by Oliver Sacks
1. It had been found that if one made small lesions in certain areas (by injecting alcohol or freezing), such lesions, far from harming patients, could break a circuit which had become hyperactive and was responsible for many symptoms of parkinsonism. Such stereotactic surgery almost ceased to be done with the coming of L-dopa in 1967 but is now enjoying a new life with the implanting of electrodes and the use of deep brain stimulation in other parts of the brain.
2. It was at Mount Zion that Libet performed his astounding experiments showing that if subjects were asked to make a fist or perform another voluntary action, their brains would register a “decision” nearly half a second before there was any conscious decision to act. While his subjects felt that they had consciously and of their own free will made a movement, their brains had made a decision, seemingly, long before they did.
3. I was interested to see that when Thom’s Collected Poems came out in 1994, this was the one poem from The Sense of Movement that he chose not to reprint.
4. The intention was genuine, but more than fifty years have passed, and I am still not a citizen. It was similar with my brother in Australia. He arrived there in 1950 but only got Australian citizenship fifty years later.
Muscle Beach
When I finally made it to New York in June of 1961, I borrowed money from a cousin and bought a new bike, a BMW R60—the trustiest of all the BMW models. I wanted no more to do with used bikes, like the R69 which some idiot or criminal had fitted with the wrong pistons, the pistons that had seized up in Alabama.
I spent a few days in New York, and then the open road beckoned me. I covered thousands of miles in my slow, erratic return to California. The roads were wonderfully empty, and going across South Dakota and Wyoming, I would scarcely see another soul for hours. The silence of the bike, the effortlessness of riding, lent a magical, dreamlike quality to my motion.
There is a direct union of oneself with a motorcycle, for it is so geared to one’s proprioception, one’s movements and postures, that it responds almost like part of one’s own body. Bike and rider become a single, indivisible entity; it is very much like riding a horse. A car cannot become part of one in quite the same way.
I arrived back in San Francisco at the end of June, just in time to exchange my bike leathers for the white coat of an intern in Mount Zion Hospital.
During my long road trip, with snatched meals here and there, I had lost weight, but I had also worked out when possible at gyms, so I was in trim shape, under two hundred pounds, when I showed off my new bike and my new body in New York in June. But when I returned to San Francisco, I decided to “bulk up” (as weight lifters say) and have a go at a weightlifting record, one which I thought might be just within my reach. Putting on weight was particularly easy to do at Mount Zion, because its coffee shop offered double cheeseburgers and huge milk shakes, and these were free to residents and interns. Rationing myself to five double cheeseburgers and half a dozen milkshakes per evening and training hard, I bulked up swiftly, moving from the mid-heavy category (up to 198 pounds) to the heavy (up to 240 pounds) to the superheavy (no limit). I told my parents about this—as I told them almost everything—and they were a bit disturbed, which surprised me, because my father was no lightweight and weighed around 250 himself.1
I had done some weight lifting as a medical student in London in the 1950s. I belonged to a Jewish sports club, the Maccabi, and we would have power-lifting contests with other sports clubs, the three competition lifts being the curl, the bench press, and the squat, or deep knee bend.
Very different from these were the three Olympic lifts—the press, the snatch, and the clean and jerk—and here we had world-class lifters in our little gym. One of them, Ben Helfgott, had captained the British weight-lifting team in the 1956 Olympic Games. He became a good friend (and even now, in his eighties, he is still extraordinarily strong and agile).2 I tried the Olympic lifts, but I was too clumsy. My snatches, in particular, were dangerous to those around me, and I was told in no uncertain terms to get off the Olympic lifting platform and go back to power lifting.
