Reaching Down the Rabbit Hole (9 page)

“Who is the president?”

“Eisenhower.”

Caught off guard, Coronna said, “It’s not Eisenhower.
Ford
is the president!”

“What? That’s not possible.”

“I assure you, it’s Ford.”

“Gerald
Ford,” the fox said, “
that
idiot? I went to law school with him. He couldn’t
possibly
be president.”

It was amnesia—Korsakoff’s syndrome—a retrograde and anterograde amnesia, a
permanent
amnesia caused by the low blood flow to his medial temporal lobe during the cardiac arrest. He was now all finished as a lawyer, his memory had stopped, and for him it was 1960. We were stunned. The guy couldn’t remember what had happened, could not retain our names for more than thirty seconds, didn’t remember the girlfriend at all, and had no interest in who she was. She was in her thirties, nice-looking, and when she figured out what was going on, she packed up and left.

His wife had to be told, not every detail of course, and I wasn’t about to do it myself, so I handed it off to a junior resident, a guy from the Deep South named Lamont Schellerman, who possessed an odd combination of New York cynicism and southern gentility. After a long, heated, and one-sided conversation with the wife, he came back to me and said, “Do I need this?”

From that day forward, the silver fox would live in a world of past memories, unaware that he had a problem forming new ones. By way of compensation, like many Korsakoff’s sufferers, he would fill in gaps by confabulating plausible but nonetheless crazy stories. “I think I saw you at the ball park,” he might say to someone he had just met. “That hot dog was great, wasn’t it?” The urge to fabricate experiences probably grows out of a need to save face. Many alcoholics do it in the early stages of the syndrome, and while it is an interesting component of memory loss, it is not a necessary one.

C. Miller Fisher, one of my professors at Massachusetts General
Hospital at the time Godfrey’s car took a spin around Leverett Circle, was the consummate observer. He insisted on reasoning backward from the minutiae of a neurological exam to further his understanding of how the brain works and how disease destroys it. In an obituary I wrote at the time of his death, I called him the grand master of detailed neurological observation. I did not mention the fact that his equanimity was sustained by two extracurricular passions: watching professional football, and the television show
Car 54, Where Are You?
It was Fisher, along with one of my other mentors, Raymond Adams, who had given transient global amnesia its name.

On a Sunday afternoon in November, Dr. Fisher had just settled into his favorite easy chair to watch the New York Giants play the Cleveland Browns when the telephone rang. On the line was an apologetic junior resident who had drawn the short straw, and had been stuck with the job of disturbing the great doctor at home.

“This had better be good,” Fisher said.

It seems that the mother of one of the hospital’s directors had suffered a fall and was confused. The bigwigs insisted that Dr. Fisher come in and see her. So Fisher came in and did what he did best, observe the patient in a fashion that made him both admired and annoying: he sat for three hours and copied everything the woman said verbatim, and then went on to publish an influential paper about the incident.

The case was notable for the fact that the woman had fallen backward off the chair she had been standing on, had hit her head, and when confronted almost immediately by her daughter-in-law—whom she had known for the past twenty-eight years—had said, “Who are you?” It seemed to be a case of concussive amnesia, but, as often happens, no one witnessed a loss of consciousness. The odd thing was that most of the previous year was gone from her memory, along with selective bits of memory going back over thirty years.

During her examination, the woman was alert and conversed readily, if somewhat hesitantly. She gave correct details of her personal
life, including having been born in Lynn, Massachusetts, having left high school in her second year, finishing at night school, then having worked successively at the Preston shoe factory for a year, at the naval shipyard during World War II, at another shoe factory briefly, and then at a Boston bank for fifteen years. She was able to give some details of her marriage, but not of the recent death of her mother and older brother. She gave all of her children’s ages minus one year. She identified Kennedy as the president, and when challenged about it, was unaware of the assassination a year earlier. She had not heard of Barry Goldwater, but was able to give a few of the names of her grade school teachers. Most striking, she could not retain Dr. Fisher’s name for more than thirty seconds, or even recall having been told it. She kept saying, “I think I’ve seen you before.” She gave the date as six months before the actual date, and many answers were given in an uncertain fashion, usually followed by the question, “Is that right?”

