Reaching Down the Rabbit Hole

REACHING
DOWN THE
RABBIT HOLE

ALSO BY DR. ALLAN H. ROPPER

Principles of Neurology

ALSO BY B. D. BURRELL

The Words We Live By
Postcards from the Brain Museum

CONTENTS

REACHING
DOWN THE
RABBIT HOLE

INTRODUCTION:

The Queen

What neurologists do

“Hello, I’m Dr. Allan Ropper. How are you?”

“That’s a grammatical question. Plus and minus.”

“Is your mind clear?”

“I guess so. There are a lot of unexplained issues around me, but . . . I’m communicating appropriately.”

His name is Dr. Vandermeer. He is in his mideighties, and he is a genuine Boston Brahmin. I know his type well. Over the past fifty years he has built a national reputation as a top-drawer researcher and a caring physician, universally admired, all the while taking somewhat heedless care of his own body. He is a man of arts and sciences, but also a man of tastes and habits acquired from his father and grandfather, which is to say, he is a nineteenth-century Yankee living a twentieth-century life in the twenty-first century, and is only vaguely aware that he can no longer manage in the ten-room house he has occupied for the last fifty-two years. When he retired in his seventies, he settled upon a daily routine that failed to anticipate his declining faculties. He is as unwilling to accept this fact as he is to acknowledge the
unruliness of his eyebrows or his surplus of nose and ear hair, a clinical indifference that is not unusual in aging doctors.

“Do you know where you are?”

“At Brigham and Women’s Hospital.”

“The date?”

“The date? No, I couldn’t give you that.”

“Year?”

“Again, it’s such a confusing sequence of events that it has confounded me to where my orientation isn’t what it should be.”

“No problem. Are you in any pain?”

“No.”

Doc Vandermeer was brought here over his own objections after his wife found him sitting on the toilet some seven hours after he had ventured from bedroom to bathroom. He had spent the night there.

“Have you had any hallucinations?”

“I don’t think so, but then people generally don’t.”

“Touché. Have you had any convulsions?”

“No.”

“And you know about this meningioma in your right temporal lobe? You have a meningioma there about the size of a lemon. Were you aware of that?”

“I’ve had two growth issues that are pertinent. One is in the pancreas and the other is there.”

“But you seem a little off, cognitively, and our struggle is, could it be the meningioma that’s doing it?”

“As they say, that’s your problem.”

He’s right. It
is
my problem. I am his neurologist. It is my job to parse his convoluted responses, fit them into the clinical picture, prioritize his issues, and come up with a plan that lets him live the life that he wants to live, to the extent that is possible. In his peculiar way, he has indicated that he is oriented to his location, but not oriented to the time or to his situation. His highly formalized locution, entirely characteristic of his tribe, may seem quaint, but it is exaggerated
just enough to suggest that he is compensating for a language deficit that he is aware of but seems indifferent to. He knows that he has a benign tumor in his pancreas that poses less of a threat than the meningioma in his brain. The brain tumor will not kill him anytime soon, but it will continue to diminish his powers of thought.

“We’ll find out what’s what and let you know,” I say. “Great to meet you.”

It will take us some time to sort it all out, but this is a start. Hannah, my senior resident, gives me a nod, signaling that it’s time to move on to the next patient.

“Hello, there. I’m Dr. Ropper. I’m one of the neurologists. And you’ve met Hannah Ross, our chief resident. Do you mind if we visit with you?”

His name is Gary, and he is one unhappy customer. He is thirty-two years old and has spent more time in hospitals than he cares to recall.

“How are you doing?” I ask.

“I can function,” Gary replies listlessly, “but not to design specifications.”

“Interesting phrase. Where did you get that from?”

“Gene Roddenberry.”

