Locked-In Syndrome

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Introduction   

In 1966, neurologists Fred Plum and Jerome Posner created the terms persistent vegetative state, in which patients looked conscious but were not, and the term Locked-in Syndrome, in which patients looked unconscious but were not. (1, 2) 

Locked-in syndrome is an extremely rare neurological disorder that occurs after an injury/trauma to the brainstem, specifically the pons area. The pons area links the brainstem to the spinal cord, and is responsible for the proper functioning of neurotransmitters, including those involved in wake/sleep cycles, and pain perception, as well as spontaneous breathing and eye movement. (3) Severe injury to the pons area can result in total body paralysis, oftentimes irreversible. 

For purposes of this learning program, the term Locked-in Syndrome is used to describe conditions caused by both traumatic brain injury or insult (traumatic brain injury, acute stroke) as well as late stages of neurodegenerative/ demyelinating disorders such as advanced Multiple Sclerosis (MS), Guillain Barre Syndrome (GBS), and Amyotrophic Lateral Sclerosis (ALS).  

Quick Terminology Reference 

• Aphasia– absence of speech 
• Catatonia– a behavioral syndrome whose symptoms may include inability to move, and lack of speech. 
• Dysautonomia– disruption of the autonomic nervous system; may cause symptoms such as changes in body temperature, balance, and changes in swallow ability. 
• Dysphagia– difficulty speaking and swallowing 
• Mutism– absence of speech; inability to speak 
• Nystagmus – rapid, uncontrolled eye movements 
• Paraplegia– paralysis of the legs 
• Tetraplegia (also known as quadriplegia)– paralysis of all four limbs; paralysis may also extend to the chest or abdomen. 

Definition 

Locked-in Syndrome is a unique medical condition, most often occurring after injury to the brain stem. Symptoms may include quadriplegia, entire body sensory loss, and disruption of the ability to swallow, speak, chew, and control facial movements. Cognition levels, hearing, and vertical eye movement, though, are still intact and play important roles in the early identification of this condition and initiation of appropriate treatment therapies. (4) 

Assessment 

On initial assessment of a patient with suspected Locked-in syndrome, the following findings would be noted. The patient would be awake, with eyes open. They would be able to move their eyes laterally, and open and close their eyes. They can hear and see normally. They cannot, however, move their lower face, swallow, chew food, speak, or move their limbs. Due to the extent of paralysis, they would not be able to breathe on their own. (5) 

Types  

There are three subtypes of Locked-in Syndrome, based on the severity of symptoms. (6,7) They are as follows: 

• Classical 
• Incomplete 
• Total  

Classical 

• Total immobility or lack of voluntary movement 
• Vertical eye movement, and blinking eyes  
• Cognitive abilities intact 
• Hearing intact 

Incomplete 

• Total immobility or lack of voluntary movement 
• Vertical eye movement, and blink eyes  
• Cognitive abilities intact 
• Hearing intact 
• Additional symptom: some sensation and movement noted in certain areas of your body. 

Total 

• Full body paralysis and loss of eye movement 
• Cognitive abilities intact (confirmed by EEG to measure brain waves) 

Epidemiology 

According to several “rare disease” statistics sites, the occurrence of Locked-in Syndrome is indeed rare. It is believed that less than 1000 people in the United States have this disease process. The average age for onset of this condition is 30-50 years old, with 46 years of age being the average (although this condition can occur at any age, dependent on causative factors). Family members are often the first ones to identify a person as having Locked-in Syndrome, observing eye movements that appear appropriate to conversations being held with the person. 

In addition, a recent population-based study, in Norway, only reported 16 cases of Locked-in Syndrome within a population of approximately 5.4 million people.  Without early identification and proper treatment, the mortality rate for those suffering from Locked-in Syndrome is very high within the first few months, which most likely negatively impacts the aforementioned statistical findings (8,9).  

