I first met Tyler as the paramedics lifted him off the ambulance stretcher and onto the narrow table for an emergency scan of his head. He had just arrived in the pediatric ER by helicopter. I had become accustomed to meeting new people this way: the human being as a series of brain images attached to a name. Tyler was not aware of our impersonal introduction. He had recently slipped into a coma, and I, as the resident on call that Sunday afternoon, had to figure out why and what to do.
Tyler was a seven-year-old boy whose mother was recently divorced and trying to make a living as a masseuse at the local shopping mall. When Tyler came down with an aggressive ear infection, she drew upon her strong New Age sensibilities and treated the infection with carefully selected herbal remedies. Similar treatments had worked wonders for her kids in the past when they came down with colds or diarrhea (mostly self-limited viral illnesses, I would love to explain to her, and bound to “respond” to time alone). Her children, in fact, had been remarkably healthy, despite her refusal to have them immunized as infants.
What began as a typical childhood ear infection then progressed. Foul-smelling pus began to drain from his ear. She had great trust in her holistic remedies, but a growing apprehension led her to break down and consult with a pediatrician who prescribed a standard antibiotic regimen and urged prompt and complete treatment. (As an aside, while it is true that many ear infections are viral, and therefore do not require antibiotics, not all ear infections are the same.) However, still wary of commercial big-pharma antibiotics and, perhaps, of patriarchal prescriptions, Tyler’s mother decided against filling the prescription and, instead, intensified her herbal remedies. She kept Tyler home from school and dutifully wiped away the pus that formed rivulets running down his neck.
During his mother’s long weekend shifts at the mall, Tyler was entrusted to the care of his teenage brother. On a Sunday morning, several days now into his infection, Tyler woke up later than usual. Confused and lethargic, he barely made it downstairs, clinging to the railing, his eyes half-closed. His brother, roused from the couch in front of the television, found Tyler pale and clammy, babbling incoherently. He phoned his mother and, on her instruction, called the pediatrician, who met them at the suburban office. In the office, Tyler was found to be barely responsive and promptly had a seizure. He was rushed by ambulance to the nearest emergency room where he was intubated to sustain his breathing, and then transported by helicopter to our children’s hospital.
I fixated on the brain images appearing one at a time on the console of the CT scanner. All four ventricles of Tyler’s brain—the cavities filled with cerebrospinal fluid—were markedly enlarged, and his brain was clearly under considerable pressure. The story was clear: his unchecked bacterial ear infection had infiltrated through the inner ear, which lies within the temporal bone of the skull. From here, it is a short distance for bacteria to travel in order to reach the outer lining of the brain, the journey facilitated by the complete absence of antibiotics. Once this lining, known as the meninges, is violated, a raging bacterial meningitis ensues, clogging the normal circulation of cerebrospinal fluid and leading to extreme pressurization within the skull. This rarely happens in the developed world. Tyler was a cute little blond kid from the suburbs who reminded me a bit of my younger brother when he was that age. It was bizarre to think that his innocent brain was under assault from a third-world infectious complication.
Immediately after the CT scan, we wheeled him over to the intensive care unit, where I drilled a small hole through the top of his skull and inserted a thin drainage tube into one of the enlarged ventricles. Cloudy fluid spewed out of the end of the catheter with incredible force. I ordered potent broad-spectrum antibiotics to be administered stat through his IV. The herbal remedies were over. Meanwhile, Tyler’s mother had just arrived at the hospital. The social worker recommended that I go out to the waiting room as soon as I could.
People often ask physicians how we handle the emotional stress of dealing with seriously ill children. What are our defense mechanisms? Do we practice a cool detachment? Do we shut off our emotions completely? Or do we go home at the end of the day and sob over a reheated dinner? I would say that cool detachment does come in handy on occasion, but the answer is really none of the above. The truth is, we are trained to do a job: recognize a problem, come up with a solution, and execute that plan. Our ability to actually
do
something protects us from what you might expect would be a chronic depressive state. We feed off the satisfaction of being able to help and we know that things would be worse if we didn’t, or couldn’t, do anything. For that reason, the experience of taking care of sick kids is much different from a hopeless walk through a pediatric ward as a visitor.
In Tyler’s case, anger took hold before any sadness could even try to creep in. What kind of mother does this? I now had to go out and meet her, this all-knowing herbal shaman. What should I be thinking about her? What crime did she commit: child abuse or child neglect? How should I act toward her: professional, detached, and respectful, or should I promote the feelings of shame and guilt she deserves to feel?
I walked out of the ICU and opened the door of the small waiting room across the hall. She was there by herself. I shook her hand, introduced myself, and explained that I had just drilled a small hole in her son’s head and inserted a tube into his brain. I had to relieve the intense pressure buildup from his aggressive meningitis, which was caused by an untreated ear infection. I added no allegations of abuse or neglect, no calls for guilt or remorse. I was, perhaps, more blunt than usual, but that’s all I could muster. It turns out that, upon opening the door to the waiting room and taking a good look at her, sitting there by herself with the same look I had seen on so many other parents’ faces, my anger was replaced by something else: pity.