Four hours after their first meeting, she was still unable to retain Fisher’s name, but slowly brought into focus the death of her mother and brother. At five hours, she recalled the assassination of the president. One of the most unusual features of the case, similar to transient global amnesia, was that she repeated the same comment with the same inflection each time Dr. Fisher told her his name. “Oh, that’s like my maiden name. I won’t forget it.” (Her maiden name was Fistay.) Other comments she kept repeating were: “What happened to me?” “I think I’ve seen you before.” “Did I fall? I must’ve fallen on my head because I feel a bump.” She improved hour by hour, and after ten hours she was fully oriented to the time, the place, and her situation. Yet on the following morning, the woman could not recall ever having met Dr. Fisher. Her first memory was from ten hours before the concussion, her prior memories having returned, and she could now provide far more accurate details of her early schooling, the principal of her high school, and every one of her schoolteachers.

Amnesia from concussion without loss of consciousness was not unprecedented, and in his paper, Fisher recounted some notable cases
in the literature. The most spectacular took place during the Harvard-Yale football game of 1941. When Harvard got the ball to start the game, the quarterback called an incomprehensible play. His stunned teammates naturally failed to execute it, and the team lost yardage. On the next play, the quarterback repeated the same set of signals, with a similar result. On third down, one of his offensive lineman figured it out: The quarterback was calling a favorite play from four years earlier when they were both on the same prep school team. The old play was stuck in his head. “It developed that on the kickoff,” Fisher wrote, “the quarterback had received an inobvious blow on the head. By the end of the game his memory had returned, but he remained permanently amnestic for the events of the entire game.”

Similar memory losses had been reported by boxers who could remember only a few rounds of fights that had gone the distance. Clearly, it is possible to perform at a high level during an amnestic event, but you might keep calling the same play, or, in a similar vein, get stuck driving round and round a traffic rotary.

By the following morning some of Godfrey’s memory had returned, but in a Swiss cheese fashion: there were significant holes in both retrograde and anterograde memory. This is not consistent with transient global amnesia. If Godfrey did not in fact have TGA, he was then, like the silver fox, in serious danger of losing a significant chunk of his past memories, along with his ability to form new ones. The clock was winding down. If I couldn’t come up with something, I would have to discharge him at noon.

TGA is highly stereotyped. It varies little from person to person. It is one of the few neurological syndromes that has inviolate borders, and Godfrey’s form of memory loss was too spotty, going backward and forward, to fall within those borders. There was also the issue of his awkward gait. I began to worry that low blood flow to his temporal lobes was the true underlying problem, and that he might be at risk of losing a divot out of his brain with a stroke.

In the twenty-third hour of observation, the nurse called me and said that Godfrey’s speech had become slurred, and the pieces fell together. I knew instantly that he was having a stroke. When I walked in, his speech was indeed slurred. He was compos mentis, but when I asked to see him walk, I saw that his coordination had completely fallen apart. Godfrey had an occlusion, an atherosclerosis, a garden variety arterial blockage from a cholesterol plaque upon which a clot had formed. As the clot accreted, it had caused decreased blood flow to the temporal lobes, resulting in an evolving stroke instead of a sudden one. It had most likely started to evolve back in Philadelphia around the time he got into his car.

Godfrey’s story had a happy ending. We gave him an anticoagulant and an agent to raise his blood pressure, and shipped him up to the ICU. He would be fine, and he left with minimal memory trouble. Had I not held him for observation, the stroke could have cost him much of his long-term memory.

“Be very careful about what you call a TGA,” I told the residents that morning. “You’re looking for anything that doesn’t sound right for a fixed period of
complete
retrograde amnesia and
complete
anterograde amnesia.” My guess is that few of them had read Dr. Fisher’s paper. That’s why I brought it up along with Godfrey’s story.

If Godfrey came into the hospital today, the awkwardness of his gait might have been enough to earn him an MRI (which did not exist back in his day), and the stroke might have been evident. Even so, an inexperienced or untutored resident or intern might just say, “No MRI for him. It’s just TGA. Let’s move him along.” Godfrey’s was an uncommon condition that mimicked a common one. In the end, it’s not really the scan, but the painstaking examination, done Fisher-style, that tells all.

As for the Colombian woman, the residents held her in the Emergency Department for a few hours, but having no memory of why she came, and no awareness that anything was wrong, she insisted on
leaving. When Hannah was convinced that her anterograde memory had returned, that it was nothing more than TGA, she discharged her. The hole in her memory would remain, and with it, all memory of her sexual encounter. Fortunately, she had another one scheduled for the following Thursday.

