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An early account of locked-in syndrome
- Andrew N Bamji (28 February 2005)
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"Peripheral" locked-in syndrome and brainstem death
- Alberto Primavera, Fabio Bandini (10 March 2005)
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Communication Chart
- Eddie R. Ramsey (18 May 2006)
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LIS & Communication Chart
- Hana F Al-Ahmar, Manuel Longo (29 May 2006)
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The sufferings, losses and complications of a person in Locked-in - syndrome.
- Latha Rajendra kumar (13 December 2007)
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Andrew N Bamji, Consultant (rheumatology/rehabilitation) Queen mary's Hospital, Sidcup Kent DA14 6LT, UK
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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 |
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Alberto Primavera, Associate Professor Department of Neurosciences, University of Genova, Via De Toni 5, 16132 Genova, Italy, Fabio Bandini
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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
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Eddie R. Ramsey, Maintenance Supervisor U.S. Postal Service 1605 Boggs Rd. Duluth, GA 30026
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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 |
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Hana F Al-Ahmar, Consultant Clinical Neuropsychologist Intermediate Neurorehabilitation Unit,Manchester Royal Infirmary, M13 0EU, Manuel Longo
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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 |
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Latha Rajendra kumar, Physiology Unit, Faculty of Medicine Asian Institute of Medical Scince and Technology, Sungai Petani,Malaysia
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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 |
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