of his caretakers. This has saved many arguments and hurt feelings!)

For example, if he wants to spell his name BILLY, he will look up to let us know he wants to say something. We will ask Billy 'A-E?' and he will look up. Then we will ask about each individual letter: A? B? then he will look up again. This lets us know that this is the first letter of his statement. We record a 'B' on the dry-erase board.
We will then move on to the next letter by again asking the sections: 'A-E?' 'F-J?' and he will look up again. F? G? H? I? and he will look up to let us know that 'i' is the next letter. We would add the 'i' to the board, so that we would now have B-i.

We would then repeat this process until we complete his statement. We always verify that he has indeed finished his entire statement... sometimes we accidentally cut him off by responding to the statement before he is finished with it. We always ask "Is that all?" when we get to what we think is the end of his statement.

---

It sounds like this system would take forever, but over time it becomes easier for both the caretakers and the locked-in person. We spell out entire paragraphs and have complete conversations with Billy. Over the past year, he has had to deal with his wife leaving him and filing for divorce. This system has allowed Billy to handle his divorce almost entirely all on his own. He tells us and his attorneys what he wants and we make it happen for him. Even furthermore, Billy is able to joke around with his younger brothers and tell his daughter that he loves her. That is something that must be invaluable to someone in Billy's state.

We hope that this system helps someone communicate with a loved one. It has totally changed Billy's life and we just thought we had to share it. If anyone has any questions about our system, email us here: AoneKyle@aol.com and we will do our best to help you out!

Brian MacKrill's picture

Eye Gaze communication devices

I previously mentioned the prototype eye-blink device I created some years ago which I have not progressed partially due to more able and sophisticated devices emerging on the market which use eye-gaze technology. The price of these units are dropping slowly. Here are some links below which may provide a solution for you. All the best to you and your loved ones.

http://www.liberator.co.uk/products/eye-gaze/nueye.html

https://www.eyetechds.com/eyeon.shtml

Rosie's picture

Sister is Locked in

Hi My Sister is currently experiencing locked in syndrome. She has been diagnosed with ALS and CIDP. She receives limited IVIG therapy but when those treatments are received her eye and head movements become much better and she is able to flinch her fingers a bit. In between treatments when those are "wearing off" for lack of a better term, we have trouble communicating with her because we can't tell when she is blinking her eyes and using the letter chart we have to point to the letters to form words, then sentences is getting more difficult as well. She has an augmentive speech device which uses eye gaze technology, but because of the slow eye movements the machine is difficult for her to use. Is there anything that you can suggest that could help us communicate with her better. She lives in a Sub-Acute unit of a skilled nursing facility and she is completely aware of what is going on and the lack of compassionate treatment she receives from some of the caregivers. Thank you for any suggestions. I wish we lived in a different part of the country because there are lots of studies going on but we have no way to get her across the country.

Claire Ball's picture

Communication using eyes

Hello, My mother is disabled. She has no mobility, cannot swallow or speak but she is fully aware of conversations and humour etc. Her conditions has been gradual over some 12 years. She has not spoken for 3 years and is confined to a wheelchair, hoists etc. I would dearly love some advice on the technology to enable her to communicate through eye movement. I feel she has so much to say and believe her time is limited.

Please please help.

Thank you

Claire

Brian MacKrill's picture

Communication using eyes

Hello Claire

Sorry to hear about your mum. Some years ago I became interested in the technology as I was very much moved by a program on TV where I saw the plight of a poor soul with locked-in syndrome. I did some research and found it difficult to identify accessible and affordable technology. As I have a background in computing and electronics I sourced some parts over the internet and within a few weeks had a rudimentary proof of concept working which uses eye-blink technology. I then posted on some internet sights to gauge interest to take the project to the next phase. The response was disappointing so I shelved the project. I could teach school kids to use my device in two minutes but did needed to test the concept with people who actually suffered from this disposition. The concept was to develop a device which was low cost and flexible. I the aim was not to make lots of money but to help those in need.

