Musicophilia: Tales of Music and the Brain (30 page)

Gowers spoke of the susceptibility of pianists and violinists to their own “occupation neuroses” other provocative occupations included “those of painters, harpists, artificial flower makers, turners, watchmakers, knitters, engravers…masons…compositors, enamellers, cigarette makers, shoemakers, milkers, money counters…and zither players”— a veritable tally of Victorian occupations.

Gowers did not see these task-specific problems as benign: “The disease, when well developed, is one in which the prognosis is always uncertain, and often unfavourable.” Interestingly, at a time when such symptoms were either ascribed to peripheral problems with muscles, tendons, or nerves or seen as hysterical or “mental,” Gowers did not feel satisfied with either explanation (though he allowed that these factors could play a subsidiary role). He insisted, rather, that these occupational “neuroses” had an origin in the brain.

One reason for this thought was the fact that, though different parts of the body could be afflicted, all the provocative occupations involved rapid, repetitive movements of small muscles. Another was the conjunction of inhibitory features such as unresponsiveness or “paralysis” with excitatory ones— abnormal movements or spasms, which increased the more one fought against the inhibition. These considerations disposed Gowers to see “occupation neuroses” as disorders of motor control in the brain, disorders he thought might involve the motor cortex (the functions of the basal ganglia were unknown at this time).

Once “occupation neuroses” developed, there was little chance of continuing in the same occupation or profession. But despite the mysterious nature and crippling consequences of this condition, remarkably little attention was paid to it, medically, for almost a century.

Though it was well known in the world of performing musicians that this dread condition could lie in wait for anyone— perhaps one in a hundred musicians would be affected, at some point in their career— a natural reserve, even secrecy, prevailed. To acknowledge an occupation-related cramp was close to professional suicide— it would be understood that one would have to give up performing and become a teacher, a conductor, perhaps a composer, instead.
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Not until the 1980s was this veil of secrecy finally torn away, with great courage, by two virtuoso pianists, Gary Graffman and Leon Fleisher. Their stories were remarkably similar. Fleisher, like Graffman, had been a child prodigy and one of the preeminent pianists in the world from his teenage years. In 1963, at the age of thirty-six, he found the fourth and fifth fingers of his right hand starting to curl under his hand when he played. Fleisher fought against this and continued to play, but the more he fought, the worse the spasm became. A year later, he was forced to give up performing. In 1981, in an interview with Jennifer Dunning in the
New York Times,
Fleisher gave a precise and graphic description of the problems that had put a stop to his performance, including the years of misdiagnosis and sometimes mistreatment he had received. Not the least of his problems, when seeking treatment, was not being believed, for his symptoms came on only with piano playing, and very few doctors had a piano in their office.

Fleisher’s public acknowledgment of his condition came soon after Graffman acknowledged his own problem in 1981, and this spurred other musicians to admit that they, too, had been having similar problems. It also stimulated the first medical and scientific attention to the problem in almost a century.

In 1982, David Marsden, a pioneer investigator of movement disorders, suggested that writer’s cramp was an expression of disordered function in the basal ganglia— and that the disorder was akin to dystonia.
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(The term “dystonia” had long been used for certain twisting and posturing spasms of the muscles such as torticollis. It is typical of dystonias, as of parkinsonism, that the reciprocal balance between agonistic and antagonistic muscles is lost, and instead of working together as they should— one set relaxing as the others contract— they contract together, producing a clench or spasm.)

Marsden’s suggestion was taken up by other researchers, most notably Hunter Fry and Mark Hallett at the National Institutes of Health, who launched an intensive investigation of task-specific focal dystonias such as writer’s cramp and musician’s dystonia. But rather than thinking of these in purely motor terms, they wondered, too, whether rapid, repetitive movements might cause a sensory overload which could then cascade into a dystonia.
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At the same time, Frank Wilson, who had long been fascinated by the speed and skill of pianists’ hands and the “dystonic” mishaps that could befall them, found himself thinking in general terms of the sort of control systems which would have to underlie the repeated, “automatic” performance of very fast, intricate sequences of small, precise finger movements, with the activity of agonist and antagonist muscles in perfect reciprocal balance. Such a system, involving the coordination of many brain structures (sensory and motor cortex, thalamic nuclei, basal ganglia, cerebellum), would be operating, he argued, at or close to its functional capacity. “The musician in full flight,” he wrote in 1988, “is an operational miracle, but a miracle with peculiar and sometimes unpredictable vulnerabilities.”

