The Terrifying Reality of Locked-In Syndrome

Added: 2026-05-06

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[00:01:22]And now back to today's episode. Try and picture the scene. You wake up one morning thinking that it's just going to be a regular day just like any other.

[00:01:29]You open your eyes and everything just seems normal. But then you try to get out of bed and you can't. In fact, you can't move at all. Not a single muscle.

[00:01:39]Panic starts to set in. By this point, your brain is wide awake, your mind is racing, and you are completely aware of your surroundings, but it's still no use. You're trapped inside your own body. This is not a nightmare. This is the horrifying reality for people who suffer from an extremely rare neurological disorder called lockedin syndrome or LIS. They are completely conscious. Their cognitive functions remain intact. So they can hear, see, and understand everything that is happening around them, but they are struck by a near complete paralysis.

[00:02:11]There is no cure. They might stay that way for weeks. They might stay that way for years if they survive. As French novelist Alexander Dear once described the ordeal, "Their soul becomes trapped in a body that no longer obeys its commands." December the 8th, 1995 started off as just another beautiful day in the life of 43-year-old French journalist Jean Dominique Borby. He seemed to have it all. Beloved family, wealth, success, a thriving social life.

[00:02:36]He was the editor-in chief of the highly popular lifestyle magazine L. His calendar was full of glamorous events and invitations to swanky parties. But on that day, it was all taken away from him. After having dinner with his nine-year-old son, Bobby was driving them both to the theater for a night of entertainment. Then, suddenly, a shock jolted his entire body. Somehow, he managed to veer his car off the side of the road without crashing. But that was about all he could do. As his son ran out of the car to get help, the lights went out for Borby, and he lost consciousness. He woke up in the hospital 20 days later. He'd been in a coma for almost 3 weeks after suffering a stroke of the brain stem. To his horror, he realized that he could not move his body. He was completely paralyzed from head to toe. In fact, the only movement he was capable of was blinking his left eye. His right eye had to be sewn shut because it had suffered irritation. Following the stroke, he was diagnosed with lockedin syndrome. Even today, the prognosis for LIS recovery is not optimistic. Any kind of improvement of movement or motor skills is considered a triumph. Complete recovery is incredibly rare. Back in the 1990s, the outlook was considerably bleaker.

[00:03:38]Basically, Jean Dominique Borby had to get to grips with the idea that this was how he would live his life from then on, whatever little of it he had left.

[00:03:48]Ultimately, he did not let his new condition defeat him. Even though he had to rely on the staff and the machines in the hospital to take care of all of his physical needs, his mind was intact and just as sharp as ever. And as long as he could still blink his left eye, he had a means of communication with the outside world. In June 1996, half a year after being struck by lockedin syndrome, Borby decided to let everyone know that he was still alive and sound of mind if not body. Since few people had heard of Loctin syndrome at the time, it was often confused with a coma or a vegetative state. But Borby wanted to assure everyone that even though he could not move or speak, his mental faculties were still there. So, he began the arduous task of dictating a letter to inform his friends, colleagues, and acquaintances of his condition. For you and me, this would take minutes. For Bobby, it took days. He had to do it letter by letter. Someone recited the alphabet, and he blinked his eye to stop at the letter he wanted over and over again, hundreds of times until the message was finished. The journalist sent this letter to a few dozen people, which included some of the editors at Robert Lafont, a French book publisher.

[00:04:49]They were impressed with his efforts, and a bolt of inspiration struck them.

[00:04:52]They had already been in talks with Borby to write a book. It was meant to be a modernized version of the counter Monte Cristo. That was obviously not going to happen anymore. But what if they pivoted and instead published a book about Borby's experience with lockedin syndrome? It seems like a herculean task. Could someone in Borby's condition actually write a book? He took on the challenge and the end result was the diving bell on the butterfly, the definitive account of living with LIS.

