The DOI matches its Crossref record; 81 of the 82 references it cites were found in a registry or an index.
Life can be worth living in locked-in syndrome
Open accessAbstract
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.
Overview
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Citations
Cites
SF-36 health survey: Manual and interpretation guide
Ware, J. (2003) · Medical Entomology and Zoology · Cited by 11,424
Kessler, R. C., Berglund, P., Demler, O., Jin, R., Koretz, D., Merikangas, K. R., Rush, A. J., Walters, E. E., & Wang, P. S. (2003) · JAMA · doi:10.1001/jama.289.23.3095 · Cited by 7,988
(1995) · Social Science & Medicine · doi:10.1016/0277-9536(95)00112-k · Cited by 7,643
Cedarbaum, J. M., Stambler, N., Malta, E., Fuller, C., Hilt, D., Thurmond, B., & Nakanishi, A. (1999) · Journal of the Neurological Sciences · doi:10.1016/s0022-510x(99)00210-5 · Cited by 3,307
Integrating response shift into health-related quality of life research: a theoretical model
Sprangers, M. A. G., & Schwartz, C. E. (1999) · Social Science & Medicine · doi:10.1016/s0277-9536(99)00045-3 · Cited by 2,228 · cited 1×
“Such an adaptation to disease is referred to as response shift (Sprangers and Schwartz, 1999).”
BackgroundThe disability paradox: high quality of life against all odds
Albrecht, G. L., & DEVLIEGER, P. J. (1999) · Social Science & Medicine · doi:10.1016/s0277-9536(98)00411-0 · Cited by 2,146
A spelling device for the paralysed
Birbaumer, N., Ghanayim, N., Hinterberger, T., Iversen, I., Kotchoubey, B., Kübler, A., Perelmouter, J., Taub, E., & Flor, H. (1999) · Nature · doi:10.1038/18581 · Cited by 1,577
Self-citationBourke, S. C., Tomlinson, M., Williams, T. L., Bullock, R., Shaw, P. J., & Gibson, G. J. (2006) · The Lancet Neurology · doi:10.1016/s1474-4422(05)70326-4 · Cited by 1,170
The Diagnosis of Stupor and Coma.
Plum, F., & Posner, J. B. (1967) · Annals of Internal Medicine · doi:10.7326/0003-4819-66-1-244 · Cited by 1,156
Sprangers, M. A. G., & Aaronson, N. K. (1992) · Journal of Clinical Epidemiology · doi:10.1016/0895-4356(92)90052-o · Cited by 1,076
Research works printed but not linked: 3
- Häcker, S. (unpublished). Depressivität und subjektive Lebensqualität bei schwerster körperlicher Beeinträchtigung — Eine empirsche Arbeit über Patienten mit amyotropher Lateralsklerose (Depression and subjective quality of life given severest physical impairment — An empirical study on patients with amotrophic lateralsclerosis). Unpublished diploma thesis in Psychology. University of Tübingen, Germany.
- Lulé, 2008, Ergebnisse zu Depression und Lebensqualität bei der amyotrophen Lateralsklerose, Deutsches Ärzteblatt International, 105, 397
- Nedele, P. (unpublished). Kognitionen, Emotionen und Bewältigung im Alltag von Patienten mit ALS: Eine empirische Arbeit bei Patienten mit amyotropher Lateralsklerose (ALS). (Cognition, emotions and coping in daily life: An empirical study in patients with amyotrophic lateral sclerosis (ALS). Unpublished diploma thesis of Psychology. University of Tübingen, Germany.
Cited by
Sorted by citations
Brain–computer interfaces for communication and rehabilitation
Chaudhary, U., Birbaumer, N., & Ramos-Murguialday, A. (2016) · Nature Reviews Neurology · doi:10.1038/nrneurol.2016.113 · Cited by 928
Mattle, H. P., Arnold, M., Lindsberg, P. J., Schonewille, W. J., & Schroth, G. (2011) · The Lancet Neurology · doi:10.1016/s1474-4422(11)70229-0 · Cited by 584
Brain computer interface: control signals review
Ramadan, R. Α., & Vasilakos, A. V. (2016) · Neurocomputing · doi:10.1016/j.neucom.2016.10.024 · Cited by 552
Simeral, J. D., Kim, S.-P., Black, M. J., Donoghue, J. P., & Hochberg, L. R. (2011) · Journal of Neural Engineering · doi:10.1088/1741-2560/8/2/025027 · Cited by 496
“Locked-in syndrome involves even more marked disability in which individuals lose all voluntary motor control with the exception in some cases of limited eye or other trace movements (Plum and Posner 1972, Laureys et al 2004, 2005, Lule et al 2009).”
