Reaching Down the Rabbit Hole: Extraordinary Journeys into the Human Brain

by Allan H. Ropper

I noticed a small white ball, a mere eighth of an inch in diameter, which seemed to be pressing on the bottom of the spinal cord. (Most people don’t understand that the spinal cord doesn’t go all the way down the spinal column, but stops in the upper back.) I said, “What’s that?” Joelle replied, “It’s just an artifact,” by which she meant a by-product of the imaging process, like a dead bug in the lens. “I don’t think so. That looks real. I think it’s an epidural abscess.” Only now, at 3:30, did it occur to us that Harry Connaway’s

“Your patient, Mr. Connaway, has a spinal epidural abscess. It’s massive.”

cutting off its blood supply. His spinal cord was slowly being strangled. This explained all of his symptoms.

“I don’t care. It might kill him, but if we miss this we miss our only shot.”

“I’ll go down with him. We have to get this scan because I need to tell a surgeon what he has, what its extent is, and where he needs to operate. He might die down there, but we have no other choice.”

at risk of damaging his spinal cord irrevocably.

you’ve got to be able to make the calls and pull the right strings.

Part of my job is to impress upon the residents

Unlike the study of, say, mathematics or physics, where it matters little to the high-level practitioner how Newton “discovered” the calculus or his theory of gravity, the practice of medicine benefits from revisiting the discoveries of the past. For a physician, seeing further means looking over the shoulders of giants.

William Osler, a Canadian physician born in 1849, who is considered by many to be the father of modern medicine.

understanding how disease affects the nervous system, was Raymond Adams, who trained me at Mass General.

He was right, and is still right. To be a truly advanced neurologist, one could argue, you have to know about the sophisticated genetics of neurological disease and the cell biology of neurological disease and the immunology of neurological disease. That is the case for the full-fledged professional.

what will turn them into doctors instead of automatons.

A subdural is short for a subdural hematoma, a brain hemorrhage typical of traumatic brain injuries, caused in this case when the skull hit the sidewalk and the brain caromed off the inside of the skull, in the process tearing veins that run across its surface.

Here, I thought, was the poster boy for the third part of that talk.

Marty prepares all of his talks, no matter the audience, as though he were addressing the annual meeting of the American Academy of Neurology,

Imagine two scenarios. In one, the viewer, perhaps yourself, sees a dog being hit by a car, being thrown thirty feet to the side of the road, where it lies motionless, probably dead. In the other, you watch a fly land on a countertop, where it is swatted and effectively crushed. Most definitely dead. Why do we agonize over the dog but not the fly? The philosopher says, “Because the dog is conscious and the fly is not.” How do we know? Because the dog has eyes that look at you. The secret to consciousness is in the eyes. If the creature has humanoid eyes and it looks at you, then it is conscious. And if it doesn’t, it isn’t. The compound eyes of the fly do not convey any feeling to us of consciousness. Same with the worm. Philosophers and neurologists have come to the same conclusion with regard to consciousness, and that is that the secret to consciousness is in the eyes. If you understand the eyes, you will understand a lot about consciousness. In fact if you know a lot about the eyes—in great detail—then you’re practically a neurologist. This is not chance.

Remember, an overdose of barbiturates will make you look absolutely dead—no pupils, no eye movement, no vestibular ocular reflex, no calorics, no EEG, nothing—until you crawl out of the grave. So until you know with reasonable certainty that there are no drugs, that there has not been any severe hypothermia, and the criteria are fulfilled, don’t pronounce anyone dead.

But Mike was not dead yet, not legally, not biologically, not until we said he was. He was still warm, still breathing (with assistance), still digesting his last meal.

The cranial nerves are key because they come out of the brain stem segmentally, and the big question is: is the brain stem involved? That’s why we begin with the eyes. The eyes are the secret to unconsciousness. We can’t test smell in these patients because that requires cooperation. Remember that smelling salts do not test smell; they test pain, the fifth cranial nerve. You can test everything else, but do you? You don’t have to. The eyes include cranial nerves 2, 3, 4, and 6, so you can go from 2 to 6 just by looking at the eyes. Whether the person gags or swallows when you move the tube depends on the level of anesthetic and many other functions.