By the 1990s, the tools had become available for a minute exploration of this question, and the first surprise, given that focal dystonia seemed to be a motor problem, was to find that cortical disturbances in the
sensory
system were, indeed, of crucial importance. Hallett’s group found that the mapping of dystonic hands in the sensory cortex was disorganized both functionally and anatomically. These changes in mapping were greatest for the fingers which were most affected. With the onset of dystonia, the sensory representations of the affected fingers started to enlarge excessively, and then to overlap and fuse, to “de-differentiate.” This led to a deterioration in sensory discrimination and a potential loss of control— which the performer, usually, would fight against by practicing and concentrating more, or by playing with more force. A vicious cycle would develop, abnormal sensory input and abnormal motor output exacerbating each other.

Other researchers found changes in the basal ganglia (which, with the sensory and motor cortex, form an essential circuit for the control of movement). Were these changes caused by the dystonia or were they in fact primary, disposing certain susceptible individuals to the problem? The fact that the sensorimotor cortex in dystonic patients also showed changes on the “normal” side suggested that these changes were indeed primary, and that there was probably a genetic predisposition to dystonia, which might become apparent only after years of rapid, repetitive movements in adjacent muscle groups.

In addition to genetic vulnerabilities, there may be, as Wilson has pointed out, significant biomechanical considerations: the shape of a pianist’s hands and the way he holds them, for example, might play a part in determining whether or not, after years of intensive practice and performance, he gets a dystonia.
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The fact that similar cortical abnormalities can be experimentally induced in monkeys has allowed Michael Merzenich and his colleagues in San Francisco to explore an animal model of focal dystonia, and to demonstrate the abnormal feedbacks in the sensory loop and the motor misfirings that, once started, grow relentlessly worse.
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Could the cortical plasticity that allows focal dystonia to develop also be used to reverse it? Victor Candia and his colleagues in Germany have used sensory retraining to redifferentiate the degraded finger representations. Though the investment of time and effort is considerable and success is not certain, they have shown that, in some cases at least, this sensorimotor “retuning” can restore relative normality of finger movement and its representation in the cortex.

A sort of perverse learning is involved in the genesis of focal dystonia, and once the mappings in the sensory cortex have gone wrong, a massive act of unlearning is needed if a healthier relearning is to occur. And unlearning, as all teachers and trainers know, is very difficult, sometimes impossible.

A
N ENTIRELY DIFFERENT
approach was introduced in the late 1980s. One form of botulinum toxin, which in large doses causes paralysis, had been used in tiny doses to control various conditions in which muscles are so tense, or in such spasm, that they can hardly be moved. Mark Hallett and his group were pioneers in the experimental use of Botox to treat musician’s dystonia, and they found that small, carefully placed injections might allow a level of muscular relaxation that did not trigger the chaotic feedback, the aberrant motor programs, of focal dystonia. Such injections— though not always effective— have enabled some musicians to resume playing their instruments.

Botox does not remove the underlying neural and perhaps genetic disposition to dystonia, and it may be unwise or provocative to attempt a return to performance. This was the case, for example, with Glen Estrin, a gifted French horn player who developed an embouchure dystonia affecting the muscles of the lower face, jaw, and tongue. Though dystonias of the hand usually occur only in the particular act of making music (this is why it is called “task-specific”), dystonias in the lower face and jaw may be different. Steven Frucht and his colleagues, in a pioneer study of twenty-six professional brass and woodwind players affected with this type of dystonia, observed that in more than a quarter of them, the dystonia spread to other activities. This happened with Estrin, who developed disabling mouth movements not only when playing the horn, but when eating or speaking, severely disabling him in daily life.