[00:05:16]Borby wrote the book with the help of ghost writer Claude Menddeil. In the mornings before she arrived, the former journalist would compose and edit the day's text in his mind. And then, with Mendel by his side, the two employed the same method Bobby had used to write his correspondence, blinking his left eye whenever she reached the correct letter.

[00:05:32]To speed up the process, his speech therapist developed a special alphabet where the letters were arranged by frequency of use in the French language.

[00:05:38]Even so, it still took Borbby and Mendel 2 months to finish a 130page manuscript, working 3 hours a day, 7 days a week.

[00:05:45]After 200,000 blinks, the diving bell and the butterfly was finished. Borby chose that name to make reference to the duality of his existence. His mind was free and untethered like a butterfly, but it was trapped inside a rigid and confining body like a diving bell. The book was full of the wit and insight that Borby was known for prior to his stroke. It provided a never-before-seen look at a rare medical condition that most people didn't even know existed. It described day-to-day problems that would seem trivial to you or I, yet they were a constant source of frustration for someone in Bobb's shoes. problems such as being unable to swallow the saliva that the mouth kept producing or having a fly land on your nose and not being able to chew it away. There were of course far more tragic difficulties that he had to deal with. Bobby described how unbearable it was to look at his son sitting just inches away from him, knowing that he would never be able to hold him again. He lamented that he would never again see his frail housebound father since neither one could make the trip to visit the other.

[00:06:38]even something as common as having a bath could sometimes bring about existential dread as it made Borby aware of his complete physical helplessness and the life he had lost. Quoting, I can find it amusing in my 45th year to be cleaned up and turned over, to have my bottom wiped and swaddled like a newborns. I even derive a guilty pleasure from this total lapse into infancy. But the next day, the same procedure seems to me unbearably sad, and a tear rolls down the lather as the nurse's aid spreads over my cheeks. And my weekly bath plunges me simultaneously into distress and happiness. The dectable moment when I sink into the tub is quickly followed by nostalgia for the protracted wallowings that were the joy of my previous life. Armed with a cup of tea or a scotch, a good book or a pile of newspapers, I would soak for hours maneuvering the taps with my toes.

[00:07:24]Rarely do I feel my condition so crudely as when I am recalling such pleasures.

[00:07:29]The Diving Bell on the Butterfly was published in March 1997. His first run sold 25,000 copies in just a day, selling out and immediately becoming a bestseller. By the end of the week, the book had sold almost 150,000 copies in France alone, and foreign translations were put into production. It became a true testament to one man's fight against unthinkable adversity. Jean Dominique Borby died of pneumonia 2 days after the book came out. So, Borby's case became the best known example of lockedin syndrome. It was for many people the first time that they'd even heard of this terrifying condition. Now, it's 30 years later, but LIS is still a somewhat obscure disorder that few people are deeply acquainted with. So, here's the rundown on the basics.

[00:08:06]Lockedin syndrome is a rare and complex medical condition that presents itself with quadripollegia, bulb pulsy, and whole body sensory loss. Although the patient usually retains blinking, vertical eye movement, cognition, and hearing or in non-medical terms, the person is trapped inside their body, the mind is active, but the body is almost completely non-responsive. The extreme symptoms of lockedin syndrome are caused by damage to the brain stem, a small stalk-like part of the brain that connects the cerebrum to the spinal cord. The brain stem itself is made up of three components. The midbrain, the pawns, and the medulla oblongata. The anterior of the ponds is the area that most commonly suffers damage and causes LIS. Lockin syndrome is categorized into three main types. Classic, incomplete, and total immobility. The classic version is the one that Borby had that most patients have in fact where he could not move any parts of his body except for his eyes. Incomplete LIS still allows the patient to retain small motor functions and total immobility locked in syndrome. Well, you can probably guess from the name, complete body paralysis. The patients cannot move at all, not even blink their eyes. The only way to know that a person even has this awful condition is to monitor their brain functions with an electrophe or EEG. Researchers have proposed a fourth category in recent years, locked in plus, which believe it or not is even more debilitating and can be found in patients who suffer from impaired consciousness. There is so much that we do not yet understand about lockedin syndrome. Medical studies on it are few and far between. We're not even sure how many total cases there are worldwide or how many new ones are confirmed annually since there isn't a single database keeping track of them. We can say for certain though that it is an exceedingly rare condition. One nationwide study was done in Norway in 2023 and it reported 16 people living with LIS in a population of 5.42 million. that works out to roughly one case of locked in syndrome for every 339,000 individuals.