If you get a perfect score on the brain-death examination—five yeses—you can officially pronounce the patient as dead as Jacob Marley: as dead as a doornail.

We don’t call it “brain death.” We call it “death by brain criteria.” To use the term “brain dead” confuses the public because they ask the question: “If it’s only brain death, what is alive? Are the kidneys alive?” I don’t use the term alive with regard to the kidneys or the skin. The person is in the brain, and virtually everybody in every culture agrees with that. It’s death, just like death by cardiac criteria. Our job is to make sure that people don’t abuse it.

“Our primary purpose is to define irreversible coma as a new criterion of death.”

Brigham in 1954, when Nobel Prize winner Joseph Murray performed the first successful kidney transplant.

The nagging issue is whether a warm, pink, pulsating, live-looking body can or should be called dead. All of the organs are viable. The body could go through the onset of puberty, it could gestate an infant. There are such cases on record. What the Beecher Committee accomplished was to find a good reason not to utilize resources on people who would unquestionably die without ever regaining consciousness. Being able to change their classification and call them dead had virtue for society. They said, in effect, “It’s not living if your brain is irrevocably gone; it’s not living, so you can go ahead and take the organs.” They had a clear mandate to protect the physician. They recommended, for example, that the patient be declared dead before the respirator is disconnected, so as to avoid the appearance of pulling the plug on a living person.

In a moment of great clarity, the Catholic Church signed on to the idea that brain death is death. In 2006, I served on the panel of the Pontifical Academy of Sciences at the Vatican that produced a monograph entitled: “The Signs of Death.”

hair and nails continue to grow after death.

I once had a patient, a member of Hell’s Angels, who was shot in the face while driving his Harley down the interstate at eighty miles per hour—with a shotgun, no less—who then went off the road and creamed his entire cranium (no helmet), and was quite obviously brain dead. Some of his brain matter was left on the road. They might as well have decapitated him, except that when he was placed on a ventilator—intubated—his heart still pumped and the body was kept alive. Here was this outlaw, a tough guy, maybe a sociopath, and ironically, it said “organ donor” on his driver’s license. In the end, he turned out to be a humanitarian. There was no question of transplanting his corneas. They were no longer there, and that was the problem: How do you know a person like that is brain dead without the all-disclosing eyes? We couldn’t examine his pupils. We couldn’t examine his eye movements or his corneal reflexes. I recall thinking, Oh my God, you don’t want to get a diagnosis of brain death wrong because it would be like committing an innocent man to the electric chair. How am I going to finesse this? We could show that he had apnea (that he couldn’t breathe on his own), but that’s just one of the five elements of the tests for death by brain criteria. The Presidential Commission said that in certain circumstances you can use surrogate tests. So we did an EEG. He had barely enough scalp on which to place the leads, and the readout was flat, or, in tech-speak, isoelectric. We did a...

a reflex hammer, the neurologist’s favorite weapon. Women neurologists, I have noticed, tend to press harder than men, as if to insure that no one is getting out alive. In this instance there was not a whit of movement. All but a fully paralyzed, comatose patient would exhibit a straightening of the arms and pushing backward as the shoulders rotate internally. But here: nothing, no cerebral response.

we did the caloric reflex: squirting ice water into one ear, then the other. This provides a potent stimulus to eye movements through a hardwired circuit in the brainstem, if it’s functioning at all. “Nada.” Now the moment of truth, the apnea test. Will he breathe? “How do you like to do it, Dr. Ropper?” “Preoxygenate him.” The person must have an apnea test. Then you can prove to yourself that the whole brain, including the brainstem, is gone. Just remember, when you take a patient off a ventilator, either for an apnea test or after a declaration of death, make sure that family members are out of sight, and forewarn the nurses. Many brain dead patients, once the ventilator is removed, exhibit the so-called Lazarus sign, where their arms spontaneously contract and their hands come up to their chest as though they’re grasping for the endotracheal tube. It’s creepy no matter how many times you’ve seen it.

