Exploring Locked-In Syndrome

I had heard of locked-in-syndrome but I remember the occasion when I first viscerally felt locked-in-syndrome. Marcus Reichle, a brilliant neuroscientist at Washington University of St Louis, had come to give a seminar at University of Chicago. As his host, I took him out to dinner that night. He told me the story of Jean-Dominique Bauby who blinked out The Diving Bell and the Butterfly with one eyelid. I was mesmerized by the story. I immediately bought Mr Bauby’s book, read it, and re-read it. I was struck by the paradoxical juxtaposition of nearly complete loss of function with extraordinary purpose. The dignity and resilience that Mr Bauby showed in the face of hardship and tragedy filled me with awe.

The facts of locked-in syndrome forcefully express the full magnificence of the nervous system: the bodily devastation occasioned by its failure, and the soaring heights of its untouched cerebrum. There is no better way to introduce neurobiology than by telling the story of being locked in. Recently I used Mr Bauby’s story to introduce my Coursera MOOC, Understanding the Brain: The Neurobiology of Everyday Life. I was subsequently alerted to Patrick Stein’s experience with locked-in syndrome by a MOOC student. I watched the allinmyhead.com video several times. From my perspective as a neuroscientist, I immediately noticed that Mr Stein retained some facial and eye movements that Mr Bauby did not retain after his stroke. To get the students thinking about neuroanatomy, I asked them to “identify the similarities and the differences in the two cases“. Little did I know…

I was expecting students to write about facial expressions, eye movements, swallowing and the like. And of course some students did do exactly that. But MOOC students quickly turned the conversation into a deeper, more philosophical discussion of the role of attitude and determination in recovery. In response to one student’s suggestion that Mr Bauby was most interested in a life of the mind and was not trying to move, another student (MB) reminded all that “determination is only one factor in how much a person will recover.”  Undeniably, this is true. Determination cannot stave off every illness. In the case of locked-in-syndrome, the pathophysiology dictates severe disability and holds most of the proverbial cards. Recovery may occur at the margins but regaining past abilities in toto is unlikely.

Holding individual determination as the sole indication of paramount humanity inappropriately homogenizes diverse experiences. As MB pointed out, determination to go-go-go is counterproductive in situations when the most recuperative activity possible is rest-rest-rest. In fact, built into the nervous system are both go-go-go (sympathetic) and rest-rest-rest (parasympathetic) systems. One is not better than the other; they are simply different and either is most appropriate in different situations. In my view, accepting that rest is a way forward or that death approaches is just as “strong” an attitude as fighting to power through illness and stave off death’s arrival.

More philosophically, the idea that individual determination is an attitude fit for all denies us our essential individuality. Hamlet’s soliloquy:

…Whether ’tis nobler in the mind to suffer

The slings and arrows of outrageous fortune,

Or to take arms against a sea of troubles…

can be viewed as an articulation of the dilemma between a suffering acceptance and an armed struggle against misfortune such as illness. I am no literary expert but I think that Shakespeare’s point was to pose the question rather than answer it. Ranking our perceptions of peoples’ chosen attitudes toward adversity is energy that would be better spent on extending compassion to all persons who struggle against misfortune. Fighting and accepting ill health are different approaches. They are not better or worse ways of being. Both Jean-Dominique Bauby and Patrick Stein faced an incredible challenge when they were locked into their brain and both deserve our compassion, support, and respect for their journeys.

I want to close by extolling those who stay engaged with loved ones who are locked in. In Bauby’s case, there was a strong support network of friends and family. From the video available it appears that Mr Stein’s young friends, as well as his family, have remained close. I stand in awe of all of these people who have chosen to stay open, loving, and fully engaged in the face of tremendous sadness.

Guest Contributor: Peggy Mason

Peggy Mason graduated from Harvard with a BA in Biology in 1983. She received her PhD in Neuroscience from Harvard in 1987. After postdoctoral work at the University of California – San Francisco, she joined the faculty at the University of Chicago in 1992. Dr Mason is now Professor of Neurobiology. Dr Mason is committed to teaching neurobiology to anyone that will listen. She has taught medical students since her arrival at UChicago, with the exception of a 2 year hiatus during which she wrote a textbook, Medical Neurobiology (Oxford University Press, 2011). Dr Mason is now offering a massively open on-line course, Understanding the Brain: The Neurobiology of Everyday Life through Coursera (https://www.coursera.org/course/neurobio). Dr Mason’s research is focused on the neurobiology of empathy and pro-social behavior in rats.