[00:09:52]One of the biggest challenges faced by the medical world when dealing with LIS is recognizing that it's there in the first place. A doctor would have to be informed on lockedin syndrome to even suspect it to begin with and they would need access to advanced medical equipment to look at the patients brain activity in order to properly diagnose it or to confirm damage to the brain stem. Unfortunately, that's not always an option, and locked in syndrome is often mistaken for other medical conditions that present with similar symptoms. Comas are probably the most common misdiagnosis, especially in patients with total immobility locked in syndrome. While they would appear similar to the untrained eye, a coma is a state of deep unconsciousness, very much the opposite of LIS. To make matters even more complicated, some people who develop Locktin syndrome do genuinely fall into a coma first. That was the case with Jean Dominique Borby who spent almost 3 weeks in a coma before regaining consciousness.

[00:10:42]Therefore, it is possible for an unconscious patient to be correctly diagnosed with a coma initially and then for them to regain normal brain activity without anybody around them realizing it. Because of this, locked in syndrome is sometimes referred to as a pseudo coma. Other common misdiagnoses include unresponsive wakefulness syndrome or UWS, the modern term for a vegetative state, and aetic mutism. At first glance, they all look the same, but they differ in key areas such as causes, treatments, and the degrees of consciousness. Someone with UWS can exhibit spontaneous eye movement and resume autonomic functions, but they still lack conscious behavior.

[00:11:17]Meanwhile, patients with ainetic mutism will display intact cognition just like those with lockedin syndrome, but their condition is not caused by damage to the brain stem and they are not physically paralyzed unlike those suffering from LIS. According to recent studies, it takes on average around two and a half months for a patient to be accurately diagnosed with lockedin syndrome. That means 75 days of a person waking up at the same time as everyone else, being able to see, hear, and understand everything that is happening around them, but be completely unable to let anyone else know that. And in rare cases, patients had to live like that for several years before someone was able to tell that they were in fact awake. Perhaps the most extreme example is that of Belgium man Rom Hobbin. In 1983, he was injured in a car crash and fell into a coma. Eventually, a brain scan revealed that his cognitive functions were still intact. That happened in 2006, 23 years after the crash. Did he really spend all that time conscious without anyone being able to notice? Or did he regain his faculties at some point during his two decade plus ordeal?

[00:12:24]Controversially, his mental state was brought into question again years later when it was revealed that Hubin used a process called facilitated communication to share his thoughts. With this method, a facilitator assists the non-verbal patient by supporting their hand as they use a keyboard or some other device.

[00:12:39]However, many organizations have criticized and discredited this technique. They've likened it to a Ouija board where the facilitator is the one doing the communication instead of the patient. But the true state of consciousness of Ron Hobin is still uncertain. Oddly enough, the first mention in the historical record of locked syndrome does not come from a medical textbook, a treatise, or a journal, but from the pages of classic literature. In a bizarre coincidence, the first description of a person with LIS is found in the counter Monte Cristo by Alexander Dumar, the same book that Jean Dominique Borby was intending to adapt before he was struck down by LIS.

