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

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Ó 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. Ó 2011 John Wiley & Sons A ⁄ S. Objectives – A stroke that affects 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 benefits 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 (1–6 years later): computer assisted communication was used by two persons, a letter board by one and Ôordinary communicationÕ 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 different 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, Gçteborg, Sweden

Key words: stroke; rehabilitation; quadriplegia K. S. Sunnerhagen, Rehabilitation Medicine, Per Dubbsgatan 14, 3rd floor, Sahlgrenska University Hospital, S-413 45 Gçteborg, 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 finger or toe movement. We have not been able to find 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 influences 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

the prevalence of LIS should be about one to two per million. there is a need to establish the preferences and personal goals of the person with LIS in 2 . Sunnaas Rehabilitation Hospital has an LIS team in which different skills are represented. The reception of the patient at the ward is important. Data were gathered from patient charts. Sunnaas Rehabilitation Hospital has country wide responsibility for Norway (4. The team consists of a nurse and nursesÕ aid. Comprehensive team work is needed to assess the personÕs situation from different perspectives. have tailor-made communication devices. an occupational therapist. To illustrate the rehabilitation process. Assessment – The basis for care of the person with LIS is threefold. 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. Concretely. During the period 2000–2007. as well as the capacity of next of kin to assess rehabilitation and participation in the long run. The person is then asked to look at a letter on a letter board or a picture containing a figure. communication skills and cognition. There is a need to assess the personÕs feeling of security on the ward. we present four cases of LIS at hospital admission. which can be used for training before establishing communication. about 15 persons with LIS were treated at Sunnaas Hospital. we discuss persons with LIS caused by a stroke in the brainstem ⁄ pons. It is important to have knowledge of prior problems in reading and writing as well as of computer experience. At admission Above all. the occupational therapist and the communication team try to assess how the patient copes with instructions and whether there is a delay in the patientÕs responses. Prior reports on persons with LIS have focused on the impairments that follow the lesion and ⁄ or experiences of living with LIS (5). the nurse. The feeling of being unable to communicate and knowing that breathing and swallowing are difficult are stressful. The aim of this article is to discuss the need and benefits of a comprehensive rehabilitation and a standardized approach to the person with LIS. needs and goals. Material and methods setting goals for the rehabilitation process. The triceps skin fold is sometimes assessed as a measure of subcutaneous fat. The prestroke weight is documented and followed closely for an assessment of nutritional status. a swallowing specialist (speech and language therapist). all personnel on the ward need to have some knowledge of LIS and the person to help when necessary. together with occasional arterial oxygen sampling. Many persons with LIS express the need to establish communication and. It is difficult to achieve a feeling of safety in persons in need of total care when there are frequent staff changes. However. There is also a focus on the functions and resources these persons might have. Basic assessment of cognition is always a part of admission procedures where the physician and the In this article. the second to give nutrition and the third to establish some means of communication. which are necessary components of good quality of life. another person observes whether the patient can start and stop an action or motion with the eyes or the head. The basic goal of rehabilitation is for the person to feel safe and confident in the environment and with the team. However. These four LIS cases were selected because they showed diversity in capacity. nutritional status. if possible. and his or her endurance.Schjolberg & Sunnerhagen LIS in France participated. the team has only one person per skill area. which emphasizes the need of dedicated personnel. The results of this basic screen are used by the neurophysiologist to tailor the test methods for cognition. The first need is to establish sufficient breathing. Inner security is often coupled with self-assurance and mastering. During this time. this implies showing a two-coloured card and asking the person to look at one of the colours. Assessments must be made of O2 saturation. Eye blink and other eye movements are examined to assess communication proficiency. The physiotherapist. knowledge of psychological reactions and access to information and communication technology. Pulse oximeters are employed to assess O2 saturation. The length of stay for in-patient rehabilitation among persons with LIS is currently about 3 months. This requires different professional skills. We have not found information on how to treat ⁄ train these patients. a physiotherapist. a physician and the hospitalÕs communication team (a speech and language therapist and an engineer). 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. motivation and interests.5 million inhabitants) for this patient group.