Besides the Maccabi, I trained occasionally at the Central YMCA in London, which had a weight-lifting gallery supervised by Ken McDonald, who had lifted for Australia in the Olympic Games. Ken carried a good deal of weight himself, especially in his lower half; he had enormous thighs and was a world-class squatter. I admired his squatting ability, and I too wanted to develop such thighs and to develop the back power crucial for squatting and overhead lifts. Ken favored stiff-legged deadlifting—a lift designed, if any lift is, to injure the back, for the entire load is focused on the lumbar spine and not taken, as it should be, by the legs. As I improved under his tutelage, Ken invited me to join him in a weight-lifting exhibition—the two of us alternating our dead lifts. Ken deadlifted 700 pounds. I could only manage 525, but this was applauded, and I felt a brief pleasure and pride that despite being a novice, I had been able to companion Ken on his record-breaking dead lift. My pleasure was very short-lived, for a few days later I developed a pain in my lower back so intense that I could hardly move or breathe; I wondered if I had fractured a vertebra. Nothing amiss was seen on X-ray, and the pain and spasms resolved in a couple of days, but I was to have excruciating back attacks for the next forty years (they only let up, for some reason, when I was sixty-five, or were then, perhaps, “replaced” by sciatica).
My admiration of Ken’s training schedule extended to the special, largely liquid diet he had designed in order to bulk up. He would come to his workouts with a half-gallon bottle filled with a thick, treacly mixture of molasses and milk supplemented by assorted vitamins and yeast. I decided to do the same, but I overlooked the fact that yeast ferments sugar, given enough time. When I pulled my bottle out of my gym bag, it was bulging ominously. It was clear that the yeast had fermented the mixture; I had put it in hours beforehand, while Ken (as he told me later) sprinkled it in just before he came to the gym. The contents of the bottle were under pressure, and I felt a little frightened; it was as if I had suddenly found myself in possession of a bomb. I thought that if I unscrewed the top very slowly, there could be a gentle decompression, but as soon as I loosened the top a little, it blew; the whole half gallon of sticky black (and now slightly alcoholic) muck went up like a geyser high into the air, landing all over the gym. There was laughter at first and then rage, and I was sternly warned never again to bring anything but water to the gym.
—
The Central YMCA in San Francisco had particularly good weight-lifting facilities. The first time I went there, my eye was caught by a bench-press bar loaded with nearly 400 pounds. No one at the Maccabi could bench-press anything like this, and when I looked around, I saw no one in the Y who looked up to such a weight. No one, at least, until a short but hugely broad and thick-chested man, a white-haired gorilla, hobbled into the gym—he was slightly bowlegged—lay down on the bench, and, by way of warm-up, did a dozen easy reps with the bench-press bar. He added weights for subsequent sets, going to nearly 500 pounds. I had a Polaroid camera with me and took a picture as he rested between sets. I got talking to him later; he was very genial. He told me that his name was Karl Norberg, that he was Swedish, that he had worked all his life as a longshoreman, and that he was now seventy years old. His phenomenal strength had come to him naturally; his only exercise had been hefting boxes and barrels at the docks, often one on each shoulder, boxes and barrels which no “normal” person could even lift off the ground.
I felt inspired by Karl and determined to lift greater pound-ages myself, to work on the one lift I was already fairly good at—the squat. Training intensively, even obsessively, at a small gym in San Rafael, I worked up to doing five sets of five reps with 555 pounds every fifth day. The symmetry of this pleased me but caused amusement at the gym—“Sacks and his fives.” I didn’t realize how exceptional this was until another lifter encouraged me to have a go at the California squat record. I did so, diffidently, and to my delight was able to
set a new record, a squat with a 600-pound bar on my shoulders. This was to serve as my introduction to the power-lifting world; a weight-lifting record is equivalent, in these circles, to publishing a scientific paper or a book in academia.
—
By the spring of 1962, my internship at Mount Zion was coming to an end, and my residency at UCLA was due to begin on July 1. But I needed time to visit London before the residency started. I had not seen my parents for two years, and my mother had just broken a hip, so I was very glad to be with her soon after her operation. Ma dealt with the trauma, the surgery, and the ensuing weeks of pain and rehabilitation with great fortitude and was determined to get back to seeing her own patients as soon as she was off crutches.
