When the Air Hits Your Brain: Tales from Neurosurgery

by Vertosick, Frank, Jr.

  The history-taking on the neurology service was equally fastidious. One afternoon, after taking a history from a man with headaches, I was grilled by the hospital’s chief neurologist on teaching rounds.

  “What does Mr. Hughes do for a living, Doctor?” the kindly professor asked.

  “He says he works in a shop.” I replied. At home, that would have been the end of the discussion on that topic.

  “What sort of shop?”

  “I don’t know, some sort of small store, maybe.”

  “Well, what sort of shop might make a difference, don’t you think? If he works in a paint store all day mixing paint with hydrocarbon thinners, might not that be a cause of his headaches?”

  “Well, yes,” I admitted.

  “Does he own the shop?”

  “I don’t know”

  The professor removed his glasses and began cleaning them slowly, squinting up at the high ceiling as he continued his dissertation. “These facts matter a great deal. What a patient does for a living, what his background is, what level of education he has achieved…all of these issues must be addressed in great detail in order to put his complaints and his disease in the proper context. If I ask a man to take the square root of 100 and he cannot, I might take this as proof of a left-hemispheric brain tumor, unless I know that he has worked on a farm since childhood and never attended school. Likewise, I might find it normal that a patient could not tell me the current exchange rate of the pound in Japanese yen. But if I knew that person was a merchant banker, on the other hand, ignorance of this fact would indicate a grave illness indeed! Americans have grown so dependent upon their scanning toys that they fail to view the patient as a multidimensional person. To have the audacity to cut into a person’s brain without the slightest clue of his life, his occupation…I find that most simply appalling.”

  These words came back to me years later in the States. I was operating on a woman for a spinal tumor when a medical student in the room casually asked me how old the patient was. I couldn’t remember! Here I was, staring at the woman’s spinal cord, and I didn’t even know how old she was! The professor was right; it was most simply appalling. I was treating an MRI image on a piece of photographic film, not a person. I’ve tried hard not to repeat that arrogance again.

  Multiple sclerosis, or MS, is more common in the U.K. than it is in the United States, and we saw a great deal of it during my three months in London. The disease affects the insulating fat, or myelin, around nerve fibers, punching holes (called plaques) into the deep white matter of the brain and spinal cord. The severity of the disease depends upon where the plaques occur. A large plaque in a tolerant area of the brain, such as the right frontal lobe, may be asymptomatic, while a minuscule plaque in a critical area of the spinal cord or brain stem may leave the patient wheelchair-bound.

  MS is capricious, striking quickly and then retreating in a series of random exacerbations scattered over a lifetime. The patient may be disabled for months, then recover and be unaffected for years before another episode occurs. At the time I was doing my neurology rotation, there was no accepted treatment save for high-dose steroids and physical therapy. (Other therapies are now available, such as interferon.) For most patients, fortunately, the tincture of time was the best cure.

  The most dramatic MS patient I ever saw was Andrew, a Nigerian foreign exchange student at the University of London. He was admitted with his very first attack of the illness. The hospital’s newly installed MRI scanner (dedicated by the Prince of Wales himself!) disclosed a half-inch plaque dead-center in his midbrain. The midbrain sits atop the brain stem, where it serves as a conduit for the output of the hindbrain cerebellum, the main balance and coordination computer of the brain. This output had been effectively amputated by the plaque, leaving Andrew with no cerebellar function at all.

  The cerebellum, or “little cerebrum,” fine-tunes the gross motor signals emanating from the upper brain. Its output is purely inhibitory: the neurons in the cerebellum serve only to dampen or inhibit the activity of other neurons in the brain. My neuroanatomy professor in medical school put this into perspective by observing that Michelangelo had carved the statue of David using only “subtraction” of stone. Thus, the big cerebrum’s motor outflow is like raw marble which the cerebellum deftly chisels into coordinated movements.