Pathophysiology 

Basic Brain Anatomy and Physiology  

The human brain is divided into three main parts, the cerebrum, brain stem, and cerebellum. The cerebrum is responsible for coordination of movement, and body temperature regulation, as well as speech, vision, and hearing. The cerebellum is responsible for voluntary muscle movement, as well as balance. The brainstem connects the cerebrum with the spinal cord and contains the midbrain, the pons, and the medulla. (10,11) 

The pons is an area involved with cranial nerve pathways, which facilitate multiple daily activities, including (but not limited to) blinking, vision, facial expression, and chewing.  

Four cranial nerves intersect with the pons area of the brain. They include the following:  

• Trigeminal nerve (5th) – Involved with the sensory and motor function of the mouth, teeth, facial muscles, and chewing ability. Injury to the 5th nerve would result in interference with normal function of the nerve fibers associated with forehead movement, upper eyelid movement, and corneal/ lacrimal glands reflexes. 
• Abducens nerve (6th) – Involved in ocular abduction (ability to move eyes outward towards sides of head; injury would affect horizontal eye movement). 
• Facial nerve (7th) – Involved in facial movement (injury would cause facial paralysis, preventing patient from performing any facial movements such as wrinkling their forehead, smiling, grimacing, and in some cases, inability to close their eyelids, which leads to severe corneal irritation). 
• Vestibulocochlear nerve (8th) – Responsible for balance and hearing (injury may cause nystagmus {involuntary eye movements}, and tinnitus {a ringing or buzzing sensation in the ears}, as well as hearing loss, vomiting, or a sense of motion sickness). 

Pathology behind Locked-in Syndrome (12) 

In Locked-in Syndrome, it is believed that injury to the pons area of the brain leads to disruptions in the nerve fibers (neurotransmitters) and results in widespread effect. The pons contains nerve fibers that connect with the medulla oblongata and the cerebellum. Damage to the pons interrupts all the nerve fibers throughout the spinal cord; thus, resulting in paralysis of the body, including those involved in facial control, expressions, and speech. (13)   

Locked-in Syndrome has a very high mortality rate within the first 4-6 months. Survival rates are thought to be negatively impacted by the lack of early identification of this syndrome. Hence, there are (potentially) many missed opportunities for medical stabilization and aggressive therapies to lower complication risks in the long term. 

Research has shown that if the patient with Locked-in Syndrome survives the first year, there are significant improvements in long-term survival (86% in the 5-year survival rate; 80% in the 10-year survival rate). Age at the time of injury would also impact survival rates, as well as overall health and well-being. Of the cases reported, those who began aggressive rehabilitation in the acute recovery stages of this condition had lower mortality rates. (14) 

Clinical Signs and Symptoms 

Patients experiencing Locked-in Syndrome are initially often thought to be in a coma. They cannot consciously chew, swallow, or make even the most basic facial movements, much less speak. There is usually no observed voluntary body movement below the level of the eyes. Concerning actual eye movement, the patient with Locked-in Syndrome can blink their eyes and move their eyes vertically (up and down) but not horizontally (side to side).  

The patient is capable of hearing and comprehending what people are saying to them. Due to the global lack of motor response to stimuli (such as withdrawal from painful stimuli), the patient with Locked-in Syndrome is often thought to be comatose or unconscious, further adding to a difficult, timely diagnosis and treatment plan. (15) 

Etiology 

The most common causes of Locked-in Syndrome are vascular lesions and trauma. In addition, infections, neurological disorders and demyelinating conditions may also cause this syndrome. 

Any lesion or injury that affects the pons area of the brain has the potential to cause signs and symptoms of this condition. (16, 17) 

• Vascular lesions (the most common cause is a stroke, obstructing the basilar artery. The stroke can be either hemorrhagic or ischemic.  
• Masses/tumors including brainstem tumors 
• Infections including encephalitis and botulism 
• Trauma including traumatic brain injuries, as well as blunt force and penetrating trauma 
• Demyelinating disorders including poliomyelitis, polyneuritis, myasthenia gravis 

Vascular Lesions  

The most common vascular lesion that can cause Locked-in Syndrome is stroke. Both ischemic and hemorrhagic strokes that affect the vertebrobasilar artery area of the brain and interfere with blood flow to the basilar artery can affect this condition. CT and MRI studies would confirm the presence and extent of a stroke. (18)  

A stroke to the pons area of the brain would result in quadriplegia and bulbar palsy (due to additional nerve impairment) in most patient populations. Signs and symptoms would include flaccid paralysis, absent reflexes, interruption of normal gag reflex and breathing patterns, as well as the inability to handle oral secretions (leading to a heightened risk of aspiration). 