The schedules of the staff must be changed according to the personÕs needs. Instructions are given to the patient to move the eyes before moving sides. A next step can be that two persons are together with the LIS patient on a tilt table to train head and trunk control. Feeding requires an elevated head end of the bed or sitting. 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. Caring procedure: There is a high risk of infections (pneumonia. Communication: For basic communication. This type of contact can be used actively in treatment. which will not only be of use for his ⁄ her tasks but also for the rest of the team. The testing takes place in a secluded environment in which the person is relaxed. urinary tract infections or pressure sores). Continued assessments of cognition are a joint responsibility of the team. For respiratory function. Positioning is important for breathing. needs stillness both physically and mentally. This is necessary to ensure the patient that he ⁄ she is seen as cognitively adequate and that we as staff are ready to accept everyday communication in different ways. a person with LIS can often communicate by winking the eyes. Knowledge and understanding of the background to the outburst. does the patient respond to a smile. Feeling the floor during training gives a feeling of security. and one person is in front to guide and to stimulate movements in the trunk. Formal neuropsychological testing aims to assess capacity to take instructions.Rehabilitation of the locked in nurse form a first impression. Using the treadmill with body weight support needs to be tailored to the personÕs stamina. and the personnel must take time with the patient. Among other things. One person sits behind the patient to give support. where one wink is yes and two is no. Being put in bed with extra pillows and cushions will make the person secure there. or can look up for yes and down for no. mimicking and postures. to train the motor deficits and to motivate the person mentally to apply the resources available. There is a need to reduce stress and anxiety. The nurse can gather much information on cognition while caring for the patient. finger movement and body stability to be able to use equipment for communication. Oral hygiene is important because infections in the mouth can affect respiration and feeding. The person needs to be looked after. the neck and all four extremities. does he ⁄ she understand a joke? In everyday conversation. Letter boards and eye pointing boards are a simple and 3 . Especially initially. We have to be open minded to different modes because communication takes place not only in conversations but also in behaviour. It is important to try to understand the person with LIS. many words ⁄ sayings also have a double meaning. stamina increases with an increase in time sitting up in a chair or longer periods of training. to prepare the body for turning in bed. Pathological laughter and ⁄ or crying sometimes occur (6) and information about this and the probable linkage to the damage in pons is also necessary. Motor function: It is important to move all the joints. is a key component of treatment (6). the stability of the treatment group is very important. Intervention – After the different functions and skills are identified. there is a need to measure the oxygen saturation in the blood (supplementary oxygen delivered when needed). The use of a standing bed. which is important (this can be done by using shoes and having the feet on the floor while sitting in a wheelchair). which gives a feeling of safety. Surveillance cameras can sometimes be used (with the permission of the patient) to give security. Establishing safe and sufficient nutrition reduces the risk of infection and is necessary for physical activity. Feeding (no gastric feeding is totally safe for the LIS patient) is usually performed through a percutaneous gastric tube. and later a body supported treadmill. tendons and muscle groups to prevent spasms and contractures. but only after a trustworthy relationship has been established. Typically. There is also a need for assessment of head and trunk control. the use of a peak expiratory pressure mask must be considered. psychological level and memory. but also needs tailored information. gives the person support from the floor and increases the tolerance for variations in blood pressure. the team work towards a common goal: to compensate for the shortcomings in the environment. Each person in the team observes different things according to the professional background. 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. as well as a cough machine. The caring procedure should aim to give a feeling of security. which is not always emotional. oral cavity and pharynx must be sucked often to reduce the risk of infection and increase the chance of optimal respiratory function. This also helps the person to allow him ⁄ herself to be touched and to become aware of this ÔnewÕ body. Phlegm in the trachea.

He had 4 months of primary rehabilitation and two additional sessions. who had a brain stem infarct resulting in tetra paresis and LIS. and thus a great deal of time was spent on trying to reduce anxiety. special training had to be given for control of the chin. This is satisfactory to many patients because they can then tell more. which reduced the problem. The eye pointing board is constructed of a system where the letters have different colours and are split into groups with a colour code in the middle. The letter is confirmed with a wink of the eye or next of kin can act as an intermediary with the person with LIS present. because spasticity interfered with her oral communication and eating. 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 benefits of a comprehensive rehabilitation and a standardized way of approaching the person with LIS. where the emphasis was on alterations ⁄ development of different communication devices (Table 1). His main problem was anxiety. neck and trunk control. he gained confidence. but it is probably low. 40 years old. He had a weak voice at the beginning. she had a weak voice. The incidence and prevalence of LIS is not known. Her problem during the whole period was spasticity. He had problems in breathing and swallowing and suffered pneumonia more than once. had started to take some nourishment orally and had some finger movements. as she controlled her PC and her home environment. felt more secure and could live at home with the support of his family and a personal assistant. 70 years old. The training to use this equipment is given by the communication team and the other personnel in the LIS team. The first rehabilitation period lasted for 2 months. who had a brain stem infarct resulting in tetra paresis and LIS. He had a supportive but worried family. She had 3 months of primary rehabilitation and three additional sessions. and it is easier and faster than using the traditional letter board. and this eventually led to him being able to utter a few words. the staff can do the training. At admission. Over time. The first step is to give a possibility to call for help. The last case was a woman of 40. with a brain stem infarct resulting in tetra paresis and LIS. Different physical treatments were used. at follow-up. in the same way. Adapting more advanced equipment for communication is a long process. which is of the utmost importance. she was fed orally twice a day with mashed food and had a better sitting position. the time in the different hospitals and getting back into life.Schjolberg & Sunnerhagen fast means of communication for persons with LIS. She spent 3 months in primary rehabilitation. The person has to move the sight twice per letter. neck and trunk because walking on this enhances the postural reflexes. The third case was a man of 60 years. She managed early to use electronic equipment and switches and wanted to manage herself. She applied for an adapted car with different switches and now drives using finger and head switches. She claims that the only inconvenience in having suffered LIS is that you are paralysed and that everything takes too long – however. she had an electrical wheelchair that she controlled with head and finger 4 switches. the same electrodes or switches can be used for the computer. and the next of kin are also instructed. Eye pointing boards are easy to use and to carry. which interfered with her training and her rest. and his main goal was to establish communication. with a brain stem infarct resulting in LIS. we often suggest that we are allowed to guess what word is coming when we believe we know. The next of kin are suggested to be taught by LIS persons. Later. The focus was on feeding and on head. this gives a feeling of having mastered the skill. In this case. which limited the rehabilitation. The board needs to be tailored according to vision. communicated with a letter board. If needed. visual field and other things. She was very fit prior to her stroke and cognitively fine and determined to train. To make communication faster. She could communicate vocally to some extent and used her computer when she got tired. and she was put on Baclofen. The second case was a woman. including the use of a surveillance camera. There is thus a risk that the person with LIS will not be correctly assessed and trained. Later. The different electrodes and switches are first applied in the motor functions that are identified and there is an evaluation of the movements (large or strong enough to activate the equipment). He then returned three more times. Four cases The first case was a man. . At discharge. She trained on a body weight supported treadmill to increase control of the head. She currently works 50% and has released a book on her experience of stroke. manoeuvring the wheelchair or controlling the indoor environment. and video cameras for surveillance of the traffic situation.

