Cover image for Reaching down the rabbit hole : a renowned neurologist explains the mystery and drama of brain disease
Reaching down the rabbit hole : a renowned neurologist explains the mystery and drama of brain disease
Ropper, Allan H.
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First edition.
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New York : St. Martin's Press, 2014.
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vi, 263 pages ; 25 cm
"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"--
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RC351 .R66 2014 Adult Non-Fiction Biography

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"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.

Author Notes

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.

Reviews 3

Booklist Review

The brain is mysterious and complicated, but longtime Harvard Medical School professor and neurologist Ropper (who counts Michael J. Fox among his former patients) and coauthor Burrell clearly explain what can go wrong, from Parkinson's disease to brain tumors, concussions, and strokes. They also throw in many fascinating tidbits. For example, Scandinavians have the fewest strokes, and the Japanese have the most, and few autopsies are performed today because everyone believes scans have already given the answer and because Medicare and Medicaid do not pay for them. As the title suggests, Ropper's patients have fallen into an Alice in Wonderland-like hole and find themselves in a bizarre realm in which nothing is what it seems. The authors disguise the identities of almost everyone (except Michael J. Fox) and say that 70 percent of the dialogue in the book is verbatim, 20 percent recollected, and 10 percent extrapolated, which seems like a stretch for the scenes from three decades ago. Still, they successfully convey what makes neurology the queen of medical specialties and the brain the most complex, fascinating organ in the body.--Springen, Karen Copyright 2014 Booklist

Publisher's Weekly Review

Ropper, a professor at Harvard Medical School and founder of neurological intensive care, and Burrell (Postcards from the Brain Museum) present an intriguing, if meandering, account of neurology's real-world applicability. The authors repeatedly emphasize, that proper diagnoses of neurological issues require both intensive study and exquisite intuition because the brain is so mysterious. Among the many patients featured is a chronic-pain faker who tries to score drugs and gets caught red-handed as well as a psychotic whose confusion, unlike other patients with severe cognitive problems, has its "own internal logic," leading him to believe Ropper was two different people despite exhibiting full awareness of his surroundings. Perhaps the biggest takeaway from Ropper's work is that every individual's mind, both in sickness and in health, is as unique as the proverbial snowflake. "Up on the ward," he says, "every grand theory of mind and every sweeping generalization about consciousness falls apart when exposed to the cold, hard truth of a single case." The book struggles to coalesce around a central claim or message of particular intrigue, though Ropper and Burrell still lead the reader a captivating stroll through the concepts and realities of neurological science. (Oct.) (c) Copyright PWxyz, LLC. All rights reserved.

Library Journal Review

It is hard to walk away from this retelling of cases from Ropper's (neurology, Harvard Medical Sch.) experience as a neurologist that speak directly to our understanding of ourselves and those around us. The people he is treating are fascinating, and each case presented is, like the individuals the clinicians treat, unique. The stories are written like a good detective story and read by Paul Boehmer, who expertly voices Ropper and his patients. Describing what it is like to be a neurologist and how diagnoses are made, the book gives readers a glimpse of what patients and their families go through. As with many works by eminent clinicians, there is a bit of self-promotion here, but all of it is well deserved. This work can be listened to whole or a bit at a time. VERDICT Recommended for those interested in neurology, clinical medicine, or, more generally, how the human brain works-or doesn't.-Eric D. Albright, Tufts Univ. Hirsh Health Sciences Lib., Boston © Copyright 2015. Library Journals LLC, a wholly owned subsidiary of Media Source, Inc. No redistribution permitted.



