Acta Neurol Scand DOI: 10.1111/j.1600-0404.2011.01552.

2011 John Wiley & Sons A S

ACTA NEUROLOGICA SCANDINAVICA

Unlocking the locked in; a need for team approach in rehabilitation of survivors with locked-in syndrome
Schjolberg A, Sunnerhagen KS. Unlocking the locked in; a need for team approach in rehabilitation of survivors with locked-in syndrome. Acta Neurol Scand: DOI: 10.1111/j.1600-0404.2011.01552.x. 2011 John Wiley & Sons A S. Objectives A stroke that aects the medulla oblongata and or pons can result in tetra pareses and paralysis of the lower cranial nerves while other parts of the brain remain intact, thus locking the person in. The incidence and prevalence is not known. The aim of this article is to communicate the need for and benets of a comprehensive rehabilitation and a standardized way of approaching the locked-in person. Material and methods To illustrate the rehabilitation process, we present four cases to highlight the needs of the person and what is required of the team. Results Communication at arrival: three persons communicated through eye movement, one by weak voice. At follow-up (16 years later): computer assisted communication was used by two persons, a letter board by one and ordinary communication by one. Conclusion There is a need for follow-up not only to re-assess skills and needs partly owing to new technologies but also to see whether the person needs more assistance to adapt to the alternative means of communication or whether the carers of the person need extra information about communication. We conclude that the low incidence of the syndrome necessitates a skilled team in which dierent professionals can together assess the person. This probably requires some centralization.
Introduction

A. Schjolberg1, K. S. Sunnerhagen1,2
1 Sunnaas Rehabilitation Hospital and Faculty of Medicine, University of Oslo, Oslo, Norway; 2Section for Clinical Neuroscience and Rehabilitation, The Institute of Neuroscience and Physiology, University of Gothenburg, Gteborg, Sweden

Key words: stroke; rehabilitation; quadriplegia K. S. Sunnerhagen, Rehabilitation Medicine, Per Dubbsgatan 14, 3rd floor, Sahlgrenska University Hospital, S-413 45 Gteborg, Sweden Tel.: +46 31 342 29 24 Fax: +46 31 41 54 33 e-mail: ks.sunnerhagen@neuro.gu.se Accepted for publication May 15, 2011

Locked-in syndrome (LIS) is a condition in persons struck by a lesion (spontaneous or traumatic) in the brain stem, normally corresponding to the ventral pons lesion. While the most common cause is an infarct caused by an obstruction of the basilar artery, more unusual phenomena can occur such as haemorrhages, traumas, tumours or encephalitis. LIS is characterized by upper motor neurone quadriplegia, paralysis of lower cranial nerves, bilateral horizontal gaze and anarthria but with preserved cognitive capacity. This can also lead to alterations in the steering mechanisms of respiration, blood pressure, temperature, swallowing and speech. The LIS has saved eye eyelid movement, and the locked-in state also saved nger or toe movement. We have not been able to nd any statistics on the frequency of LIS after stroke, which can be taken as an indication of its

rareness. Little information is found on the outcome of LIS: how often do the persons remained locked in or does some kind of recovery occur as in many other cases after a lesion (1)? There are also reports that not all persons who have been diagnosed as having LIS have maintained cognition (2, 3). Luckily, not all lesions in the brainstem lead to LIS. LIS sufferers after traumatic lesions in pons often have extra problems because of diffuse brain damage elsewhere and or anoxic brain damage (and therefore cognitive impairments) that inuences rehabilitation and communication as well. A survey was carried out in France (4) in which 44 persons with LIS participated. The distribution of sexes was equal, and most reported normal cognition but stated they were quite isolated. The conclusion drawn from this survey is that there is little treatment and rehabilitation (4). If this survey is representative, and we presume that about 50% of persons with 1