A brain-computer interface for long-term independent home use
Sellers, E. W., Vaughan, T. M., & Wolpaw, J. R. (2010) · Amyotrophic Lateral Sclerosis · doi:10.3109/17482961003777470 · Cited by 400
An Accurate and Rapidly Calibrating Speech Neuroprosthesis
Card, N. S., Wairagkar, M., Iacobacci, C., Hou, X., Singer-Clark, T., Willett, F. R., Kunz, E. M., Fan, C., Vahdati Nia, M., Deo, D. R., et al. (2024) · New England Journal of Medicine · doi:10.1056/nejmoa2314132 · Cited by 268
Brain–computer interface–based communication in the completely locked-in State
Chaudhary, U., Xia, B., Silvoni, S., Cohen, L. G., & Birbaumer, N. (2017) · PLOS Biology · doi:10.1371/journal.pbio.1002593 · Cited by 235
“Patients F, G, and B answered open questions containing quality of life estimation repeatedly with a “yes” response, indicating a positive attitude towards the present situation and towards life in general, as reported in larger samples of ALS patients [43,44].”
BackgroundRetractedBruno, M., Bernheim, J., Ledoux, D., Pellas, F., Athena Demertzi, A., & Laureys, S. (2011) · BMJ Open · doi:10.1136/bmjopen-2010-000039 · Cited by 225
Self-citationWolpaw, J. R. (2013) · Handbook of clinical neurology · doi:10.1016/b978-0-444-52901-5.00006-x · Cited by 231
Lulé, D. E., Noirhomme, Q., Kleih, S. C., Chatelle, C., Halder, S., Athena Demertzi, A., Bruno, M., Gosseries, O., Vanhaudenhuyse, A., Caroline Schnakers, A., et al. (2012) · Clinical Neurophysiology · doi:10.1016/j.clinph.2012.04.030 · Cited by 191
“In spite of what healthy subjects might think, LIS patients can experience a good quality of life (Bruno et al., 2011a; Lule et al., 2009).”
Self-citationMugler, E. M., Ruf, C. A., Halder, S., Bensch, M., & Kübler, A. (2010) · IEEE Transactions on Neural Systems and Rehabilitation Engineering · doi:10.1109/tnsre.2010.2068059 · Cited by 181
“It is worth noting that these scores are above the average for healthy subjects [ 48 ].”
BackgroundSelf-citationThe body social: an enactive approach to the self
Kyselo, M. (2014) · Frontiers in Psychology · doi:10.3389/fpsyg.2014.00986 · Cited by 170
The Neurology of Consciousness
Tononi, G., Boly, M., Gosseries, O., & Laureys, S. (2015) · Elsevier eBooks · doi:10.1016/b978-0-12-800948-2.00025-x · Cited by 178
Self-citationAttitudes towards end-of-life issues in disorders of consciousness: a European survey
Athena Demertzi, A., Ledoux, D., Bruno, M., Vanhaudenhuyse, A., Gosseries, O., Soddu, A., Caroline Schnakers, A., Moonen, G., & Laureys, S. (2011) · Journal of Neurology · doi:10.1007/s00415-010-5882-z · Cited by 166
Self-citationDepression in epilepsy: a critical review from a clinical perspective
Hoppe, C., & Elger, C. E. (2011) · Nature Reviews Neurology · doi:10.1038/nrneurol.2011.104 · Cited by 143
Brain–computer interfaces for communication with nonresponsive patients
Naçi, L., Monti, M. M., Cruse, D., Kübler, A., Sorger, B., Goebel, R., Kotchoubey, B., & Owen, A. M. (2012) · Annals of Neurology · doi:10.1002/ana.23656 · Cited by 139
“Following injury, it is often possible for clinicians to confirm the presence of preserved sensory, cognitive, and emotional abilities in these patients on the basis of small but reproducible movements.(1,2) In the acute phase of LIS, consciousness is frequently impaired,(3,4) especially if there is brain swelling bey…”
Self-citationDecoding spoken phonemes from sensorimotor cortex with high-density ECoG grids
Ramsey, N. F., Salari, E., Aarnoutse, E. J., Vansteensel, M. J., Bleichner, M. G., & Freudenburg, Z. V. (2018) · NeuroImage · doi:10.1016/j.neuroimage.2017.10.011 · Cited by 124
“Disorders leading to severe communication disability include afflictions leading to total niversity Medical Center Utrecht, De ey). ber 2017; Accepted 6 October 2017 paralysis resulting from trauma, stroke and neurodegenerative diseases (Locked-In Syndrome)(Lul e et al., 2009), and loss of muscle coordination due to t…”
Howell, K., Grill, E., Klein, A.-M., Straube, A. M., & Bender, A. (2013) · Resuscitation · doi:10.1016/j.resuscitation.2013.05.015 · Cited by 116
Silva, A. B., Littlejohn, K. T., Liu, J. R., Moses, D. A., & Chang, E. F. (2024) · Nature Reviews Neuroscience · doi:10.1038/s41583-024-00819-9 · Cited by 103
Review: Human Intracortical Recording and Neural Decoding for Brain–Computer Interfaces
Brandman, D. M., Cash, S. S., & Hochberg, L. R. (2017) · IEEE Transactions on Neural Systems and Rehabilitation Engineering · doi:10.1109/tnsre.2017.2677443 · Cited by 104
“Despite the dramatic limitations in motor control, studies indicate that quality of life markers for individuals with ALS and LIS consistently demonstrate positive life attitudes [138], [139].”