Strokes are the leading cause of Locked-in Syndrome. The NIHSS is the preferred assessment scale for the evaluation of stroke. Consider the following scenario and what concerns you may have.  

The NIHSS (National Institutes of Health) Stroke Scale was developed in 1989 and consists of a 15-item impairment scale. Each item is scored, with the score range of 0-42 points. Used in the evaluation of strokes, the NIHSS scores are thought to be reflective of the clinical outcomes. Total scores less than 4 indicate favorable outcomes, while scores greater than 21 indicate severe strokes. NIHSS scores of greater than 22 are at greater risk of hemorrhagic conversion, if given tPA (tissue plasminogen activator) protocol. (19,20,21) 

Given that an acute stroke patient may present to the Emergency Department with a NIHSS of greater than 22, consider the following questions: 

Masses/ Tumors (22) 

The growth of a mass (tumor) in the anterior pons or midbrain area can affect blood flow and lead to Locked-in Syndrome. Fibrillary astrocytomas and pontine reticulum cell sarcomas have also been implicated in the development of Locked-in Syndrome. Additionally, metastasis from adenocarcinoma of the lung, and malignant melanoma (skin cancer) have been reported as possible precipitating factors in causing Locked-in Syndrome. As with stroke, CT and MRI studies would confirm the presence of a mass or tumor growth.  

Infections (23)  

In rare instances, the following infections can cause Locked-in Syndrome-like symptoms. Serum blood tests (complete blood counts with differential) would confirm the presence of inflammation or infection. Blood cultures would confirm bacterial infections. Lumbar punctures would confirm increased intracranial pressures. With appropriate treatment of underlying infections, there are usually significant improvements in recovery to a patient’s baseline, but long-term effects (on cognitive abilities) may linger.  

• Meningitis can cause increased intracranial pressure that affects blood flow to the pons area of the brain 
• Brainstem abscesses   
• Pseudomonas systemic infection affecting brain stem blood flow 
• Meningovascular syphilis causing pontine (pons section of the brain) infarction  

Trauma (24) 

Any external trauma / traumatic brain injury can cause Locked-in Syndrome. Both blunt force trauma and penetrating trauma (stab wounds and gunshot wounds), causing damage to the vertebrobasilar artery blood flow, can result in Locked-in Syndrome. A tear in the vessel lining or developing a thrombus post-trauma would also affect blood flow. CT and MRI scans would confirm the presence and extent of external/penetrating trauma or blunt trauma contusions. Currently, traumatic brain injury is the 2nd largest cause of Locked-in Syndrome.  

Demyelinating Disorders (25) 

Central Pontine Myelinolysis (secondary to rapid correction of hyponatremia) (CPM) 

Central pontine myelinolysis (CPM) is also known as Osmotic Demyelination Syndrome (ODS). It results in damage to sections of the brain, including the pontine tracts. It occurs after rapid correction of hyponatremia (sodium deficiency). During rapid correction of serum sodium levels, water is pulled from brain cells and can cause injury/destruction to the myelin sheath covering nerve fibers.  