Schjolberg & Sunnerhagen LIS in France participated, the prevalence of LIS should be about one to two per million. Prior reports on persons with LIS have focused on the impairments that follow the lesion and or experiences of living with LIS (5). We have not found information on how to treat train these patients. The question is whether an organized care rehabilitation process is available that can start when the person needs it or whether every unit has to re-invent the wheel. Sunnaas Rehabilitation Hospital has country wide responsibility for Norway (4.5 million inhabitants) for this patient group. The length of stay for in-patient rehabilitation among persons with LIS is currently about 3 months. This requires a rapid assessment of the resources that are needed and of what areas must be emphasized to be able to give intensive treatment and rehabilitation. Many persons with LIS express the need to establish communication and, if possible, have tailor-made communication devices. This requires dierent professional skills, knowledge of psychological reactions and access to information and communication technology. The aim of this article is to discuss the need and benets of a comprehensive rehabilitation and a standardized approach to the person with LIS.
Material and methods

setting goals for the rehabilitation process. Comprehensive team work is needed to assess the persons situation from dierent perspectives. The reception of the patient at the ward is important. The feeling of being unable to communicate and knowing that breathing and swallowing are dicult are stressful. The basic goal of rehabilitation is for the person to feel safe and condent in the environment and with the team. Inner security is often coupled with self-assurance and mastering, which are necessary components of good quality of life. It is dicult to achieve a feeling of safety in persons in need of total care when there are frequent sta changes, which emphasizes the need of dedicated personnel. However, all personnel on the ward need to have some knowledge of LIS and the person to help when necessary. There is a need to assess the persons feeling of security on the ward, and his or her endurance, motivation and interests, as well as the capacity of next of kin to assess rehabilitation and participation in the long run.
Assessment The basis for care of the person with LIS is threefold. The rst need is to establish sucient breathing, the second to give nutrition and the third to establish some means of communication. Assessments must be made of O2 saturation, nutritional status, communication skills and cognition. Pulse oximeters are employed to assess O2 saturation, together with occasional arterial oxygen sampling. The prestroke weight is documented and followed closely for an assessment of nutritional status. The triceps skin fold is sometimes assessed as a measure of subcutaneous fat. Eye blink and other eye movements are examined to assess communication prociency. Concretely, this implies showing a two-coloured card and asking the person to look at one of the colours. The person is then asked to look at a letter on a letter board or a picture containing a gure. During this time, another person observes whether the patient can start and stop an action or motion with the eyes or the head. It is important to have knowledge of prior problems in reading and writing as well as of computer experience. The physiotherapist, the nurse, the occupational therapist and the communication team try to assess how the patient copes with instructions and whether there is a delay in the patients responses. The results of this basic screen are used by the neurophysiologist to tailor the test methods for cognition. Basic assessment of cognition is always a part of admission procedures where the physician and the

In this article, we discuss persons with LIS caused by a stroke in the brainstem pons. There is also a focus on the functions and resources these persons might have, which can be used for training before establishing communication. Sunnaas Rehabilitation Hospital has an LIS team in which dierent skills are represented. However, the team has only one person per skill area. The team consists of a nurse and nurses aid, a physiotherapist, an occupational therapist, a swallowing specialist (speech and language therapist), a physician and the hospitals communication team (a speech and language therapist and an engineer). To illustrate the rehabilitation process, we present four cases of LIS at hospital admission. Data were gathered from patient charts. During the period 20002007, about 15 persons with LIS were treated at Sunnaas Hospital. These four LIS cases were selected because they showed diversity in capacity, needs and goals.
At admission

Above all, there is a need to establish the preferences and personal goals of the person with LIS in 2

Rehabilitation of the locked in nurse form a rst impression. Continued assessments of cognition are a joint responsibility of the team. The nurse can gather much information on cognition while caring for the patient. Among other things, does the patient respond to a smile, does he she understand a joke? In everyday conversation, many words sayings also have a double meaning; does the patient understand this? The psychologist usually masters the means of communication used but might need someone in the team to act as an interpreter. The testing takes place in a secluded environment in which the person is relaxed. Formal neuropsychological testing aims to assess capacity to take instructions, psychological level and memory. Pathological laughter and or crying sometimes occur (6) and information about this and the probable linkage to the damage in pons is also necessary. Knowledge and understanding of the background to the outburst, which is not always emotional, is a key component of treatment (6). There is also a need for assessment of head and trunk control, nger movement and body stability to be able to use equipment for communication. Instructions are given to the patient to move the eyes before moving sides, to prepare the body for turning in bed. Each person in the team observes dierent things according to the professional background, which will not only be of use for his her tasks but also for the rest of the team.
Intervention After the dierent functions and skills are identied, the team work towards a common goal: to compensate for the shortcomings in the environment, to train the motor decits and to motivate the person mentally to apply the resources available. Caring procedure: There is a high risk of infections (pneumonia, urinary tract infections or pressure sores). For respiratory function, there is a need to measure the oxygen saturation in the blood (supplementary oxygen delivered when needed). Positioning is important for breathing; the use of a peak expiratory pressure mask must be considered, as well as a cough machine. Phlegm in the trachea, oral cavity and pharynx must be sucked often to reduce the risk of infection and increase the chance of optimal respiratory function. Oral hygiene is important because infections in the mouth can aect respiration and feeding. Feeding (no gastric feeding is totally safe for the LIS patient) is usually performed through a percutaneous gastric tube. Feeding requires an elevated head end of the bed or sitting. Establishing safe and sucient nutrition reduces the