Claassen, J., Velazquez, A. G., Meyers, E. E., Witsch, J., Falo, M. C., Park, S., Agarwal, S., Schmidt, J. M., Schiff, N. D., Sitt, J. D., et al. (2016) · Annals of Neurology · doi:10.1002/ana.24752 · Cited by 102
Quality of life in patients with locked-in syndrome: Evolution over a 6-year period
Rousseau, M.-C., Baumstarck, K., Alessandrini, M., Blandin, V., Billette de Villemeur, T., & Auquier, P. (2015) · Orphanet Journal of Rare Diseases · doi:10.1186/s13023-015-0304-z · Cited by 93
“Several studies have previously explored the QoL of LIS patients [5, 9–14] and surprisingly have shown that the”
BackgroundResultEffects of training and motivation on auditory P300 brain-computer interface performance
Baykara, E., Ruf, C. A., Fioravanti, C., Käthner, I., Simon, N., Kleih, S. C., Kübler, A., & Halder, S. (2016) · Clinical Neurophysiology · doi:10.1016/j.clinph.2015.04.054 · Cited by 91
Self-citationChaudhary, U., Mrachacz‐Kersting, N., & Birbaumer, N. (2020) · The Journal of Physiology · doi:10.1113/jp278775 · Cited by 88
Towards a user-friendly brain-computer interface: initial tests in ALS and PLS patients
Bai, O., Lin, P., Huang, D., Fei, D.-Y., & Floeter, M. K. (2010) · Clinical Neurophysiology · doi:10.1016/j.clinph.2010.02.157 · Cited by 80
“Loss of motor function significantly affects patients' quality of life (QoL) (Mockford et al., 2006; Bromberg, 2008; Williams et al., 2008; Lule et al., 2009) and increases the financial burden for the cost of care (Mutsaarts et al., 2004).”
Quality of life in fatal disease: the flawed judgement of the social environment
Lulé, D. E., Ehlich, B., Lang, D., Sorg, S., Heimrath, J., Kübler, A., Birbaumer, N., & Ludolph, A. C. (2013) · Journal of Neurology · doi:10.1007/s00415-013-7068-y · Cited by 77
Self-citationDirect Brain Control and Communication in Paralysis
Birbaumer, N., Gallegos-Ayala, G., Wildgruber, M., Silvoni, S., & Soekadar, S. R. (2013) · Brain Topography · doi:10.1007/s10548-013-0282-1 · Cited by 76
Comprehensive rehabilitative care across the spectrum of amyotrophic lateral sclerosis
Paganoni, S., Karam, C., Joyce, N. C., Bedlack, R., & Carter, G. T. (2015) · Neurorehabilitation · doi:10.3233/nre-151240 · Cited by 75
Persistent Vegetative State and Minimally Conscious State
Bender, A., Jox, R. J., Grill, E., Straube, A. M., & Lulé, D. E. (2015) · Deutsches Ärzteblatt international · doi:10.3238/arztebl.2015.0235 · Cited by 74
Self-citationWalter, U., Fernández-Torre, J. L., Kirschstein, T., & Laureys, S. (2018) · Clinical Neurophysiology · doi:10.1016/j.clinph.2018.08.009 · Cited by 70
Self-citationAn observational study on quality of life and preferences to sustain life in locked-in state
Kuźma‐Kozakiewicz, M., Andersen, P. M., Ciećwierska, K., Vázquez, C., Helczyk, O., Loose, M., Uttner, I., Ludolph, A. C., & Lulé, D. E. (2019) · Neurology · doi:10.1212/wnl.0000000000008064 · Cited by 69
“Still, low physical function is not necessarily associated with low well-being, as outlined in previous work.(11,12) Also,”
BackgroundResultInfluentialSelf-citationBrain-computer interfaces for communication
Vansteensel, M. J., & Jarosiewicz, B. (2020) · Handbook of clinical neurology · doi:10.1016/b978-0-444-63934-9.00007-x · Cited by 72
“Indeed, preserved social interaction — particularly via preserved communication — is a strong determinant of QoL in LIS (see Lul (cid:1) e et al., 2009).”
