Trapped but Aware: Understanding Locked-In Syndrome and Its Treatments

Apurva Sudesh Abitkar, Sarah Fathima Khateeb, Maria Tiana Shenella Paiva, Murad Majdi Farah, Raashed Ramzan, Dr. Sethulakshmi Keelathara Sajeev

Abstract


Locked-In Syndrome (LIS) is a rare neurological disorder with quadriplegia and anarthria accompanied by the preservation of cognitive function and vertical eye movement. It results from injury to the ventral pons, most commonly by stroke, trauma, or demyelinating disease. LIS is very challenging to diagnose, and advanced neuroimaging techniques like fMRI, EEG, and CT scanning are required to differentiate it from coma and vegetative states. The quality of life is relatively good for most LIS patients, especially with regard to the availability of assistive communication devices and psychological counseling, despite the motor disabilities being catastrophic. The management of LIS also includes neurorehabilitation, which consists of physical therapy aimed at avoiding complications such as muscle atrophy and deep venous thrombosis, combined with mental fortitude, communication, and cognitive enhancement therapies. Emerging technologies, including brain-computer interfaces and neural implants, show immense potential for boosting autonomy. Ethical considerations involving patient autonomy and end-of-life decisions are still complex, with the majority of LIS patients initially expressing a wish for hastened death but later accepting their situation. Advances in neuroimaging, electrophysiology, assistive technologies, and multidisciplinary rehabilitation have significantly improved the outcomes of LIS patients. Societal myths and ethical issues continue to impact clinical care. Individualized rehabilitation strategies and further incorporation of neuroprosthetic solutions are areas where future research must be focused to encourage patient independence and quality of life.
Keywords: Locked-in Syndrome, quadriplegia, neuroimaging, electrophysiology, brain-computer interface, quality of life, neurorehabilitation, patient autonomy, assistive technology, ethical considerations.


Keywords


Locked-in Syndrome, quadriplegia, neuroimaging, electrophysiology, brain-computer interface, quality of life, neurorehabilitation, patient autonomy, assistive technology, ethical considerations

Full Text:

PDF

References


Schnetzer L, McCoy M, Bergmann J, Kunz A, Leis S, Trinka E. Locked-in syndrome revisited. Ther Adv Neurol Disord. 2023;16:17562864231160873. Published 2023 Mar 29.

Halan, T., Ortiz, J. F., Reddy, D., Altamimi, A., Ajibowo, A. O., & Fabara, S. P. (2021). Locked-In Syndrome: A Systematic Review of Long-Term Management and Prognosis. Cureus, 13(7), e16727.

Das JM, Anosike K, Asuncion RMD. Locked-in Syndrome. In: StatPearls. Treasure Island (FL): StatPearls Publishing; July 24, 2023.

Smith E, Delargy M. Locked-in syndrome. BMJ. 2005 Feb 19;330(7488):406-9.

Law, Y. M., Feng, L. F., Liang, Q., Meng, L. J., Shen, P., Yu, S. J., & Pao, W. Y. (2018). Effect of Exercise on Physical Recovery of People with Locked-In Syndrome after Stroke: What Do We Know from the Current Evidence? A Systematic Review. Cerebrovascular diseases extra, 8(2), 90–95.

Lulé D, Zickler C, Häcker S, et al. Life can be worth living in locked-in syndrome. Prog Brain Res. 2009;177:339-351.

Yen, C., Lin, C. L., & Chiang, M. C. (2023). Exploring the frontiers of neuroimaging: a review of recent advances in understanding brain functioning and disorders. Life, 13(7), 1472.

Markand, O. N. (1976). Electroencephalogram in “locked-in” syndrome. Electroencephalography and Clinical Neurophysiology, 40(5), 529-534.

Roquet, D., Foucher, J. R., Froehlig, P., Renard, F., Pottecher, J., Besancenot, H., ... & Kremer, S. (2016). Resting-state networks distinguish locked-in from vegetative state patients. NeuroImage: Clinical, 12, 16-22.

Khanna, K., Verma, A., & Richard, B. (2011). “The locked-in syndrome”: Can it be unlocked? Journal of Clinical Gerontology and Geriatrics, 2(4), 96-99.

Nieto-Salazar, M. A., Osman, S. D. M. K., Jain, H., Blanco, R. M., Montecino, J. N. G., Pineda, M. I. M., ... & Aleman, A. M. (2023) Unlocking the Clinical Manifestations and Classifications of Locked-In Syndrome: A Comprehensive Review.

Laureys, S., Pellas, F., Van Eeckhout, P., Ghorbel, S., Schnakers, C., Perrin, F., ... & Goldman, S. (2005). The locked-in syndrome: what is it like to be conscious but paralyzed and voiceless? Progress in brain research, 150, 495-611.

Lulé, D., Zickler, C., Häcker, S., Bruno, M. A., Demertzi, A., Pellas, F., ... & Kübler, A. (2009). Life can be worth living in locked-in syndrome. Progress in brain research, 177, 339-351.

Kuzma-Kozakiewicz, M., Andersen, P. M., Ciecwierska, K., Vázquez, C., Helczyk, O., Loose, M., ... & Lulé, D. (2019). An observational study on quality of life and preferences to sustain life in locked-in state. Neurology, 93(10), e938-e945.