risk of infection and is necessary for physical activity. There is a need to reduce stress and anxiety. The caring procedure should aim to give a feeling of security. Surveillance cameras can sometimes be used (with the permission of the patient) to give security. The person needs to be looked after, needs stillness both physically and mentally, but also needs tailored information. Especially initially, the stability of the treatment group is very important. The schedules of the sta must be changed according to the persons needs. Physical contact can be used to comfort and give a feeling of safety and can be used as a way to achieve good communication with the patients. This type of contact can be used actively in treatment, but only after a trustworthy relationship has been established. Being put in bed with extra pillows and cushions will make the person secure there, which gives a feeling of safety. Feeling the oor during training gives a feeling of security, which is important (this can be done by using shoes and having the feet on the oor while sitting in a wheelchair). It is important to try to understand the person with LIS, and the personnel must take time with the patient. This is necessary to ensure the patient that he she is seen as cognitively adequate and that we as sta are ready to accept everyday communication in dierent ways. We have to be open minded to dierent modes because communication takes place not only in conversations but also in behaviour, mimicking and postures. Motor function: It is important to move all the joints, tendons and muscle groups to prevent spasms and contractures. This also helps the person to allow him herself to be touched and to become aware of this new body. A next step can be that two persons are together with the LIS patient on a tilt table to train head and trunk control. One person sits behind the patient to give support, and one person is in front to guide and to stimulate movements in the trunk, the neck and all four extremities. The use of a standing bed, and later a body supported treadmill, gives the person support from the oor and increases the tolerance for variations in blood pressure. Using the treadmill with body weight support needs to be tailored to the persons stamina. Typically, stamina increases with an increase in time sitting up in a chair or longer periods of training. Communication: For basic communication, a person with LIS can often communicate by winking the eyes, where one wink is yes and two is no, or can look up for yes and down for no. Letter boards and eye pointing boards are a simple and 3

Schjolberg & Sunnerhagen fast means of communication for persons with LIS. The letter is conrmed with a wink of the eye or next of kin can act as an intermediary with the person with LIS present. Eye pointing boards are easy to use and to carry, and it is easier and faster than using the traditional letter board. The eye pointing board is constructed of a system where the letters have dierent colours and are split into groups with a colour code in the middle. The person has to move the sight twice per letter. The board needs to be tailored according to vision, visual eld and other things. The next of kin are suggested to be taught by LIS persons; this gives a feeling of having mastered the skill, which is of the utmost importance. If needed, the sta can do the training. To make communication faster, we often suggest that we are allowed to guess what word is coming when we believe we know. This is satisfactory to many patients because they can then tell more. Adapting more advanced equipment for communication is a long process. The dierent electrodes and switches are rst applied in the motor functions that are identied and there is an evaluation of the movements (large or strong enough to activate the equipment). The rst step is to give a possibility to call for help. Later, the same electrodes or switches can be used for the computer, manoeuvring the wheelchair or controlling the indoor environment. The training to use this equipment is given by the communication team and the other personnel in the LIS team, and the next of kin are also instructed.
Four cases

The rst case was a man, 70 years old, who had a brain stem infarct resulting in tetra paresis and LIS. The rst rehabilitation period lasted for 2 months, and his main goal was to establish communication. He then returned three more times, where the emphasis was on alterations development of dierent communication devices (Table 1). The second case was a woman, 40 years old, who had a brain stem infarct resulting in tetra paresis and LIS. She spent 3 months in primary rehabilitation. The focus was on feeding and on head, neck and trunk control. She trained on a body weight supported treadmill to increase control of the head, neck and trunk because walking on this enhances the postural reexes. At discharge, she was fed orally twice a day with mashed food and had a better sitting position. Later, at follow-up, she had an electrical wheelchair that she controlled with head and nger 4