Patients at high risk of hyponatremia include the following: 

• Elderly patients taking multiple medications (rule out drug-to-drug interactions, and medication side effects as causative factors) 
• Patients with multiple medically complex conditions such as liver disease, endocrine disorders (hypothyroidism), advanced kidney disease 
• Patients taking long-term medication therapies such as diuretics, antihypertensives, and antidepressants 
• Patients being treated for pneumonia and urinary tract infections  
• Patients involved in long-distance outdoor activities, such as ultramarathons (may consume significant levels of water while exercising and dilute their serum sodium levels) 

Symptoms can occur within days of hyponatremia and include the following: 

• Decreased level of alertness 
• Difficulty speaking and swallowing (dysphagia) 
• Difficulty with coordination 
• Weakness of extremities  
• Restlessness and irritability 
• Headaches and confusion 
• Muscle weakness with muscle spasms and cramps 
• Hypotension (low blood pressure) 

As sodium levels continue to decrease, the following symptoms may emerge: 

• Disorientation 
• Depressed reflexes 
• Seizure activity 
• Comatose states-> respiratory arrest 

Sodium replacement (treatment of hyponatremia) guidelines: 

• Sodium correction no greater than 8-12 mEq/L per 24 hours 
• Serum sodium level checks every 4-6 hours during replacement therapy. 

Levels of hyponatremia based on serum blood levels. 

• Mild 130-135 mEq/L 
• Moderate 120-129 mEq/L 
• Severe Below 120 mEq/L 

*Normal range for serum sodium level 135-145 mEq/L. 

Severe cases of myelinolysis can lead to comatose states, and Locked-in Syndromes, as well as death. Although recovery is possible, patients often experience long-term effects of intellectual impairment and movement disorders. (26,27) 

Multiple Sclerosis (MS) (28,29) 

Multiple Sclerosis is a chronic autoimmune condition that attacks the Central Nervous System. Damage occurs to the myelin sheath which surrounds and protects the nerve fibers. Chronic inflammation of these areas then causes scarring, leading to further disruption of normal pathways.  

Current diagnostics for Multiple Sclerosis include a lumbar puncture to do antibody testing, and MRI studies to evaluate brain lesions. In addition, Evoked Potential testing is used to check limb movement as it relates to nerve impulse stimulation. 

Guillain-Barre Syndrome (GBS) (30) 

Guillain-Barre Syndrome is a rare neurological disorder where the immune system attacks the Peripheral Nervous System, causing progressive weakness, numbness and paralysis. Although there is no known definitive cause, it has been noted that Guillain-Barre Syndrome often occurs in patients after exposure to certain viral and bacterial illnesses, including those of the gastrointestinal and respiratory tracts. Damage occurs to the myelin sheath, causing disruptions in the transmission of nerve signals. GBS is diagnosed through physical examination, lumbar puncture, and nerve conduction studies.  

There are three main types of GBS and are distinguished by continent of origin/ occurrence as well as the progression “direction” of muscle weakness (ascending/ descending). 

• Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) 
  • The most common form in North America and Europe.  
  • The most common sign of AIDP is muscle weakness that starts in the lower part of the body and spreads upward (ascending weakness/paralysis) 
• Miller Fisher Syndrome (MFS) 
  • Most common form in Asia 
  • The most common sign is paralysis, which starts in the eyes, accompanied by an unsteady gait. In contrast to Guillain-Barre Syndrome, Miller-Fisher Syndrome tends to affect the upper body.  
• Acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN) 
  • More common in China, Japan, and Mexico. 
  • The most common signs are acute paralysis and loss of reflexes. Unlike other forms of Guillain-Barre Syndrome, AMAN syndrome has a sudden onset with minimal demyelination. 

Amyotrophic Lateral Sclerosis (ALS) (31,32) 

Amyotrophic Lateral Sclerosis, also known as Lou Gehrig’s disease, is a fatal, neurological disorder that results in weakness, loss of voluntary movements, and eventual paralysis. Chronic nerve inflammation disrupts normal neurotransmitter pathways, resulting in increasing loss of motor neurons. This progression of the disease causes an increasing inability to control voluntary muscle movement. Diagnostic testing for ALS may include lumbar punctures, MRI, muscle biopsy, and nerve conduction studies.  

Akinetic Mutism (33) 

Akinetic mutism is a rare neurological syndrome. Patients diagnosed with akinetic mutism have an intact level of cognition/ consciousness and sensory/motor capacity but often lack spontaneous speech or movement. Although they appear to be unable to move or speak, they are not in the true form of Locked-in Syndrome, as they have the physical ability to perform daily tasks. Akinetic mutism may occur as the result of injury to the frontal lobes of the brain, as in the case of traumatic contusions. 

