Cincinnati, 3/8/2010

The following article on Proprioception Deficit Disorder appeared in a recent issue of Utne Reader.
 
 
Medicine
Coming Apart at the Seams A Teacher with PDD What Is Proprioception
Premonitory Symptoms Onset Symptoms The Search for a Cure
 

 


Sacks


Wittgenstein

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
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


Rheingold

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

PREMONITORY  SYMPTOMS
Up to six months prior to onset, victims may experience one or more of the following: 1) Foreboding dreams of the loss of physical control; 2) Decreasing physical coordination—a growing inability to walk or even to stand upright; a feeling of being "all thumbs" in the manipulation of objects; general clumsiness; 3) Sporadic "phantom limb" delusions— the conviction that an appendage does not "belong"; 4) Time distortion—the delusion that all events take place as if in slow motion; 5) A sudden fear of mirrors—sufferers often find themselves inexplicably terrified at the sight of their own reflected image; 6) Increasing "spaciness" and absent-mindedness— sometimes mistaken as symptoms of Alzheimer's disease; 7) A general sense of unreality—a mysterious doubt about the "actuality" of everyday objects and even people (victims often complain of a change in spatial orientation that makes everything further away, physically and psychologically more distant).
ONSET SYMPTOMS
Those who suffer from the syndrome in its full-blown form may continue to experience any or all the above (often in more pronounced manifestations) and, in addition, may succumb to the following: 1) total, or near total, loss of proprioception; an inability to perform even the most ordinary tasks without conscious surveillance; 2) graphic distortion in body image—comparable to that undergone by victims of anorexia nervosa; 3) the complete or near-complete loss of color vision (victims complain that the world begins to appear as if in a black and white photograph); 4) a multiplicity of strange "tics," habitual, uncontrollable, and inexplicable random movements—nose twitching, eye-blinking, jerks of the head, shoulders, and hands, and inappropriate noises (sniffs, whistles, screams, hissing, clicking, barks, and other inarticulate noises)—usually brought on by spasmodic muscular movements; 5) frigidity and impotence.
THE SEARCH FOR A CURE
Thus far, Proprioception Deficit Disorder has baffled medical scientists. Initially reluctant (in the early of '90s) to recognize it as a consistent, diagnosable ailment (it was often dismissed as "merely psychosomatic"), researchers quickly discovered that 1) it is neither contagious nor hereditary; 2) there is no known medical cure or even recommended treatment (sufferers ordinarily seek psychiatric help soon after the onset of their symptoms and require the regular assistance of a physical therapist); 3) though initially incapacitating, victims are able in half the cases to make partial recoveries, substituting, like Christina (see main story), conscious monitoring of bodily capacities for natural proprioceptive control. 

Although PDD remains a mysterious (idiopathic) illness, epidemiologists have nevertheless established some of its demographic parameters: a) victims are as likely to be male as female; b) the disorder can strike at any time in the life-cycle beginning with adolescence, though victims tend to be in their 40s; c) the more education an individual has, the more likely he or she is to be afflicted; d) 95% of all identifiable cases have been in the Northern Hemisphere.
 

Utne Reader January/February 2010
 
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