It fits. Gary is a computer programmer who has so immersed himself in
Star Trek
that he knows the Starship Design Specifications by heart. He has the body of a Buddha, the eyes of a koala, and an air of resignation that is probably his baseline. He might as well be wearing a T-shirt emblazoned with the motto:
IT IS WHAT IT IS
. He has had epilepsy since childhood, culminating in the surgical removal of part of his brain when he was seventeen years old. A U-shaped scar stands out prominently on the left side of his close-cropped scalp. He has enough experience with his condition to know when he needs to go to the hospital, although he would rather not go. When he does, he most definitely does not want to be admitted, but Hannah has admitted him anyway, and there is a lingering animus on his part.

“I understand you had several events yesterday,” Hannah says. “A lot of seizures?”

“Well, not many for me. What’s considered many?”

“It all depends on how many is many for you. I was under the impression that you did have many for you . . . in a
row
. Is that not true?”

“No. Even when I have clusters it’s probably four or five.”

“Is that what you had yesterday? Four or five?”

“Well, that’s not what brought me to the hospital, if that’s what you’re getting at.”

Gary is in a mood, so I’m happy to let Hannah carry the ball. This is her ward, these are her patients, I am the attending physician, and while I took the lead with Dr. Vandermeer in deference to his stature, I now take up a position at the foot of the bed and watch.

“That
is
what I’m getting at,” Hannah continues in a sing-song tone as if trying to engage a curious child. “So what brought you to the hospital today?”

A pause. Gary seems bothered less by Hannah’s tone than by her question. “It was late in the afternoon,” he replies with mock sarcasm. “I had a really bad headache and I was feeling very tired. I was clumsy and I couldn’t stand up straight and my hands were vibrating.”

“And that’s not what usually happens with your seizures?”

“Well, I don’t get so much warning with the seizures, although some days I can tell if I’m walking up to the edge of a seizure, but it doesn’t always lead to a seizure. Sometimes it leads to a lot of seizures, sometimes nothing.”

“How many seizures do you usually have in a given day?”

“There is no usual. I can go as long as five days without any seizures at all.”

Hannah’s directed line of inquiry seems to have snared his attention. He is clearly not used to having anyone take such a keen interest in his troubles.

“And what’s your usual when you do have them?” she asks.

“My record is eighty-four seizures in a day.”

“Is that typical?”

“There is no rhyme or reason to it. Some days I have none, some days I have a lot.”

“But say in the course of a month.”

“You keep
wanting
me to do that. You keep
wanting
me to put some pattern to it. Believe me, I wish I
could
put a pattern to it. Would you like to see my seizure log?”

“That won’t be necessary.”

I’m staying out of it. Hannah is firm yet restrained and unflustered; Gary is exasperated to the point of annoyance, almost anger, without the emotional edge to attain real anger. He acts besieged, yet lacks the reserves of panic that fuel true paranoia. It’s as though he has programmed himself to be confrontational, but the confrontational part of his brain is so compromised that it can’t run the code.

Hannah presses on. “What I really want to understand is whether you ever have a month without seizures.”

“A month? No. I’m lucky if I go a week.”

“And then when they come you can have one or you can have a ton?”

“Yes.”

“Any missed doses of your meds?”

“Everyone wants to blame the epileptic. ‘You wouldn’t have seizures if you didn’t miss your doses.’ Is that it?”

She doesn’t bite. “No. You’d probably have seizures anyway.”

“That’s true. I don’t miss doses, and I have seizures.”

We have the picture now. Fortunately, Gary does not. If he did, he would know that Hannah suspects, as do I, that some of his seizures are fake. It is time for us to get going again.

“It was really great to meet you,” I say. “I wish the rest of our patients had your outlook.”

“You mean felt that there’s nothing you guys can do?”

“No, I mean had your level of self-awareness.”

Gary has what was once called a temporal-lobe seizure personality. He is intelligent, mildly paranoid, has good word usage, and most of
all is extremely literal. He is wrong that there’s nothing we can do, although this has long been a knock on neurology: that we can usually tell what’s going on in someone’s brain, but that we can’t do much about it. I won’t bother to disabuse him of the idea because it would take a book to do so. This book, for example.