BackgroundDepression and wish to die in a multicenter cohort of ALS patients
Rabkin, J. G., Goetz, R. R., Factor-Litvak, P. R., Hupf, J. C., McElhiney, M. C., Singleton, J., Mitsumoto, H., & The Als Cosmos Study Group (2014) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.3109/21678421.2014.980428 · Cited by 73
Single-session communication with a locked-in patient by functional near-infrared spectroscopy
Abdalmalak, A., Milej, D. F., Norton, L. E., Debicki, D., Gofton, T. E., Diop, M., Owen, A. M., & Lawrence, K. S. (2017) · Neurophotonics · doi:10.1117/1.nph.4.4.040501 · Cited by 64
“In general, locked-in patients represent an ideal case to test the method in the intensive-care unit (ICU) given they are awake and aware, unlike DOC patients but lack almost all ability to respond to commands.(14) At the time of this experiment, the patient had regained limited eye movement, which provided a unique o…”
MethodPositive Clinical Neuroscience
Kapur, N., Cole, J. D., Manly, T., Viskontas, I. V., Ninteman, A., Hasher, L., & Pascual‐Leone, Á. (2013) · The Neuroscientist · doi:10.1177/1073858412470976 · Cited by 60
Emotional adjustment in amyotrophic lateral sclerosis (ALS)
Lulé, D. E., Pauli, S., Altintas, E., Singer, U., Merk, T., Uttner, I., Birbaumer, N., & Ludolph, A. C. (2011) · Journal of Neurology · doi:10.1007/s00415-011-6191-x · Cited by 61
“ALS patients present a shift of priorities from themselves towards social aspects [15, 18, 21].”
InfluentialSelf-citationImpaired Conscious Recognition of Negative Facial Expressions in Patients with Locked-in Syndrome
Pistoia, F., Conson, M., Trojano, L., Grossi, D., Ponari, M., Colonnese, C., Pistoia, M. L., Carducci, F., & Sarà, M. (2010) · Journal of Neuroscience · doi:10.1523/jneurosci.6300-09.2010 · Cited by 60
“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).”
BackgroundLocked-in syndrome: a challenge for embodied cognitive science
Kyselo, M., & Paolo, E. A. D. (2013) · Phenomenology and the Cognitive Sciences · doi:10.1007/s11097-013-9344-9 · Cited by 59
“Reports on their quality of life show that what matters to LIS patients is not so much their physical bodily state or integrity but their relation to other people and that they remain recognized as a person to be engaged with (Bruno et al. 2008; Gosseries et al. 2009; Lulé et al. 2009).”
BackgroundA Brain-Computer Interface Based on Bilateral Transcranial Doppler Ultrasound
Myrden, A., Kushki, A., Sejdić, E., Guerguerian, A., & Chau, T. T. (2011) · PLoS ONE · doi:10.1371/journal.pone.0024170 · Cited by 48
“Moreover, BCI control bypasses the muscular system entirely and thus may allow communication even for those who are completely locked-in due to conditions such as stroke or amyotrophic lateral sclerosis (ALS) [7].”
BackgroundSchwartz, C., Pfefferkorn, T., Ebrahimi, C., Ottomeyer, C., Fesl, G., Bender, A., Straube, A. M., Pfister, H., Heck, S., Tonn, J., et al. (2017) · Neurosurgery · doi:10.1093/neuros/nyw138 · Cited by 47
Kuehlmeyer, K., Racine, É., Palmour, N., Hoster, E., Borasio, G. D., & Jox, R. J. (2012) · Journal of Neurology · doi:10.1007/s00415-012-6459-9 · Cited by 47
“[36] reported that despite their extreme motor impairment, a significant number of LiS patients maintain a good quality of life that seems unrelated to their state of physical functioning.”
BackgroundIndividual quality of life in spousal ALS patient-caregiver dyads
Galvin, M., Gavin, T., Mays, I., Heverin, M., & Hardiman, O. M. (2020) · Health and Quality of Life Outcomes · doi:10.1186/s12955-020-01551-5 · Cited by 43
“social, spiritual, and existential [12, 13].”
Amyotrophic lateral sclerosis progression and stability of brain-computer interface communication
Silvoni, S., Cavinato, M., Volpato, C., Ruf, C. A., Birbaumer, N., & Piccione, F. (2013) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.3109/21678421.2013.770029 · Cited by 43
A Fate Worse Than Death? The Well-Being of Patients Diagnosed as Vegetative With Covert Awareness
Graham, M. (2017) · Ethical Theory and Moral Practice · doi:10.1007/s10677-017-9836-8 · Cited by 42
Evaluation of Quality of Life in Complete Locked-In Syndrome Patients
Rousseau, M., Pietra, S., Nadji, M., & de Villemeur, T. B. (2013) · Journal of Palliative Medicine · doi:10.1089/jpm.2013.0120 · Cited by 42
Rehabilitation Outcome of Unconscious Traumatic Brain Injury Patients
Klein, A.-M., Howell, K., Vogler, J., Grill, E., Straube, A. M., & Bender, A. (2013) · Journal of Neurotrauma · doi:10.1089/neu.2012.2735 · Cited by 41
“This dichotomization takes into account that a good QOL is not necessarily dependent on functional status, as it was shown in the case of patients in the locked-in syndrome.(15) The temporal pattern of CRS improvements were analyzed by determining the week during which the first significant CRS increase occurred (Fig.”
