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

by Allan H. Ropper

There are at least three broad categories of stroke: one involving blocked blood vessels, another involving bleeding into the brain, and a third—an aneurysm—involving a ruptured bulge in a blood vessel. Although these are all called strokes, they are as different from each other as hepatitis is from gall bladder disease (both of which give you jaundice). And their treatments are entirely different.

when I pried open her right eyelid, I 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.

Elsewhere on the ward, we had Mrs. Newlin, a ninety-year-old woman with such explosive headaches that Elliott suggested putting her on a terrorist watch list.

A shunt is an internal one-way drainage valve that relieves and controls the internal pressure of cerebrospinal fluid.

The skull is like a fixed container. If pressures within the skull are unevenly distributed, there is a breaking point at which the entire brain gets squeezed downward like a plunger, compressing the brain stem.

Mannitol, a drug administered by IV, is a sugar alcohol used to pull fluid out of the brain in order to reduce the internal pressure. In this case it was just a stopgap.

Status is short for status epilepticus, a state in which a patient is constantly seizing. In this case, without physical convulsions, it would properly be called nonconvulsive status epilepticus. But Gordon wasn’t seizing. The Haldol, an antipsychotic, had made him worse, suggesting that he was also not psychotic.

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. Wally Maskart’s thinking might have been crazy (in fact it was crazy), but it wasn’t stupid. My job was to step over the boulders and out into the middle of the current, and try to lead him back to shore.

Concluding that it was an episode of transient global amnesia, or TGA, a dramatic but entirely benign condition, the resident came to me to approve a discharge in anticipation of the expected return of the patient’s memory. Although it may sound serious, transient global amnesia can be set off in a variety of ways, has no obvious cause, and will usually resolve within hours, leaving no permanent damage.

“If this isn’t a transient amnesia,” I asked myself, “what else could it be?” There were only a few possibilities: a concussion, a viral infection in his brain, ongoing seizures, or a stroke. All four affect memory. Three out of four are life-threatening. His CAT scan was normal, seizures seemed unlikely, it wasn’t drug-induced, so it had to be either transient global amnesia or a stroke.

aphasia or agnosia, that is, someone whose perception functions properly, but whose processing does not,

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. In the Boston area, I tell the residents, there’s a big spike in these cases in early summer when people start swimming in the ocean. Cold water may be the opposite of sex, but it can create a shocking experience nonetheless, and can trigger TGA.

In Through the Looking-Glass, the second adventure in Wonderland, Carroll tells of a frustrating conversation between Alice and the White Queen. The Queen, it seems, claims to live backwards. “Living backwards!” Alice says, “I never heard of such a thing!” The great advantage in it, the Queen replies, is that “one’s memory works both ways.” When Alice counters that her memory only works one way, the Queen says, “It’s a poor sort of memory that only works backwards.” She was right. 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.

It was amnesia—Korsakoff’s syndrome—a retrograde and anterograde amnesia, a permanent amnesia caused by the low blood flow to his medial temporal lobe during the cardiac arrest.

By the following morning some of Godfrey’s memory had returned, but in a Swiss cheese fashion: there were significant holes in both retrograde and anterograde memory. This is not consistent with transient global amnesia. If Godfrey did not in fact have TGA, he was then, like the silver fox, in serious danger of losing a significant chunk of his past memories, along with his ability to form new ones.

TGA is highly stereotyped. It varies little from person to person. It is one of the few neurological syndromes that has inviolate borders, and Godfrey’s form of memory loss was too spotty, going backward and forward, to fall within those borders.

“She’s aphasic. She must be seizing. Let’s put on a hairline.” A hairline is a quick and dirty electroencephalogram done with an abbreviated set of sticky electrical leads connected to an EEG machine. The object is to find out if she’s in status epilepticus, a fancy way of saying that she is seizing uncontrollably.

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.

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.

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.

“Dr. Ropper, let’s get her going for the CT scan so we can start her TPA.” He’s referring to tissue plasminogen activator, a powerful clot-busting drug given to stroke victims. Because it is a blood thinner, it poses a serious risk of hemorrhage.

I move to the other side of the bed, and with my hand now under her left heel, I ask her to try to lift up her right leg while I try to resist. When she does this, I can feel downward pressure in her left heel. 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. She is using her left leg without realizing it.

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.

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“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.

Pseudoseizures, or P-NES, are probably the most common form of hysteria or conversion symptom seen in a neurology ward. The majority of them occur in patients who have genuine epilepsy. They know what a real seizure should look like, and are pretty good at producing fake ones. The siblings of patients with epilepsy, who have seen plenty of real seizures, also account for a significant number of cases.

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.