Symptoms that are characteristic of this form of mutism include the following: 

• Intact level of consciousness and sensorimotor capacity 
• Decease in emotions, most notably a profound sense of apathy and lack of goal directed actions or behaviors 
• Absence of any spontaneous movement 
• Absence of any spontaneous speech 
• Indifference to pain, thirst and even hunger 

Diagnostics 

• Rule out metabolic disturbances (osmotic demyelination) and brain injury activity (such as brain herniation and brainstem lesions)  
• Rule out drug overdoses (intentional or accidental)  

Treatment (34,35) 

Transcranial magnetic stimulation treatment, which is a non-invasive treatment, uses external magnets (magnetic fields) to stimulate nerve cells. This therapy is often used in the treatment of depression and associated mental health disorders. Additional therapies, dependent on the underlying causative factors, may include pharmaceutical components such as benzodiazepines, antipsychotics, and antidepressants. 

Catatonia (36) 

Catatonia is a neuropsychiatric syndrome that may cause a person to exhibit unusual behaviors and movements. It is thought to be the result of a variety of conditions, including depression, autism, dementia, multiple sclerosis, and encephalitis, as well as medication side effects and even strokes.  Symptoms can range from grimacing facial expressions to mutism (aphasia) and posturing and stupor (no response to external stimuli). Catatonia differs from akinetic mutism with additional behaviors such as echolalia (involuntary repetition of words spoken by another person) and echopraxia (involuntary repetition of the actions of another person). 

Diagnostics  

• CT and MRI scans 
• Blood and urine cultures 
• Lumbar puncture studies 
• EEG studies brain activity.  

Treatment  

• Medications (benzodiazepines, followed by mood stabilizers and antipsychotics)  
• ECT treatments (electroconvulsive) 
• Transcranial Magnetic Stimulation  

Locked-in Syndrome Diagnostics  

The following diagnostics can be done to assist in the confirmatory diagnosis of Locked-in Syndrome as well as to assist in ruling out other conditions causing similar symptoms. Many of these tests would most likely be done during the customary initial stabilizing treatment of an acute stroke. (37, 38) 

MRI/CT (Magnetic resonance imaging/ Computed Tomography)  

Advanced radiologic studies, such as MRI and CT scans, are performed to ascertain damage to the brain as the result of a stroke. Detailed imaging, indicating specifically damage (isolated lesions) to the pons area of the brain would heighten the possibility of Locked-in Syndrome. MRIs are useful in the differential diagnosis of Locked-in Syndrome versus other conditions such as coma, vegetative states, and minimally conscious states.  

EEG (Electroencephalogram) 

The EEG is done to evaluate electrical activity in the brain. This examination can measure normal brain activity as well as sleep-wake cycles.  

EEG in Locked-in Syndrome-> level of consciousness is evaluated by eye movement or blinking response to questions asked. Patients with Locked-in Syndrome will show appropriate activity to various stimuli as well as normal sleep-wake cycles. By comparison, patients in a chronic vegetative state would show a slow response to various stimuli. 

Evoked Potentials 

This examination procedure is done to evaluate electrical activity in the brain and spinal cord as it relates to outside/external stimulation (pain, auditory, and/or visual stimulation).  

EP in Locked-in Syndrome->EP studies are used to assess patient awareness; could also be used to assess pain and sensation in patients to determine the extent of the syndrome. 

CSF (Cerebrospinal Spinal Fluid Evaluation) 

This examination can be performed to determine if the presenting symptoms are related to an underlying infection, or an autoimmune disease process.  

Lumbar punctures would be done in patients with suspected Locked-in Syndrome to rule out other conditions. The presence of inflammatory markers related to autoimmune disease processes would lower the suspicion of a trauma-related brain insult. 