  As long as Andrew kept very still, he was fine. However, the moment he tried to do even the slightest task, even to scratch his nose, his arms and legs became uncontrollable, flailing about like octopus tentacles. He had to be fed, since he could not be trusted with a fork or knife. His face was swollen from attempts to brush his teeth, attempts which resulted only in a self-inflicted pummeling. He could walk short distances, but his gait was wild. More often than not, he ended up draped over the ward’s central radiator, crying from frustration.

  This uncoordinated movement, called ataxia, even involved Andrew’s speech. Although the melodic Nigerian inflections of his perfect English were still detectable, the words came out herky-jerky, with the tone monotonous and the sentences incorrectly parsed, a pattern known as cerebellar scanning speech. Scanning speech has a hollow, mechanical quality, like the diction of robots and computers in science fiction films. Like the stare of the acute schizophrenic, scanning speech is difficult to describe in words, but once you have experienced it you remember it forever.

  As we sat drinking our afternoon coffee (we still called it “tea time,” although few registrars actually drank tea), William, David, and I watched Andrew as he ricocheted around the ward, walking into beds and walls and bouncing away in a different direction like a mechanical toy.

  “Poor bastard,” remarked David.

  “Not to worry,” observed William, “he’ll get better. They all do from the first episode, no matter how bloody awful they look. In six or eight months he’ll be back at school again. The question is how to manage him until this plaque remits. The physical therapy people have fitted him for a lead jacket.”

  “A lead jacket?” I asked.

  William continued. “Yes, a lead jacket. It’s a suit coat that’s been fitted with lead bars to make the arms very heavy. If Andrew has to work harder to move his arms and gets more proprioceptive feedback from them, he can control his movements a little better. Quit beating himself up all of the time. It’s quite lowtech, you know, but it works.”

  “The main drawback,” added David, “is they get dreadfully tired, as you can well imagine. Ah, but just think! If he wears it for a few months, he may end up looking like the Incredible Hulk!”

  The jacket was delivered the following day. It was of heavy green cloth and looked identical to the protective “burner’s jackets” worn by the acetylene torch operators in the steel mill. Numerous slots were sewn along the arms. Along with the jacket came a carton of thin lead bars to be placed into the slots, allowing us to titrate the added weight so that we could give Andrew some motor control without rendering him unable to lift his arms at all.

  We spent the morning experimenting with the jacket, until finally Andrew was able to lift his arm, slowly and agonizingly, and run a comb through his short hair. His mouth blossomed into a broad African grin.

  “Ah, this is very much more to my liking!” he said in his robot voice, beaming at his regained arm control.

  He could only wear the jacket a few hours at a time, and by the end of each day his arms ached so much that he had to take narcotics to sleep. But he stopped crying the day he got the jacket. As William predicted, Andrew’s speech slowly returned to normal, his drunken gait began to sober, and his need for the jacket diminished weekly. Two months after he came to us a limp jellyfish of a man, the Nigerian strode out the hospital door, erect and controlled, the only remnant of his disease the increased power in his well-exercised arms. He shook our hands briskly.

  “You know, I am a philosophy student,” Andrew observed just as he exited, flexing his biceps in a bodybuilder’s pose. “Nietzsche said ‘Whatever doesn’t kill me make
s me stronger.’ I now know this as fact. Thank you, my friends.”

  He would be back, no doubt—in a year, maybe ten, depending upon the mercy of his personal disease. Had we, as Hippocrates postulated, simply “entertained” Andrew until he got better on his own? Perhaps, but we had managed to stop his tears, and any therapy which accomplishes that feat for a suffering patient is damned good medicine.

  The U.K. is hardly a classless society. Nevertheless, I was amazed at the degree to which nationality, race, and socioeconomic status factored into and at times hindered the clinical reasoning of the registrars and attending neurologists.

  London has a very large number of people from the Indian subcontinent, a vestige of the days of the empire. Whenever recent immigrants from India or Pakistan showed up at our doorstep, they were immediately given the diagnosis of tuberculosis, regardless of their symptoms. A middle-aged Pakistani grocer, for example, came to the casualty department (British for ER) with a three-month history of progressive weakness and stiffness in his legs. An MRI scan revealed a mass within the center of his thoracic spinal cord, between the shoulder blades. I recognized it as an astrocytoma, a tumor arising from the spinal tissues. To my chagrin, the attending surgeon, Mr. Royston, diagnosed it as a tuberculoma, a swelling caused by the tuberculosis bacterium, even though the patient had a negative chest X ray and no other evidence or history of tuberculosis. The patient’s nationality was cited as the sole reason for this bizarre conclusion.

