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

by Allan H. Ropper

But shortly after a visit to a chiropractor, she had suffered a vertebral artery dissection, a form of stroke. Chiropractic neck adjustments are not a common cause of stroke (maybe one in every twenty thousand treatments produces one), but the high rotary force involved, one with just the right vector and amplitude, can strip off the inner layer of a blood vessel, causing it to tear and collapse into the channel, impeding the flow of arterial blood to the brain.

There is an old joke among stand-up comics that goes: “Dying is easy, comedy is hard.”

Vincent’s form of speech difficulty, known as Wernicke’s aphasia, sounds like gibberish, but not pure nonsense. It can include halting phrases that almost make sense, echolalia (repeating someone else’s just-used words), perseveration (giving the same answer to a succession of different questions), and play association (cracking wise). While he knew the answers to many of our questions, most of his responses didn’t come out quite right, yet he seemed unaware and unconcerned.

The phrase A and O times three means “awake, oriented to self, oriented to place, and oriented to time.” Some people add a fourth: oriented to situation. The problem is that everybody is “oriented times one” unless they are hysterical or dead.

A teratoma is an unusual tumor that contains cells from the brain, teeth, hair, skin, and bone. Most teratomas are harmless, but they have the potential to wreak havoc by causing encephalitis.

saw that the pupil was enlarged and had lost its natural reflex of constricting in response to light. We call this a blown pupil, and it is a neurological sign that the brain is about to collapse.

“Just the news,” I kept telling the residents, “not the weather.”

The last case of this I saw was a guard at the Egyptian room at the Museum of Fine Arts. He got Nocardia from the mummy.” Nocardia is a bacterium, typically found in soil that, if inhaled, can cause a slowly progressing pneumonia. In rare cases, it can cause an inflammation of the meninges, the brain’s protective sac, and lead to hydrocephalus. The museum is just down the street from the hospital.

A code is a highly choreographed performance executed in a small space measuring approximately eight by twelve feet. Among the dozen people who rushed into the room in the next few minutes, each one had a specific part to play, much like the musicians in an ensemble.

“He’s either got a hair up his ass or something’s really wrong with his head.

It may sound trite to say that confusion is the most confusing syndrome in medicine, but it is. A confused person behaves in a way so foreign to common experience that it can be unnerving, even for professionals. It is an alternate state of being. Portrayals of confusion in popular culture—the town drunk, for example—may look funny, but in the case of a truly confused person, the sight of someone who can’t find his own mind can be overwhelming.

Clinically, confusion is defined as a loss of the usual clarity, coherence, and speed of thinking, but this description, while accurate as far as it goes, captures only a snapshot of confused behavior.

Marsel Mesulam, the prominent and prolific Northwestern University neurologist, has taken the lead in trying to define confusion, and has focused on what he calls “the attentional matrix.” The matrix, as he views it, is a place that serves as a temporary register of items that make up links in a sequential thought process. Confusion, according to Mesulam, disrupts tasks that require attention, and by inference, it represents a disruption within the matrix. I think that’s far too limited. When you sit at the bedside of confused patients, this is not what you see. They are inattentive, to be sure, and you can use that as an identifying feature, but many other things enter into it. For example, misperception and illusion (one thing being misinterpreted as another), topographic and spatial difficulty (they can’t draw, they can’t copy boxes), a loss of temporal coherence (difficulty connecting one moment to the next), and a hard-to-define language trouble. Those are all elements of various confusional states, and they do not derive from inattention so much as accompany it. Disorientation, for example, a staple of confusion, is not simply a result of inattention. Confused people have little or no insight into their predicament, and they cannot absorb their circumstances when I tell them that their minds are not working. They get lost and, most distressingly, become irascible, agitated, wildly unmanageable, or in some cases, their minds collapse inward, and they become silent and...

Psychosis is a special type of confusion with its own reality, an internal reality that is consistent only with itself. It does feature a connected, ever-flowing “stream of thought,” to use William James’s term (not a stream of consciousness, a phrase that James came to dislike). In his Principles of Psychology, James claimed that all of us carry on a virtually continuous internal conversation. While a psychotic’s internal stream may seem bizarre and disconnected, it has its own internal logic. Anyone could follow it, according to James, if they were standing in the waters of the stream.

