Locked-in Syndrome
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Locked-in syndrome (LIS) is a condition were people “lose all output from the brain to both muscles and the autonomic nervous system” (Kalat & Shiota, 2007, p. 22). LIS patients are said to be "locked in" because they become paralyzed from every muscle in their body while still being alert (Kubler, Kotchoubey, Kaiser, Wolpaw, & Birbaumer, 2001). In 1966, Plum and Posner introduced the term “locked in syndrome” to describe a patient that was conscious but had no “voluntary motor activity other than vertical eye movements” (Shivji, Streletz, Baeesa, & Girvin, 2003). There are two types of LIS; "classic" locked-in syndrome, were vertical eye movement and eye blinks remain undamaged and "total" locked-in syndrome were patients lose all ability of movement (Kubler et al., 2001). LIS can also be known as; Ventral Pontine Syndrome, State of Supra-nuclear Motor De-efferentation, and False Coma by Cerebro-medullary Spinal Disconnection (Leon-Carrion, Eackhout, Del Rosario, Dominguez-Morales, & Perez, 2002).
What generally causes LIS is damage to the ventral pons and medulla (Kalat & Shiota, 2007, p. 22; Doble, Haig, Anderson, & Katz, 2003). Why LIS is caused is more difficult to define. Tumors, encephalitis, infections or brain injuries localized in the ventral midbrain can cause LIS, but researchers have not yet figured out why only some people that suffer from damage to the ventral midbrain get LIS, while others do not (Kubler et al., 2001). In a study done by Leon-Carrion et al. (2002), found that in their study 86.4% of LIS patients obtained LIS from a stroke, and 13.6% of LIS patients in their study were from the result of a traumatic brain injury.
Even though people with LIS lose the ability to move their body and to speak, many still remain conscious, and after being diagnosed with LIS, they continue to suffer from various other problems. For instance, by asking the LIS patient’s most intimate family members, research (Leon-Carrion et al., 2002) has shown that when it comes to their emotional state, some (12.5%) were found to be depressed, but being in a good mood was found in the majority (47.5%) of the patients. A large amount of LIS patients (85%) were however found to be more emotionally sensitive since the onset of LIS. Research (Leon-Carrion et al., 2002) also shows that patients with LIS suffer from various cognitive problems. In the Leon-Carrion et al. (2002) study, they demonstrated that 95.3% of LIS patients claimed they could pay attention for more than 15 minutes and 76.7% could read. Memory problems were described by 18.6% of LIS patients and 14% showed visual deficits. When it came to sleeping patterns, 9.3% reported a tendency to remain asleep, and only 2.3% slept for the majority of the time.
Although one of the primary cognitive problems that LIS patients suffer from is a verbal speech deficit, 78% of the patients can produce sounds and 65.8% could communicate using a code system with vertical eye movements and blinking (Leon-Carrion et al., 2002). Technical aids were found as an uncommon method to help LIS patients communicate (Leon-Carrion et al., 2002). Kubler et al. (2001) described several brain-computer interfaces to help LIS patients communicate, but none were found to fit LIS patients perfectly. For example, some brain-computer interfaces show high accuracy, but have never been used on LIS patients; the P300 event related potential and the µ-rhythm brain-computer interface are a couple of technical aids that have this problem. On the other hand, the Thought Translation Device has been used by LIS patients, but has been found to be somewhat inaccurate (Kubler et al., 2001). In addition to technical aid, there are different treatments available for LIS patients such as pharmacological treatment, for instance Lioresal (baclofen) and Sintrom (acenocumarin), physiotherapy treatment, neuropsychological treatment, and combinations of pharmacological and neuropsychological treatments or pharmacological and physiotherapy treatments (Leon-Carrion et al., 2002).
One of the major problems with LIS is that there is very little recent literature concerning the course and prognosis of LIS (Leon-Carrion et al., 2002). In consequence, many aspects of LIS are not definite. However, some researchers have tried to provide some answers. The survival rate for LIS found in Doble et al. (2003) study was 83% for 5 and 10 years and for a 20 year survival rate of 40%. Doble et al. (2003) also found that out of their 15 deceased patients, the causes of deaths included heart disease (4), multisystem failure (2), 1 pneumonia, 1 subsequent cerebrovascular infarct, 1 respiratory failure, 1 internal bleeding, and 1 aspiration/asphyxiation. According to Leon-Carrion et al. (2002), LIS has shown to occur in about the same frequency in men (51.2%) and women (48.8%). They (Leon-Carrion et al., 2002) also found that 20.5% of patients in their study had LIS for less than 2 years, 63.6% between 2-5 years and 15.9% between 6- 10 years. Two patients had had LIS for over 20 years
Another problem with LIS identified by Leon-Carrion et al. (2002) is that people are left unable to speak. Due to this, patients with LIS tend to be misdiagnosed and are generally confused with having akinetic mutism or with a vegetative state. Sometimes, it takes doctors days or months before they correctly diagnose the LIS patient. For example, in their study, the mean time elapsed from onset until the diagnosis of the syndrome was made was 78.76 days.