InfluentialPhenomenology of the Locked-In Syndrome: an Overview and Some Suggestions
Vidal, F. (2018) · Neuroethics · doi:10.1007/s12152-018-9388-1 · Cited by 41
SCHNETZER, L., McCoy, M. R., Bergmann, J., Kunz, A. B., Leis, S., & Trinka, E. (2023) · Therapeutic Advances in Neurological Disorders · doi:10.1177/17562864231160873 · Cited by 40
Nizzi, M., Athena Demertzi, A., Gosseries, O., Bruno, M., Jouen, F., & Laureys, S. (2011) · Consciousness and Cognition · doi:10.1016/j.concog.2011.10.010 · Cited by 41
“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 (Demertzi et al., 2011; Lule et al., 2009).”
Self-citationLinse, K., Rüger, W., Joos, M., Schmitz‐Peiffer, H., Storch, A., & Hermann, A. (2017) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2017.1392576 · Cited by 35
“Moreover, even severely impacted patients are able to find meaning (32,33).”
BackgroundCorallo, F., Bonanno, L., Buono, V. L., Salvo, S. D., Rifici, C., Pollicino, P., Allone, C., Palmeri, R., Todaro, A., Alagna, A., et al. (2017) · Journal of Stroke and Cerebrovascular Diseases · doi:10.1016/j.jstrokecerebrovasdis.2017.06.026 · Cited by 34
“034* NA Bodily pain T0 25 (10-50) 60 (60-100) <.”
Klein, A.-M., Howell, K., Straube, A. M., Pfefferkorn, T., & Bender, A. (2013) · Clinical Neurology and Neurosurgery · doi:10.1016/j.clineuro.2013.08.004 · Cited by 34
Medical Decision Making by Patients in the Locked-in Syndrome
Bernat, J. L. (2018) · Neuroethics · doi:10.1007/s12152-018-9358-7 · Cited by 33
A European survey on attitudes towards pain and end-of-life issues in locked-in syndrome
Athena Demertzi, A., Jox, R. J., Racine, É., & Laureys, S. (2014) · Brain Injury · doi:10.3109/02699052.2014.920526 · Cited by 33
“implications, such as reduced satisfaction with quality of life on the patient’s behalf [15, 20, 35].”
BackgroundResultSelf-citationMeditation Training for People with Amyotrophic Lateral Sclerosis and Their Caregivers
Pagnini, F., Credico, C. D., Gatto, R., Fabiani, V., Rossi, G., Lunetta, C., Marconi, A., Fossati, F., Castelnuovo, G., Tagliaferri, A., et al. (2014) · Journal of Alternative and Complementary Medicine · doi:10.1089/acm.2013.0268 · Cited by 30
“It is possible that mindfulness meditation, which is sometimes seen as a relationship with elements that we experience, may provide a coping strategy for this condition.(22) Because it is difficult to understand the subjective experience of a person who cannot communicate, this hypothesis is difficult to prove.”
BackgroundAn Ethics of Welfare for Patients Diagnosed as Vegetative With Covert Awareness
Graham, M., Weijer, C., Cruse, D., Fernández‐Espejo, D., Gofton, T. E., González-Lara, L. E., Lazosky, A., Naçi, L., Norton, L. E., Peterson, A. H., et al. (2015) · AJOB Neuroscience · doi:10.1080/21507740.2015.1014072 · Cited by 32
“Nevertheless, our focus on physical pain is consistent with the majority of the neuroscientific and neuroethical literature, and, more importantly, consistent with an aspect of welfare that is likely to be highly relevant to these patients.”
BackgroundResultPhysician driven variation in the care of children with spinal muscular atrophy type 1
Oskoui, M., Ng, P., Liben, S., & Zielinski, D. (2016) · Pediatric Pulmonology · doi:10.1002/ppul.23616 · Cited by 31
Snoeys, L., Vanhoof, G., & Manders, E. (2012) · Disability and Rehabilitation · doi:10.3109/09638288.2012.705950 · Cited by 30
“LIS can arise suddenly or can develop progressively, implying a gradual evolution towards the condition [16,17].”
BackgroundInference and Inductive Risk in Disorders of Consciousness
Johnson, L. S. M. (2016) · AJOB Neuroscience · doi:10.1080/21507740.2016.1150908 · Cited by 28
Diagnosing Consciousness: Neuroimaging, Law, and the Vegetative State
Fisher, C. E., & Appelbaum, P. S. (2010) · The Journal of Law Medicine & Ethics · doi:10.1111/j.1748-720x.2010.00496.x · Cited by 27
Grill, E., Klein, A.-M., Howell, K., Arndt, M., Bodrozic, L., Herzog, J., Jox, R. J., Koenig, E., Mansmann, U., Müller, F., et al. (2013) · Archives of Physical Medicine and Rehabilitation · doi:10.1016/j.apmr.2012.10.040 · Cited by 28
Graham, M. (2021) · The Journal of Medicine and Philosophy A Forum for Bioethics and Philosophy of Medicine · doi:10.1093/jmp/jhab026 · Cited by 27
“In fact, research suggests that the most powerful predictor for good quality of life in locked-in patients is perceived social support (Lule et al., 2009; Rousseau et al., 2013).”