  I went to the library the next day and could unearth only two previous cases of a solitary tuberculous mass in the spinal cord, and both of those reported cases had documented pulmonary TB. When I confronted the surgeon with this fact, he shrugged and pointed to the good clinical response the patient had made to the antituberculous antibiotics as further proof of the correctness of the original diagnosis. The patient truly was better, but I argued that the improvement could be from the steroid medication that was being administered as well. I made no headway. Three weeks later, the patient returned to the casualty department in a state of near-paraplegia and emergency surgery was performed, at which time his astrocytoma was removed and he made an uneventful recovery.

  In another incident, we were gathered to hear a patient demonstration by one of the most senior and respected neurologists in all of southern England. Donning our white jackets, I and the other trainees crammed into the archaic demonstration hall, sitting in ascending concentric circles around the small exam table below. A registrar brought in the patient, a scrawny laborer in his fifth decade. The patient staggered to the table and sat down, but proceeded to bob and sway even while seated. The stately neurologist conducted a detailed interview with the man, listening keenly to his complaints: unsteady gait, dizziness, and nausea. He conducted a very cursory exam and then had the patient escorted from the lecture hall. The neurologist turned to us, paused for a dramatic effect, and asked, “What single piece of information you have heard this morning tells you what is wrong with this man?”

  “His age,” answered a registrar.

  “No, that’s not correct.”

  “His occupation?” I volunteered.

  “No, wrong once again.”

  This continued for a few minutes, until the exasperated teacher told us his expected answer: “His nationality! His name is O’Brien and he’s from Belfast. Since he’s obviously of the Irish lower classes, it’s a very good bet that he’s a sot. He’s clearly afflicted with alcoholic cerebellar degeneration.”

  In fact, this diagnosis was correct, but the bluntness of this deduction would not have been tolerated in America. The neurologist never even asked the patient if he drank; he simply assumed it to be true. I couldn’t see one of our, neurologists standing before an open forum of medical students and announcing that a patient must be a user of crack cocaine simply because he was black and from an urban area.

  “I can’t see!” The woman spoke in a measured tone, trying to conceal her panic in proper British fashion.

  “Keep calm and tell us what’s been going on,” William reassured her.

  The patient, a pretty young woman still wearing her street clothes, arrived on the neurology ward just as we were returning from lunch at a local pub. The casualty department, where she had gone seeking help for progressive blindness, had sent her directly to our service.

  “I was fine this morning when I woke up, but after breakfast I began having a headache, right here.” She motioned to the top of her head. “My vision began to blur, but I could still see well enough to get here. Now I can only see dark forms moving about, and things are getting darker all the time!”

  William turned to David and me, speaking in low tones. “David, notify the CT scan facility that we will need an immediate pituitary study. Frank, go to the Jefferson ward and find Mr. Cunningham and bring him here at once. Quickly now.” Mr. Cunningham was a senior attending surgeon and the hospital’s pituitary specialist.

  I dashed off to the Jefferson ward and found Mr. Cunningham in his office; we were back on the neurology ward within minutes. The young woman, Alice Weathers, recounted her story to the surgeon. Her health was good, although her menstrual periods had become irregular during the preceding year and she had noticed a slight discharge from her nipples recently. Unmarried, she typed for a living and lived alone in the Tottenham Court area.

  Mr. Cunningham examined her vision. She could make out his large hand waving in front of her face, but she could not count his fingers or tell the color of his necktie. The surgeon’s elegant face turned dour.

  “Young lady,” he began, “I believe that a tumor has hemorrhaged within your pituitary gland, that small bit of tissue located behind your eyes. We call this hemorrhaging ‘pituitary apoplexy.’ The blood clot is pushing on the optic nerves, the nerves that connect the eyes to the brain. I will have to operate immediately to remove the clot and tumor or you will remain blind.”