EMG  

Electromyography studies can be done to evaluate muscle and nerve functioning. The paralysis occurring in Locked-in syndrome would interfere with normal muscle and nerve functioning; the EMG would confirm the extent (type) of Locked-in Syndrome. 

Laboratory Testing 

• Complete Blood Counts with differential to rule out inflammation, infection 
• Baseline chemistries to rule out underlying metabolic imbalances (especially sodium levels to rule out pontine myelinolysis) 
• Urinalysis with cultures to rule out underlying infection 
• Therapeutic drug levels (if applicable) to rule out other causes of symptoms.  

Ongoing Treatment of Locked-in Syndrome  

At this time, there is no actual “cure” for Locked-in Syndrome, and there are no specific “plans of care” for those diagnosed with Locked-in Syndrome. Treatments for this syndrome are primarily focused on treating the cause and long-term prevention of complications. (39,40) 

As the patient with Locked-in Syndrome is unable to perform the basics of daily living (self-care), ongoing, around-the-clock supportive care and communication therapies are top priority.  

The following therapies require prompt initialization and ongoing utilization to reduce the heightened risk of long-term, potentially life-threatening complications: 

• Tracheotomy airway support – Airway management reduces the risk of pneumonia and aspiration. 
• Gastrostomy tube nutritional support – to ensure proper nutrition and hydration. 
• Bowel and bladder elimination – to prevent constipation, obstipation, obstruction, urinary retention, and urinary tract infections that could lead to systemic sepsis)  
• Physical Therapy to preserve range of motion and lower risk of limb contracture; ongoing assessment of voluntary movements (rehabilitation/ maximal point of recovery) 
• Speech Therapy to evaluate alternate methods of communication (through eye movements, communication boards, infrared and computer-enhanced prosthetic devices) 
• Occupational Therapy to evaluate assessments/ serve in consulting role to facilitate caregiver duties in extended care/ home care 
• Prevention of pressure ulcers, limb contractures and thromboembolic disease due to immobility  

Secondary Prevention (to lower the risk of recurrent stroke) 

A high percentage of the cases of Locked-in Syndrome occur after a person suffers a catastrophic stroke. In these cases, there should also be a dedicated focus on reducing the risk of recurrent stroke.  

The following guidelines should be taken into consideration: 

• Antihypertensive therapies (preexisting history of hypertension) 
• Hyperlipidemia therapies (statin therapies in high-risk individuals) 
• Hyperglycemia therapies (preexisting diabetes medical condition) 
• Physical inactivity therapies (preexisting lifestyle behaviors versus post stroke limited mobility) 
• Proper nutritional therapies (to address any preexisting metabolic conditions {prediabetes, diabetes, metabolic syndrome} as well as to maintain optimal nutrition post stroke) 
• Lifestyle behavioral assessment and intervention. If the patient has a history of alcohol and nicotine usage prior to stroke, patient should be assessed for possible substance withdrawal in the acute phase of rehabilitation (and treated accordingly) 
• Antiplatelet and anticoagulant therapies if applicable (* may be contraindicated in cases of hemorrhagic stroke conditions) 

As patients with Locked-in Syndrome are believed to have full cognitive function, they should be included in their own healthcare decision-making. Through the use of alternate communication assistive devices, with the guidance of speech therapists, the patient can communicate through eye movements. 

Self-Management/ Care Management 

The following quote (and its sentiments) were found in many of the sites researched for this learning program: “Due to supportive care and advancements in technology, many people with Locked-in Syndrome live full and meaningful lives”. 

Ethics/ Ethical Dilemma (Active, Assisted, and Passive Euthanasia) 

Currently, in the United States, active euthanasia is illegal in all 50 states.  

10 states have legislation in place to support active or assisted euthanasia (California, Colorado, Oregon, Vermont, New Mexico, Maine, New Jersey, Hawaii, Washington, D.C.). All 50 states; however, have legislation in place to support passive euthanasia (DNR/ do not resuscitate; pain management even if it hastens the person’s death). (43) 

Palliative and Hospice Care for Locked-in Syndrome (44) 

Palliative Care 

Palliative care is focused on the improvement of the quality of life for people with serious illnesses as well as their care partners. It may begin at the time of diagnosis (or at any time throughout the duration of the illness/ chronic condition) and often involves an interdisciplinary team to address all facets of ongoing care (including advanced directives, individual and family support therapies, and end of life planning) Palliative care can be initiated alongside of curative care; one does not exclude the other.  