Luckily, Tyler woke up within a few hours of the drain insertion. The antibiotics did their job and his meningitis cleared up within days. Still, at that point, he was not yet quite the same kid that he had been. He was a blunted version of the old Tyler, not as quick-witted or as cheerful, according to his family. As much as I am amazed by the normal brain of a child, though, I am equally amazed by its resilience once rendered abnormal by infection, trauma, or other catastrophe. Tyler was sent off to rehabilitation and I had high hopes for his future. Kids tend to bounce back over time. He may not become an A student, but I, for one, was satisfied knowing that he’d at least be a student.
An older, adult brain doesn’t fare nearly as well under duress. His mother’s brain, I was sure, had suffered a long-lasting injury of a different sort, and I couldn’t predict how well she would bounce back.
TWELVE
Slices
Neurosurgeons have a love-hate relationship with the other professionals they depend on. Consider anesthesiologists. We can’t do an operation without them. On one hand, we enjoy the friendly banter back and forth across the sterile drapes, and we like to tease them about doing crossword puzzles at their posts while we’re slaving away. But on the other, such camaraderie doesn’t prevent us from complaining bitterly when they delay our cases with the slow, tortured placement of endotracheal tubes and central lines (even though a patient’s challenging anatomy may be the main culprit). And, anesthesiologists serve as great scapegoats when a case doesn’t go well, even when they have done a fine job and have nothing to do with the complication. “Blame anesthesia,” we might say to all present in the operating room when, for example, the total blood loss is announced at the end of the case and is twice the expected amount.
In a similar fashion, failure to make a definitive diagnosis on a brain biopsy is often blamed on the neuropathologist, even though he has to make do with the quality of material supplied by the surgeon. Garbage in may mean garbage out. This concept is especially critical when it comes to the tiny brain specimens obtained by needle biopsy. There’s not much for the pathologist to work with, so it’s up to the surgeon to obtain the best possible sample.
As one of my mentors puts it, a brain biopsy is “two scared people separated by a needle.” It involves the scared surgeon inserting a long, thin biopsy needle into the brain lesion of an even more scared patient through the smallest of stab wounds in the scalp and a hole in the skull no wider than a pencil eraser. It’s one of the most minimally invasive forms of brain surgery. Very small lesions, deep within the brain—even within the brain stem—can be sampled with the aid of precise localizing techniques. Luckily, it’s quite rare for something to go wrong—like bad bleeding—but we surgeons tend to worry about the rare.
During a brain biopsy, then, the mood in the OR can sometimes be a little tense. If the neuropathologist enters the room after his preliminary inspection of the specimen and requests more tissue, he may receive a sharklike glare from the surgeon no matter how politely the request is made.
After spending a couple years at the safer end of the needle, the time had come for me to play the much-abused soul receiving these tiny biopsy specimens of questionable quality in plastic petri dishes from the OR. During our required four-month rotation in neuropathology, we learn to be gentler to our neuropathology colleagues. We see that it’s not always easy to make a diagnosis, especially when the surgeon doesn’t give you much to work with. We learn the basis of staining techniques and microscopic diagnosis, and we participate in “brain cutting” autopsy sessions in which the specimens are much larger—the entire brain—and the surgeon is out of the picture.
The world of the neuropathologist is different from the world of the neurosurgeon. For better or for worse, there is no personal patient contact. This shields the neuropathologist from a certain type of stress pervasive in most other medical specialties. Also, because they have no direct patient contact, they can wear pretty much whatever they want. Some even get away with wearing jeans to work.
On a more serious note, the concept of hedging your bets is not necessarily frowned upon in neuropathology. A biopsy report might read something like: “could be A but cannot rule out B.” This type of report drives a surgeon nuts. Neurosurgeons, on the other hand, are often ridiculed for being “frequently wrong but never in doubt.” They don’t do well with doubt or indecision.
Many neurosurgery residents merely tolerate this four-month requirement. They miss the OR, are bored by the pace, and feel a bit out of place in the nonsurgical milieu. I thoroughly enjoyed it. Often, I was intrigued by the aesthetics of what I saw under the microscope, the best of it truly worthy of display in a Chelsea gallery of abstract art. Equally, though, I loved the sane hours, which gave me plenty of time to immerse myself in popular culture, enjoy magazines and books again, see movies, check out the new restaurants I had missed, and spend time outdoors during daylight hours. As a cultural anthropology major in college I learned that leisure is the basis of culture, and I definitely felt more cultured during these leisurely four months, if only as part of popular culture and in catching up on who was dating whom. Neurosurgery residency has a way of making the outside world seem foreign at times, and this nice, easy rotation allowed me to feel like a native again.
Apart from enjoying the hours, hedonism, and aesthetics, I was lucky to work with a brilliant Cuban-born senior neuropathologist who maintained a fresher sense of wonder about the brain than anyone else I had worked with before, even though he was nearing retirement. In studying specimens with him under the multiheaded teaching microscope, I was delighted to hear his frequent “Look…look how
be-zarrrre
!” as his eyebrows rose up above the rims of the eyepieces. He was a patient teacher. If I didn’t understand what I was looking at, I could anticipate the “Look…look, I show you,” as he explained a subtle finding for the third time. He would marvel at rosette formations, perfect mitotic figures, and double nuclei, and I would find myself marveling at all those little things with him.