switches, in the same way, as she controlled her PC and her home environment. The third case was a man of 60 years, with a brain stem infarct resulting in tetra paresis and LIS. He had 4 months of primary rehabilitation and two additional sessions. His main problem was anxiety, which limited the rehabilitation. He had a supportive but worried family, and thus a great deal of time was spent on trying to reduce anxiety, including the use of a surveillance camera. He had problems in breathing and swallowing and suered pneumonia more than once. He had a weak voice at the beginning, and this eventually led to him being able to utter a few words. Over time, he gained condence, felt more secure and could live at home with the support of his family and a personal assistant. The last case was a woman of 40, with a brain stem infarct resulting in LIS. She had 3 months of primary rehabilitation and three additional sessions. At admission, she had a weak voice, communicated with a letter board, had started to take some nourishment orally and had some nger movements. She was very t prior to her stroke and cognitively ne and determined to train. Her problem during the whole period was spasticity, which interfered with her training and her rest. Dierent physical treatments were used, and she was put on Baclofen, which reduced the problem. In this case, special training had to be given for control of the chin, because spasticity interfered with her oral communication and eating. She managed early to use electronic equipment and switches and wanted to manage herself. She could communicate vocally to some extent and used her computer when she got tired. She applied for an adapted car with different switches and now drives using nger and head switches, and video cameras for surveillance of the trafc situation. She currently works 50% and has released a book on her experience of stroke, the time in the different hospitals and getting back into life. She claims that the only inconvenience in having suffered LIS is that you are paralysed and that everything takes too long however, that is not that much of a thing to worry about!
Discussion

The aim of this article is to try to communicate the need for and benets of a comprehensive rehabilitation and a standardized way of approaching the person with LIS. The incidence and prevalence of LIS is not known, but it is probably low. There is thus a risk that the person with LIS will not be correctly assessed and trained.

Table 1 Description of four cases During rehabilitation Cognitive screen was performed Training balance and head control in treadmill and standing table Adjusted glasses were tested At discharge Follow-up Used head controlled electrical wheelchair Stable situation for feeding and elimination Less mucous in throat

Cases

At admittance

Man, 70 years Brain stem infarcts

Vertical eye movements Some head motion PEG was used Letter chart was used Had an active family

Woman, 40 years Brain stem infarcts Face and oral motor training Breathing training Training of head, trunk and finger movements Body weight support on treadmill for stamina Trained sitting balance Resting positions in bed Was given information and psychological support Started to use PC

Very weak voice Eye movements Could move 1 finger Had some head motion Fed through PEG and ate some mashed food Used letter chart

Had an adjusted home Had an electrical wheelchair with head and finger switch Used a PC with sound, SMS function and could control the indoor environment with this

Man 60 years, with a brain stem infarct Cognitive screen was performed Had large security and safety needs in communication information Was monitored by a surveillance camera to feel secure Received reading glasses Was given facial massage Had facial and oral motor training Performed a spirometry Had breathing training Trained the head, trunk and finger movement Was put on body weight support on treadmill for stamina Was taught that movements could be applied to reduce spasms Was given adjustments of surfaces close to head, cheek and fingers Used taped books, eye pointing board, roll talk Had to be treated for pneumonia

Weak voice Could say yes no Had some eye movements Had motion in one thumb Had an active family

No signs of cognitive impairment Used adjusted PC with switches Managed keyboard on the screen Mastered purposeful head movements Used an electrical wheelchair Used eye movement chart Had an alarm call The family and personal assistants were trained Improved adjusted sitting position in wheel chair Applied for electrical wheel- chair in the home community Used an eye pointing board Managed her PC with head switch Ate 2 meals day with mashed food The family and personal assistants were trained Community health care and family physician informed and involved Home visit was performed A personal plan for continuous rehabilitation was put into action Could use PC Had been training in the home setting Applied for an electrical wheel chair with a finger switch in the home community Used eye pointing board and body language for communication Had better facial mimic Could utter single words Felt more secure

Did not want to leave home for a follow-up period at the hospital. The discharge planning had worked and the family managed

Rehabilitation of the locked in

Schjolberg & Sunnerhagen

Had adjusted living quarters Had adjusted car and was driving herself Was working part time Has written a book about LIS