[00:13:10]Of course, Dumar did not name the condition locked in syndrome in his book. That term wouldn't exist for another hundred years, but his character Mossu Noier de Wilford had been left paralyzed and mute following a stroke and could only communicate by blinking his eyes. The description of the character perfectly matches that of a person suffering from Lis. Demar wrote, "Mossure Noatier, although almost as immovable as a corpse, looked at the newcomers with a quick and intelligent expression, perceiving at once by their ceremonious courtesy that they were come on business of an unexpected and official character. Sight and hearing were the only senses remaining, and they, like two solitary sparks, remained to animate the miserable body which seemed fit for nothing but the grave. It was only, however, by means of one of these senses, that he could reveal the thoughts and feelings that still occupied his mind. And the look, by which he gave expression to his inner life, was like the distant gleam of a candle, which a traveler sees by night across some desert place, and knows that a living being dwells beyond the silence and obscurity. in his eyes, shaded by thick black lashes, was concentrated, as it often happens, with an organ which is used to the exclusion of the others, all the activity, address, force, and intelligence which were formally diffused over his whole body. And so although the movement of the arm, the sound of the voice, and the agility of the body were wanting, the speaking eye sufficed for all, his whole appearance produced on the mind the impression of a corpse without living eyes. And nothing could be more startling than to observe the expression of anger or joy, suddenly lighting up these organs, while the rest of the rigid and marble-like features were utterly deprived of the power of participation. Two decades after Alexander Dumar published his seinal work, a second description of a person with lockedin syndrome found its way into the pages of another French author.

[00:14:57]This time it was a Mill Zola in his novel Theresa Rakin. In the book, the character Madame Raken appears to suffer from lockedin syndrome described as buried alive in a dead body following a stroke but still able to communicate using eye movements. 10 years after Zola published through his reckon the first clinical case of lockedin syndrome was presented in an 1875 meeting of the society at an amik in Paris the man responsible was Camil doles at the time there's still a hospital intern under the guidance of French physician Fransis Damashino his presentation titled softening of the pawns thrombosis of the basil trunk is now regarded as the first case report of LIS the condition has been known under many different names over the last century and a half as previously mentioned And it has sometimes been referred to as a pseudo coma, but also as a deefferented state, vententral pontine syndrome, pontine disconnection syndrome, vententral brain stem syndrome, and even Monte Cristo syndrome in recognition of Alexander Dumar and his original mention of the disorder. It wasn't until 1966 that the medical term lockedin syndrome was coined by American neurologists Fred Plum and Jerome B. Pausner in seinal work on neurological disorders. They described it as quote a state in which the patient is deefferented resulting in paralysis of all four limbs and the lower cranial nerves. Although not unconscious, locked in patients are unable to respond to most stimuli. A high level of clinical suspicion is required on the part of the examiner to distinguish a lockedin patient from one who is comeomaosse. Such patients usually retain control of vertical eye movements and eyelid opening which can be used to verify their responsiveness.

[00:16:32]It is important to identify lockedin patients so that they may be treated appropriately by the medical and nursing staff at the bedside. Discussion should be with the patient, not as with an unconscious individual about the patient. Patients with large midpontene lesions often are awake most of the time with greatly diminished sleep on physiologic recordings. They may suffer greatly if they are treated by hospital staff as if they are non-responsive.

[00:16:55]Now, in order to have a chance at improving the lives of people with Loctin syndrome in the future, we need to understand the condition better. Even today, probably the most influential and comprehensive study on lockedin syndrome comes from 1986 performed by doctors James R. Patterson and Martin Graoir and published in the medical journal stroke.

[00:17:11]It reviewed 139 cases of LIS over a 25-y year period to get a better grasp on the condition. A similar but smaller survey was conducted by the Association of Lockin Syndrome of France, which collected information about 44 cases.