A confusion of this type is in most cases a reversible state because it is a reflection of the dynamic functioning of nerve cells. If the causes are addressed, the patient will get better. It just takes time. If the causes are not addressed, the confusion takes over.

The gravity of memory problems is often disguised by their risibility. Someone in the throes of aphasia or agnosia, that is, someone whose perception functions properly, but whose processing does not, can unintentionally crack up a room full of trained specialists. Cases like these, replete with malapropisms and verbal absurdities, are more bizarre than scary. Transient amnesias in particular may last a few hours, almost always less than a day. Any number of things can trigger an episode, or nothing at all. Sometimes a heightened emotional experience, even sex, can set one off.

She didn’t know that the one thing people never forget is who they are. She had no idea that this curious thing we call memory works two ways. She would have benefited from reading Lewis Carroll.

Memory works both forward and backward. Forward, or anterograde memory, is the ability to form memories going forward. Backward, or retrograde memory, is the ability to retain memories of the past. The two are inextricably linked: when you lose one, you lose the other.

unaware that he had a problem forming new ones. By way of compensation, like many Korsakoff’s sufferers, he would fill in gaps by confabulating plausible but nonetheless crazy stories. “I think I saw you at the ball park,” he might say to someone he had just met. “That hot dog was great, wasn’t it?” The urge to fabricate experiences probably grows out of a need to save face. Many alcoholics do it in the early stages of the syndrome, and while it is an interesting component of memory loss, it is not a necessary one.

The majority of hysterical symptoms—symptoms that have no basis in disease and are subject to suggestibility—look like real neurological diseases. These include paralysis, inability to walk or speak, blindness, deafness, seizures, and weakness. All are manifestations of an organ that sometimes fabricates problems. But it gets even crazier. People who cannot feel on one side of the body will say they are deaf on that side, or blind on that side, unaware that this is an anatomical impossibility. The hardwiring of the human nervous system cannot produce these defects. This is not disease doing something to the nervous system, but rather the brain doing something to itself. The stomach doesn’t have a mind of its own to create stomach problems, nor do the colon, the lungs, or the skin. Ulcers, asthma, psoriasis, eczema, once thought (incorrectly) to be psychosomatic, or originating in the mind, were all shown to have tangible causes, and have been reclassified as nonpsychological diseases. Only one organ has a mind of its own, and it is constantly causing problems for itself. These problems, once termed “hysterical” and “psychosomatic,” are now called “functional” or “somatoform.” The conditions themselves are referred to as conversion disorders, implying the conversion of psychic distress into physical symptoms. It is one of the last vestiges of Sigmund Freud’s legacy still lurking in mainstream medicine.

Symptoms are what a patient reports. Signs are what a physician sees in an examination. Symptoms are thus subjective, and signs objective. When a patient reports a symptom, we have to take it at face value: a headache, dizziness, numbness, lower back pain. We have no tests for such things, and accept them as real until something in the patient’s behavior gives the game away. The claim of blindness, on the other hand, can be tested. People follow the image of their own eyes in a mirror. Not only that, even if they don’t flinch when I bring my hand toward their face quickly, most will involuntarily glance at a $100 bill that I wave in front of them. An old-school neurologist tested this effect. What denomination will get a blind hysteric to follow the bill? A $1 bill doesn’t work; a $100 bill works almost all of the time. So he always carried a C-note in his wallet just for that purpose.

“Let’s try some other tests. Follow my finger.” She stares blankly ahead as I move my finger back and forth. I pull a small mirror out of my bag, and move it from left to right in front of her face about a foot away. Her eyes follow their own images in the mirror. It’s gimmicky, like the $100 bill trick, but seeing eyes almost always follow the mirror. Elliott has his own unfortunate variant: he will take a Post-it note, and in small letters write GO FUCK YOURSELF on it, and then stick it on his forehead while he interviews the patient.