Hospice Care  

Hospice care is traditionally initiated when the person with a serious illness is no longer undergoing curative treatment/any longer responding favorably to attempts to cure/ slow down (halt) the disease progression. The physician(s) has determined a life expectancy of less than 6 months. Treatments at this time may also be interdisciplinary, but shift focus to comfort, individual and family support therapies (including bereavement and aftercare), and guidance with end-of-life decisions.  

Ongoing Care Decisions in Locked-in Syndrome 

Locked-in Syndrome is associated with a poor prognosis overall.  

The following comment was found online in an article (45) discussing patients who had suffered with LiS: 

“…It is quite common for health professionals to infer that a LIS person quality of life is poor and, therefore, consider their lives worthless. Research with LIS patients report significant levels of self- perceived quality of life and their demand for euthanasia is surprisingly infrequent.”  

The article discussed the various challenges that these patients, as well as their families, experienced as the result of having Locked-in Syndrome, including but not limited to changes in physical, and psychological well-being, as well as challenges in self-identity, self-worth, and overall quality of life. Coupled with an article (46) that reported the following statistics, how does this affect your thoughts regarding the care of patients with Locked-in Syndrome?  

“…Statistically, “Mortality and recovery are highly variable depending on the underlying cause of the locked-in syndrome. Locked-in syndrome generally has a high mortality rate in the acute setting. Retrospective studies and analysis models have estimated the 5-year and 10-year mortality rates of 84% and 31%, respectively.”  

As healthcare professionals, we are well-trained to care for medically complex patients. The latest advances in “modern medicine” have been able to extend the number of years one lives, but the question remains regarding the quality found within those final years. The patients who have experienced Locked-in Syndrome, as well as their caregivers, have described many challenges to the ongoing medical care required to treat this condition. How best can we serve our patients during these most challenging times and ensure both the patient, and their family are living a life of quality? 

The following external website references are listed to provide additional resources to healthcare providers caring for medically complex patients. They offer therapies, support groups, clinical trials and ongoing research. Utilize them to build your workplace resource center.  

The final two case studies presented involve key insights into the lives of two men who experienced Locked-in Syndrome as the result of catastrophic strokes. They both used their remaining life to share unique glimpses into the daily challenges of living in a “Locked-in” world.  Through their efforts, they were able to provide us with a rare, first-hand patient experience.  

Research and Resources 

• Brain Foundation 
  • https://brainfoundation.org.au/disorders/locked-in-syndrome-lis/  
• Shirley Ryan AbilityLab  
  • https://www.sralab.org/conditions/locked-syndrome-lis  
• The Brain Charity 
  • https://www.thebraincharity.org.uk/condition/locked-in-syndrome/ 
• The Christopher and Dana Reeve Foundation 
  • https://www.christopherreeve.org/  
• The National Association for Rare Disorders
  • https://rarediseases.org/rare-diseases/locked-in-syndrome/ 

Case Studies 

The following two cases, in many ways, introduced the world to the inner workings of Locked-in Syndrome, from a patient’s point of view. These two gentlemen experienced this syndrome after suffering a catastrophic stroke and adjusted to the challenges forced upon them from this disease process. The cases are truly insightful as they are told from the patient/ family perspective and should serve further in opening discussions on the right to die and what constitutes true “quality of life”.  

Reflection Questions: 

• How do you describe “quality of life”? 
• What is more important to you, to live a long life, or a good life? 
• How do you describe a “good death”? 
• What factors should be considered in a patient’s request to end their life? 
• Do you feel that an individual has the right to determine when/ how they will die (through any form of euthanasia)? 