BackgroundLocked-In Syndrome: Case Report and Discussion of Decisional Capacity
Maiser, S., Kabir, A., Sabsevitz, D. S., & Peltier, W. (2015) · Journal of Pain and Symptom Management · doi:10.1016/j.jpainsymman.2015.10.021 · Cited by 27
Spoken and Inner Speech-related EEG Connectivity in Different Spatial Direction
Kiroy, V. N., Бахтин, О. М., Krivko, E., Lazurenko, D. M., Aslanyan, E. V., Shaposhnikov, D. G., & Щербань, И. В. (2021) · Biomedical Signal Processing and Control · doi:10.1016/j.bspc.2021.103224 · Cited by 30
Lemaire, J., Sontheimer, A., Nezzar, H., Pontier, B., Luauté, J., Roche, B., Gillart, T., Gabrillargues, J., Rosenberg, S. D., Sarret, C., et al. (2013) · Annales Françaises d Anesthésie et de Réanimation · doi:10.1016/j.annfar.2013.11.007 · Cited by 25
“Even so, the severity of handicap in patients emerging from consciousness disorders must also be anticipated as best we can, and it is important to integrate the experiences of the patient’s relatives, healthcare workers, and wherever possible the patient too [92].”
BackgroundLocked-in Syndrome and BCI - Towards an Enactive Approach to the Self
Kyselo, M. (2011) · Neuroethics · doi:10.1007/s12152-011-9104-x · Cited by 25
“This seems to imply that the “ physical state does not predict psychological adjustment ” (p.344) [37].”
BackgroundBrain–computer interfaces and dualism: a problem of brain, mind, and body
Lee, J. (2014) · AI & Society · doi:10.1007/s00146-014-0545-8 · Cited by 23
“The mind–body problem is accentuated for dualism, in short, ‘‘how can you move your bodily part by willing that you move it, given that moving a bodily part is a physical event while thinking that you move a bodily part is a mental event?’’”
BackgroundCan they suffer? The ethical priority of quality of life research in disorders of consciousness
Johnson, L. S. M. (2013) · Bioethica Forum · doi:10.24894/bf.2013.06035 · Cited by 23
Wilkinson, D. J. C., & Savulescu, J. (2014) · Bioethics News · doi:10.1007/s40592-014-0002-y · Cited by 23
“For example, a hospital policy might state that antibiotic B should not be provided, or that patients with B-type problems should not be admitted to intensive care.”
BackgroundConsciousness in the Locked-In Syndrome
Bruno, M., Nizzi, M., Laureys, S., & Gosseries, O. (2015) · Elsevier eBooks · doi:10.1016/b978-0-12-800948-2.00012-1 · Cited by 24
Self-citationTowards decoding speech production from single-trial magnetoencephalography (MEG) signals
Wang, J., Kim, M., Hernandez-Mulero, A. W., Heitzman, D., & Ferrari, P. (2017) · IEEE International Conference on Acoustics Speech and Signal Processing · doi:10.1109/icassp.2017.7952714 · Cited by 20
“Providing a level of communication offers these patients a chance to resume a meaningful life [3].”
BackgroundKageyama, Y., He, X., Shimokawa, T., Sawada, J., Yanagisawa, T., Morris, S., Sakura, O., Kishima, H., Mochizuki, H., Yoshimine, T., et al. (2020) · Journal of Neurology · doi:10.1007/s00415-020-09903-3 · Cited by 22
Ethics of neuroimaging after serious brain injury
Weijer, C., Peterson, A. H., Webster, F., Graham, M., Cruse, D., Fernández‐Espejo, D., Gofton, T. E., González-Lara, L. E., Lazosky, A., Naçi, L., et al. (2014) · BMC Medical Ethics · doi:10.1186/1472-6939-15-41 · Cited by 22
“In another study, the selfreported scores of locked-in patients for mental health, general health, and bodily pain were close to those of healthy controls [57].”
ResultSilencing The Brain May Be Better Than Stimulating it. The GABA Effect
Pistoia, F., Sarà, M., Sacco, S., Franceschini, M., & Carolei, A. (2013) · Current Pharmaceutical Design · doi:10.2174/13816128113196660649 · Cited by 22
“Depression and anxiety-related symptoms are apparently lacking if a pure ventral pontine lesion is responsible for the syndrome [57,59].”
