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Cincinnati, 3/8/2010 The following
article on Proprioception Deficit Disorder appeared in a recent issue of
Utne
Reader.
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COMING
APART AT THE SEAMS
In "The Disembodied Lady," one of the case-histories that make up The Man Who Mistook His Wife for a Hat, and Other Clinical Tales (1985), neurologist Oliver Sacks tells the story of a patient named Christina. Prior to the affliction which brought her to see Dr. Sacks in 1977, Christina was a healthy, intelligent woman of twenty seven. Admitted to a hospital for a routine operation (removal of a gall bladder), she found herself bothered by anxiety- producing pre-operation dreams in which she foresaw herself losing all control of bodily functions. After the operation, she discovered her dreams coming true before her very eyes. At first she found herself unable to walk. Then she became unable to hold things in her hands. Then she could not even stand. Her hands began to wander about of their own accord "unless she kept an eye on them." She lost nearly all coordination of body movements. Sacks describes the end result: "She could scarcely even sit up--her body 'gave way.' Her face was oddly expressionless and slack, her jaw fell open, even her vocal posture was gone." "Something awful's happened," Christina tried to explain in what Sacks described as "a ghostly flat voice." "I can't feel my body. I feel weird --disembodied." What happened, doctors were quick to conclude, was that Christina had lost her "proprioception" (see box story). She was suffering from what has only recently been named Proprioception Deficit Disorder (PDD), or Sacks Syndrome. At first, her doctors were ready to blame Christina's strange symptoms on that old bugaboo hysteria. Then, after running a battery of tests, they sought a physiological explanation: an acute "polyneuertis affecting the sensory roots of spinal and cranial nerves throughout the neuraxis" was suspected. More philosophically inclined than his colleagues (he begins his essay by discussing Wittgenstein on the question of doubting the reality of our bodies), Sacks was not content to accept a merely physical explanation. He wrote of the need to understand Christina's disease as "a genuine phenomenon," in which her state-of-body and state-of-mind are not fictions but a psychophysical whole. Christina, too, began to philosophize about her condition, as Sacks reported. Undergoing therapy, she showed real insight into its its cause: "This 'proprioception' is like the eyes of the body, the way the body sees itself. And if it goes, as it's gone with me, it's like the body's blind. My body can't "see" itself if it's lost its eyes, right? So I have to watch it--be its eyes. Right?" Gradually, Christina learned to control her movements with "almost painful conscientiousness and care." Though at first she seemed (Sacks notes) as floppy as a ragdoll," she managed to become a fairly successful self-engineered automaton. Her brain's body-image gradually gained conscious control over her intuitive "proprioceptive body-model." But such adaptations, Sacks observes, "made life possible--they did not make it normal." Her recovery, Sacks comments, represented a success "in operating, but not in being." Gradually, Christina did become her body's eyes. With "every move made by artifice," she learned to function by keeping watch on her own substance. She continued to feel her body to be dead and, consequently, to be dumbfounded by what Sacks calls her "bereftness, [a] sensory darkness (or silence) akin to blindness or deafness." "Her de-afferentiation," Sacks concludes, "has deprived her of her existential, epistemic basis--and nothing she can do, or think, will alter this fact." Later, Christina, watching home movies of herself before her disembodiment, movies of a happier time, when she moved and acted normally, was overcome by her sense of loss, as Sacks poignantly records. "I can't identify with that graceful girl any more! She's gone, I can't remember her, I can't even imagine her. It's like something's been scooped right out of me, right at the centre. . . that's what they do with frogs, isn't it? They scoop out the centre, the spinal cord, they pith them . . . That's what I am, pithed, like a frog . . . Step up, come and see Chris, the first pithed human being. She's no sense of herself--disembodied Chris, the pithed girl." Christina's
"case" began in 1977; since 1985 (the year Sacks' case study was completed),
her condition has not changed appreciably. Sacks' ends his essay with the
observation--true at the time--that Christina's life was "unprecedented":
"She is, so far as I know, the first of her kind, the first 'disembodied'
human being." In an alarming "Postscript" (alarming in retrospect at least,
for if The Man Who Mistook His Wife for a Hat were a novel, his
words would have to be considered-- given current developments--as rather
heavy-handed "foreshadowing"), Sacks went on to note that "large numbers
of patients are turning up everywhere now with severe sensory neuronopathies,"
brought on, he believed, by healthfaddist overdoses of Vitamin B6. He speculates
that there might be, worldwide, "some hundreds of 'disembodied' men and
women. . . ." Now, almost fifteen years later, an estimated one hundred
thousand worldwide suffer from the syndrome named after its first chronicler.
Utne
Reader January/February
2010
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| A
TEACHER WITH PDD
Before his nightmarish dreams began, Anthony Kirk taught first grade at Campus Elementary School at the University of Memphis in Memphis, Tennessee. A much loved and highly respected veteran educator--he was honored as "Elementary School Teacher of the Year" in the Disney--sponsored National Teacher Awards, he is also the father of three boys. In a series of recurring dreams a year ago, he found himself in school on a typical day, but inexplicably unable to move. He could not tie a shoe, open a stubborn milk carton, erase a blackboard, snap his fingers. Despite his students' disturbed entreaties, he could not even get up from his desk. Within a week, his dreams had become real. Within a month his disability had made it impossible to continue teaching. Granted a leave of absence, he remained at home, where his normal duties as a father had likewise become impossible. Various attempts at drug therapy failed to produce results. Numerous psychiatric sessions were no more successful. Three months after the first onset of symptoms, Anthony's color vision had disappeared. Like Christina (see main story), he gradually learned to manipulate himself by careful, hyper-self-conscious attentiveness to each task, but he came to find every excursion beyond the walls of his home a source of great anxiety and was incorrectly diagnosed for a time as agoraphobic. Unable to return to the classroom he loved, he is now in semi-retirement--a housebound invalid. |
Sherrington |
WHAT
IS PROPRIOCEPTION?
The term "proprioception" was coined by British biologist Charles Sherrington in the early years of this century. For Sherrington, the word named something already known to each of us, though largely ignored. He thought of it as "our secret sense, our sixth sense"--as the ability to "feel our bodies as proper to us, as our property, as our own." Neurologist and author Dr. Oliver Sacks provides a somewhat more technical but nevertheless lucid definition: "the continuous sensory flow from the movable parts of our body muscles, tendons, joints), by which their position and tone and motion is continually monitored and adjusted but in away which is hidden from us because it is automatic and unconscious. "Each human joint and muscle is monitored by internal sensors (proprioceptors) capable of registering even minute changes of position and pressure and passing on messages concerning the state of the organism back to the brain's processing system, which discerns in an almost infinite number of combinations meaningful patterns. As science writer Howard Rheingold explains: "This pattern of messages from this particular set of sensors means that your body is going to topple forward if you don't do something about it; that pattern of messages means that you are pushing something heavy and polished across a low-friction surface." The organism cannot continue to interact with its environment --without proprioception's unconscious tuning, its silent partnership. Without it, we don't have a leg to stand on, a hand to give. January/February 2010 Utne Reader |