Reaching Down the Rabbit Hole: A Renowned Neurologist Explains the Mystery and Drama of Brain Disease - Hardcover

Ropper, Dr. Allan H.; Burrell, Brian David

Hardcover

ISBN 10: 1250034981 ISBN 13: 9781250034984

Publisher: St. Martin's Press, 2014

Synopsis

"Tell the doctor where it hurts." It sounds simple enough, unless the problem affects the very organ that produces awareness and generates speech. What is it like to try to heal the body when the mind is under attack? In this book, Dr. Allan Ropper and Brian Burrell take the reader behind the scenes at Harvard Medical School's neurology unit to show how a seasoned diagnostician faces down bizarre, life-altering afflictions. Like Alice in Wonderland, Dr. Ropper inhabits a world where absurdities abound:

· A figure skater whose body has become a ticking time-bomb
· A salesman who drives around and around a traffic rotary, unable to get off
· A college quarterback who can't stop calling the same play
· A child molester who, after falling on the ice, is left with a brain that is very much dead inside a body that is very much alive
· A mother of two young girls, diagnosed with ALS, who has to decide whether a life locked inside her own head is worth living

How does one begin to treat such cases, to counsel people whose lives may be changed forever? How does one train the next generation of clinicians to deal with the moral and medical aspects of brain disease? Dr. Ropper and his colleague answer these questions by taking the reader into a rarified world where lives and minds hang in the balance.

About this title

About the Author:

Dr. Allan H. Ropper is a Professor at Harvard Medical School and the Raymond D. Adams Master Clinician at Brigham and Women's Hospital in Boston. He is credited with founding the field of neurological intensive care and counts Michael J. Fox among his patients.

Brian David Burrell is the author of Postcards from the Brain Museum. He has appeared on the Today Show, Booknotes, and NPR's Morning Edition. He divides his time between writing and statistical research with neuroscientific applications.

Excerpt. © Reprinted by permission. All rights reserved.:

1

Six Improbable Things Before Breakfast

 

Arrivals, departures, and delays on the ward

On the third of July, a day after a routine colonoscopy, Vincent Talma was playing right field in a company softball game. A short, intense man with thick gray hair and a perpetual scowl, he did not look as though he was having fun, or even capable of having any fun. Whenever a teammate said something amusing or cracked a joke, Vincent would laugh without smiling, as if to say, “funny, funny, ha, ha.” When he disagreed with a call by the umpire, he would throw up his arms in disbelief, kick the dirt, and swear under his breath, not for show or for the approbation of his teammates, but out of real anger and disgust. No one called him Vinnie, few called him Vince, and when he stood at the plate, none of his teammates dared to cheer him on by name.

As the game wound into the late innings, Vincent’s behavior began to change, subtly at first, then dramatically. By the time he was dropped off at his house, his wife was startled to see a bemused look on his face, an air of innocence in place of his usual gruffness. He gave one-word answers to her questions, avoided eye contact, and seemed quite unlike himself. He was smiling too much.

“Are you okay?” she asked.

“Sure, fine,” he replied.

“Did you win?”

“Fine, yes.”

“Did something happen?”

“Fine.”

The more she persisted, the more Vincent perseverated.

“Did anything happen at the game?”

“Fine, yeah fine,” he mumbled with a sheepish smile.

She called their primary care physician, who told her to get him to the emergency room immediately.

“Vincent, we need to go,” she said.

“Fine. Okay.” Still smiling.

At East Shore Hospital an MRI showed an ambiguous blotch on the left frontal lobe of Vincent’s brain, and at the suggestion of one of his sons, a pediatrician, the family requested a transfer to us. He arrived sometime around 10:00 that morning and was brought up to the ward.

*   *   *

A week earlier, Cindy Song, a sophomore at Boston College, had started acting a bit withdrawn. Her roommate was concerned enough to call Cindy’s sister. The first phone call was not too worrisome. “Not a big deal,” the sister said. “She gets that way. Just give her time. She’ll be okay.” The next call could not be taken so lightly.

By morning, Cindy wouldn’t leave her room, and would not or could not tell her roommate why. Alternately anxious and distracted, uncharacteristically morose and sullen, she spent the day in bed. That evening she refused to eat, and her roommate made the second call, this one to Cindy’s mother, a first-generation Korean immigrant. Despite the language difficulty, there was no mistaking the concern in the roommate’s voice. Cindy’s mother took the next commuter train from Framingham, exited at Yawkey Station, took the Green Line out to Chestnut Hill, walked up the steep hill from the terminal, past the Gothic spire of Gasson Hall, and down the long, winding road to the dorms. When she got to Cindy’s room and sat down in front of her daughter, all she got back was a blank stare focused on the wall behind her. Her daughter’s eyes were wide open and her pupils dilated. She was shivering mildly and sweating all over. Finally, she spoke.