As we can see LIS is an extreme condition with many aspects of it still unanswered. LIS is pretty well defined but there is still room for research for LIS such as; ways to help LIS patients communicate, cause of death for LIS patients after survival, the prognosis of LIS, who is more likely to get LIS, and ways to improve survival rate.
Example-Research
Doble et al., (2003) investigated the long term outcome of patients with locked-in syndrome (LIS). They hypothesized that LIS is not directly related to the quality of life. In other words, people with LIS may be able to live a long and satisfied life. The method for this study included phone interviews asking specific questions about the patients’ condition (i.e. date of onset, cause, hospitalizations per year, ability to verbally communicate, limb movement, moods, emotional states, euthanasia and resuscitation wishes, feeding methods, bowel/bladder management, how independent they are, and method of mobility) as well as questions about their present environments (i.e. where they live, vocational activities, how often and how long they are left alone during the night/day, and how much family is involved). Most phone interviews were the opinions of the primary caregivers of people with LIS, except for one person, who was an LIS patient but had the ability to communicate by phone. The operationalization of Doble et al., (2003) study was to follow up on LIS patients form an earlier study which was conducted 11 years before the present study. The results that were found for Doble et al., (2003) study were consistent to their hypothesis. The study concluded that LIS patients may live for many decades and the majority is satisfied with their life. Most do not wish to be euthanized and they come to develop meaningful lives involving vocational activities and significant involvement with family and community. Some of the implications for this research are that survival rate and life satisfaction for LIS patients could dictate family and medical decisions. Accurately knowing an LIS patients survival rate and long term outcomes can help doctors, family, or even insurance companies determine how much time and effort should be spent on a LIS patient’s care.
Example-Real Life
A real life example of locked in syndrome is displayed in a clip from a popular TV series on NBC called “Scrubs”. This clip shows two characters from the TV show, one nurse and one doctor, walking into the patient’s room to check on the patient. As they are walking towards the patient’s room, the nurse announces that the patient has locked-in syndrome (LIS) and describes the condition as “a paralysis so severe that he could only communicate through a computer that responds to his eye movements”. When they are walking into the patient’s room the doctor asks the nurse to check the patients’ vitals, but calls the nurse a girl’s name when the nurse is a male. The LIS patients is shown to be connected to a computer from his head and simultaneously, while he is blinking, the computer types what he wants to say and a monotone voice produced by the computer verbally says what he wishes to say. In this clip the LIS patient asks the nurse, “Why does he call you a girl’s name?” The nurse answers his question and the patient follows by responding “Oh my God. I get it now. CarolCarolCarolCarolCarolCarolCarol etc.” The nurse gets angry because he believes the patient was purposely teasing by calling him a girl’s name repeatedly. The doctor then points out that the computer must be broken.
Although this clip was meant for amusement purposes, it illustrates many aspects of people with locked-in syndrome. In the clip, the LIS actor is shown to be conscious, unable to speak, while laying perfectly still, and only moving his eyes to blink, which is consistent with research describing how LIS patients’ look. Research on LIS also shows that computer interfaces, such as the Thought Translation Device, are used by LIS patients to communicate, as is displayed in the “Scrubs” clip. However, the computer from the “Scrubs” clip appears to be broken, which is also consistent to LIS research; most technical aids for LIS patients are not a hundred percent accurate.
References
Doble, J.E., Haig, A.J., Anderson, C., & Katz, R. (2003). Impairment, activity, participation, life satisfaction, and survival in persons with locked-in syndrome for over a decade. Journal of Head Trauma/Rehabilitation, 18(5), 435-444.
Ghettobrownie88. (2007). Locked in syndrome. Retrieved February 12, 2010, from http://www.youtube.com/watch?v=MadRK3PElpI&feature=related
Katal,J.W., & Shiota, M.N. (2007). Emotion. Thomson Wadsworth.
Kubler, A., Kotchoubey, B., Kaiser, J., Wolpaw, J.R., & Birbaumer, N. (2001). Brain-computer communication: Unlocking the locked-in. Journal of Psychological Bulletin. 127(3), 358-375.
Leon-Carrion, J., Eeckhout, P.V., Del Rosario, M., Dominguez-Morales, R., & Perez-Santamaria, J.F. (2002). The locked-in syndrome: A syndrome looking for a therapy. Journal of Brain Injury. 16(7), 571-582.
Shivji, Z. M., Streletz, L. J., Baeesa, S., & Girvin, J. (2003). Electrophysiological investigations in the locked-in syndrome: A case report. Journal of Electroneurodiagnostic Technology.43(2), 60-69.
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