Locked-In Syndrome (Locked In Syndrome)

Acquired neuromuscular disorder characterized by complete paralysis of voluntary muscles and lower CRANIAL NERVES except for limited voluntary eye movements. It is due to various cerebrospinal disconnections at or near the PONS and the POSTERIOR CRANIAL FOSSA, typically secondary to pontine hemorrhage or infarct. Because cognitive function is intact it is sometimes referred to as a pseudocoma state.

Also Known As:

Networked: 139 relevant articles (4 outcomes, 16 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Thrombosis (Thrombus)
2.	Stroke (Strokes)
3.	Persistent Vegetative State (Vegetative State)
4.	Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease)
5.	Opsoclonus-Myoclonus Syndrome (Kinsbourne Syndrome)

Experts

1.	Laureys, Steven: 6 articles (10/2015 - 01/2013)
2.	Branco, Mariana P: 5 articles (10/2021 - 01/2019)
3.	Vansteensel, Mariska J: 5 articles (10/2021 - 01/2019)
4.	Aarnoutse, Erik J: 4 articles (10/2021 - 01/2019)
5.	Ramsey, Nick F: 4 articles (10/2021 - 01/2019)
6.	Pels, Elmar G M: 4 articles (08/2021 - 01/2019)
7.	Chatelle, Camille: 4 articles (01/2018 - 01/2013)
8.	Pistoia, Francesca: 4 articles (01/2016 - 06/2010)
9.	Peters, Betts: 3 articles (10/2018 - 05/2014)
10.	Cash, Sydney S: 3 articles (01/2018 - 06/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Locked-In Syndrome:

1.	2- benzylidene- 3- (cyclohexylamino)- 2,3- dihydro- 1H- inden- 1- oneIBA 10/01/2022 - "Implantable BCI offers promise for individuals with severe disease, such as locked-in syndrome, but also raises important ethical issues. " 01/01/2014 - "In future work, it is hoped that by integrating the three signals into a hybrid BCI a significant improvement in performance will be forthcoming leading to an easily usable real time communication device for patients with severe disabilities such as Locked-In Syndrome (LIS)." 10/01/2018 - "The results of this study imply that children with severe motor disabilities (such as locked-in syndrome) may use an SSVEP-based BCI to restore/replace the ability to communicate." 01/01/2014 - "The aim of this study was to determine whether patients with locked-in syndrome (LIS) could elicit a P300 wave, using a vibrotactile oddball paradigm for establishing somatosensory BCI-based communication. " 01/01/2013 - "The present study provides evidence that EEG based BCI can detect command following in patients with altered states of consciousness and functional communication in patients with locked-in syndrome. "
2.	levodopa drug combination carbidopa (Nakom)FDA LinkGeneric 09/01/1995 - "In this study we report two cases of locked-in syndrome which were treated successfully with Sinemet (DuPont Pharmaceuticals, Wilmington, DE). " 09/01/1995 - "Even though the exact neuropharmacological mechanism is unclear, the dramatic improvement in these cases, as well as in one other published case report, dictates that use of Sinemet should be considered in the management of locked-in syndrome." 05/01/1996 - "Sinemet in locked-in syndrome." 09/01/1995 - "Use of Sinemet in locked-in syndrome: a report of two cases." 04/01/1989 - "Recovery in non-vascular locked-in syndrome during treatment with Sinemet."
3.	Tissue Plasminogen Activator (Alteplase)FDA Link 08/22/2019 - "As the alteplase was being infused, the patient started declining with an examination suggesting locked-in syndrome. " 05/12/2011 - "The authors present a case in which intravenous thrombolysis with recombinant tissue plasminogen activator (rt-PA) potentially saved a young man from locked-in syndrome or life threatening consequences. " 03/01/2004 - "We report two female patients aged 63 and 26 years, with Locked-in syndrome due to a basilar thrombosis who were treated successfully with intra arterial thrombolysis using recombinant tissue plasminogen activator (r-TPA). " 02/01/1992 - "This report describes a recipient of single-lung transplantation surviving extraordinary complications: (1) early graft failure mandating retransplantation; (2) left atrial thrombus formation, which resolved by recombinant tissue plasminogen activator lysis; (3) and development of a "locked-in-syndrome." Possible underlying mechanisms are discussed."
4.	Gabapentin (Neurontin)FDA LinkGeneric 06/01/2010 - "In this study, 4 patients presenting with locked-in syndrome complicated by opsoclonus-myoclonus syndrome were continuously treated with gabapentin up to 1200 mg/d. " 06/01/2010 - "We evaluated whether off-label treatment with gabapentin could help patients with locked-in syndrome and opsoclonus-myoclonus symptoms regain voluntary control of full eye movements. " 06/01/2010 - "Opsoclonus-myoclonus syndrome in patients with locked-in syndrome: a therapeutic porthole with gabapentin." 06/01/2010 - "The experience with a patient with locked-in syndrome suffering from opsoclonus-myoclonus symptoms is described: gabapentin successfully reversed the symptoms, just favoring the regaining of eye-dependent communication strategies." 06/01/2010 - "Gabapentin therapy for ocular opsoclonus-myoclonus restores eye movement communication in a patient with a locked-in syndrome."
5.	SodiumIBA 09/09/2022 - "Based on his underlying conditions, present episode of sodium correction, and MRI finding, the patient was diagnosed as having CPM, which progressively worsened, resulting in locked-in syndrome after 12 days. " 06/01/2016 - "After 10 days of treatment with no improvement, it was noted that the patient had locked-in syndrome and that her sodium levels had rapidly risen 2 days prior. " 02/01/1990 - "After initial improvement under careful sodium correction, the patients neurologic condition degraded progressively and within 4 weeks she developed a "locked-in"-syndrome. " 11/01/1994 - "While the initial serum sodium value of 186 mEq/L was corrected to 139 mEq/L in 5 days, locked-in syndrome, bilateral hand tremor and tetraparesis appeared. "
6.	SolutionsIBA 01/01/2018 - "Restoring communication for people with locked-in syndrome remains a challenging clinical problem without a reliable solution. " 10/01/2017 - "In cases of complete loss of muscle control, termed "complete locked-in syndrome," a BCI may be the only viable solution to restore communication. "
7.	BaclofenFDA LinkGeneric 04/01/2002 - "Motor function improvement following intrathecal baclofen pump placement in a patient with locked-in syndrome." 04/01/2002 - "This case study suggests that intrathecal baclofen pump therapy might improve motor function in select patients with locked-in syndrome." 01/01/2015 - "Intrathecal baclofen: effects on spasticity, pain, and consciousness in disorders of consciousness and locked-in syndrome."
8.	Calcineurin InhibitorsIBA 01/01/2013 - "Recovery of locked-in syndrome following liver transplantation with calcineurin inhibitor cessation and supportive treatment."
9.	Therapeutic UsesIBA 01/01/2013 - "While we previously focused on devices facilitating communication in the rather few patients with locked-in syndrome, much interest is now devoted to the therapeutic use of BCI in rehabilitation. "
10.	Sirolimus (Rapamycin)FDA Link 01/01/2023 - "Physicians should recognize a rapid progression of central pontine myelinolysis and locked-in syndrome in the context of heart transplant and although several factors likely contributed to this outcome, adjustment of immunosuppression including by substituting tacrolimus with sirolimus could be effective."