[00:17:23]The results were similar and provided the medical community with a decent look into a debilitating disorder that we knew very little about. Those insights are still valuable and pertinent today, 40 years later, especially since few other extensive studies on the topic have been done since then. Let's start with the causes of Loctin syndrome. By far the biggest culprit are strokes of the brain stem. Be they hemorrhagic caused by bleeding or eskeemic caused by a clot. Out of 139 cases examined by Patterson and Groir, 105 of them were brought on by vascular complications in the form of a stroke. That's around 76%, a number backed up by the French study where the percentage was even higher at 86%. A very distant second place goes to traumatic brain injuries, accounting for approximately 14% of all cases. In all but one of these cases, the patients developed lockedin syndrome after sustaining physical direct damage to the brain stem. These two types of complications are responsible for roughly 90% of LIS cases. Other causes are possible such as infections and masses albeit unlikely. When we're talking about a disorder as rare as lockedin syndrome, there are some instances where the cause has only been reported in a single case. For example, the metastasis of adenos saroma of the lung or the metastasis of a malignant melanoma. Metastasis happens when cancer cells start spreading from the primary tumor to other organs and tissues of the body. In these cases, from the lungs and the skin respectively into the brain stem. Extremely severe cases of inflammations such as menitis and enapilitis have also been documented in the leadup to lockedin syndrome. One documented case was brought on by heroin abuse while another was classified as dasopam toxicity. Undoubtedly the most unusual case was that of a patient who developed LIS after ingesting the tetrototoxin of the puffer fish.

[00:19:03]Statistics show that BTI syndrome affects men more than women, although there is some discrepancy between the studies. While in the French survey, men and women were almost 50/50. The one conducted by Patterson and Gravoir was roughly 6535 in favor of men. Given the relatively small number of cases analyzed for the studies, especially the French one, it is difficult to say with certainty that either one paints an accurate picture of the demographics of people suffering from LIS. Far more worrying is their age range, as it shows that lockedin syndrome can strike almost anyone at any time. Both studies agreed that the mean age of LIS patients at the onset of lockedin syndrome was middle ages, either late 40s or early 50s.

[00:19:41]However, in the French study conducted by ALIS, the youngest person they surveyed developed lockedin syndrome at 22 and in another case only 16.

[00:19:50]Coexisting conditions that the patients had along with the LIS were fairly common. Hypertension was the most frequent coorbidity presenting in almost a third of cases. Other conditions that were reported in multiple patients included atheroscerotic heart disease, diabetes, and atrial fibrillation.

[00:20:05]Unsurprisingly, the prognosis for a person who has developed lockedin syndrome isn't great, but it's also not the automatic death sentence that some might fear it would be. Admittedly, a full recovery would be almost miraculous. It's happened in roughly 5% of all cases. The exceedingly rare best case scenario involves something described as a transient lockedin state where the symptoms last only for a few days and the patient usually makes a full recovery within the month. But even for those patients who are trapped by lockedin syndrome for prolonged periods of time with intense early rehabilitation, a significant percentage can not only survive for years, even decades after the onset of the lockedin state, but regain motor skills to a limited degree. What exactly this percentage might be is again almost impossible to tell with any degree of certainty due to a lack of research. The study published in stroke strongly indicates that the first few months are crucial. In that survey, 83 of the total 139 people had died. That is an overall mortality rate of about 60%. But of those 83 mortalities, 52 occurred within the first month and 73 of them died within the first four months. It seems that if a patient can receive timely treatment for the underlying cause of the lockedin syndrome and proper rehabilitation care immediately after, their chances of survival go up considerably. There are of course variables that affect the survival rate.