There were many stories of his uncanny diagnostic skills, and it was hard to know which were apocryphal. Before starting the rotation, I was told by a senior resident that this guy could diagnose a meningioma (a benign brain tumor) “by smell alone.” I found this claim hard to believe at first, but thought it plausible once I had gotten to know him. While I can’t vouch for his olfactory skills, I can say he was an absolute master of the slick “touch prep” technique.
1
This method for rapid preliminary diagnosis was perfect for the tiny needle-based specimens as it involved simply touching the sample very lightly to a glass slide, thus preventing any artifact from freezing, crushing, or smearing the tissue as in other methods, and allowing the maximum amount of specimen to be left for permanent studies and special staining. He could glean all sorts of things from examining the individual cells that stuck to the glass. In contrast to some of his colleagues, he was more likely to deliver a solid, definitive diagnosis to the surgeon waiting in the OR with a needle in someone’s brain: “This is A.”
This professor had made his mark in the neuropathology world decades earlier for his work on a little parasite, an amoeba known as
Entamoeba histolytica,
a so-called free-living amoeba, which can invade the brain in certain cases. He had perfected his neatly packaged talk on the topic, notable mostly for the historic-appearing slides and the fact that this was a disease we would probably never see in our own country anymore with our sanitation standards and well-filtered water. Nonetheless, this tiny independent amoeba had helped secure his place in academia.
During my four months of learning and leisure, a two-year-old rhinoceros died at the local zoo. Nobody knew why. A full-body autopsy was performed at the zoo, but the brain was set aside and handed over to this senior sleuth for a specialized opinion. Right off the bat, we were puzzled by a clean slash through part of the cortex, and the zoo official explained in a matter-of-fact way that a chain saw and crowbar were required to open the skull, which was reportedly half a foot thick, so the process wasn’t exactly delicate. With that, we had no further questions and got to work.
We took the fresh brain and weighed it: 650 grams, considerably less weighty than a human brain, which is usually around 1300 to 1400 grams, or about three pounds, in an adult. (To be fair, though, I don’t know if a two-year-old rhino’s brain would be considered adult size yet.) Neither of us had ever seen a rhino brain. We could roughly make out most of the structures—they were similar enough in location, but not exactly in size and shape—to a human brain. We were stuck, though, on the strange flaplike appendages protruding from part of the cerebellum, or hind part of the brain. There were no correlates in the human brain and I was inspired to find out what they were.
No one really kept tabs on my whereabouts during long tracks of the day, so I felt comfortable spending a few hours in the library looking for anything I could find on rhino brain anatomy, for anything that might link form to function. I found a few sparse references, but nothing detailed enough to answer my pressing question. In the end, the rhino case, although a fun diversion, proved ultimately frustrating: no clear answer as to cause of death, and no clues as to the what and why of those strange appendages.
In my animal brain research, though, which had become more wandering by the hour, I was enlightened to a tangential yet interesting curiosity regarding the brains of household pets: veterinarians will actually operate on brain tumors in cats and dogs, and the owners of those cats and dogs will gladly shell out cash for these delicate and expensive operations. (Are these the same humans who grumble about their own co-pays and hospital bills?) I wondered if I should have gone to vet school instead.
Near the end of my four-month rotation, our Cuban neuropathologist threw me a curveball. A group of residents was sitting around in the microscope room, shooting the breeze about the brain, primates, and evolution. I asked him his thoughts. He answered: “Evolution I don’t believe in. How could it be true? I don’t believe.” His answer was as definitive as: “This is a glioblastoma. Period.” I filed his creationist beliefs away as a personal quirk, and respected his willingness to speak his mind despite going against the grain. When it comes to the fundamental questions, I guess you can’t make assumptions, even when dealing with a scientist and fellow brain-lover.
Incidentally, some scientists believe that the human brain is still evolving.
2
This theory is based on population studies of two genes, both known to be somehow involved in brain size. A variant (or “allele”) of one of these genes, known as ASPM, likely emerged in the human brain for the first time only about 5,800 years ago—relatively recently on an evolutionary scale—and is already present in about half of certain populations. This means that the new gene has spread quite rapidly.
One controversy among many, though, is whether or not its spread is truly a reflection of the natural selection of evolution. It could possibly be via a more random process known as genetic drift, whereby certain individuals, or populations, leave behind more offspring—thus, genes—by chance. Also, given the fact that human culture, rather than brute biology, plays such a strong role in who survives for how long, how well, and passes genes on to how many offspring, the idea may be a stretch. Furthermore, we don’t even know how important this gene is. Although its mutated form can affect brain size, the normal form may not. Apparently, the average human brain size has not increased any further over the past two hundred thousand years.
A couple years after my four-month rotation, I went back to my neuropathology friend’s office to ask him for a favor. I needed to borrow a few brain slices. I was preparing for a lecture about the brain that I was going to give to an undergraduate class and I figured it would be helpful to pass around the real thing as a hands-on demonstration of look and feel. He was happy to oblige.