Locked-in syndrome
BMJ 2005; 330 doi: https://doi.org/10.1136/bmj.330.7488.406 (Published 17 February 2005) Cite this as: BMJ 2005;330:406
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The following information is a variation on the LIS communication
chart. My friend Manuel (Oggie) suffered a thrombosis of the basilar
artery in 2000 and subsequent LIS. He developed the system below for
communication.
"Some weeks after my stroke I could only open and close my eyes. I
used a chart like this :
E T A O I N S
R H L D C U M
F P G W Y B V
Z X K P J
The person I would be communicating with would read the letters and I
would close my eyes when the letter I want is read. That way, letter by
letter , I would spell the word I want. This system is very slow but it's
the only way. After some months I regained some head movement and thanks
to that I can now communicate much faster. A system devised for me to be
able to use a computer was based on a small laser mounted on the leg of a
pair of glasses without lenses. With this laser I can now point at the
letters which is much faster and now the letters can be in any order.
In fact the new letter card I want to make will have the letters in
alphabetical order and I will make a couple of additions :
1) Numbers
2) the "@" sign
3) the space
4) the dot
These signs are very useful.....try spelling an email address without
them. Another advantage of using the letters in alphabetical order is that
someone who has never communicated like this will find it easier and more
logical. On the back of the card I will have instructions on how to use
it.
Now I have a small mains adaptor when I am in bed and a power source
from the wheelchair's battery for the laser. In bed I have a laser mounted
on the leg of a pair of glasses without lenses while on the wheelchair I
have another laser mounted on a headband.
An A2 sized board is often used and it's a good system. Since I point
the laser, my board is much smaller (A5 - half A4 or half letter size for
USA people)".
Competing interests:
None declared
Competing interests: No competing interests
The chart we used for my son, Erik, was designed taking the vowels
out of the alphabet and the dividing the rest of the alphabet into lines
an columns, reducing eye movements to convey responses. Here is the chart
we used:
1. B C D F G H
2. J K L M N P
3. Q R S T V W
4. X Y Z
5. A E I O U
We would ask the line first, then column 1 (left column) or column 2
(right column). Then ask each letter until the right one was identified
with an upward eye movement. Erik lost the ability to use the chart after
approximately 3 years from the time he was diagnosed with LIS. This
ability was lost after his second bout with pneumonia. We have now had to
resort to the guessing game of what he wants to convey.
Competing interests:
None declared
Competing interests: No competing interests
Smith and Delargy (18 February issue)1 provide a thorough review of
the locked-in syndrome, describing, inter alia, causes and mechanisms of
the syndrome. However, they fail to mention the “peripheral” causes of
locked-in syndrome, which include neuromuscular blockade, drug
intoxication, advanced amyotrophic lateral sclerosis and iperacute
polyneuropathy2. We have recently observed two patients with a fulminant
polyneuropathy who rapidly deteriorated to a clinical state of “total
locked-in syndrome”. The neurological picture of the second patient
resulted in an apparent “brainstem death”.
Patient 1. Ten days after a flu-like syndrome, a 57 year old woman
developed paresthesia and leg weakness. The patient became progressively
weaker in the four extremities, areflexic and ataxic over 24 hours. Blood
chemistry findings were normal. The cerebrospinal fluid (CSF) examination
revealed an albuminocytological dissociation. The nerve conduction studies
revealed an inexcitability of all nerves. A diagnosis of iperacute
Guillain-Barré polyneuropathy was made. In a few ours the patient
developed a flaccid quadriplegia and had bilateral involvement of all
motor cranial nerves with complete (horizontal and vertical)
ophthalmoplegia and bilateral ptosis (“total locked-in syndrome”), but
with initial preservation of consciousness. Subsequent reduction in vital
capacity with hypoxia and lethargy necessitated transfer to the intensive
care unit for intubation and plasmapheresis. The Electroencephalogram
(EEG) showed an alpha and theta activity responsive to sensory
stimulation. Due to impaired eye closure, the patient later developed a
left corneal ulceration. The patient was weaned off the ventilator after
three weeks. Her motor functions slowly improved with prolonged
rehabilitation and ten months later she almost fully recovered. The
patient could partially remember the period when she was clinically
unresponsive.
Patient 2: One week after influenza vaccination followed by diarrhoea, a
78 year old woman suddenly experienced double vision, difficulty in
swallowing and gait ataxia. Laboratory investigations were negative. A
provisional diagnosis of brainstem stroke was made but a MRI of the brain
was normal. In the ensuing hours the patient became quadriplegic and
clinically unresponsive, lost all motor cranial nerve functions and
brainstem reflexes, did not breathe spontaneously and required intubation.
