Life can be worth living in locked-in syndrome

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Abstract

The locked-in syndrome (LIS) describes patients who are awake and conscious but severely deefferented leaving the patient in a state of almost complete immobility and loss of verbal communication. The etiology ranges from acute (e.g., brainstem stroke, which is the most frequent cause of LIS) to chronic causes (e.g., amyotrophic lateral sclerosis; ALS). In this article we review and present new data on the psychosocial adjustment to LIS. We refer to quality of life (QoL) and the degree of depressive symptoms as a measure of psychosocial adjustment. Various studies suggest that despite their extreme motor impairment, a significant number of LIS patients maintain a good QoL that seems unrelated to their state of physical functioning. Likewise, depression is not predicted by the physical state of the patients. A successful psychological adjustment to the disease was shown to be related to problem-oriented coping strategies, like seeking for information, and emotional coping strategies like denial — the latter may, nevertheless, vary with disease stage. Perceived social support seems to be the strongest predictor of psychosocial adjustment. QoL in LIS patients is often in the same range as in age-matched healthy individuals. Interestingly, there is evidence that significant others, like primary caregivers or spouses, rate LIS patients' QoL significantly lower than the patients themselves. With regard to depressed mood, ALS patients without symptoms focus significantly more often on internal factors that can be retained in the course of the disease contrary to patients with depressive symptoms who preferably name external factors as very important, such as health, which will degrade in the course of the disease. Typically, ALS patients with a higher degree of depressive symptoms experience significantly less “very pleasant” situations. The herein presented data strongly question the assumption among doctors, health-care workers, lay persons, and politicians that severe motor disability necessarily is intolerable and leads to end-of-life decisions or euthanasia. Existing evidence supports that biased clinicians provide less-aggressive medical treatment in LIS patients. Thus, psychological treatment for depression, effective strategies for coping with the disease, and support concerning the maintenance of the social network are needed to cope with the disease. Novel communication devices and assistive technology now offers an increasing number of LIS patients to resume a meaningful life and an active role in society.

Introduction

The term locked-in syndrome (LIS) was first introduced by Plum and Posner in 1983 and describes patients who are awake and conscious but selectively deefferented, that is, have no means of producing speech, limb or facial movements. The American Congress of Rehabilitation Medicine (1995) defined LIS as a neurological impairment characterized by the presence of sustained eye opening (bilateral ptosis should be ruled out as a complicating factor), quadriplegia or quadriparesis, aphonia or severe hypophonia, preserved cognitive function, and a primary and elementary code of communication that use eye movement or blinking. The LIS can be subcategorized according to the severity of the motor impairment in: (1) classical LIS, characterized by quadriplegia and aphonia with preserved consciousness and vertical eye movements or blinking; (2) incomplete LIS, characterized by remnants of voluntary motion other than vertical eye movements; and (3) total LIS, characterized by complete immobility (including eye movements) with preserved consciousness (Bauer et al., 1979). Acute pontine lesions following vascular pathology are its most common cause (Plum and Posner, 1983; Laureys et al., 2005). LIS can also be observed in progressive neurologic pathologies like end-stage amyotrophic lateral sclerosis (ALS) (Ludolph and Dengler, 1999). It has been traditionally stated that long-term survival in LIS is rare (Ohry, 1990). Mortality is indeed high in acute LIS of vascular origin (87% within the first 4 months; Patterson and Grabois, 1986) and mean survival rate in ALS is 3–5 years with only a short period of time in LIS (Ludolph and Dengler, 1999). However, individuals in LIS may survive for long periods of time, sometimes exceeding 20 years (Doble et al., 2003; León-Carrión et al., 2002; Laureys et al., 2005). Doble et al. (2003) reported a 10-year survival of 83% and 20-year survival of 40% in 29 stabilized (i.e., surviving more than 1 year) LIS patients. Data from the French Association for Locked-In Syndrome (ALIS; www.alis-asso.fr) on 250 patients showed that mean time spent in LIS was 6±4 years (range 14 days–27 years; Laureys et al., 2005). With improving medical technology, life with severe physical impairment can be significantly prolonged — for example, by application of noninvasive and invasive ventilation in ALS. Noninvasive ventilation prolongs life for 250 up to 300 days and has therefore the same life-prolonging effect as Riluzole, the only approved drug in ALS (Bourke et al., 2006). However, motor recovery is futile in a progressive motor neuron disease like ALS and the hope for motor recovery is limited in LIS of vascular origin (Doble et al., 2003). Despite the severe persisting motor deficits in classic LIS, some patients may present improvement (classically showing a distal to proximal progression) and recover voluntary control of head, finger, or foot (Richard et al., 1995; Laureys et al., 2005). Overall, the level of care remains extensive in chronic LIS and patients classically remain dependent on others for activities of daily living.