The discharge planning had worked and the family managed Rehabilitation of the locked in 5 . 40 years Brain stem infarcts Face and oral motor training Breathing training Training of head. SMS function and could control the indoor environment with this Man 60 years. 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. 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. cheek and fingers Used taped books. 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. 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.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.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. eye pointing board. 70 years Brain stem infarcts Vertical eye movements Some head motion PEG was used Letter chart was used Had an active family Woman.

Good communication in the team (8). which transfers the need for special competence to the next link. 3) is extremely important because this will influence the possibility for the person to use communication devices. because one of the major goals is to establish stable communication. In recent years. is important for the outcome. LIS.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. a few 20 years. Some of them have lived for 5–10 years with LIS. There is a need for follow-up to 6 Cases . We have steady contact with some of the persons with LIS. During the week of the follow-up. 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 Ôdischarge apartmentÕ at the hospital to gain confidence to manage herself Had a clearer. One common challenge is the complicated IT system that is sometimes constructed. the hospital has offered followup periods for persons with LIS. chin control. A limitation in this study is the low number of persons for whom we have complete data. difficulties were experienced in the next link in the chain. 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. 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. 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 different professionals together can assess the person and help find alternative ways of communication. In one of the cases in this study. considering the limitations. 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. However. This is as important and rewarding for the persons with LIS as for the team. Most of them state that they live a good life. next of kin and the next link in the chain. The assessment of cognitive function (2. 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 fill expected roles in daily life in a different but still a good way. all four had success in communication. The result of a provision of a dedicated skill team for assessment and subsequent interventions is better communication skills. which agrees with the experience of the LIS team in the past 10 years. Locked-in syndrome. which forces us to present only four persons. as well as between the person. We as a team get information about the experiences of those with LIS.

8.39:2515–21. House A. Grol RP.1002/14651858. 4. Schouten LM. Castelnot E. Stroke 1986. Cochrane Database Syst Rev 2008. Acknowledgments We acknowledge the support of the stroke rehab ward at Sunnaas Rehabilitation Hospital. Rigaux P. Huijsman R. Young J. Stroke 2008. Sources of funding The Sunnaas hospital allowed AS with leave of absence for part of this work. Arch Phys Med Rehabil 2008. 6. Factors that influence the stroke care teamÕs effectiveness in reducing the length of hospital stay. References 1. Akkermans R.86:338–43. J Neurol Neurosurg Psychiatry 2009. Dominguez-Morales Mdel R.80:166–70. Leon-Carrion J.330:406–9. Evidence of persisting cognitive impairment in a case series of patients with locked-in syndrome.CD001919. Issue 2. Albertini G.pub2. van Everdingen JJ. 7 . Brain Inj 2002. Cognitive impairments in the locked-in syndrome: a case report. Smith E. Wright J. Hulscher ME. Delargy M. Management of pathologic laughter and crying in patients with locked-in syndrome: a report of 4 cases. Rousseaux M. Sacco S. Sara M. Carolei A. Conson M. Locked-in syndrome: a review of 139 cases. van Eeckhout P. Conflict of interest The authors declare no conflict of interests. 7. Kozlowski O. BMJ 2005. Knapp P. Patterson JR.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. DOI: 10. Danze F. Forster A. 2. Smith J. Pistoia F. 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.89:775–8. Grabois M. The locked-in syndrome: a syndrome looking for a therapy.17:758–64. CD001919. Information provision for stroke patients and their caregivers. Thomas SJ. 3. Locked-in syndrome. New PW. There was no other funding. Arch Phys Med Rehabil 2005.16:555–69. which allowed AS with leave of absence for part of this work. 5.

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