What Causes Locked-In Syndrome?

Locked-in syndrome, or LIS, arises when lesions form on the pons regions and the midbrain. This condition can also arise from strokes, brain stem infections, masses, demyelinating diseases, or brain trauma. This rare neurological disorder is characterized by paralysis, bulbar palsy, and quadriplegia. The patient retains vertical eye movements and cognitive functioning, however. 

Pons help coordinate the physical movement of all the voluntary body muscles. In diseased states, the damaged or diseased pons cannot relay signals to and from the body muscles. That breakdown results in paralysis, which affects the person’s facial movements, swallowing, chewing, talking, and speech. 

Causes of Locked-in Syndrome

There are several known causes of locked-in syndrome. They include:  

Vascular Causes 

The leading cause of LIS is ischemic and hemorrhagic strokes. Vascular complications cut communications to the corticobulbar, corticospinal, and corticopontine regions of the brain stem. Ischemic strokes arise when blood flow gets impeded by an obstructed brain blood vessel. A hemorrhagic stroke arises from a burst brain blood vessel. 

Brain Trauma 

The second leading cause of locked-in syndrome is brain trauma. Blunt force or penetrating trauma may result in a blood vessel tear or vessel occlusion. The tears or occlusion, in turn, cause whole- or partial-body paralysis. This trauma can also arise from substance abuse. 

Masses

Another cause of locked-in syndrome is masses forming on the midbrain and pons areas. Masses may originate from lung adenocarcinomas, cell sarcomas, or fibrillary astrocytoma finding their way to the brain stem. 

Demyelinating Diseases

Sometimes, this neurological condition gets triggered by demyelinating diseases, such as amyotrophic lateral sclerosis (ALS) or multiple sclerosis. 

Infections 

Brain stem infection transmissions have been shown to cause LIS, but are rare. The brain makes it difficult for brain stem abscesses to spread to adjacent structures. In an isolated case, a person developed LIS after the meningococcal infection caused the intracranial pressure to rise. This elevated pressure, in turn, allowed the infection to pass to adjacent brain stem structures. 

Forms of Locked-in Syndrome

Locked-in Syndrome manifests in three forms. 

Classical LIS 

Here, the patient’s brain stem experiences irreparable damage, leading to complete paralysis. The stroke or coma survivor can perform vertical eye movements. The survivor also retains cognitive abilities, like reasoning and hearing. 

Incomplete LIS

In this form of locked-in syndrome, the patient loses partial sensation. Only selected muscle movements get affected. 

Total or Complete LIS

Here, the patient’s voluntary muscles have been completely damaged. The patient experiences whole-body paralysis accompanied by total loss of eye movement. The patient still retains his or her cognitive abilities as shown by the EEG test. 

Consequences of Locked-in Syndrome 

People with LIS have different symptoms. The symptoms depend on the severity of the brain stem damage or disease. Several neurological diseases share similar symptoms. That makes it difficult to distinguish a regular coma from locked-in syndrome.

Locked-in Syndrome symptoms include immobility in the lower face and other body parts. Inability to speak, breathe, chew, perform facial expressions, or even move the eyes normally are other symptoms of this neurological condition. However, this neurological impairment preserves the patient’s cognitive functions.

The affected person remains alert, aware, and conscious of his or her environment. This LIS survivor reasons and has logic. Also, the LIS patient has regular sleeping and waking cycles. The patient can, however, only communicate through vertical eye movements or blinking. 

What Is the Treatment for Patients With Locked-in Syndrome?

Treating people with locked-in syndrome is a multi-tier process. It starts with the initial diagnostic tests. These include MRIs, CTs, EEGs, and PT scans to confirm this neurological condition. The treatment module then seeks to address the immediate symptoms of locked-in syndrome. Then, the patient undergoes physical and respiratory therapy and speech therapy. He or she is also provided with assisted technologies to help compensate for the lost motor functions. 