Gary is right about one thing. His mind is not working to design specifications, although in that regard he is hardly unique. There are twenty-nine other people on the ward right now who could say the same thing.

They call this place the Brigham, short for Brigham and Women’s Hospital, an amalgam of the names of a few historic hospitals that merged decades ago—the Peter Bent Brigham, the Robert Breck Brigham, and the Boston Hospital for Women—and it spans the length of a city block that abuts the Harvard Medical School campus. This massive enclave of teaching hospitals, which also includes Beth Israel Deaconess Hospital, the Dana Farber Cancer Institute, Boston Children’s Hospital, and the Joslin Diabetes Center, serves as a training ground for Harvard’s medical students. It is a city within a city, or at least a city on the very edge of a city: a district of glass, stone, and steel towers that occupies the eastern bank of a lazy, muddy waterway that separates Boston from the suburb of Brookline. At clinics and community hospitals throughout New England, whenever a doctor looks over a patient chart, sighs, and says, “Send it to Boston,” there is a good chance that the patient will end up here.

On any weekday morning the inbound traffic is daunting, like a rising tide in a teeming estuary, bearing with it an influx of patients, visitors, and family members, not to mention staff, that will be carried away with the ebb at day’s end, leaving behind isolated pools and puddles of low-level activity.

This is one of those pools—the neurology inpatient ward. It occupies the tenth floor of the hospital tower. Built in the 1980s, the tower
has a cross section in the shape of a four-leafed clover, each leaf being a pod, each pod consisting of a dozen fanned-out rooms, each room visible and easily accessible from a semicircular nurses’ station, a novel idea that now seems somewhat dated. The patients here, like Gary and Doc Vandermeer, are in tough shape, and they spend much of their morning waiting for our visit, and the rest of the day wishing they were someplace else, even though this is the best place they could be. It is a place where the strangest and most challenging cases are sent to be sorted out, cases whose complexity would tax the resources of smaller hospitals.

I am a clinical neurologist and a professor of neurology. Most people have little idea what that means, but among other things it means that I am an authority on what the brain does right and does wrong: language, sensation, and emotion; walking, falling, weakness, tremors, and coordination; memory, mental incapacity, delays in development; anxiety, pain, stress, even death. The practice of my craft, the clinical part of it, is the systematic, logical, deductive method that was in the past applicable to all branches of medicine, but now resides mainly in neurology. The paradoxical part of it, the unique challenge, is that my primary sources of information—my patients’ brains—are quite often altered, sometimes bizarrely, as a result of disease. This creates an incredible and self-referential conundrum. How do you begin to understand a sick brain? The only viable answer, as this book will show, is that you do it by engaging the person inside, and you do it on a case-by-case basis.

“He hasn’t felt emotion for at least a month.”

This is the girlfriend speaking. She is in her midthirties, as is her boyfriend, the man without emotion. They are not well-to-do, but they get by. She speaks with a strong North Shore accent, and despite her downscale style of dress, she has above-average verbal skills: she listens carefully, processes information quickly, and answers helpfully.
They have lived together for five years. They have a child. They are resigned to their fate.

“What do you mean by that?” I ask her.

“No sadness over what’s happening. No anger anymore. When he was first diagnosed he had a lot of anger issues. But recently he’s just been happy, not out of control.”

“Happy, or just nothing?”

“I would say content,” she replies. “He’s easygoing. The TV used to make him laugh. I saw happiness then. Not now.”

“When he expresses an emotion . . . ?”

“He may smile, nothing else.”

“In speaking to you, speaking to each other?”

“No. He really hasn’t initiated a conversation in the last month.”

“That’s typical for a butterfly glioma infiltrating the frontal lobes,” I say, referring to his spreading, malignant brain tumor. “So we’re really seeing him in good shape now. Is that true?”