BackgroundDisease progression but not physical state per se determines mental wellbeing in ALS
Vázquez, C., Aho-Özhan, H. E. A., Weiland, U., Uttner, I., Ludolph, A. C., & Lulé, D. E. (2020) · Journal of Neurology · doi:10.1007/s00415-020-10027-x · Cited by 22
Self-citationThe Role of Specialty Palliative Care for Amyotrophic Lateral Sclerosis
Sethi, A., Everett, E. A., Mehta, A. K., Besbris, J. M., Burke, C., Pedowitz, E., Kilpatrick, M. M., Foster, L., & Maiser, S. (2021) · American Journal of Hospice and Palliative Medicine® · doi:10.1177/10499091211049386 · Cited by 21
Severely affected ALS patients have broad and high expectations for brain-machine interfaces
Kageyama, Y., Hirata, M., Yanagisawa, T., Shimokawa, T., Sawada, J., Morris, S., 水島, 希., Kishima, H., Sakura, O., & Yoshimine, T. (2014) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.3109/21678421.2014.951943 · Cited by 21
Brandman, D. M., & Hochberg, L. R. (2016) · Wiley eBooks · doi:10.1002/9781118816028.ch9 · Cited by 23
Barć, K., & Kuźma‐Kozakiewicz, M. (2020) · Neurologia i Neurochirurgia Polska · doi:10.5603/pjnns.a2020.0081 · Cited by 20
Vansteensel, M. J., Branco, M. P., Leinders, S., Freudenburg, Z. V., Schippers, A., Geukes, S. H., Gaytant, M. A., Gosselaar, P. H., Aarnoutse, E. J., & Ramsey, N. F. (2022) · Neurorehabilitation and neural repair · doi:10.1177/15459683221125788 · Cited by 21
A Symbols Based BCI Paradigm for Intelligent Home Control Using P300 Event-Related Potentials
Akram, F., Alwakeel, A. M., Alwakeel, M. M., Hijji, M., & Masud, U. (2022) · Sensors · doi:10.3390/s222410000 · Cited by 22
Lulé, D., Kübler, A., & Ludolph, A. (2019) · Frontiers in Neurology · doi:10.3389/fneur.2019.00259 · Cited by 14
“This may even be true in the final state of complete immobility, the locked-in state [LIS; (30, 31)].”
BackgroundSelf-citationBasilar Artery Occlusion: Diagnosis and Acute Treatment
Buchman, S. L., & Merkler, A. E. (2019) · Current Treatment Options in Neurology · doi:10.1007/s11940-019-0591-0 · Cited by 19
An accurate and rapidly calibrating speech neuroprosthesis
Card, N. S., Wairagkar, M., Iacobacci, C., Hou, X., Singer-Clark, T., Willett, F. R., Kunz, E. M., Fan, C., Vahdati Nia, M., Deo, D. R., et al. (2023) · doi:10.1101/2023.12.26.23300110 · Cited by 244
“As communication fails, people report increased rates of isolation, depression, and decreased quality of life 2,3 ; losing communication often determines if a person will pursue or withdraw life-sustaining care in advanced ALS.”
BackgroundNizzi, M., Blandin, V., & Athena Demertzi, A. (2018) · Neuroethics · doi:10.1007/s12152-018-9375-6 · Cited by 18
“ening scenario which leads to questions of whether a Blife is worth living^ after severe brain injury [6].”
BackgroundEliminating ‘life worth living’
Fumagalli, R. (2017) · Philosophical Studies · doi:10.1007/s11098-017-0892-7 · Cited by 18
Real-time decoding of full-spectrum Chinese using brain-computer interface
Qian, Y., Liu, C., Yu, P., Ran, X., Li, S., Yang, Q., Liu, Y., Xia, L., Wang, Y., Qi, J., et al. (2025) · Science Advances · doi:10.1126/sciadv.adz9968 · Cited by 23
Brain Computer Interfaces for Assisted Communication in Paralysis and Quality of Life
Chaudhary, U., Chander, B. S., Ohry, A., Jaramillo-Gonzalez, A., Lulé, D. E., & Birbaumer, N. (2021) · International Journal of Neural Systems · doi:10.1142/s0129065721300035 · Cited by 19
Self-citationEvaluation of quality of life in individuals with severe chronic motor disability: A major challenge
Rousseau, M., Baumstarck, K., de Villemeur, T. B., & Auquier, P. (2016) · Intractable & Rare Diseases Research · doi:10.5582/irdr.2016.01017 · Cited by 17
“It has been previously observed that patients' subjective QoL is not related to physical impairments; this observation agrees with previous studies of different motor neuron disorders (1,2,25,34,38,48-53), SCI patients (54), and LIS patients (5,9,49,53,55).”
ResultCommunication and Well-Being Considerations in Disorders of Consciousness
Lazaridis, C., Desai, M. J., & Johnson, L. S. M. (2021) · Neurocritical Care · doi:10.1007/s12028-020-01175-z · Cited by 17
“Research has repeatedly shown, however, that disabled persons report that their own quality of life is not diminished by disability [10].”
BackgroundPrecedent Autonomy and Surrogate Decisionmaking After Severe Brain Injury
GRAHAM, M. (2020) · Cambridge Quarterly of Healthcare Ethics · doi:10.1017/s0963180120000286 · Cited by 16
Wilson, E., Lee, J., Wenzel, D., & Faull, C. (2022) · Brain Sciences · doi:10.3390/brainsci12091162 · Cited by 16
“As other aspects of the neurological condition worsen, such patients may become unable to communicate other than via eye movements (locked-in syndrome) [9,10] or may even lose all ability to communicate (a total locked-in state) [11].”
