Frequently Asked Questions about the Locked-In Syndrome

The Locked-In Syndrome (LIS)

Frequently Asked Questions:

1. What is LIS?

2. What causes LIS?

3. How many people have LIS?

4. Can people with LIS communicate ?

5. Can you live with LIS? Can you steel feel happy at times?

6. Are these people better of dead?*

7. Can LIS be cured?

8. How do people with LIS spend their day?

9. Can people with LIS be in love and be intimate?

10. Where can I find more information on LIS?

* This question is phrased quite harsh. I left it uncensored, since i know this is what most healthy people are thinking and this document attempts to answer the FAQ. Even if painful.

DR. FEMKE NIJBOER | HEALTH, MEDICAL AND NEUROPSYCHOLOGY UNIT | LEIDEN UNIVERSITY

What is Locked-In Syndromeor Locked-In State (LIS)

Most people say:

“A person with locked-in syndrome is completelyparalyzed, but has intact cognition. That is.. he can think

and feel normally, but he cannot move”

“So, you are locked-in your own body”


Definition of the Locked-In Syndromeor Locked-In State (LIS)

Classical LIS+ eye movement

Complete/TotalLIS

Residual LIS+ eye movement

+ few residual muscles

Quadriplegiaparalysis of limbs

& torsofacial muscles and

speech intactAnarthria

However, we need to distinguish between different degrees of severity


(some) Causes of the LIS

• Infarct or haemorrhage to ventral pons

• Late-stage Amyotrophic Lateral Sclerosis (ALS)

• Trauma


How many people with LIS are there?

• Prevalence classic LIS: 0.7/10.0000 somaticnursing home beds (Kohnen et al, 2013)

• However....we do not really know

• Many people live at home and go unnoticed

• Nobody properly counted (we’re working on that)


Can people with total LIS communicate?

• Total LIS:no commication possible.

• So how do we know they are conscious?We don’t.

• What can be done to diagnose consciousness?Since these people do not show any behavior, we need toestimate their level of consciousness by directly assessing theirbrain functions. For this we need neuroimaging.

• What else can be done?Assume that they are conscious and treat them with respect, dignity and kindness.


Can people with classic LIS communicate?

• Classic LIS:Alphabet system and maybe eye tracking system

• Do we know they are conscious?Yes! They can give sensible answers to yes and no questions. And they can select letters suggested to them (alphabetsystem). This way they can form words, sentences, emails, love letters and complaints. Just like the rest of us

• I find it scary to talk to them like that.Understandable, but try. After a few rounds you’ll see the alphabet system is not difficult. I can be even fun to guess the word. Plus, it’s worthwile to get to know the person inside


Can people with residual LIS communicate?

• Residual LIS:If you have a 1 or 2 muscle functions then assistive technologyspecialists can offer a “switch” that allows to give a command. With a command you can control just about every technologyyou can think of. Also communication programs.

• What kind of muscle function can be there?Some people can move their lower lip, use a thumb or tilt theirhead a bit to the right. If so, specialists can attach sensitivebuttons to that area.

• Why can’t they use the alphabet?They can, but sometimes they might prefer a technology tocommunicate. For example, when you want to communicteprivate messages to your wife or kids.


Can you live with LIS? Can you still feel happy?

• Large majority rates quality of life as satisfactoryor good

• Minority rates quality of life as poor

• This surprises many able-bodied people (it is counter intuitive to most people)

• Factor “time” is important

• “You never get used to it, but you accept it” (comment

from woman with > 30 yrs LIS)

• “You have to accept it as soon as possible, andthen move on” (advise from woman with >20 yrs LIS)


Are these people better of dead?

• A very personal and complex question• This question should not be ignored

– Do not make it a taboo issue

• But it should not be overemphasized either– The majority of people is relatively okay (previous slide)– Imagine the effect if you, as a person with LIS, are told 20 times a day that it is

so absurd that you would want to live… You would get depressed or annoyedquite easily

• Studies show that most people with LIS find life worth living

Personal opinion• I tend to say: “most people are better of living, and we should use our

energy making life possible for them”• I also think: “if people really want to die (and they are well-informed,

autonomous and capable of judging their situation and prospects), thenwe should make death possible for them”


Can LIS be cured?

• After LIS it is unlikely that you will ever be as ableas you once were

• If people can improve after LIS depends on the cause of LIS:– After a brain stem stroke /infarct: small improvements

are possible. Don’t expect miracles, but keep hope and try rehabilitation. Factor “age” seems important too

– When you reach LIS after a progressive disease likeALS or SMA, then the chance that you will improve are unfortunately very small. Functional decline mightstop for a while, so you have a stable period


How do people with LISspend their day?

• This varies from person to person and with degree of LIS:

• Total LIS: in bed

• Classical and residual LIS: mostly upright in a wheelchair; after a few years most people establish a routine with care acivities, hobbies, corresponce with friends, and occassionally trips outside the house (friends, concerts, museums)


Can people with LIS work?

• Yes, some can. A few examples:– A woman who did voluntary work to lobby for

accessible public transport– A man who continued to run his company – A man who continued to run his research lab

• Complicating factors:– Amount of time needed for care– Fatigue– Need for adapted tools– Content of the work (physical work is no longer

possible)


Can people with LIS be in love and be intimate?

• Yes, definitely. Living with a disability does notmean you cannot be in love. Obviously.

• People can fall in love and be in love with peoplewith LIS as well. Really.

• People with LIS can also be intimate with partners– Literature on this is scarce as you may imagine

– Therefore, I imagine that also help is scarce whenpeople experience sexual problems or have unsatisfied needs

– Taboo subject


Where can I find more information on LIS?

• Due to the rare disease, you may find out thatphysicians, nurses, general practitioners do not have adequate information

• The advise of a person with LIS: “Be pro-active!”.

• Check the facts yourself, find opportunitieswhere others don’t see them.

• Find peers.

Would you like to ask a quesion? Let me know.

[email protected]

Disclaimer: I am not a medical expert. I am a neuropsychologist. I have seen and interviewed many people with LIS, but they

might not be representative of all people with LIS. These answers are not neccesarily the truth, but an educated guess.

Health & Medicine

Frequently Asked Questions about the Locked-In Syndrome