  “Tumor? I have cancer?”

  “No, no, my dear young lady. The tumor is almost certainly a benign adenoma, a growth quite common in young women. In your case, the tumor is making a hormone called prolactin. Excess prolactin hormone will produce exactly the symptoms you have noted—the irregular menstruation, the slight nipple discharge. That discharge is milk. Prolactin is a hormone normally made during pregnancy. It stimulates the fatty breast tissue to manufacture milk.”

  “Do what you have to do, just don’t let me go blind. Please!”

  Mr. Cunningham examined the patient’s breasts and confirmed the milk secretion. “Have her taken to the operating theaters straightaway and cancel the scan. There is little doubt what needs to be done here. I will scan her after I have decompressed the optic nerves. Be sure to administer a large dose of hydrocortisone, won’t you? I will have my senior registrar lend a hand.”

  I followed the patient to the holding area outside the operating suite. She wept quietly into a handful of tissues.

  “Is there anyone I can call to be with you?” I asked.

  “My dad died two years ago and my mum’s got a bad heart. I’d rather not tell her anything right now.”

  I couldn’t begin to imagine her despair as she faced this, crisis alone. After several minutes of waiting, an OR nurse whisked her into the room and an anesthesiologist ended her suffering with a whiff of gas.

  Little more than fifty minutes had passed since she had arrived in the casualty department. British medicine at its finest. Although mired in red tape, the U.K. system avoids the legal wrangling that hamstrings American medicine. In the United States, taking a woman to the OR without objective studies, such as MRI or CT scans, invites malpractice action should anything go wrong. True, Gary had cut Billy’s spine without a myelogram, but such fortitude is rare. Back home, anesthesia would balk at doing her so soon after she had eaten breakfast. And the operating rooms would have been overbooked. In London, the mighty Mr. Cunningham spoke and things happened.

  Mr. Cunningham and his registrar positioned Alice in the head clamp, th
en scrubbed her mouth and nose with soap. The pituitary, an embryonic relic of the nasal passages, lies buried in the hard palate, just above the uvula. The neurosurgeon approaches the gland through the nose, with the aid of a microscope and some truly long instruments. To enter the hypophysis (the correct name for the gland), the surgeon traverses the sphenoid sinus; hence the operation’s tongue-twisting name—transsphenoidal hypophysectomy.

  First used by Cushing in the early twentieth century, the transsphenoidal procedure is arguably the most bizarre operation in the neurosurgeon’s repertoire. To enter the nose, the surgeon cuts under the upper lip and peels the face up and away from the “nares,” the openings of the nasal passages. Cracking the nasal septum to one side, the surgeon advances a large speculum to the base of the skull, the sphenoid sinus is removed, and the floor of the pituitary chamber is chiseled away.

  Cushing had little luck with this approach and abandoned it, preferring to take the tumors out through the head rather down through the nose. Superb a technician though he was, Cushing lacked a microscope, and the fiber-optic lighting, needed to do the operation safely. Jules Hardy and his colleagues in Canada resurrected the procedure in the 1960s, with superb results, and the nasal approach soon became standard. Hardy’s use of the microscope and intraoperative X rays overcame Cushing’s difficulties.

  Mr. Cunningham deftly dissected the pretty woman’s face and exposed her pituitary gland in less than an hour. The gland looked blue and taut on the video screen. With a swift poke of his pointed microblade, thick clot and purulent yellow tumor gushed from the gland and into Mr. Cunningham’s thin suction tip. He spent several minutes rummaging around with small currettes, until satisfied that all tumor and blood clot were evacuated. The task of reassembling the face fell to the registrar.

  Alice’s broad smile in the recovery room told more about her eyes than any formal vision test could do. A few hours after surgery, her vision returned to normal. The nasal packing was removed on the third postoperative day and she went home on the fifth—pretty as ever. Her mother never learned of Alice’s brief flirtation with disaster.