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, did the anesthesia bring it on? My guess is that it was just a coincidence. A straw poll of the team leaned toward a diagnosis of tumor, possibly stroke, maybe a seizure, but they were basing their guesses on Vincent's MRI. I had seen the scans and knew they did not hold the answer. On the other hand, Vincent's wife, who was sitting in an armchair at the foot of his bed, did. "He had a bad headache from the beginning," she told me, "and a fever." The residents had neglected to mention this, but it was important. "How about a virus?" I suggested. "I think this is probably an infection." Herpes encephalitis was my hunch. It would connect the headache and low-grade fever, neither of which fit with a tumor or a stroke. "Ignore the scan for now," I told Hannah. "When there's nothing obvious there, it can be a distraction. Stick with the patient's story and the bedside exam." We started him on acyclovir, an antiviral medication, and he soon improved. Five days later, Vince was discharged, talking normally again, and, for better or worse, just like his old self. "I just ran into your Mr. Talma in the elevator lobby." Elliott, a colleague who seems to keep closer tabs on my patients than I do, had buttonholed me in the corridor outside of the ward. "When I gave him a shout-out," he said, "you'd think I'd asked him to put up bail for the Unabomber. The guy comes in here a pussycat, and when you finish with him he's Mr. What's-It-To-You-Pal. No more smiles, no more jokes. What did you do to him?" "We cured him," I said. "Apparently, that's his baseline. I told his wife that if he started being nice to her again she should bring him back in immediately." * * * I was out on the ward at about 9:30 that morning when the call about Cindy Song arrived from the other hospital. "Is she salivating like she has rabies?" That was my first question, and would turn out to be my only one. "Yes, like a dog," was the reply. "Holy cow!" I said. "It's an ovarian teratoma. You'd better send her over." It was a snap diagnosis, possibly wrong, but there was no harm in raising on a pair of aces. I had a pretty good idea what the other cards would be: memory deficits, gooseflesh, a high heart rate, and no family history of psychosis. The drooling alone was a tip-off. 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. When you see it, the syndrome is unmistakable: an ovarian teratoma stimulates an antibody that will produce the very ensemble of symptoms that were described to me over the phone. Two hours later, when she was wheeled into the ICU, Cindy looked toxically ill, with a heart rate of 135 beats per minute and blood pressure of 160/90. She was sweating, salivating, and shivering wildly. Her eyes were wide open but she was by now entirely unresponsive. Her jittery limbs seemed as if they wanted to convulse. Joelle, the senior ICU resident, Hannah's counterpart down on the ninth floor, immediately intubated her. The toxicology screen from the other hospital was negative, so I called the gynecology service to get an emergency ultrasound of Cindy's pelvis. They thought I was crazy. Moreover, I insisted that they do it transvaginally in order to get a good look at the ovaries. An ovarian teratoma can produce memory loss, seizures, and confusion--what neurologists call "limbic encephalitis," or sometimes the "Ophelia syndrome" (not for Hamlet's beloved, but for the daughter of the neurologist who described a similar condition). The psychotic symptoms are due to autoimmune antibodies that attach to a receptor in the brain, where they simulate the effects of PCP (aka "angel dust" or "wet"). When that receptor became blocked in Cindy's brain--when the antibodies hit their target--all of her symptoms became manifest. She went nuts. "Remove her ovary?" the gynecologist said. "Right. Do you see that cyst on the ultrasound? It's not so benign." I had to insist that there was now no doubt about it: the ovary-brain connection. First--"Who would have thought?" Then--"What do you know? It's a real thing." Eventually, both the resident and the attending gynecologist were convinced, and they were comfortable knowing that Cindy could still have children with her remaining ovary. This was a rare, rare thing. No one fully understands it, but I know it clinically when I see it, or even hear it over the phone, because I collect arcana. If the problem is properly framed, there are very few other things it could be. It took a bit of cajoling, but in the end, they removed her ovary. The sweating, the salivating, and the wild swings in blood pressure were gone within hours. Her psychosis resolved within days. * * * Back on ten, Arwen Cleary, our ice skater with the multiple strokes, had gone deeper into the rabbit hole than anyone else on the ward, and I wasn't confident that we could pull her out of it. According to the notes in her chart, she had by now had three separate strokes, clearly visible on MRI scans, in addition to the vertebral dissection from her neck manipulation. An angiogram had been interpreted as showing vasculitis, an inflammation of the blood vessels. She had a subplural lesion in her left lung, according to the pulmonary specialist. She had a low platelet count, according to the hematologist. "The patient uses humor to cope with her situation," according to the social worker, and on and on for thirty pages of cut-and-pasted