Yoshiki, H., Morimoto, N., Urayama, K. Y., & Urayama, K. (2023). Understanding the psychological well-being of patients with locked-in syndrome: a scoping review. Cureus, 15(1).

Hordila, M. L., García‐Bravo, C., Palacios‐Ceña, D., & Pérez‐Corrales, J. (2024). Locked‐in syndrome: A qualitative study of a life story. Brain and Behavior, 14(8), e3495.

Bai, O., Nakamura, M., Ikeda, A., et al. (2020). Functional electrical stimulation in neurological rehabilitation: Current use and future directions. Journal of NeuroEngineering and Rehabilitation, 17(1), 56.

Bruno, M. A., Pellas, F., Schnakers, C., et al. (2011). Preservation of cortical processing in locked-in syndrome. Brain, 134(3), 2475-2487.

Hochberg, L. R., Bacher, D., Jarosiewicz, B., et al. (2012). Reach and grasp by people with tetraplegia using a neurally controlled robotic arm. Nature, 485(7398), 372-375.

Kübler, A., & Birbaumer, N. (2008). Brain-computer interfaces and communication in paralysis: Extinction of goal directed thinking in completely paralysed patients? Clinical Neurophysiology, 119(11), 2658-2666.

Owen, A. M., Naci, L., & Fernández-Espejo, D. (2012). Restoring cognitive function in disorders of consciousness. The Lancet, 379(9832), 1634-1641.

Piccione, F., Scandola, M., Pavone, E. F., et al. (2006). Neurorehabilitation strategies for locked-in syndrome: Role of robotics and motor imagery. Restorative Neurology and Neuroscience, 24(2), 153-161.

Vansteensel, M. J., Pels, E. G., Bleichner, M. G., et al. (2016). Fully implanted brain-computer interface in a locked-in patient for independent home use. New England Journal of Medicine, 375(21), 2060-2066.

Schnetzer L, McCoy M, Bergmann J, Kunz A, Leis S, Trinka E. Locked-in syndrome revisited. Therapeutic Advances in Neurological Disorders. 2023;16.

Voity, K., Lopez, T., Chan, J. P., & Greenwald, B. D. (2024). Update on How to Approach a Patient with Locked-In Syndrome and Their Communication Ability. Brain Sciences, 14(1), 92.

Das JM, Anosike K, Asuncion RMD. Locked-in Syndrome. [Updated 2023 Jul 24]. In: Stat Pearls Treasure Island (FL): StatPearls Publishing; 2025 Jan.

Vansteensel MJ, Branco MP, Leinders S, et al. Methodological Recommendations for Studies on the Daily Life Implementation of Implantable Communication-Brain–Computer Interfaces for Individuals With Locked-in Syndrome. Neurorehabilitation and Neural Repair. 2022;36(10-11):666-677.

Sacha Leinders, Mariska J. Vansteensel, Giovanni Piantoni, Mariana P. Branco, Zac V. Freudenburg, Tineke A. Gebbink, Elmar G.M. Pels, Mathijs A.H. Raemaekers, Anouck Schippers, Erik J. Aarnoutse, Nick F. Ramsey,Using fMRI to localize target regions for implanted brain-computer interfaces in locked-in syndrome, Clinical Neurophysiology, Volume 155, 2023, Pages 1-15, ISSN 1388-2457.

Bonin, E. A. C., Delsemme, Z., Blandin, V., Alnagger, N. L., Thibaut, A., Faymonville, M.-E., Laureys, S., Vanhaudenhuyse, A., & Gosseries, O. (2022). French Survey on Pain Perception and Management in Patients with Locked-In Syndrome. Diagnostics, 12(3), 769.

Yoshiki H, Morimoto N, Urayama KY. Understanding the Psychological Well-being of Patients With Locked-in Syndrome: A Scoping Review. Cureus. 2023;15(1):e34295. Published 2023 Jan 27.

Laureys S, Pellas F, Van Eeckhout P, et al. The locked-in syndrome: what is it like to be conscious but paralyzed and voiceless? In: Laureys S, ed. Progress in Brain Research. Vol 150. Elsevier; 2005:495-611.

Bruno MA, Pellas F, Schnakers C, et al. Le Locked-In Syndrome : la conscience emmurée. Rev Neurol. 2008;164(4):322-335.

Yan Y, Demertzi A, Xia Y, et al. Ethics of life-sustaining treatment in locked-in syndrome: A Chinese survey. Ann Phys Rehabil Med. 2020;63(6):483-487.

Kompanje EJO. Ethical decision-making in two patients with locked-in syndrome on the intensive care unit. Clin Ethics. 2009;4(2):98-101.

Lulé D, Zickler C, Häcker S, et al. Life can be worth living in locked-in syndrome. In: Laureys S, Schiff ND, Owen AM, eds. Progress in Brain Research. Vol 177. Elsevier; 2009:339-351.




DOI: http://dx.doi.org/10.52155/ijpsat.v49.2.7099

Refbacks

  • There are currently no refbacks.


Copyright (c) 2025 Apurva Sudesh Abitkar, Sarah Fathima Khateeb, Maria Tiana Shenella Paiva, Murad Majdi Farah, Raashed Ramzan, Dr. Sethulakshmi Keelathara Sajeev

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.