To answer your question, there are a number of solutions, depending on how much coherent movement of the head and eyes your mum still has. The most impressive I have seen is where there are cameras mounted on a personal computer. The computer tracks where on the screen the user is looking and by blinking, selects the character or picture on the screen to form words or answer with preset phrases. To see what is possible, look at http://www.grinbath.com/eyeguide?gclid=CMmstMbjgbICFYRLpgoddSoA-Q and
http://www.eyegaze.com/applications/eye-controlled-assistive-technology/

I had planned my device to cost under $500 with the addition of the user's own laptop computer. I have not yet seen a comparable device to mine using simple technology, eye-blinking and low cost.

Hope this helps. Let me know if you need further information.

Serendip administrator, please feel free to pass on my email address to Claire as communicating via this website is a huge challenge.

Thanks

Emma's picture

ERICA

Hello Brian

I am also interested in your project, as my mother has the same condition, and we would love to be able to communicate with her again.

Please advise if you are carrying out any projects currently, and would you be interested to test out your product on a real patient.

Hope to hear from you, and any advise is valuable.

Serendip Visitor's picture

Locked-in

Hello Emma

Yes I would be happy to work with you. This would provide real insights and provide me with a valid reason to progress the project if the results are promising.

If the Serendip administrators would kindly pass on my details to you we can take it further.

Brian
bmackrill@bigpond.com

Serendip Visitor John NEISH Ritchie's picture

A quick Way to learn Morse Code

1. I had a stroke in 09 ,met a guy who still is unable to speak.
2.I 've been thinking around this subject for some time
3. Had to learn Morse for DTI Yachtmaster, (I think in 70s) developed my own system [passed]
4. Biggest drawback to morse is that, not only the patient must learn but also anyone else who wishes to make contact.
5. Example---"what is the Morse code made up of?"
"Dots & dashes." {nobody ever seems to say dashes first}
"We'll start the alphabet with one of each then; a dot & a dash for A" ._ is A
"That's our first letter, but it is also our first word. It is one form of the indefinite article
the other form is 'an' and there is n't any difficulty here because N is just A the other way round." _. is N
" I printed my middle name in caps for the good reason that E,I,S,H, in morse, respectively are . is E
one dot, two dots, three dots and four dots." .. is I
{These are the only letters that comprise only dots} ... is S
{it was noticing this that was the beginning of my system} .... is H
"A Lorry with a Load has a wheel in front and two wheels at the back,so it Looks like this; ._.. is L
"If the lorry is, on the other hand, going to France with Freight it is going the other way .._. is F
"A Wheelbarrow also has a Wheel at the front With a body and a shaft; ._ _ is W
"To put the barrow away in the Garden shed it has to be turned to face the other way so _ _. is G

6. That's the first 10 letters the rest go much the same way.
7. I would like to know if it was thought that there was any mileage in this system for your purposes and how this could be
actioned?

Brian MacKrill's picture

Locked-in and ALS communication devices

Hi Andrea Goldstein

I recently found this website as some years ago I first came to know of people with the Locked-in Syndrome and wanted to do something to help. I have developed a low cost eye-blink computer interface protype and I am wondering if this technology has been surpassed and if I should develop my interface any further. I am aware of the Eyegaze technology which is currently rather costly but obviously will reduce in price as time goes by. I am wondering if people are finding it effective technology as I hear about too many cases where the person is lying helpless and not making use of eyegaze devices instead.

Thanks
Brian

Carla's picture

your system

How is your prototype going? I am interested as I have a friend in the later stages of ALS who cannot communicate effectively, although he has plenty to say! He can blink, but not move his hands or his body. He cannot speak in any understandable way now. He lives in Italy, in Chianti, outside a small village and speaks English and Italian fluently.
Hope to have the pleasure of hearing from you and hope you are well. Carla ..

Brian MacKrill's picture

ALS

Hi Carla
Somehow I missed your message as I haven't checked in on this site for some time. Did you find a communication solution for your friend with ALS?

Brian

Karen's picture

help

Brian, Please contact me! I have a "sister" afflicted with Lou Gerigs and I need help for her to communicate! This may be just the beginning. Thank You!