notes from more than two dozen doctors who had examined her over the past two months: too many specialists weighing in with too many disconnected analyses, not adding up to a complete picture. Most of her file consisted of blind alleys and misinterpretations. She was in rough shape, virtually blind in her right field of vision, and now aphasic. What worried me was that she didn't have any reserve left, and any little chip-shot stroke was going to be a disaster. The next one, I was convinced, could wipe her out. "I don't see any vasculitis here," I told Hannah. The low platelet count, which if anything would tend to protect against clotting and stroke, was another red herring. "I think the thing to do is just start from scratch. Something is missing. We've got a new team, so just make like she's being seen for the first time, make believe she hasn't been worked up, fill in all the holes. I think there's a single origin of these multiple emboli. That's what it sounds like, that's what it looks like. There's something upstream that's flicking off debris into the blood vessels of the brain, and we just haven't found it yet. If you told me she had a myxoma, I wouldn't be surprised." Something had to be giving off small flecks that lodged on the walls of blood vessels, effectively narrowing them. That was what had caused the strokes, and that was what had been misinterpreted as vasculitis. It was happening now, and would continue to happen, and the most logical source for the flecks had to be a thrombus (a clot of some kind), a tumor (a myxoma or fibroelastoma), or a bacterial growth due to an infection, probably in or near one of the valves of her heart. Yet my residents insisted that there was nothing wrong on the echocardiogram. After sifting through the case file, we finally got around to visiting her. "Hello. This is the neurology team. How are you?" "Not so hot." Unlike most of our patients, Arwen Cleary did not look sick. Not only did she look physically fit, but physically vibrant. Rather than sagging into the hospital bed, she balanced on it like a coiled spring, ready to jump out of it if necessary. At the same time she was shy, somewhat abashed at being here. She had had no visitors for over a day, possibly because she did not want her children to see her like this, or, more accurately, to hear her like this, for although she could talk, she could only do so with halting fluency, mostly in monosyllables. She struggled and usually failed to come up with the longer words that best expressed her thoughts. "I know, it's tough, not being able to express yourself easily." "Oh, yeah. I'm off ... Oh, my gosh!" "The dissections ... I read in the chart that that happened after you got chiropractic treatment. Is that true? "Right. Yes." "How much time elapsed, between the two." "It was ... just a few days." "You know your spirits have been marvelous despite all this. How are you doing it?" "I ... I..." "You stay optimistic." "You have to." She spoke with an unnatural monotone, somewhat like a deaf person, without accenting any of her words. That was the aphasia. She struggled with all but the simplest responses, and settled for tropes. "Do you find yourself getting down sometimes?" I asked. "Some ... times." "Are you depressed?" "No, not depressed ... just sick of all this." "Discouraged?" "Yeah." "Well, thanks for letting us spend some time with you. We're racking our brains to figure out what's going on." * * * Stroke offers the most precise and restricted indicator of damage to the brain that nature produces, and therefore allows an understanding of brain function like no other disease. It is highly "readable," and reading strokes reveals a tremendous amount about the nervous system. One of my professors used to say that the residents learn neurology stroke by stroke. But it is not a simple thing. Six thousand people have a stroke in the United States every day. The numbers are overwhelming. The country has a stroke belt which runs from North Carolina right through Oklahoma. There are genetic factors and dietary ones. Scandinavians have the fewest strokes, the Japanese have the most. 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. My experience told me that Arwen Cleary's echocardiogram had missed something, not just once, but twice. I turned to Hannah after we had left the room, and asked her this crucial question: "Can you put your finger on what's different in this case?" She replied that it was the angiogram, which showed the alarming number of constricted blood vessels and cutoffs in the cerebral arteries. "No," I said. "It's the recurrence of many, many small strokes over time . That's what's different. You have to think about what could cause this. There must be a cardiac source for the emboli. Do the echo over. It was wrong. If it doesn't show something abnormal on one of her heart valves or in one of the chambers, I'll eat my hat." Medical textbooks teach you what tests to do to make a diagnosis, but they do not dwell on the simple reality that humans are interpreting the tests. Hannah ordered the echocardiogram yet again. This would be the third one. The hospital would have to eat the cost. The cardiology fellows initially balked, but when we showed them the echocardiogram from the other hospital, they came around. It was incomplete. By way of proof, the repeat TEE showed a mass sitting on her mitral valve--a papillary fibroelastoma, the