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A subarachnoid hemorrhage, or burst aneurysm, is one of the most treacherous diseases in medicine. Although considered a type of stroke (or to use the Boston vernacular, a “shock”) because it happens suddenly and occurs in the head, it bears no resemblance to the paralysis or speech trouble usually associated with a stroke. An aneurysm is a rounded pouch on one of the blood vessels at the bottom of the brain. If it reaches a critical size and form, it can burst open with the entire force of the body’s blood pressure. Blood then fills the spaces around the brain in a split second and causes a thunderbolt of a headache that no one forgets and many don’t survive. One-third of patients who do survive have a re-rupture in days, and half of those people die. Ruby had dodged the thunderbolt twice. He had had two bleeds, which resolved, although his blood had now mixed with the spinal fluid surrounding his brain—not enough, apparently, to show up on the CT scan (in about 5 percent of patients it does not), but enough for us to confirm it if we could get a lumbar puncture.

If the spinal fluid revealed the red-brown color typical of broken-down blood cells, it would have confirmed the diagnosis. After that, an angiogram—essentially an X-ray that reveals the outline of the cerebral arteries—would have isolated the aneurysm, and led to a rapid treatment.

If God calls, you are never satisfied until you find yourself doing what you feel you are called to do.

“Do people ask you about deep existential, theological issues or problems? Do they ask you: Is there a God?” “Many times. You always have that question: ‘Is there a God? And if there is a God, why am I going through this?’ Back when I was the chaplain on duty, just starting part-time, a young lady was med-flighted here. She was pregnant. They had to rush her into surgery, open her up, take out the baby, all wrapped up in a blanket, and they gave it to me to bless because they knew the baby was not going to live. And one of the nurses said, ‘You need to go in there and pray for the mother.’ She was coding, blood was going everywhere, and this was the first time I’d ever seen someone with a human heart in their hand, pumping it to try to keep her alive. I blessed the baby, I prayed for the mother. She lived, but the baby died. That stuck with me. I met another young lady just the other day. She had lost her daughter a few months earlier. Her husband was in the hospital, and his prognosis was not too good. She said, ‘I’m mad as hell with God. As a matter of fact, I don’t want to hear nothing about God.’ And this is a person who professed to go to church. So there are times when you have to be able to let them pour out exactly how they feel without condemning them. You don’t have to prove to them that there is a God. Sometimes it’s just what it is. They have these questions—‘I’m serving God. Why am I sick? I’ve been a good person, why is this happening? Why has my child died?’...

The definitive cure for an anterior cerebral aneurysm is an extremely delicate procedure. It involves the placing of a clip that looks like a miniature distorted clothespin on the neck of the aneurysm. The trick of it lies in choosing the right clip, choosing the right place to put it, getting just the right angle, and making sure that you seal off the neck of the aneurysm so that no blood can get into it, all the while avoiding all of the surrounding vessels.

All of them. The clips are made with little cutouts, allowing them to put pressure only on the aneurysm and not on any surrounding blood vessels. But in that area of the brain it is very easy to nab tiny vessels that feed the frontal lobes.

There is an alternative to clipping called endovascular coiling, in which the surgeon gets at the aneurysm via a micro-catheter fed through the artery, but that procedure has problems, too.

Tuskegee syphilis experiment.”

The Tuskegee syphilis experiment was one of the most notorious clinical studies ever conducted. From 1932 to 1972, the U.S. Public Health Service offered free health care and meals to rural black men in return for being allowed to follow the progress of their untreated syphilis, without informing them of their disease or the existence of treatments to cure it.

“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.”

we treated a farmer from the Sacramento area who had coccidioidomycosis meningitis, also known as valley fever. Stockton, California, was ground zero for the disease. It is carried by airborne dust particles that settle into the lungs, causing a pneumonia-like infection that can develop into an inflammation of the brain’s lining. The treatment involves a lumbar spinal tap that allows medicine to be injected into the spinal canal daily, a very invasive regimen requiring a long stay in the hospital.

We immediately brought her up to the intensive care unit where she continued to have seizures intermittently without waking up. In other words, she was in status epilepticus.

Out of the depths of a partially formed instinct, and even though she did not look pregnant, I called the pharmacy and asked them to send up a drip with magnesium, the tried-and-true treatment for eclampsia. We started the drip and, miraculously, the seizures stopped.

Eclampsia, a problem associated with pregnancy, causes a spectrum of symptoms similar to hers, but I hadn’t even thought to check whether she was pregnant,

“Never shoot a singing bird. Keep the magnesium going.” We did not have a diagnosis, I had no more hunches, but it seemed to help. So I let the bird sing.

I thought she was in Addisonian shock—that she had Addison’s disease, a disorder of the adrenal glands that can lead to coma.