Nomenclature: Hysteria, psychosomatic, and pseudoseizure are OUT. Neurologists still use these words all of the time, just not in front of patients and their families. Other words we take pains to avoid are psychiatric and psychiatrist. People tend to hear these as crazy and shrink, and this rarely goes over well with anyone. Terms that are IN include: conversion disorder instead of hysteria, functional instead of psychosomatic (the two are not equivalent in any case), and psychological non-epileptic seizure— or P-NES (I kid you not)—instead of pseudoseizure, as in, “This lady has a P-NES.” That’s now a term of the art. It was coined either by someone with a very devious sense of humor, or no sense at all.

The Brits call this sort of thing Functional Neurological Symptoms, or FNS, the psychiatrists call it conversion disorder, and almost everyone else just calls it hysteria. There are three generally acknowledged, albeit uncodified, strategies for dealing with it. The Irish strategy is the most emphatic,

“What am I going to do?” he said. “I’ll tell you what I’m goin’ to do. I’m going to get her, and her family, and her husband, and the children, and even the feckin’ dog in a room, and tell ’em that they’re wasting my feckin’ time. I want ’em all to hear it so that there is enough feckin’ shame and guilt there that it’ll keep her the feck away from me. It might not cure her, but so what? As long as I get rid of them.”

In order for anyone to lift one leg up, they have to begin by pushing the other leg down, by way of bracing. This is called Hoover’s sign.

In many cases of hysteria, the ideal treatment would be hypnosis. We used it when I was a resident, and it worked, just as it worked for Sigmund Freud and his teacher, the French neurologist Jean-Martin Charcot over a century ago. It worked because patients with hysterical symptoms are suggestible, and, having fooled themselves into the symptoms, they can be fooled out of them. Deception works, but in the modern age, in the age of informed consent, we are not allowed to fool patients about anything, even if it is the only way we can help them.

“Look, seizures stop themselves after a couple of minutes. The cells exhaust themselves and use up all the ATP, so it’s almost impossible to convulse for this long without stopping and starting again.

The average American has at least one unexplained symptom every week or two, and less than one-fifth of survey subjects report no symptoms at all during the three days prior to a random query. Headache, tingling, pain, dizziness, briefly blurred vision, a slight imbalance when walking, loss of train of thought, feelings of jabs and jolts: these are the most common symptoms, and they befall any healthy nervous system, then disappear and are forgotten. In some instances, however, there is no way to disabuse a patient of his or her symptom. Where a normal, functioning person might say, “Well, things happen,” some patients develop an attachment to or an excessive worry about a specific extrinsic cause of their suffering. They fixate on it. Depending on the decade, it might be electrical fields or Lyme disease, environmental allergies or chronic candida infection, hypoglycemia or sick buildings, perfumes, or even alien abduction. The trick is to communicate the fact that you know, and that the patient needs to know that you know, and the family also needs to know, that the problem is not organic, somatic, neurological, medical, life-threatening, contagious, critical, or cataclysmic. That is not to say that it isn’t disabling in its own way. Hysterias and factitious disorders prevent people from getting on with their lives, and prevent their loved ones from getting on with theirs. They are highly disruptive. Whatever strategy we choose to take is obliged to acknowledge that it i...

Baloney is not hysteria or a conversion reaction. Unlike conversion, which has everything to do with overt neurological dysfunction, the more outrageous the better, baloney involves the exaggerated reporting of personal neurological experiences that is focused on nonsense, usually abetted by the hyperbolic popular press, all bordering on the delusional.

There is a wealth of Web sites and chat rooms devoted to it. Sadly, when it comes to dealing with, much less treating, such borderline theories, I have no spiel to offer, and sometimes revert to being a jerk. In this case, I suggested that they both might be magnetized. As an experiment, I said, he and his wife should float on their backs in their swimming pool to see if they both pointed north. I was guessing that they had a pool. I was right. They never came back.

Two things give him away as a drug seeker. The first is his preemptive insistence that he is not one. The second is his familiarity with drug names and dosages. His frustration seems to stem not so much from the pain as from our refusal to give in to his requests for drugs.

Children under about the age of eight do not get hysteria, probably because they are guileless. On the other hand, if a young woman, especially an adolescent, is suspected of hysteria, a pink bunny or teddy bear next to her pillow is a tell. It is almost a guaranteed sign of a conversion disorder.

“Mothers who poison their daughters so that they can nurse them through recovery.”

