Reaching Down the Rabbit Hole (3 page)

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.