“Mom, they’ve been after me for weeks, creeping in through the cinderblocks, taking my clothes off.”

“What are you talking about, honey?”

“My clothes, my clothes,” she said desperately, “can’t you see them?”

Like all universities, Boston College has a health center that provides minimal services overnight, on holidays, on weekends, and during the summer, relying on referrals to local emergency rooms for anything serious. The after-hours nurse, who was used to such things, assumed that Cindy had been using recreational drugs and was “just flipping out.” Nothing unusual as far as the nurse was concerned, but Cindy’s mother was outraged. Convinced simply from cultural experience that there were no drugs involved, she would not let that stand. Cindy was so jittery and sweaty that the nurse gave in and called an ambulance to take her to the Brookline Hospital emergency room. Once there, Cindy remained agitated, stopped responding to questions, and started thrashing, as though reacting to hallucinated visions. This prompted a round of phone calls to the eight local psychiatric hospitals to see if there was a bed for an acutely psychotic young woman. Such beds are hard to come by, and it took a hard sell by the emergency room doctor to secure the promise of one by the next afternoon, “if you could just hang onto her and give her Haldol in the meantime.”

As daylight broke, Cindy was close to berserk. Her arms had to be restrained with straps, and she was soaking up tranquilizers like a sponge. Finally, the ambulance came to bring her to the psychiatric hospital. After a short interlude of relative calm, the psych nurses became alarmed when Cindy’s jitteriness escalated into full-blown myoclonus—arms and legs flinging up off the bed, her head jerking back violently. Her pupils were huge. If it had been a drug overdose, they realized, this would have abated by now. Instead, the hallucinations continued, and Cindy was excessively restless and sweaty. I got a call at about 9:30 a.m.

“Is she salivating like she has rabies?” I asked the psychiatry resident.

“Yes, like a dog,” was the reply.

“You’d better send her over.”

*   *   *

By the time Vincent Talma and Cindy Song had settled in at the Brigham, Arwen Cleary had been there for four days. She came by ambulance on the morning of July 1, and was admitted to neurological intensive care from the Emergency Department later that evening. Of the three cases, hers was the least clear-cut, the most troubling, and one that had the potential to become an absolute shambles. According to her medical records, her problems had begun two years earlier, when she showed up at a central Massachusetts hospital with disabling nausea, difficulty walking, and vomiting.

Arwen Cleary had been a professional figure skater as a teenager, had retired from the Ice Capades upon its dissolution in 1995, had then raised three children, gotten divorced, and moved with her two younger children to a ranch house in Leominster, a distant suburb, where she worked part-time at a local health club. Her medical history was unremarkable: once a smoker, she had quit ten years earlier. Her travels had taken her no place more exotic than Bermuda and no more distant than Orlando. Her only hospitalizations to that point had been in maternity wards. She was remarkably fit and in seemingly good cardiovascular health, if judged only by her appearance and vital signs. 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. At her local hospital, Ms. Cleary was started on a blood thinner, and after a long inpatient and rehab stay, she recovered her motor skills and balance, and was sent home.

All went well for two years, until she returned to the hospital with sudden right facial drooping and difficulty finding words, sure signs of another stroke, but this time a stroke of a very different kind. A portion of one of the language centers of her brain had been deprived of its blood supply. Her speech was now noticeably impaired. Within a few days, she showed signs of improvement, and was again discharged on a blood thinner.

Ten weeks later, to her infinite frustration, it happened yet again, and she arrived at the same hospital in the middle of the night with another language problem, this time even more pronounced, as well as right arm weakness. The scans now showed that several other blood vessels had been stopped up, causing a scattering of new strokes. At that point her doctors became even more worried. Why would this be happening in someone so young? But they could locate neither a cause nor a source. They subjected her to exhaustive tests, the usual suspects for stroke were rounded up, an echocardiogram was ordered, and she was given a portable heart monitor. Everything came back normal. It was decided that the previous chiropractic stroke (the dissection) was unrelated to her current problem. Among the staff, the consensus was: “We’re going to need a bigger boat.” So they sent her to us.

*   *   *

There is an old joke among stand-up comics that goes: “Dying is easy, comedy is hard.” If we were as inner-directed as comedians, we neurologists might say, “Trauma is easy, neurology is hard.” Every one of our patients has, in effect, fallen into a hole, and it’s our job is to get them out again.