[00:21:16]Many of them we don't yet fully understand, but the three we know have a significant impact are cause, age, and rehab. The cause for the lockin syndrome is important because patients whose condition was brought on by a vascular episode such as a stroke have a worse outlook than those whose li was caused by trauma or another outlying cause. In fact, the majority of patients who went on to recover fully from lockedin syndrome had a non-vascular issue. The age of the patient also makes a big difference. Younger people who developed lockedin syndrome had a higher rate of survival and recovery. The quality and intensity of the rehab program they underwent also improved their odds significantly. At the opposite end of the survival spectrum, the cause of death for people with LIS is usually a complication that occurs either due to the underlying cause of Loctin syndrome or the day-to-day problems of living with the condition rather than the disorder itself. By a significant margin, pneumonia is the leading cause of death reported in 26 of the 83 mortalities analyzed by Patterson and Gravoir. The frequency of pneumonia in LIS is increased by the need for long-term intubation. But other pulmonary complications such as respiratory arrest and pulmonary emblei have also been listed as relatively common causes of death. For non-respiratory issues, the extension of the brain stem lesion was the leading cause of death reported in 15 out of 83 mortalities. It typically occurred in patients who died soon after the onset of the lockedin state. It simply meant that the original damage done to the brain stem was too great to overcome and those people never really had a chance to survive. The unfortunate reality is that the vast majority of people who experience such a debilitating disorder as locked in syndrome, will never be able to return to the lives that they had enjoyed previously. On the plus side, many strides have been made in recent times, both in medical and technological terms. Remember that the study published in the stroke journal is from 1986, so all the cases presented in it are at least 40 years old. Although there's still plenty of room to improve, the world understands Loctin syndrome much better now than it did decades ago.

[00:23:03]They know how to treat it better and they know how to better remediate the lives of those who have it. Take for example the case of former English footballer Gary Parkinson who mainly played for Middlesborough and Burnley during the 80s and 90s. In 2010 he suffered a stroke at the age of 42 and was diagnosed with lockedin syndrome.

[00:23:20]Still alive today he lives at home with his family and has even worked as a scout for Middlesbrer watching footage of potential signings and rating them by blinking. Devon man Howard Wixs was left in a lockedin state following a stroke in 2011 when he was only a teenager.

[00:23:32]Since then he has not only founded the lockedin trust charity in Britain but has also written his own book on life with lis. Then there's the case of New Zealand rugby player Nick Chrysom. He suffered a brain stem stroke in 2000 and developed locked in syndrome. Since then he has regained the ability to walk assisted. He has become a father and has dedicated his life to bodybuilding winning the New Zealand wheelchair bodybuilding championship six times. He also founded the Iron Warriors to help other people with long-term injuries train and get back into shape. The inability to communicate with the outside world is one of the biggest challenges for people who have to live with this syndrome. However, new technologies enable them to get their message across a lot faster than in the past. Eyetracking software makes it easier for them to construct these messages using only their eye movements and without the need for a second person to go through the alphabet. Then, thanks to texttospech software, they can even vocalize their thoughts. South African man Martin Ptorius, who's had locked in syndrome since he was a teenager, has even given a TED talk about his condition. One new technology even allows non-verbal people to control a device by sniffing. So even patients with the most extreme form of lockedin syndrome who are incapable of moving their eyes or blinking could still find a way to communicate. On a more somber note, for years people with lockedin syndrome have been at the forefront of the right to die debate. Should they have access to assisted euthanasia if they desire it? What about those who are unable to communicate? Should someone else be allowed to decide for them? One famous example in Britain was that of Tony Nichlson who described his life with lockedin syndrome as a living nightmare and wanted to end it. His case was first heard in the high court who ruled against him. Then his appeal went all the way to the Supreme Court of the United Kingdom who dismissed it in 2014.

[00:25:08]Not that it really mattered for him anymore as Tony Nicholson had died of pneumonia in 2012. Just to end on a more positive note, it seems that situations like that of Tony Nicholson are more outliers than the standard. A more recent study conducted by ALIS in 2010 has provided us with an up-to-date picture of life with lockedin syndrome.

[00:25:25]Out of the 65 patients surveyed for the study, 70% of them had recovered some limb movement and over half of them partially regained their speech. More importantly, only 7% admitted to wanting to end their lives, whereas 72% of the participants described themselves as being happy.

[00:25:41]And on that more positive note, thank you for watching.