Therapies and Procedures

1.	Brain-Computer Interfaces 11/01/2022 - "Methodological Recommendations for Studies on the Daily Life Implementation of Implantable Communication-Brain-Computer Interfaces for Individuals With Locked-in Syndrome." 10/01/2018 - "Ethical Considerations in Ending Exploratory Brain-Computer Interface Research Studies in Locked-in Syndrome." 06/01/2015 - "This study demonstrates the first use of an intracortical brain-computer interface for neural point-and-click communication by an individual with incomplete locked-in syndrome. " 01/01/2013 - "We study the applicability of a visual P3-based and a Steady State Visually Evoked Potentials (SSVEP)-based Brain-Computer Interfaces (BCIs) for mental text spelling on a cohort of patients with incomplete Locked-In Syndrome (LIS). " 11/18/2023 - "Boosting brain-computer interfaces with functional electrical stimulation: potential applications in people with locked-in syndrome."
2.	Therapeutics 01/01/2005 - "The locked-in syndrome: a challenge for therapy." 07/01/2002 - "The locked-in syndrome: a syndrome looking for a therapy." 07/01/2002 - "The locked-in syndrome: a syndrome looking for a therapy." 04/01/1989 - "A case of successful pharmacological therapy of non-vascular locked-in syndrome (LIS) is described. " 12/01/1985 - " Franklin disagrees, contending that it is unfair to withhold life-sustaining therapy because a patient cannot communicate and that there is an increasing number of cases on record of patients with locked-in syndrome who have recovered or have learned to communicate through Morse code or computers."
3.	Implanted Electrodes 01/01/2011 - "In one study, neural signals recorded by an intracranial electrode implanted in a speech-related region of the left precentral gyrus of a human volunteer suffering from locked-in syndrome were transmitted wirelessly across the scalp and used to drive a formant synthesizer, allowing the user to produce vowels. " 12/09/2009 - "Neural signals recorded by a Neurotrophic Electrode implanted in a speech-related region of the left precentral gyrus of a human volunteer suffering from locked-in syndrome, characterized by near-total paralysis with spared cognition, were transmitted wirelessly across the scalp and used to drive a speech synthesizer. "
4.	Anesthesia 01/01/2021 - "We address these questions by investigating the brain's intrinsic timescales in healthy controls, motor (amyotrophic lateral sclerosis, locked-in syndrome), sensory (anesthesia, unresponsive wakefulness syndrome), and progressive reduction of sensory processing (from awake states over N1, N2, N3). " 01/01/2014 - "These studies invariably show that the complexity of the cortical response to TMS collapses when consciousness is lost during deep sleep, anesthesia and vegetative state following severe brain injury, while it recovers when consciousness resurges in wakefulness, during dreaming, in the minimally conscious state or locked-in syndrome. "
5.	Neural Prostheses 11/14/2018 - "Clinical studies evaluating neural prostheses in amyotrophic lateral sclerosis (ALS) patients have been performed; however, to date, no study has reported that ALS patients who progressed from locked-in syndrome (LIS), which has very limited voluntary movement, to a completely locked-in state (CLIS), characterized by complete loss of voluntary movements, were able to continue controlling neural prostheses. "