All about locked-in syndrome
The first recorded description of locked-in syndrome is found in an unexpected place: Alexandre Dumas’s 1846 classic The Count of Monte Cristo.
Of the fictional patient, Dumas wrote, “Sight and hearing were the only senses remaining…. 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 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.”
Other readers might be familiar with the condition from the 1996 memoir The Diving Bell and the Butterfly by Jean-Dominique Bauby, which the author wrote by blinking to indicate which letters to use after he developed locked-in syndrome following a stroke.
These portraits of patients who could not move their bodies but could use their eyes to communicate remains accurate for many cases of locked-in syndrome as we know it today, but not all of them.
Read on to find out more about what locked-in syndrome is, how it happens, and what life with this condition can look like.
What is locked-in syndrome?
Locked-in syndrome is a rare neurological disorder in which part of the brainstem is damaged, causing a disconnect between the brain and body. The result is that the person’s cognitive function remains intact, but they have near-complete paralysis. Most people will be able to hear and blink or move their eyes to communicate.
There are three main types of locked-in syndrome:
- Classic locked-in syndrome: The patient is paralyzed but remains conscious and retains their hearing and the ability to blink and move their eyes vertically.
- Incomplete locked-in syndrome: Similar to classic, except the patient will also have some small motor functions or the ability to make tiny movements.
- Total immobility locked-in syndrome: The patient is not even being able to move their eyes, but brain wave testing showing preserved cognitive function.
What causes locked-in syndrome?
The primary cause of locked-in syndrome is stroke that affects the brain stem, with one small study showing 86.4% of locked-in syndrome cases were from stroke. These strokes can be either ischemic (caused by a clot) or hemorrhagic (caused by a brain bleed.)
The next most common cause of locked-in syndrome is traumatic brain injuries to the brain stem, making up 13.6% of cases.
Other, even rarer causes include infections, masses such as tumours, or inflammation in the brain.
Learn how our team saved Sophie Leblond Robert’s life, and helped her get back, following a stroke and diagnosis of locked-in syndrome.
How common is locked-in syndrome?
Locked-in syndrome is extremely rare. While more research is needed, one study suggests it might occur once in every 339,000 people. Although numbers in Canada are hard to come by, data suggests fewer than 1,000 people in the United States have locked-in syndrome.
What is the treatment for locked-in syndrome?
The initial treatment for locked-in syndrome usually addresses the acute cause of the condition: whether that is treating the stroke, brain injury, infection, etc. Following the onset of locked-in syndrome, treatment might involve a multidisciplinary team of physicians, respiratory therapists, physiotherapists, nurses, and other allied health professionals working together to help the person regain certain skills or feel comfortable. For people who do not regain mobility, they might require ongoing supportive care.
What is the prognosis for locked-in syndrome?
The mortality rate for locked-in syndrome is relatively high at 60%. This is usually due to other issues or the issue that caused the locked-in syndrome rather than locked-in syndrome itself. Only one in 20 cases experiences a complete recovery of their motor skills.
But there is hope: people who stabilise and survive their first year have an 86% chance of surviving beyond five years. People who are younger at the time of their diagnosis and who receive immediate care have the highest rates of recovery. For people who receive early, intensive rehabilitation, it is estimated 21% will see a recovery of motor skills or movement, while 42% will recover enough to use devices to communicate.
Physiotherapist, Linda Powers
While much of the information on locked-in syndrome can sound negative or scary, one study showed that quality of life for all locked-in syndrome patients, but especially those who are able to communicate, remains high when compared to other conditions, such as Alzheimer’s disease. This promising data combined with the constantly developing care for stroke and rehabilitation means there’s hope for life with, and treatments for, locked-in syndrome.
The fictional and real cases of locked-in syndrome reflected in The Count of Monte Cristo and The Diving Bell and the Butterfly weren’t just accurate depictions of locked-in syndrome medically, but accurate depictions of how a life with the condition can still be rich and fulfilling.