It seems that rehabilitation of persons with LIS has an enormous impact on life quality, because one of the major goals is to establish stable communication. The assessment of cognitive function (2, 3) is extremely important because this will inuence the possibility for the person to use communication devices. Gaining better information about what to expect strengthens the possibilities of the person and the family to better handle the future after the stroke (7). The aim of the rehabilitation team is to help the person to focus on his her skills and mastering and how to ll expected roles in daily life in a different but still a good way. Good communication in the team (8), as well as between the person, next of kin and the next link in the chain, is important for the outcome. A limitation in this study is the low number of persons for whom we have complete data, which forces us to present only four persons. However, all four had success in communication, which agrees with the experience of the LIS team in the past 10 years. The result of a provision of a dedicated skill team for assessment and subsequent interventions is better communication skills. In one of the cases in this study, diculties were experienced in the next link in the chain. One common challenge is the complicated IT system that is sometimes constructed, which transfers the need for special competence to the next link. In recent years, the hospital has oered followup periods for persons with LIS. This is as important and rewarding for the persons with LIS as for the team. We as a team get information about the experiences of those with LIS. During the week of the follow-up, the team receives information about whether what we suggested worked and what is needed to make things work better in the home environment for the persons as well as for the communities in which the persons live. The follow-up also makes it possible for the next of kin and personal assistants to get more information and support to be able to handle the challenges that they meet. We have steady contact with some of the persons with LIS. Some of them have lived for 510 years with LIS, a few 20 years. Most of them state that they live a good life, considering the limitations.
LIS, Locked-in syndrome.

Follow-up At discharge During rehabilitation At admittance

Woman 40 years with brain stem infarcts

She had a weak voice Used letter board Had some finger movements Had good physical form prior to stroke Appeared normal cognitively Was fed through PEG and ate some mashed food

Performed a spirometry Received breathing voice training Learnt to do exercises to reduce spasms Was put on Baclofene medication Had training of head control, chin control, trunk and all 4 extremities Had balance training There was adaptation of the electrical wheelchair Needed adaptation of keyboard and mouse for PC She was training to eat Performed training in a discharge apartment at the hospital to gain confidence to manage herself

Had a clearer, stronger voice Did not need letter board Used a PC with keyboard and mouse Could control the internal environment by her computer Manages to feed by eating (important for psychological reasons)

Table 1 Continued

Conclusion

The person who is locked in requires a skilled team in which dierent professionals together can assess the person and help nd alternative ways of communication. There is a need for follow-up to

Cases

Rehabilitation of the locked in re-assess skills and needs partly because of new technologies but also to see whether the person needs more assistance in adapting to the alternative way of communicating or more information about communication for carers of the person. An international network of sites involved in the rehabilitation of LIS patients should be of help for improving professional skills and thereby the quality of rehabilitation for these patients.
Acknowledgments
We acknowledge the support of the stroke rehab ward at Sunnaas Rehabilitation Hospital, which allowed AS with leave of absence for part of this work.

References
1. Smith E, Delargy M. Locked-in syndrome. BMJ 2005;330:4069. 2. New PW, Thomas SJ. Cognitive impairments in the locked-in syndrome: a case report. Arch Phys Med Rehabil 2005;86:33843. 3. Rousseaux M, Castelnot E, Rigaux P, Kozlowski O, Danze F. Evidence of persisting cognitive impairment in a case series of patients with locked-in syndrome. J Neurol Neurosurg Psychiatry 2009;80:16670. 4. Leon-Carrion J, van Eeckhout P, Dominguez-Morales Mdel R. The locked-in syndrome: a syndrome looking for a therapy. Brain Inj 2002;16:55569. 5. Patterson JR, Grabois M. Locked-in syndrome: a review of 139 cases. Stroke 1986;17:75864. 6. Sacco S, Sara M, Pistoia F, Conson M, Albertini G, Carolei A. Management of pathologic laughter and crying in patients with locked-in syndrome: a report of 4 cases. Arch Phys Med Rehabil 2008;89:7758. 7. Smith J, Forster A, House A, Knapp P, Wright J, Young J. Information provision for stroke patients and their caregivers. Cochrane Database Syst Rev 2008; Issue 2. DOI: 10.1002/14651858.CD001919.pub2. CD001919. 8. Schouten LM, Hulscher ME, Akkermans R, van Everdingen JJ, Grol RP, Huijsman R. Factors that inuence the stroke care teams eectiveness in reducing the length of hospital stay. Stroke 2008;39:251521.

Conflict of interest
The authors declare no conict of interests.

Sources of funding
The Sunnaas hospital allowed AS with leave of absence for part of this work. There was no other funding.