In Alice’s Adventures in Wonderland, Alice jumps into a rabbit hole and finds herself in a bizarre realm in which nothing is what it seems, where everything bears little relation to the outside world. It is a place where, as the Red Queen mentions to Alice, it helps to believe six impossible things before breakfast. Unlike the Queen, I have no need to believe six impossible things before breakfast because I know that, on any given day, I will be confronted by at least six improbable things before lunch: a smiling man whose speech difficulties seemed to have been brought on by a colonoscopy, a thrashing young woman whose psychosis seemed to come out of nowhere, a figure skater with a slow-fuse time bomb in her body that was knocking off her faculties one by one. The first of these, I should note, was indeed impossible, and I didn’t believe it for a second, but the next two were quite possible, and by the end of the morning, I would encounter at least three more improbabilities: a woman who could only be cured by a hole in the head, a case of amnesia brought on by sex, and a man who was adamant that I was two very different doctors.

We treat people with seemingly implausible ailments all of the time. Each day they show up in a predictable parade of signs, symptoms, and diseases: an embolus, a glioma, a hydrocephalus; a bleed, a seizure, a hemiplegia. That’s how the residents refer to the cases, as in: “Let’s go see the basilar thrombosis on 10 East.” When viewed in terms of actual patients, however, no day is quite like any other. After the bedside visit, the thrombosis suddenly has a name, the glioma has a wife and children, the hydrocephalus writes a column for a well-known business journal. Our coed suffering from psychosis turned out to be a Rhodes Scholarship candidate, the case of multiple strokes became a charming woman who had competed in the Junior Olympics, and the man for whom a smile was a troubling symptom owned a personal empire of six Verizon wireless stores.

*   *   *

“Good morning, Mr. Talma,” Hannah said, “do you remember me?”

“Yes, good, good, fine,” Vincent replied. He was sitting up in bed, watching television with a smile of bemused innocence. Vincent Talma was a picture of contentment. His room on the tenth floor of the hospital tower commanded an outstanding view of Fort Hill Park in Boston’s Roxbury section, but Vincent took no notice. Along with twenty-nine of our other patients, he had been waiting for a visit from the neurology team on their morning speed rounds.

Hannah was in charge. Her service, the culmination of three years as a neurological resident, had started a week before I came on board. A “service” involves running the neurology inpatient ward, admitting and discharging the patients, and directing a team consisting of three junior residents, two medical students, and a physician’s assistant—a cohort that could barely squeeze into Vincent’s curtained-off half of the room.

My colleagues and I had some doubts about Hannah when she first came to the program three years earlier. The most superficial of these doubts focused on her style of dress. In a profession where sartorial flair is an unexpected and somewhat suspect concept, Hannah’s clogs, leggings, and wraps seemed needlessly exotic, and sowed uneasiness among the Dockers, Skechers, and scrubs crowd. Perhaps even more alienating was the fact that Hannah did not drive a car, and instead rode her bike from her apartment in Boston’s North End to the Brigham, usually well before the sun rose or long after it had set, in any kind of weather short of a blizzard. Such stoicism flew in the face of the unhealthy lifestyle adopted by most of the residents and teaching faculty, who tend to favor pastries over granola, Coke over water, and elevators over stairs.

I could see that over the course of the previous week, Hannah had begun the transition from resident to full-fledged physician. I could see it in her bearing, in the assertive physicality with which she carried out her examinations, in the firmness of her tone with some of the more difficult patients, and in the controlled sympathy she adopted in family meetings when she had to deliver bad news. She had turned out to be one of our strongest clinicians.

Although she hails from the Midwest, Hannah Ross has a northern European flair, somewhat Dutch, in that she is tall, lithe, wears fashionably businesslike glasses, and seems indifferent to the possibility that anyone might appreciate the effort she has made in choosing her look, probably because the effort is now merely a habit. She moves swiftly from room to room, from pod to pod, from the nurses’ station to the rolling laptop cart, where she displays an instantaneous command of electronic medical records, and can bring up an MRI scan and zoom in on a tumor or a cerebral hemorrhage with no wasted effort.

“What are you watching?” Hannah asked Vincent, in an inflection she would later inform me was Kansan rather than Missourian.

“The Bunkers.”

“Do you mean All in the Family?”

“Yes, yes,… the Bunk … Yes.”

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.

“What’s your name?” Hannah said.

“Vincent.”

“Good. Where are we? What place is this?”

“Vincent … uh, yeah … Vince.”

“What day is it?”

“Avince … Vince.”

“Okay. Look at my hand. Now follow my thumb.”

“Gee, you’re so dumb.”

Gilbert, the medical student who had made the initial exam, recorded this as “orientation times one.”

“To one what?” I later asked him.

“To himself,” he said.

“Have you ever met a patient who wasn’t?”

“I don’t think so.”

“No, you haven’t. It doesn’t exist.”

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.

Vincent knew who he was. He was sharp enough to find himself amusing. Did his colonoscopy earlier in the week bring this on, or, more to the point, ...

About this edition

• Publisher: St. Martin's Press
• Publication date: 2014
• ISBN 10: 1250034981
• ISBN 13: 9781250034984
• Binding: Hardcover
• Edition number: 1
• Number of pages: 272