Diagnosing Locked-in Syndrome

Typically, clinicians will perform a motor response assessment to affirm the condition. Affected persons cannot respond to external stimuli while in a conscious state. For instance, he or she fails to withdraw his or her limbs from cold or hot stimuli. The examiners also conduct other diagnostic tests to confirm this neurological condition. For instance, doctors will recommend an MRI or CT brain imaging scan to rule out signs of stroke not caused by LIS, but by other conditions. 

Other tests carried out on the LIS patient include SPECT, or single-photon emission computed tomography, and PET, or positron emission tomography. Additionally, an EEG can be administered to see if the patient has regular sleep-wake patterns. 

Other confirmatory tests required for locked-in syndrome include cerebral angiography, Cerebrospinal (CSF), evoked potentials exam, and blood tests. 

Currently, there exists no definitive treatment for locked-in syndrome. As of today, the best the doctors can do is to manage the symptoms. That move prevents a further escalation of the situation, leading to dire complications. 

Experts have perfected both urgent and long-term management strategies to address this condition. The urgent or early diagnosis LIS interventions include speech and supportive therapies. These measures restore normal eating and breathing in a stroke survivor. 

Short-Term Treatments 

Supportive Therapies 

Prompt commencement of supportive therapy for LIS victims is highly recommended. This swift action increases the chances of recovery and containing further complications. The patient is immediately placed under artificial breathing and feeding aids. 

Other supportive therapy solutions for LIS patients include:

  • Addressing opportunistic infections like UTIs, thrombosis, and pneumonia in the immobilized patient. 
  • Taking precautions to prevent bedsores. 
  • Physiotherapy to prevent muscle and joint complications from prolonged immobilization. 

Communication Therapy

Speech therapy helps strengthen and restore the LIS survivor’s speech muscles. People with LIS often need communication devices, such as eye motion sensors and voice modulators. 

Long-Term Treatments 

After stabilizing the locked-in syndrome victim and making him or her comfortable, it is time to address the long-term consequences. Here are the long-term treatment modalities for LIS patients: 

Physical Therapy 

Here, the patient gets subjected to various physical routines. That therapy helps to strengthen and heal his or her muscles. 

Respiratory Therapy

LIS patients are also placed on respiratory therapy. The person undergoes chest cavity expansion training and breathing exercises. These interventions are ideal for cardiovascular and respiratory system functioning. 

Assistive Devices 

These devices allow mobility and communication. They include neural-computer links, eye motion sensors, and AI-inspired voice prosthetics. 

Who Is at risk of LIS? 

Generally, this neurological condition affects anyone. The effects are, however, more pronounced in persons aged 30 to 50 years. 

What Is the Prevalence of LIS? 

Locked-in syndrome is one of the most commonly misdiagnosed and unrecognized neurological disorders. That makes it difficult to figure out the prevalence of this serious and rare condition. 

What Damages Can You Recover in a Locked-In Syndrome Lawsuit?

Locked-in syndrome results in irreversible damage to the brain stem. It is usually viewed as one of the worst possible injuries in medicine. Only a handful of LIS patients attain full recovery. In most cases, the brain stem damage is usually too severe, and the patient faces lifetime consequences. 

You could recover economic and non-economic damages if you were diagnosed with LIS. This is especially true if your condition arose from medical negligence. For example, if a doctor prescribed the wrong medication and triggered a stroke or coma. 

You have the right to hold the negligent doctor or health provider financially accountable for the resulting medical condition and other losses. After all, the doctor’s negligent actions caused you to develop this life-threatening neurological condition characterized by loss of speech and mobility. 

Furthermore, the incompetency led to your pain and anguish of living with paralysis. If your claim is successful, the payout will go toward paying for the arising medical treatment and restorative therapies. You can also use a portion of the financial recovery to purchase devices to help you communicate, walk, and eat properly. Additionally, the settlement covers wages or income lost and you will continue to lose if this condition keeps you from working.  

The medical malpractice case gets resolved through an out-of-court settlement or before a jury or judge. The two processes consider the total economic and non-economic expenses incurred. They also consider other extenuating circumstances, such as the level of negligence. 

