The Frontal Cortex

Locked-In Syndrome

I’m pretty sure that if Dante had known about locked-in syndrome he would have rewritten the chapter in the Inferno devoted to the ninth circle of hell. In the most recent Esquire, Joshua Foer has an excellent profile of Erik Ramsey, who suffered a devastating injury to his brain stem, leaving him entirely paralyzed. (The only muscles Erik could consciously control were the ones that moved his eyeballs up and down.)

There are stories of people being locked-in for years before anyone notices the fully conscious person hiding inside the paralyzed body. In 1966, a thirty-two-year-old woman named Julia Tavalaro became locked-in after a brain hemorrhage and was sent to Goldwater Memorial Hospital on Roosevelt Island, New York, where the staff took to calling her “the vegetable”. It wasn’t until six years later that a family member noticed Tavalaro trying to smile after she heard a dirty joke. She was immediately taught to communicate with eye blinks and became a poet and author. She died in 2003 at the age of sixty-eight, having never spoke for thirty seven years.

Comments

  1. #1 Theo
    October 6, 2008

    Odd, I just watched The Butterfly and the Diving Bell this past weekend, and now this post pops up.

    “The true story of Elle editor Jean-Dominique Bauby who suffers a stroke and has to live with an almost totally paralyzed body; only his left eye isn’t paralyzed.”

    He has locked-in syndrome. Highly recommended movie.

  2. #2 Anibal
    October 6, 2008

    Your remark about Dante is, unfortunately, very certain.
    I hope that our neuroscientists investigating human consciousness from a clinical perspective, and not only theoretical, could one day reverse all disorders of consciousness.

  3. #3 Matt Springer
    October 6, 2008

    “He has locked-in syndrome. Highly recommended movie.”

    He had locked-in syndrome. Unfortunately he died shortly after the completion of his book. It was truly amazing that he wrote a book like that, and it’s sad that he didn’t get to see its success.

  4. #4 Celeste
    October 6, 2008

    The Butterfly and the Diving Bell is an incredible book and movie, though I’m glad I watched the film first. The cinematography really captures the terrifying claustrophobia of locked-in-syndrome.

    I’m curious, though, why some/all voluntary eye muscle control is still preserved in many locked-in-patients?

  5. #5 Anibal
    October 6, 2008

    Im not a neurologist but reading the work of some of the big names in the study of disorders of conciousness (Steven Laureys, Adrian Owen, J. Giacino, N. Hirsch…) is due to some lesion in the corticobulbar and corticospinal pathways that leave intact the occulomotor nerves controling eye-lids movements, but neverthless the condition produces quadriplegia.

  6. #6 jb
    October 6, 2008

    Looking in my big fat medical book I see that this eye movement phenomenon also happens with ALS (Lou Gehrig’s Disease); patients gradually lose all voluntary muscle control as well as the ability to breathe and eat. Sensory nerves and mental function are unaffected. They can “end up on a respirator unable to communicate except with eye movements”. A friend of mine came to such an end. I don’t recall that her grown children or husband were actively using her eye movements as a way of communicating at the end; I never learned to do that with her and am feeling remiss now. I would visit, meditate with her, and read the 23rd Psalm. She was a devout Christian.

    At her funeral, in talking to her daughters I learned that 2 of the 3 had a dream about her the night she died, in which she told them that there was money to pay for her funeral in her savings account, something they had not known
    and something they were grateful to learn as the family did not have much money.

  7. #7 Tony Jeremiah
    October 6, 2008

    (The only muscles Erik could consciously control were the ones that moved his eyeballs up and down.)

    Stephen Hawking has a specially designed wheelchair that allows him to communicate with his eyes. They are his only remaining functioning body part as a consequence of amyolateral sclerosis.

    I’m curious, though, why some/all voluntary eye muscle control is still preserved in many locked-in-patients?

    That’s a good question. This would be a guess on my part, but it might be related to an inherent property of the nervous system.

    When we go to bed and enter REM sleep, there’s a biological mechanism that shuts down voluntary motor brain regions, whose purpose is believed to prevent us from acting out our dreams. In some people, this mechanism fails during sleep and shows up as sleepwalking. When it (temporarily) fails upon waking or just going to sleep, it shows up as a common (and scary) condition called sleep paralysis, whereby people report being unable to move even though they are conscious (with eyes open).

    When the mechanism is working as it should, it is interesting that the only voluntary muscles not deactivated are the ones attached to the eyes; which explains the rapid eye movements (REM) that occur when we dream. So I’m guessing there’s some connection between that and the observation that eye movements are still possible with locked-in-syndrome.

  8. #8 Rachel
    October 16, 2008

    My father used a computer program called Brainfingers as his method of communication when he had bulbar ALS. By lifting his eyebrows or clenching his jaw, he was able to click and spell out words with the text prediction system. Beyond that, he was able to email, and create websites and Power Point presentations for his job as the school system’s technology supervisor. Had he not been able to lift his eyebrows, Brainfingers could also read alpha and beta brainwaves. He could have used a heightened state of emotion to then control his words.

    The power of communication and his ability to work is why my father outlasted his 18-24 month diagnosis and lived for 5 years.
    I can’t imagine the frustration of someone like Erik Ramsey. And I can only hope that this emerging science will give him a voice once more.

    On a side note: Besides The Diving Bell and the Butterfly, one of the more powerful stories about losing the power to communicate is from Seth Carey, a man living with ALS, on a 2004 edition of NPR’s Weekend Edition. To think if he’d never been able to write this:

    http://www.npr.org/templates/story/story.php?storyId=3926143

    (I also find it surreal that I now have to have an emotional attachment to a computer voice like Seth’s.)

  9. #9 güzel sözler
    February 28, 2009

    I�m not a neurologist but reading the work of some of the big names in the study of disorders of conciousness (Steven Laureys, Adrian Owen, J. Giacino, N. Hirsch…) is due to some lesion in the corticobulbar and corticospinal pathways that leave intact the occulomotor nerves controling eye-lids movements, but neverthless the condition produces quadriplegia.

  10. #10 francesca
    December 1, 2009

    locked-in

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