There are three kinds of patients who show up on the ward: the risk-neutral, the risk-averse, and the risk-resistant. We rarely see a fourth kind, the risk-takers, who instead show up in the morgue, if they show up at all. Most of the time they go straight to the funeral home. Risk-takers don’t come to the hospital of their own volition. They simply refuse to go to a doctor for anything. By contrast, risk-neutral people, a term that describes most of our patients, go to the doctor because they believe in modern medicine. They listen, they question, they comprehend, and for the most part, they cooperate. Risk-averse patients, on the other hand, need reassurance. These are the people who have to know everything. They have to call everyone. They need a better opinion. They won’t have the operation until they’ve had five second opinions. They anguish, they’re neurotic, but in the end they do what all normal people do when confronted with choices. Then there are the risk-resistant, who are simply recalcitrant, and give us the most trouble. They act as if they have something to hide, they often do have something to hide, and they hide it even when it would be in their best interest to let us know about it up front.

Medicine is like the laughing and crying masks of the theater—comedy and drama. There is never one side that is right and another side that is wrong. The risk calculations are in the doctor’s head, and no algorithm has yet to do better. Yet ultimately, as far as the hospital is concerned, the patient is always right because personal autonomy trumps probabilistic outcomes. You have to respect their wishes as human beings, we are told. But if you ask me whether the customer is always right, I would say, “Not at all.” The patient is so very often dead wrong, and very much so when it comes to his own brain.

“The patients are holding out their troubles. They are not really asking you to take them. You should only take them if you want or need to take them. Otherwise, leave it. They’ll get along without your suffering. You have another job to do.”

Tricking people means knowing and controlling what’s on their minds.

“Never shoot a singing bird.

(“treat-’em-and-street-’em,”

Every doctor can recall a transcendent moment of arrival, a moment of breaking through the cloud cover, leaving behind the shaky confidence and self-doubt that hung over their residency and fellowship. In neurology, it takes four, five, sometimes seven years to make that transition, and once you make it, once you hit your cruising altitude, those below you in the fog might think you are performing diagnostic miracles. It is important to point out to them that you are not.

There is a constant physical and emotional distance that separates a doctor and a sick patient, as though a rising tide were filling a channel that lay between them. But that space is not entirely empty. It is filled with emotion—hers of anguish, mine of a welling sense of dread. As long as my dread remains hidden, I can muster the degree of calmness and patience that allows me to stay at the bedside and be effective. Knowing how high the tide will go, I can sometimes see that it will take my patient with it. At such moments I have the comfort of knowing that I will be going home to my wife, my kids, and my dog, with all of my faculties intact.

Crises always focus the mind,

Air hunger is one of the most uncomfortable symptoms known to man or woman. Struggling to breathe, the awareness of breathing, contemplating the difficulty of each breath—something that a healthy, resting person never has to think about—is the feeling of dying. It probably contributes to the imminent sense of death (angor animi) of a panic attack.

I can only assume it requires a long internal conversation to make a confident decision to die.

We rarely stop to think about how much of our persona is created by the forty-three or so facial muscles at our disposal, especially those that encircle our eyes. When we think of eyes, other than their color, we think mainly about their frame: the lids, lashes, and brows; a squint, a glint, an arched brow, a purposeful asymmetry. We speak with our eyes. We read other people’s faces through a myriad of micro-expressions. One of the cruelties of ALS is that it not only forces its victims onto ventilators, thus robbing them of speech, but it eventually neutralizes most of the facial muscles, reducing the expressive palette to a few basic gestures.

George reminds me often that our role is to give Sophie balloons, not anchors.

“Tough. Very tough. The two extremes in neurological practice, as I see it, are the result of the incredible damage that can be done to the brain and spinal cord—dehumanizing effects. One extreme is to save life at any cost; the other is to participate in ending somebody’s life in order to reduce their suffering. You’d think the two extremes can be reconciled, but they can’t. You have to live with both.

Happiness is a decision.

The spinning motor in her head has slowed down, and the clutch won’t engage. Independent of her knowing how to get to the subway entrance, the mechanism that effectuates the giving of directions isn’t working. Most people do not have to think about this, but Tikvah does.