Building a successful locked-in syndrome claim requires an in-depth understanding of your state’s medical malpractice laws and years of handling such cases. A lawyer provides credible evidence to apportion blame to one or more parties. If successful, the lawyer compels the at-fault party to pay you damages and losses arising from the injury or medical condition caused by his or her negligence. 

In medical malpractice cases, the responsible party, in this case, a doctor or healthcare provider, must be shown to have abandoned his or her duty of care as per the industry’s set standards. In cases where the locked-in syndrome got triggered by an accident, such as an auto crash, the same legal elements apply as the responsible driver, like the doctor, still owes you a duty of care. 

Can You File a Medical Malpractice Lawsuit for Locked-in Syndrome?

You can file a medical malpractice lawsuit if a doctor’s or hospital’s negligence damaged your brain stem and caused you to develop locked-in syndrome. First, you need to establish that you entered into a doctor-patient relationship with the involved doctor or healthcare provider. A medical bill or invoice with the name and signature of your treating doctor can help demonstrate a doctor-patient relationship. 

You must show that the at-fault party violated the established standard of care. Here, your lawyer must show that stroke, seizures, or coma could have been avoided had the doctor or healthcare professional provided proper treatment. Your lawyer may hire a medical expert witness to help the judge (in a bench trial) or jury (in a jury trial) understand how your treating doctor departed from the applicable standard of care.

With the help of your lawyer, you will also need to determine the losses you have suffered and provide evidence for the same. Medical bill receipts, your employer’s letter, wage statements, receipts for out-of-pocket expenses, and other financial documents are instrumental in proving your LIS-related damages.  

Filing a medical malpractice claim with the appropriate local court is the first step of the lawsuit process. It is important to remember that a statute of limitations applies to such personal injury claims in Illinois. A knowledgeable medical malpractice lawyer is your best bet at ensuring your claim is filed on time. The lawyer can also help you learn more about your case, including how long such a case takes to resolve and the possible payouts. 

Your legal team handles the complicated out-of-court settlement negotiations with the insurance companies. If the case goes to trial, your attorney gathers the relevant evidence and does the necessary investigations. That ensures you build a robust case and get a favorable court ruling or jury verdict. 

Living With LIS 

Living with LIS is both challenging and costly. The patient requires lifelong care and assisted living equipment. It costs to provide respiratory machines, physical and speech therapy, and other medical interventions for recurrent UTIs, bedsores, and pneumonia attacks. You need a seasoned personal injury lawyer to get sufficient compensation for lifetime expenses. Otherwise, you risk going bankrupt and being unable to cover the costs arising from locked-in syndrome treatment. 

You can sue the negligent medical practitioners for failing to diagnose or making an inaccurate diagnosis. You can also file a claim if your medical condition arose from wrong medications, which triggered the brain damage. Also, you can file a medical malpractice claim if your condition arose from the unsanitary hospital environment. Or, your medical condition stemmed from human errors by the poorly trained medical staff at the hospital. 

Do not worry about the cost of a medical malpractice lawyer. Most medical malpractice lawyers operate on a contingency fee basis. That means you do not pay legal representation fees until the case is resolved. The lawyer usually deducts 33% of the awarded payout as legal fees. Other case expenses also get deducted from the awarded payout.

Chicago personal injury and workers’ compensation attorney Howard Ankin has a passion for justice and a relentless commitment to defending injured victims throughout the Chicagoland area. With decades of experience achieving justice on behalf of the people of Chicago, Howard has earned a reputation as a proven leader in and out of the courtroom. Respected by peers and clients alike, Howard’s multifaceted approach to the law and empathetic nature have secured him a spot as an influential figure in the Illinois legal system.

Years of Experience: More than 30 years
Illinois Registration Status: Active
Bar & Court Admissions: Illinois State Bar Association, U.S. District Court, Northern District of Illinois, U.S. District Court, Central District of Illinois