Silence. I could hear my pocket watch ticking.

Alan Shewmon, a respected neurologist and professor at UCLA Medical Center, has the audacity to claim that brain death is not death. He didn’t always believe this, but his worldview changed about a decade ago when he was presented with a patient, a fourteen-year-old boy, who suffered a severe head trauma after jumping onto the hood of a slow-moving car, falling off, hitting his head on the curb, and eventually being confirmed as brain dead. Yet he “lived” for another sixty-three days on a respirator and vital fluids. Dr. Shewmon was called in to examine the boy, and he agreed with the brain death determination. By the standards of the State of California, the boy was a corpse, but at the family’s insistence, the body was kept going in a facility under the care of nurses who were baffled and unsettled by that diagnosis. Shewmon reported that the boy began to go through puberty and eventually died, not from his head injury, but of pneumonia. How long can a body survive in a brain-dead state? Not indefinitely, but longer than most people think.

he is a scientist doing what scientists are supposed to do: question the status quo. Alan Shewmon claims, rightly, that there are three concepts of death: a biological one that speaks of the organism, a psychological one that speaks of the person, and a sociological one that speaks of the legal person.

“Is it? If he’s dead, in what sense is he dead?” “In the dead sense,” Trey replied. “Well, his brain may be dead, but his other organs are alive. They can be transplanted.” “But they’re just organs. Organs can be sustained, even grown outside of a body, independent of a body.” “The gash on his neck where the transplant surgeon cuts out a lymph node would heal.” “Those are just cells,” Trey countered. “They’re on automatic pilot. You provide them with blood, they keep going, but there’s nothing meaningful going on.”

a brain-dead patient will come to the same conclusion, just as we had: this entire brain will never recover, and all the king’s horses and men can’t do a damn thing about it.

translating what they tell him into usable form,

That’s how a patient frames a problem in his own vernacular.

Too much Mirapex, so I lowered his dose.”

It can be a very intense chess game—speed chess—because on rounds, we have to act fast. The value we add is the intellectual act of making those moves, and the clarity with which we can do it.

complications following the surgery to remove his meningioma. It was worth trying, because the growing tumor would have further clouded his mind.

personality that warped the sensibilities of their acolytes. The ethos served a social good,

supplanted personality.

Part of my role today is regrettably to lead the next generation of neurologists through the transition from the old school to a new one, to turn them into interchangeable parts in the new health-care system. Yet I want them all to be best in show. I want them to live the vita medicalis, and get their neurology from their patients’ stories, not from books and Web sites.

insufferable circumlocution, the lifestyle bordering on self-negligence, the elitism tucked behind a mask of benevolence, but also a dedication to science and service verging on the messianic.

I said, “Did you flex his neck?” They said, “We don’t do that in the MRI scanner. That’s not the protocol.” “You don’t get it,” I said. “The disease is defined that way.” I bring the new scans up on my monitor to show Elliott. This is one of those cases where the right picture really is worth a thousand words, and the wrong picture is worth one. I show Elliott what the MRI looked like when they had him flex his neck. The ligament buckles, it bunches, it causes venous congestion, and it pinches the spinal cord. “Every time the kid leaned forward he compressed his spinal cord, and he’s a plumber. What it’s done over a year or two is damage the anterior horn cells in the cord, and caused what looks like ALS, but most definitely is not.”

It’s not like being a butcher, a baker, or a candlestick maker. We’re trying to understand how the brain works so that we can fix it.

Instead of theories, we need clinical truths: What’s wrong with this person’s brain? Can we get it back to normal or some semblance of normal? Can we at least get them on the right track? When we can’t, the question turns grim: How will I walk this patient and this family through this all the way to dementia or death? Because the job doesn’t end with the diagnosis.

We all invent notions to reassure us and lies to protect us. Elliott calls these distancing techniques. His are largely cinematic.

telling the patient, “and Death has told us your time has come.”

contemplative time afforded a trial lawyer: time to scrutinize and adjudicate every detail, time to follow clearly enumerated rules of evidence, time to adjourn if necessary.