Our winding staircase, with worn carpet and sometimes loose stair rods, was not safe for someone on crutches, so I carried her up and down as she needed—she had been against my heavy lifting, but now she was glad of my strength—and I delayed my return until she could manage the stairs herself.3
—
At UCLA, we residents had a weekly “Journal Club”; we would read the latest papers in neurology and discuss them. I sometimes annoyed the group, I think, by saying that we should also discuss the writings of our nineteenth-century forebears, relating what we were seeing in patients to their observations and thoughts. This was seen by the others as archaism; we were short of time, and we had better things to do than consider such “obsolete” matters. This attitude was reflected, implicitly, in many of the journal articles we read; they made little reference to anything more than five years old. It was as if neurology had no history.
I found this dismaying, for I think in narrative and historical terms. As a chemistry-mad boy, I devoured books on the history of chemistry, the evolution of its ideas, and the lives of my favorite chemists. Chemistry had, for me, a historical and human dimension too.
It was similar when my interests moved from chemistry to biology. Here, of course, my central passion was for Darwin, and I read not only the Origin and the Descent, not only The Voyage of the Beagle, but all his botanical books, too, with Coral Reefs and Earthworms thrown in. I loved his autobiography most of all.
Eric Korn had a similar passion and eventually gave up a career in academic zoology to become an antiquarian bookseller specializing in Darwiniana and nineteenth-century science. (He was consulted by booksellers and scholars the world over for his unique knowledge of Darwin and his times, and he was a good friend of Stephen Jay Gould’s. Eric was even asked—no one else could have done it—to reconstruct Darwin’s own library in Down House.)
I was not a book collector myself, and when I bought books or articles, it was to read them, not to show them. So Eric reserved his torn or damaged books for me, books which might lack a cover or a title page—books no collector would want but which I could afford to buy. As my interests moved to neurology, it was Eric who got me Gowers’s 1888 Manual, Charcot’s Lectures, and a host of lesser-known but to me beautiful and inspiring nineteenth-century texts. Many of these became central to the books I was later to write.
—
I was fascinated by one of the first patients I saw at UCLA. It is not uncommon to have a sudden myoclonic jerk as one is falling asleep, but this young woman had a much more severe myoclonus and would react to flickering light of a certain frequency with sudden convulsive jerks of her body or, occasionally, with full-blown seizures. These problems had been in her family for five generations. With my colleagues Chris Herrmann and Mary Jane Aguilar, I wrote my first paper (for the journal Neurology) about her, and, fascinated by myoclonic jerks and the many conditions and circumstances in which these could occur, I wrote a small book about them all, which I titled “Myoclonus.”
When, in 1963, Charles Luttrell, a neurologist noted for his outstanding work on myoclonus, visited UCLA, I told him of my own interest in the subject and said I would be most grateful for his opinion on my little book. He was agreeable, and I gave him the manuscript; I did not have a copy. A week passed, and another, and another, and at six weeks I could contain my impatience no longer and wrote to Dr. Luttrell. I learned that he had died. I was shocked. I wrote a letter of condolence to Mrs. Luttrell, in which I spoke of my admiration for her husband’s work, but I thought it would be unseemly, at this juncture, to ask for the return of my manuscript. I never asked, and the manuscript was never returned. I do not know whether it is still in existence; it was probably tossed out, but perhaps it still resides quietly in some forgotten drawer.
—
In 1964, I saw a puzzling young man, Frank C., in the neurology clinic at UCLA. He had incessant jerking movements of his head and limbs which had started when he was nineteen, getting gradually worse over the years; his sleep, more recently, had been disturbed by massive jerks of his entire body. He had tried tranquilizers, but nothing seemed to help the jerks, and Frank, depressed, had started drinking heavily. His father, he said, had had identical movements starting in his early twenties, had become depressed and alcoholic, and had finally committed suicide at the age of thirty-seven. Frank himself was thirty-seven now, and he said he could imagine exactly how his father felt; he feared that he might take the same step himself.
He had been in the hospital six months earlier, where various diagnoses were considered—Huntington’s chorea, postencephalitic parkinsonism, Wilson’s disease, etc.—but none of these could be confirmed. So Frank and his strange disease presented an enigma. I gazed at his head at one point, thinking, “What’s going on inside there? I wish I could see your brain.”