Estrin has been treated with Botox but has stopped playing, given the danger of recurrence and the disabling nature of his symptoms. Instead, he has turned his attention to working with Musicians with Dystonia, a group that he and Frucht founded in 2000 to publicize the condition and help musicians who are struggling with it. A few years ago, musicians like Fleisher and Graffman, or the Italian violinist who wrote to me in 1997, might go for years without a proper diagnosis or treatment, but now the situation has been transformed. Neurologists are much more aware of musician’s dystonia, as are musicians themselves.

R
ECENTLY LEON FLEISHER
came to visit me a few days before he was to give a performance at Carnegie Hall. He spoke of how his own dystonia had first hit him: “I remember the piece that brought it on,” he began, and described how he had been practicing the Schubert
Wanderer
Fantasy for eight or ninehours a day. Then he had to take an enforced rest— he had a small accident to his right thumb and could not play for a few days. It was on his return to the keyboard after this that he noticed the fourth and fifth fingers of that hand starting to curl under. His reaction to this, he said, was to work through it, as athletes are often told to “work through” the pain. But “pianists,” he said, “should not work through pain or other symptoms. I warn other musicians about this. I warn them to treat themselves as athletes of the small muscles. They make extraordinary demands on the small muscles of their hands and fingers.”

In 1963, however, when the problem first arose, Fleisher had no one to advise him, no idea what was happening to his hand. He forced himself to work harder, and more and more effort was needed as other muscles were brought into play. But the more he exerted himself, the worse it became, until finally, after a year, he gave up the struggle. “When the gods go after you,” he said, “they really know where to strike.”

He had a period of deep depression and despair, feeling his career as a performer was over. But he had always loved teaching, and now he turned to conducting as well. In the 1970s, he made a discovery— in retrospect, he is surprised he did not make it earlier. Paul Wittgenstein, the dazzlingly gifted (and immensely wealthy) Viennese pianist who had lost his right arm in the Great War, had commissioned the great composers of the world— Prokofiev, Hindemith, Ravel, Strauss, Korngold, Britten, and others— to write piano solos and concertos for the left hand. And this was the treasure trove that Fleisher discovered, one that enabled him to resume his career as a performing artist— but now, like Wittgenstein and Graffman, as a one-handed pianist.

Playing only with the left hand at first seemed to Fleisher a great loss, a narrowing of possibilities, but gradually he came to feel that he had been “on automatic,” following a brilliant but (in a sense) one-directional course. “You play your concerts, you play with orchestras, you make your records…that’s it, until you have a heart attack on stage and die.” But now he started to feel that his loss could be “a growth experience.”

“Suddenly I realized that the most important thing in my life was not playing with two hands, it was
music.
…In order to be able to make it across these last thirty or forty years, I’ve had to somehow de-emphasize the number of hands or the number of fingers and go back to the concept of music as music. The instrumentation becomes unimportant, and it’s the substance and content that take over.”

And yet, throughout those decades, he never fully accepted that his one-handedness was irrevocable. “The way it came upon me,” he thought, “might be the way it would leave me.” Every morning for thirty-odd years, he tested his hand, always hoping.

Though Fleisher had met Mark Hallett and tried Botox treatments in the late 1980s, it seemed that he needed an additional mode of treatment, in the form of Rolfing to soften up the dystonic muscles in his arm and hand— a hand so clenched that he could not open it and an arm “as hard as petrified wood.” The combination of Rolfing and Botox was a breakthrough for him, and he was able to give a two-hand performance with the Cleveland Orchestra in 1996 and a solo recital at Carnegie Hall in 2003. His first two-handed recording in forty years was entitled, simply,
Two Hands.

Botox treatments do not always work; the dose must be minutely calibrated or it will weaken the muscles too much, and it must be repeated every few months. But Fleisher has been one of the lucky ones, and gently, humbly, gratefully, cautiously, he has returned to playing with two hands— though never forgetting for a moment that, as he puts it, “once a dystonic, always a dystonic.”