Life can be worth living in locked-in syndrome

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.

From armchair to wheelchair: How patients with a locked-in syndrome integrate bodily changes in experienced identity

“…From an exterior point of view, it seems difficult to endure such a handicap without failing in experiencing a continuous self. When asked if they themselves would accept to be kept alive in such a situation, medical teams often answer that they would not, predicting a negative experience for the patients Lule et al, 2009). Most of the patients, on the contrary, report a positive wellbeing Bruno et al, 2011).…”

Impaired Conscious Recognition of Negative Facial Expressions in Patients with Locked-in Syndrome

“…LIS patients show quadriplegia, mutism, and bilateral facial palsy with preservation of vertical gaze and upper eyelid movements; consciousness and cognitive abilities are spared (Schnakers et al, 2008). LIS patients are able to interact with the environment solely through eye-coded communication; despite this extreme degree of motor disability, LIS patients appear emotionally adapted to their severe physical conditions (Cappa et al, 1985;Lulé et al, 2009). The causal ventral pontine lesion in LIS patients usually spares neural structures involved in producing spontaneous facial expressions (Hopf et al, 1992;Töpper et al, 1995).…”

A Fate Worse Than Death? The Well-Being of Patients Diagnosed as Vegetative With Covert Awareness

“…A recent study by Bruno and colleagues surveyed a group of 65 patients with locked-in syndrome, 72% of whom indicated that they were happy, with a greater period in the locked-in state positively correlated with patient happiness (Bruno et al 2011). Other studies have found that patient-evaluated quality of life was not significantly different from healthy controls (with the exception being on scales of physical function) (Lule et al 2009;Rousseau et al 2013). The fact that these patients report that they are happy in their state suggests that it may not be in their best interests to withdraw them from life sustaining treatment.…”