Tendon reflexes and plantar responses were abolished. She then no longer
triggered the ventilator, apparently meeting the criterion of brainstem
death. The EEG, however, revealed diffuse alpha activity partially
reactive to passive eye opening, acoustic stimulation and sternal rub. The
CSF examination revealed an albuminocytological dissociation. A diagnosis
of fulminant Guillain- Barrè polyneuropathy was made and intravenous
immunoglobulins were started on day 3 of symptom onset. The findings of
electromyography and nerve conduction studies confirmed an iperacute
axonal neuropathy. The patient remained clinically unresponsive until the
30th day when she started showing slight eye movements on the vertical
plane and some movements of the lower extremities. The patient slowly
recovered, and was weaned off the ventilator after four months. With
intensive rehabilitation the patient gradually regained the motor function
and, after two years, she was able to walk. She was able to recall the
period of her unresponsiveness.
These two patients suffered a severe acute paralysis with rapid clinical
deterioration, such that they ended to meet the clinical criteria for the
diagnosis of total locked-in syndrome and brainstem death, respectively.
In Patient 1, the diagnosis of Guillain-Barré syndrome was easier, based
on clinical history, CSF analysis and electrophysiologic studies,
demonstrating a severe acute polyneuropathy. In Patient 2, the initial
diagnosis of an acute brainstem disease was strongly considered. However,
the normal MRI imaging of the brain did not support this hypothesis.
Instead, subsequent investigations (CSF and nerve conductions) were
consistent with an fulminant axonal polyneuropathy.
The Guillain-Barré syndrome commonly leads to admission to an intensive
care unit for mechanical ventilation. Our patients illustrate that the
clinical picture of the disease can progress to a “total” locked-in
syndrome and a clinical state mimicking brainstem death, as pointed out by
other authors3. They also emphasize the importance of the EEG in cases of
unresponsiveness of uncertain origin. Nerve conduction studies and EMG are
obviously required for the recognition of the neuropathy. Clinicians
should be reminded that, although unusual, a fulminant polyneuropathy has
to be considered in the differential diagnosis of locked-in syndrome and
even brainstem death. Indeed, the erroneous diagnosis of both conditions
in cases of fulminant polyneuropathy may lead to inappropriate management
of a treatable and reversible condition, whose prognosis is often
favourable, provided that a prolonged rehabilitation is guaranteed.
References
1 Smith E, Delargy M. Locked-in syndrome. BMJ, 2005 Feb 19;
330(7488): 406-9.
2 Wijdicks EFM. Neurologic states resembling brain death. In EFM
Wijdicks (ed.) Brain death, 2001. Lippincott Williams & Wilkins,
Philadelphia: pp115-34.
3 Friedman Y, Lee L, Wherrett JR, Ashby P, Carpenter S. Simulation of
brain death from fulminant de-efferentation. Can J Neurol Sci 2003; 30
(4): 397-404
Competing interests:
None declared
Editorial note
The patients whose cases are described have given their signed informed consent to publication.
Competing interests: No competing interests
Alexandre Dumas gives what I believe is the first (and certainly most
poetic) account of locked-in syndrome in the world literature - admittedly
in the fictional form of M. Noirtier de Villefort. This appears in "The
Count of Monte Cristo", written in 1844.
"M. Noirtier was sitting in an arm-chair, which moved upon casters,
in which he was wheeled into the room in the morning, and in the same way
drawn out again at night. He was placed before a large glass, which
reflected the whole apartment, and so, without any attempt to move, which
would have been impossible, he could see all who entered the room and
everything which was going on around him. M. Noirtier, although almost as
immovable as a corpse, looked at the newcomers with a quick and
intelligent expression, perceiving at once, by their ceremonious courtesy,
that they were come on business of an unexpected and official character.
Sight and hearing were the only senses remaining, and they, like two
solitary sparks, remained to animate the miserable body which seemed fit
for nothing but the grave; it was only, however, by means of one of these
senses that he could reveal the thoughts and feelings that still occupied
his mind, and the look by which he gave expression to his inner life was
like the distant gleam of a candle which a traveller sees by night across
some desert place, and knows that a living being dwells beyond the silence
and obscurity. Noirtier's hair was long and white, and flowed over his
shoulders; while in his eyes, shaded by thick black lashes, was
concentrated, as it often happens with an organ which is used to the
exclusion of the others, all the activity, address, force, and
intelligence which were formerly diffused over his whole body; and so
although the movement of the arm, the sound of the voice, and the agility
of the body, were wanting, the speaking eye sufficed for all. He commanded
with it; it was the medium through which his thanks were conveyed. In
short, his whole appearance produced on the mind the impression of a
corpse with living eyes, and nothing could be more startling than to
observe the expression of anger or joy suddenly lighting up these organs,
while the rest of the rigid and marble-like features were utterly deprived
of the power of participation."
The book's denouement depends upon the diagnosis; those who have not
read the book should do so, as it is a classic portrayal also of of
revenge that will not be quenched - pathological in its expression. That
Dumas was only a legal clerk makes it all the more interesting.