second most common benign tumor of the mitral valves, and one which took the shape, as I had predicted, of a peduncular (or branched) growth. I wasn't entirely right. The cardiologists did not think it was consistent with an atrial myxoma, a very different kind of tumor, but at least I didn't have to eat my hat. The course of treatment seemed clear to me: the tumor would have to be removed as soon as possible, before another stroke occurred, and her mitral valve would have to be replaced. Although this seemed to be an answer, when I ran it by the head of cardiothoracic surgery, he balked. "There's too high a risk she'll have a cerebral hemorrhage on the heart pump. We'll have to wait six weeks so her last stroke won't turn into a brain hemorrhage." * * * Alice in Wonderland is an absurdistan story. Beyond fantasy, it's ridiculous. That's neurology in a nutshell. Your patient disappears down a rabbit hole. You've got to do something. You can't just sit there, so you go down the hole after the patient. Sometimes you can do it right away: you go to the gynecologist and say, "Take out her ovary," and that gets her out of the hole. It may not get her back out the same hole she went in, but in a case like Cindy Song's, it gets her out relatively quickly. Same with Vincent Talma. We brought him out, not quite as good as new, with a slight speech deficit that most people wouldn't even notice, but we got him out. With Arwen Cleary it would be a longer journey. Although it went unmentioned at morning rounds, her case would offer a sobering reminder that there are significant limits to our knowledge of diseases of the human nervous system. Anyone expecting a clean resolution and a quick turnaround was in for a disappointment. Arwen Cleary remained on the ward for five weeks. She did suffer a cerebral hemorrhage, but it resolved with almost no consequence. The fibroelastoma that looked so ominous on her TEE somehow disappeared, or perhaps it wasn't a fibroelastoma to begin with. Because of the blood thinner we gave her, she suffered no more strokes. Eventually she went to rehab, and from there she went home. She did not get the heart surgery. She would have to stay on the blood thinners for the rest of her life, and I may never be able to say what caused her problem, or whether it was still a problem, until she suffers the big stroke that wrecks her. Six months later, she came to the outpatient clinic, her speech much better, but still frustratingly limited. Her vision had not fully returned. She was making very slow improvement. "What's your account of what's going on?" I asked her. "I think it stinks." "Are you optimistic you're going to get better?" "Yup." "Can you tell me what you think of your experiences in the hospital?" "I feel like I've never left here. Some days, I can wake up and say, 'Oh, it's going to be a very good day.' Then, it sucks." "What kind of person were you before all these strokes, and what kind of person are you now?" "I ... I was always on the go. Four hours of sleep. Upbeat. Dancing ... Yeah. I used to walk. Now I can't see clear." "That's because you're missing the right side of the world. You might not be aware of it, but your vision on the right is diminished because of one of the very early strokes. Are you a different person now?" "Once I'm home, I'm good. Like, I just..." She trailed off. "Are you very weepy?" "Kind of." "I think this will settle down and there will be a new equilibrium where you're better than you are now. And I hear you, that the dizziness is what's driving you crazy. I know it's frustrating, but your kind of case can't be solved by a book, or it would have been solved by now." * * * "It's not simple," I tell Gilbert, the third-year medical student, "almost nothing is routine, but if at the right moment you can combine experience, logic, and leaps of imagination, you'll get your patients where they need to go." That's the pitch. Gilbert has to decide on which specialty to choose by the end of the year, and that is the extent of the effort I will make to sell him on clinical neurology. Rounding on the ward will either appeal to him or it won't. It's not for everyone. Among the residents on the team, who have already chosen neurology, some will concentrate on research and try to find the causes and cures of Parkinson's disease, Alzheimer's, or multiple sclerosis. Some will go into pediatric neurology. Others will become epilepsy or stroke specialists, some will go into psychiatry. But a few special ones, like Hannah, will carry on the clinical tradition, one case at a time. Back on the ward, she comes up to me with the patient list. I am waiting at the nurses' station with the rest of the team. "Elliott thought you ought to see this lady first before we make rounds," she tells me. "Her name is Mrs. G, and she's making me nervous." "Why?" "She's the lady with the hydrocephalus." In other words, she has too much water in the cavities of her brain, a serious problem. "Lead on," I reply. "I'm at your service." Copyright © 2014 by Dr. Allan H. Ropper and Brian David Burrell Excerpted from Reaching down the Rabbit Hole: A Renowned Neurologist Explains the Mystery and Drama of Brain Disease by Brian Burrell, Allan Ropper All rights reserved by the original copyright owners. Excerpts are provided for display purposes only and may not be reproduced, reprinted or distributed without the written permission of the publisher.