Half an hour after Frank had left the clinic, a nurse rushed in and said, “Dr. Sacks, your patient has just been killed—hit by a truck—he died instantly.” An immediate autopsy was performed, and two hours later I had Frank’s brain in my hands. I felt awful—and guilty. Could my wish to see his brain have played a part in his fatal accident? I could not help wondering, too, whether he had decided to end things and stepped deliberately in front of the truck.
His brain was of normal size and showed no gross abnormalities, but when I got some slides under the microscope a few days later, I was startled to see gross swelling and tortuosity of nerve axons, pale spherical masses, and rusty brown pigmentation from iron deposits in the substantia nigra, the pallidum, and the subthalamic nuclei—all parts of the brain that regulate movement—and nowhere else.
I had never before seen such huge swellings confined to the nerve axons or bits of detached axon; these did not occur in Huntington’s disease or any other disease I had encountered. But I had seen pictures of such axonal swellings, photographs illustrating a very rare disease described by two German pathologists, Hallervorden and Spatz, in 1922—a disease that started in youth with abnormal movements but then, as it advanced, caused widespread neurological symptoms, dementia, and finally death. Hallervorden and Spatz observed this fatal disease in five sisters. At autopsy, their brains contained axonal swellings and lumps of detached axon, as well as brownish discolorations in the pallidum and substantia nigra.
It looked, then, as if Frank might have had Hallervorden-Spatz disease and that his tragic death had allowed us to see the neural basis of this at a very early stage.
If I was right, we had a case which exemplified, better than any previously described, the initial and fundamental changes in Hallervorden-Spatz disease, uncontaminated by all the secondary features seen in more advanced cases. I was intrigued by the strangeness of a pathology which seemed aimed only at the nerve axons, leaving the bodies of the nerve cells and the myelin sheaths of the axons untouched.
Just the year before, I had seen a paper by David Cowen and Edwin Olmstead, neuropathologists at Columbia University, describing a primary axonal disease in infants: here the disease might present itself as early as the second year and was usually fatal by the age of seven. But in contrast to Hallervorden-Spatz disease, where the axonal abnormalities were confined to small but crucial areas, in infantile neuroaxonal dystrophy (as Cowen and Olmstead called it),
there was widespread distribution of axonal swellings and fragments.
I wondered whether there were any animal models of axonal dystrophy, and here, by chance, I found that two researchers in our own neuropathology department were working on precisely this.4 One of them, Stirling Carpenter, was working with rats maintained on a diet deficient in vitamin E; these pathetic rats lost control of their hind limbs and tails because sensation from these was blocked by axonal damage in the sensory tracts of the spinal cord, and their nuclei in the brain—a distribution of axonal damage completely unlike that seen in Hallervorden-Spatz disease but perhaps casting some light on the pathogenic mechanism involved.
Another colleague at UCLA, Anthony Verity, was working on an acute neurological syndrome that could be produced in laboratory animals by giving them a toxic nitrogen compound—iminodipropionitrile (IDPN).5 Mice given this developed a wild excitement, incessantly turning in circles or running backwards, accompanied by involuntary jerking movements, bulging eyes, and priapism; they, too, had gross axonal changes, but these were in the arousal systems of the brain.
The term “waltzing mice” was sometimes used of such driven and incessantly active mice, but this decorous term gives no picture of the overwhelming severity of the syndrome. The usual quiet of the neuropathology department was punctuated by shrill cries and squeaks from the overexcited mice. While the IDPN-toxic mice were so different from the vitamin-E-deficient rats dragging their spread-eagled hind limbs behind them, and so different from the human conditions of Hallervorden-Spatz disease and infantile neuroaxonal dystrophy, they all seemed to share a common pathology: severe damage confined to the axons of nerve cells.
Could one get any insight from the fact that human and animal syndromes so different were generated, seemingly, by the same sort of axonal dystrophy, albeit in different regions of the nervous system?