Competing interests:
None declared
Competing interests: No competing interests
The sufferings, losses and complications of a person in Locked-in - syndrome.
Sir,
Nothing could be more terrible than Locked-in-syndrome. My mother
Alli Subramaniam was only 38 (September 5th, 1981)when she went into this
hell. It is very difficult for an outsider to understand or comprehend the
total loss of dignity, power, and control over life. It not only changes
the life of person affected but also the family around, though the
sufferer is the person herself.
In those days in India this condition was not known and it was
labelled as "Viral Encephalities" and " Qudriplegia with aphasia". It took
dedication and sacrifice from my father to understand her condition by
reading articles and writing to people, subscribibg to journals and
educating himself.
Still we could only helplessly and foolishly watch her suffer with
out being able to bring her out of this horrible situation. Inside we
would cry and outside put a brave face before her, constantly encouraging
her, entertaining her with jokes, old memories and good times we had. It
breaks us to see her in this situation.
Over years we train ourselves to understand her. Social datings and common
talk is of no importance. Only she is considered of prime importance as
she is locked in herself.Our home is turned into a hospital with suction
machine, oxygen cylinder, nebulizer, emergency medicines and continuous
nursing support.
If it can benefit the community, here are few of our observations.
Initially she was full of energy and alert, but as days and years go by,
the sheer hopelesslessness strikes. Energy level and will power to live
reduces. Eyes which are the only hope for her gets weaker. Is there any
way she can express if a catract develops? Her hearing was above perfect.
She turned her head sweetly to any small noise or whisper. The smile which
is preserved goes away in the last few years. Even a small itching or ant
bite is a huge frustating problem for her. As age advances, osteoporosis
sets in and multiple fractures of vertebra of her thoracic and lumbar
spine occured . It requires exposure to sunlight and hormone treatment.
She has a tracheostomy for 27 years. This leads to respiartory
infections, and soreness around the tube. The worts complication is traceo
-oesophageal fistula due to the long years of insertion. This causes food
to leak out of the tracheostomy tube when she swallows(especially
liquids). I often imagine myself in her place. How she would feel when a
tube is inserted into her trachea and she cant lift her hand and touch her
neck or feel the area around? And she is aware of what is happening. How
it would feel when you wnat to do some thing( a simple activity) and
cannot? Many times when we lift her (though we are careful) the
tracheostomy tube tilts, sometimes it causes violent cough. Even
meticulous care and devotion cannot prevent such episodes. The only great
advantage of the tracheostomy is that when she needs to be hospitalised
and put on ventillator, it is very useful and painless compared to
endotracheal intubation.
In women in premenopausal age group, menstrual cycles proves to be a
irritation.
As I read in the article, she didnt develop incontinence ( inability to
hold urine or involuntary excretion of urine) . There is no dribbling of
urine. It is a spastic bladder where they learn to hold urine for 2-3
hours and then pass urine( on hot days). Infact she is very aware when
friends visit that she holds back urine in the presence of outsiders. And
when she cannot hold back, she opens her mouth wide to make us aware that
the other persons have to leave and only then passes urine. Such a
terrific control and intelligence I witnessed in her.
Common complication are fits, urinary infection due to stasis,
consolidation of lungs and right ventricular failure.Blood tests every
month is a must.
Hand movements vary and due to spasm she could hold things like remote
control of TV. It gave her immense pleasure when we tell her to change the
channels. Infact our mind gets so locked with them and their activities
that we have no other thoughts day or night or in our dreams.
I often worry how will she express her chest pain? Or headache? or
sorethroat? Or abdominal pain? Or earache? There is no communication
system existing in India for locked-in-syndrome.
Though we tell our selves God has a purpose, it is extreme torture,
pathetic and heart breaking. If you see such conditions for years and
their helpless state, you tend to loose interest in life.
Still we need to keep up the courage and continue with life.
The only promise and hope which we discuss every day in family is stem
cell research. It may or may not work miracle for the Locked-in- syndrome
victims. But when we are in that position when we are aware of every
detail around us and sensory perception is excellent, but unable to move
or communicate,then the situation is desperate. Whether or not there is a
recovery is immaterial as the sufferer has lost every thing and there is
nothing more to loose.
Injection of adult stem cell( from the own patient) or embryonic stem
in the brain stem level could be tried and seems to be the only ray of
hope. If there is any way to allevate the suffering and improve thier
communication and quality of life, it would be a greatest reward for
mankind.
For every person in Locked-in-syndrome my only message is after this
earthly suffering is over you are sure to be in heaven while the remaining
of us will not get the happiness, peace and eternal bliss you are due for.
Latha Rajendra Kumar,MD.
Competing interests:
None declared
Competing interests: No competing interests