Life can be worth living in locked-in syndrome
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.
References (78)
- et al.
The disability paradox: High quality of life against all odds
Social Science and Medicine
(1999) - et al.
Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: A randomised controlled trial
The Lancet Neurology
(2006) - et al.
Blink and you live: The locked-in syndrome
Review Neurology (Paris)
(2008) - et al.
The ALSFRS-R: A revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III)
Journal of the Neurological Sciences
(1999) - et al.
Personal values and individual quality of life in palliative care patients
Journal of Pain and Symptom Management
(2005) - et al.
Depression associated with multiple sclerosis. Looking beyond diagnosis to symptom expression
Journal of Affective Disorders
(2001) - et al.
A brain-actuated wheelchair: Asynchronous and non-invasive brain-computer interfaces for continuous control of robots
Clinical Neurophysiology
(2008) - et al.
Follow-up study of individuals with high tetraplegia (C1–C4) 14 to 24 years post injury
Archives of Physical Medicine and Rehabilitation
(1999) - et al.
Validity of the ALS-depression-inventory (ADI-12)—A new screening instrument for depressive disorders in patients with amyotrophic lateral sclerosis
Journal of Affective Disorders
(2008) - et al.
The French SF-36 Health Survey: Translation, cultural adaptation and preliminary psychometric evaluation
Journal of Clinical Epidemiology
(1998)
Patients' assessment of quality of life instruments: A randomized study of SIP, SF-36 and SEIQoL-DW in patients with amyotrophic lateral sclerosis
Journal of Neurological Sciences
Conscious perception of brain states: Mental strategies for brain–computer communication
Neuropsychologia
A P300-based brain–computer interface for people with amyotrophic lateral sclerosis
Clinical Neurophysiology
Thought'-control of functional electric stimulation to restore hand grasp in a patient with tetraplegia
Neuroscience Letters
The role of health care providers and significant others in evaluating the quality of life of patients with chronic disease: A review
Journal of Clinical Epidemiology
Integrating response shift into health-related quality of life research: A theoretical model
Social Science and Medicine
A study comparing patients with amyotrophic lateral sclerosis and their caregivers on measures of quality of life, depression, and their attitudes toward treatment options
Journal of the Neurological Sciences
Reintegration to normal living as a proxy to quality of life
Journal of Chronic Diseases
Subjective health assessment
Rehabilitation success: The interrelationships of multiple criteria
Journal of Health and Social Behavior
Recommendations for use of uniform nomenclature pertinent to patients with severe alterations of consciousness
Archives of Physical Medicine and Rehabilitation
Depression, psychological resources, and health-related quality of life
Journal of Clinical Geropsychology
Responding to requests for physician assisted suicide: These are uncharted waters for both of us
Journal of the American Medical and Association
Varieties of the locked-in syndrome
Journal of Neurology
A spelling device for the paralysed
Nature
Discontinuation of mechanical ventilation in patients with amyotrophic lateral sclerosis
Journal of Neurology
Locked-in: Don't judge a book by its cover
Journal of Neurology Neurosurgery and Psychiatry
Legalized physician-assisted suicide in Oregon — The first year's experience
New England Journal of Medicine
Evidenzbasierte Leitlinie zur Psychotherapie Affektiver Störungen (Evidence based guidelines for psychotherapy of affective disorders)
Impairment, activity, participation, life satisfaction, and survival in persons with locked-in syndrome for over a decade: Follow-up on a previously reported cohort
Journal of Head Trauma Rehabilitation
Anxiety, depression and quality of life of cancer patients undergoing radiation therapy: A cross-sectional study in a community hospital outpatient centre
European Journal of Cancer Care
Psychiatric disorders and depression in multiple sclerosis outpatients: Impact of disability and interferon beta therapy
Neurological Sciences
Physician-assisted death a-last resort?
New England Journal of Medicine
Correlates of suffering in amyotrophic lateral sclerosis
Neurology
Longitudinal predictors of psychological distress and self-esteem in people with ALS
Neurology
Cited by (155)
Spoken and Inner Speech-related EEG Connectivity in Different Spatial Direction
2022, Biomedical Signal Processing and ControlCare at the End of Life
2021, Aminoff’s Neurology and General MedicineLocked-in syndrome revisited
2023, Therapeutic Advances in Neurological DisordersFunctional recovery continues beyond 3 months post-basilar artery thrombectomy: A retrospective cohort study
2023, CNS Neuroscience and Therapeutics