Autonomy and physical functioning has long been seen as the prerequisite of a life worth living (Chin et al., 1999). In recent years this was adjusted and life was defined to be worth living if there was a perspective of gaining autonomy (e.g., Bruno, Bernheim, Schnakers, & Laureys (2008a), Bruno et al. (2008b)). Studies show that QoL often equates with social rather than physical interaction or autonomy (Laureys et al., 2005). The arising question from our definition of “a life worth living” is what the consequences are for people with such extreme motor impairment, in which the probability for regaining autonomy in daily life is very limited. We may have to revise our classical idea of autonomy emphasizing that mental autonomy can be maintained even in a state of high dependence on others, in which much if not all physical autonomy is lost. The present work will review the available literature and present new data on psychological adjustment of patients with severe states of motor impairment leading a life depending on others — including both LIS following an acute brainstem lesion and following chronic motor neuron diseases such as ALS.

Section snippets

Quality of life

Successful adjustment to a diagnosis can be measured as a degree of quality of life (QoL). According to the World Health Organization (WHO) “quality of life is defined as the individual's perception of their position in life. (…) It is a broad ranging concept affected in a complex way by a person's physical health, psychological state, level of independence and their relationship to salient features of their environment” (The WHOQOL Group, 1995). In the field of neurology and neuro-critical

Psychological adaptation in LIS

Psychological adaptation to extreme motor impairment is mediated by coping strategies, coping resources, and social support (Matuz et al., submitted) and thus is the prerequisite for a high QoL in LIS. Factors which help to cope with the disease are various and may change in the course of the disease. Avoidance showed to be a successful strategy at the beginning of the disease, but became nonadaptive as the disease progressed (Matuz et al., submitted). In accordance with our previous studies (

Depression rate

Depression is a well-described psychological disorder permitting effective psychotherapeutic and pharmacologic treatment (e.g., De Jong-Meyer et al., 2007). Similarly to QoL, the presence of depression can be regarded as a measure of adjustment to the circumstances of the disease. Comparable to the findings for QoL previously discussed, there is evidence that the incidence of depressive symptoms in patients with severe physical impairment is not associated with physical function. This was true

Social participation

One of the strongest factors which help to cope with severe motor impairment as seen in LIS and ALS is social support. Matuz and colleagues (submitted) demonstrated that perceived social support is the most powerful predictor for a good QoL and low depression rate. The same was shown by Häcker (unpublished) in a study encompassing 33 ALS patients (mean age, 58±9.0; range, 37–72 years; 18 females; ALS-FRS mean, 20.0±11.0; range, 0–38; diagnosed since 40±31; range, 1–126 months): patients

Alternative communication devices

Another decisive factor for successful adjustment strategies in LIS patients is communication — classically and most basically via an eye movement code (León-Carrión et al., 2002; Laureys et al., 2005). Those functions are usually retained in LIS patients with pontine lesions (León-Carrión et al., 2002) and are usually preserved in ALS patients (Ludolph and Dengler, 1999). However, this way of communication always requires the help of a second person who needs to be willing and capable to

Wish to die

The discussed studies on successful adaptation in severely disabled patients like LIS and ALS have strong implications for the management, end-of-life decisions, and euthanasia in these challenging patients. Reports from LIS patients contradict the widespread opinion that patients with severe physical impairment inevitably suffer from poor QoL, depression, despair, and hopelessness which, consequently lead to the wish to die. In fact, the wish to die and the request for euthanasia is low,

Conclusion

Superficially involved for the short-term of clinical surveillance clinicians may tend to assume that LIS patients will die anyway or would choose to die if they only knew what the clinicians knew (Laureys et al., 2005). Ganzini and Block (2002) suspect that this is due to a psychological defense mechanism: healthy people have difficulty imagining the feelings and experiences of a severely impaired patient and may assume that the patient's QoL is poor (McDonald et al., 1996). As a result,

Acknowledgments

This work was supported by funds by the European Commission (DISCOS, Mindbridge, and COST), McDonnell Foundation, Mind Science Foundation, and the Belgian Fonds de la Recherche Scientifique (FRS). S. Laureys and M. A. Bruno are, respectively, Senior Research Associate and Research Fellow at FRS.

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