BackgroundRezvani, S., Hosseini-Zahraei, S., Tootchi, A., Guger, C., Chaibakhsh, Y., Saberi, A., & Chaibakhsh, A. (2023) · Cognitive Neurodynamics · doi:10.1007/s11571-023-09995-3 · Cited by 18
“It is now well established from various studies (Khanna et al. 2011; Lule´ et al. 2009; Kuzma-Kozakiewicz et al. 2019) that the QoL in LIS patients is often significantly underestimated by the patient’s primary caregivers and spouses compared to what the patients claim about their condition.”
Soekadar, S. R., & Birbaumer, N. (2014) · Springer eBooks · doi:10.1007/978-94-007-4707-4_41 · Cited by 16
Stasolla, F., Caffò, A. O., Bottiroli, S., & Ciarmoli, D. (2021) · Developmental Neurorehabilitation · doi:10.1080/17518423.2021.2011457 · Cited by 16
Goodrich, E., Wahbeh, H., Mooney, A. R., Miller, M., & Oken, B. (2014) · Neuropsychological Rehabilitation · doi:10.1080/09602011.2014.970201 · Cited by 19
“Quality of life in this population is often reported to be in the same range as in age-matched healthy individuals (Lule et al., 2009), yet people with SSPI still face unique emotional struggles.”
ResultPsychophysiological correlates of coping and quality of life in patients with ALS.
Real, R. G. L., Herbert, C., Kotchoubey, B., Wessig, C., Volkmann, J., & Kübler, A. (2014) · Clinical Neurophysiology · doi:10.1016/j.clinph.2013.09.040 · Cited by 9
“, 2001), but strongly influenced by psychosocial factors (Lulé et al., 2009; Matuz et al., 2010; McDonald et al., 1994; Plahuta et al., 2002).”
BackgroundSelf-citationThe Minimally Conscious State, the Disability Bias, and the Moral Authority of Advance Directives
Napier, S. R. (2018) · International Journal of Law and Psychiatry · doi:10.1016/j.ijlp.2018.03.001 · Cited by 14
Smart, C. M., & Giacino, J. T. (2015) · Neurorehabilitation · doi:10.3233/nre-151244 · Cited by 13
“Contrary to expectations, several survey studies have found that, once past the acute stage of illness/injury and medically stable, persons with LIS report a quality of life and even happiness that is comparable to individuals with no major medical illness or injury (Lulé et al., 2009; Bruno et al., 2011).”
Schorl, M., Valerius-Kukula, S. J., & Kemmer, T. P. (2014) · Clinical Neurology and Neurosurgery · doi:10.1016/j.clineuro.2014.05.004 · Cited by 13
“self-assessment of QoL may differ substantially as depicted in the literature [70, 71, 72] e.”
Metadata
- Authors
- Lulé, D., Zickler, C., Häcker, S., Bruno, M. A., Demertzi, A., Pellas, F., Laureys, S., & Kübler, A.
- Affiliations
- University of Liège (BE)
- University of Tübingen (DE)
- Centre Hospitalier Universitaire de Nîmes (FR)
- University of Würzburg (DE)
- Corresponding author
- Lulé, D.
- Venue
- Progress in Brain Research
- Publisher
- Elsevier
- Published
- 2009
- Volume
- 177
- Pages
- 339-351
- ISSN
- 0079-6123 (ISSN-L)
- OpenAlex
- W1531824716
- Cited by
- 217
- References
- 82
- Citations by year
- 2026: 8 · 2025: 10 · 2024: 12 · 2023: 12 · 2022: 8 · 2021: 23 · 2020: 12 · 2019: 10 · 2018: 10 · 2017: 11 · 2016: 13 · 2015: 18 · 2014: 18 · 2013: 21 · 2012: 9
- FWCI
- 29.40
- Type
- book chapter
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Progress in brain research (publishedVersion)
- PubMed (publishedVersion)
- Open Repository and Bibliography (University of Liège) (submittedVersion, other-oa)
- Funders
- James S. McDonnell Foundation
- Mind Science Foundation
- European Commission
- Topics
- Health Sciences › Medicine › Rehabilitation › Stroke Rehabilitation and Recovery
- Health Sciences › Medicine › Oncology › Cancer survivorship and care
- Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Aging, Health, and Disability
- Keywords
- locked-in syndrome, quality of life, depression, psychosocial adjustment, coping strategies, social support, amyotrophic lateral sclerosis, brainstem infarction, motor impairment, end-of-life decisions, assistive communication technology
- MeSH terms
- Adaptation, Psychological, Consciousness Disorders, Depression, Persons with Disabilities, Humans, Quadriplegia, Quality of Life, Surveys and Questionnaires
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