At this time, several countries authorize assisted suicide and criminalize active euthanasia. Euthanasia (also known as active euthanasia) is thought to hasten the death of a person with an incurable/ terminal disease, to alleviate end-stage pain and suffering.  In assisted suicide, the patient would make the final decision by taking the final action (such as ingesting an overdose of medication); in active euthanasia, the physician would administer a lethal dose of medication. (47) 

Conversely, passive euthanasia is considered legal in all 50 states under US jurisdiction. The legality of this issue lies in the fact that there is no actual “act of killing someone” but rather allowing someone to die naturally. Passive euthanasia is known as a “no code” status or “do not resuscitate” status and is a legal order that is signed by a medical professional. 

Case #1 (48, 49, 50) 

Tony Nicklinson, who resided in Wiltshire, England. 

Mr. Nicklinson suffered a stroke in 2005 at the age of 51. After living in a Locked-in state for seven years, with no chance of recovery, he petitioned the British Law to allow for active euthanasia, arguing that, due to total body paralysis, he could not partake in this suicide (by willingly ingesting lethal doses of medication). Under British Law, active euthanasia is considered murder and is punishable by life in prison. 

Tony mastered computerized communication boards and often utilized social media to convey his message. He argued that the courts should allow him the right to an assisted death, in this unique situation, as he was unable to take his own life due to physical limitations imposed by Locked-in Syndrome. He described his current life as a “living nightmare”. After spending 2.5 years in a hospital undergoing post-stroke rehabilitation, he was now confined to a wheelchair at home with 24/7 caregivers, which included his spouse and teenage children.  

The British courts denied his request. Reports are that he died in 2012, at his home, after refusing food. His family continued his legal case even after his death, taking it to additional courts before it was eventually rejected by the Supreme Court.  

• Do you agree with British Law that considers active euthanasia to be an act of murder? 
• Should there be special exceptions made to active euthanasia when extenuating circumstances (in case this, quadriplegia) prevent a person from actively assisting in their own death? 
• Do you feel there should be a “time limit” to evaluate a patient for maximal recovery after an illness before allowing them to pursue an act of euthanasia for a catastrophic illness/ condition? 
• If Mr. Nicklinson lived in the United States, how might his case have been handled differently? 
• If Mr. Nicklinson had advanced directives on file at the time of his stroke, how might his case have been handled differently? 

Case Study #2 (51,52) 

Jean-Dominique Bauby, who resided in France 

In December 1995, Jean-Dominique Bauby, the current Editor in Chief of the French Elle magazine, suffered a catastrophic stroke and lapsed into a coma. He awoke 20 days later, and it was determined he was in Locked-in Syndrome, with only minimal movement in his eyes. Eventually, he had to have his right eye sewn shut due to chronic irritation. At the time of his stroke, it was reported that he had recently agreed to write a book, having actually signed a book deal agreement.  

With the use of a communication board and an assigned transcriber who repeatedly recited the entire alphabet until Bauby blinked to confirm a letter, he was able to write an entire book simply by blinking his left eye. It is reported that the book took him “approximately 200,000 blinks to write at an average rate of a word every 2 minutes”.  

The book, The Diving Bell and the Butterfly, went on to become an international best-selling book, which was then made into a movie. It described everyday events in the life of a person suffering from Locked-in syndrome.  

Jean-Dominique Bauby died of pneumonia two days after the book was published in 1997, at the age of 44.  

• How would you care for these two patients, Mr. Nicklinson and Mr. Bauby, if they were admitted to your place of employment? 
• What interdisciplinary team members would be appropriate to add to these patients’ plan of care? 

Conclusion

Locked-in Syndrome is a rare medical condition that occurs after injury or trauma to the pons area of the midbrain. This condition can cause complete paralysis of the body, except for vertical eye movement. Yet, the patient is fully awake, alert, and with intact cognition. Early identification and ongoing medically complex therapies can increase substantially the life expectancy of a patient with Locked-in Syndrome. The professional nurse plays a unique role in patient advocacy in this medically vulnerable patient scenario, offering guidance in acute, chronic, long term and even end-of-life services.  

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