Brian's picture

ALS communication device

Apologies Karen, I got your name wrong. Moderator, could you please pass on my email address to Karen who has responded to my article. It is unclear if the moderator has passed on my contact details to you so I am posting this response.

Brian's picture

Re: Please contact me

Hello Danielle
I am unsure how to contact you via this website. I can be contacted at

Danielle Hannigan RN's picture

Communication devices

I was wondering if anyone could give me their input on the best communication devices with a patient with progressive ALS. This patient has already tried the Dynavox which is a wonderful device but happens to make my patient nausea. We believe the nausea is related to another eye disease unrelated to the ALS. It is my ultimate goal to get this device as quickly as possible. Thank you for any help you may be able to give us!!!

Anonymous's picture

Advanced Bulbar ALS

Trying to find a chart to download for phrases commonly used to speed communication with my Sister-in-law who has advanced ALS. Can only blink. Would appreciate any info as to what site I can go to to get this information to copy. Thank you.

Anonymous's picture

I made a chart for my Dad

I made a chart for my Dad using Excel.

Here is a link to some premade charts:

http://tpals.org/commaids.htm

Also, you may want to visit Jason Becker's Web site. His Dad developed quite a system for him to communicate using a sort of chart.

http://jasonbeckerguitar.com/video_gallery.html

Watch "Special Report" from Nov. 2008 under the "News Stories" tab.

Anonymous's picture

Thank you- your post on Euthanasia

As a person with ALS, going on four years, this thought and have met people who have gone through what your brother did, I appreciate your concrete input on this subject.

Thank you and take care,
Juri

Charles Bradford (an individual)'s picture

locked-in syndrome

I have read two articles here re assistive
communication for those with locked-in syndrome,
one by Shadia Bel Hamdounia and one by Andrea
Goldstein.

I am a creator of free advanced single-switch
communication-aids that I would like for them to
know exist. Can you help us get in contact?

Thanks,

Charles Bradford, voice tel: 520.826.4201, email:
"chasbrad33@yahoo.com"

susan marfleet's picture

Do you have something to help with a person who is locked in?

My friend is in ICU in Toronto who just had a brain stem stroke and the doctors are saying he is locked in.He can only move his eyes and a little nodding.We cant communicate with him.Its horrible.
Thanks

Serendip Visitor's picture

Locked in syndrome and euthanasia

Do families or patients with locked in syndrome have the option of euthanasia? The thought of being or seeing somebody locked in his own body without being able to move or communicate in any manner is just petrifying.

Serendip Visitor's picture

LIS

I guess it depends on your jurisdiction, but our doctors recommended we try to establish communication with my brother and ask him what he wants.

My recommendation to everyone is to make a living will and keep it somewhere where it can be found if this ever happens to you. I am not a morbid person, and am an optimist about life, so please don't think I am being negative. But, please make one. The hardest thing I ever had to do was try to establish a yes or no communication form to my dear brother and ask him if he wanted to live this way. We asked several times, gave him days to 'think' it over...................but in the end, his decision was firm - he did not want to live that way. We take some peace in knowing at least we tried our best to communicate all the facts to him and he had an ability to direct his fate.

So, I am grateful that our doctors did give us and my brother the option of euthanasia.

Hoping few people will ever have to go through what we had to,

Regards,
Anonymous

Tricia's picture

Euthenasia

My daughter can only communicate with nods. She laughs, goes shopping with me. Enjoys her life even with her many limitations. She can not move much except one arm. I ask her all day what she would like to do, where she wants to go or to eat. I think if I kept asking her if she wanted to die, she would feel that I don't want her. I can't imagine putting that thought in someone's head.i ask her how she feels and she nods when I say the alphabet to spell things out. I think we should help people feel wanted and have life experiences even when their bodies won't move.

Kathi Donegan's picture

Your brother...

I am very sorry to hear of your loss and hope you are finding some comfort in your good memories together. I just wanted to weigh in, as a hospice volunteer, you and your family did the brave and difficult last directive for your brother. ALS is a terrible disease and you were strong enough to help him when he needed it the most.
Best Regards,
Kathi

Post new comment

The content of this field is kept private and will not be shown publicly.