5

What Seems to Be the Problem?

A politically incorrect guide to malingering, shamming, and hysteria

Her name is Lauren H, age twenty-three, white, brunette, five foot seven inches, 129 pounds. Born in North Carolina, she came to Boston as a student at age nineteen, and is currently employed in public relations.

“I understand that you suddenly became unable to speak this afternoon,” I ask her in a rhetorical vein.

“I . . . I . . . I . . . k . . . kkk . . . can . . . can . . . can’t . . . t . . . t . . . t . . . talk.”

“How come?”

No answer. She stares blankly ahead. Her eyes blink a few times.

Hannah takes me aside and says, “She’s aphasic. She must be seizing. Let’s put on a hairline.” A hairline is a quick and dirty electroencephalogram done with an abbreviated set of sticky electrical leads connected to an EEG machine. The object is to find out if she’s in
status epilepticus
, a fancy way of saying that she is seizing uncontrollably. The eye blinking could be a tip-off.

“Okay,” I tell her, “go ahead, and I’ll keep her talking while you’re setting up.”

I turn back to the patient. “Is there any reason you may not be able to speak? Has anything unusual or difficult happened to you today?”

A few tears begin to well up in her eyes, and she shifts her gaze away from me toward the window.

“Is it something you can talk about?”

“N . . . N . . . N . . . No.”

“Does that mean that nothing unusual has happened or that you don’t want to talk about it?”

She pulls the bedsheet up to her nose so that only her teary eyes are showing.

“It’s very important that we talk about this because some of the tests that would be done to sort out why you can’t speak have risks, and it would be bad for you if we did them for no reason.”

A sniffle and a passive look back toward me, but no response. A few absent minutes pass while Hannah gets the material together for the EEG. It’s fairly clear that Lauren comprehends me. This would be quite unlike any true aphasia. For one thing, the well-articulated single syllables that stutter up to a full word are very hard for the brain to do. The language areas in her brain must be calling on all of their powers to produce this bizarre speech pattern. From the first sounds out of her mouth, I conclude that it is very unlikely that we are dealing with damage to her brain from a stroke, seizure, or any other acute problem.

Again I ask, “Did anything unusual happen to you today?” Her sister, who has been sitting passively in a chair at the foot of the bed, now pipes up: “She broke up with her boyfriend this morning. Go ahead and tell them, Lauren.”

“Was that traumatic for you, Lauren?”

“M . . . m . . . may . . . may . . . b . . . be . . . may . . . be.” She sniffles.

“Is that why you can’t speak clearly?”

“I don’t know.”

“That’s very good. You
can
speak in a clear sentence. Can you try to speak to me more clearly now?”

By this point a junior resident has finished hooking up all the leads, and the EEG machine is running. It may be a primitive test, but the brain waves look pretty normal.

“Can I get you to say Massachusetts?”

“Mass . . . Mass . . . Mass . . . massmassmass.”

“How about Boston?”

“Bos . . . boss . . . boss . . . ton . . . ton . . . ton.”

The residents want to get her downstairs immediately for a CT scan and a CT angiogram to see if she’s had a stroke. I suggest they may want to slow down and see what happens, but they feel the stroke issue has great time value. They prevail, and she heads off the floor to get a big dose of radiation. She’ll be gone for a good hour. I go to see some other patients in the meantime. Later that evening I run into Hannah in the hall.

“What happened with the young woman who couldn’t speak?”

“All the studies were normal including her CT angiogram,” she tells me.

I manage to resist saying, “Aha!” On rounds the next morning, I ask, “How are you, Lauren?”

“I’m feeling pretty good. Isn’t it amazing, my speech came back.”

“Yes, it is amazing.”

The majority of hysterical symptoms—symptoms that have no basis in disease and are subject to suggestibility—look like real neurological diseases. These include paralysis, inability to walk or speak, blindness, deafness, seizures, and weakness. All are manifestations of an organ that sometimes fabricates problems. But it gets even crazier. People who cannot feel on one side of the body will say they are deaf on that side, or blind on that side, unaware that this is an anatomical
impossibility. The hardwiring of the human nervous system cannot produce these defects. This is not disease doing something to the nervous system, but rather the brain doing something to itself. The stomach doesn’t have a mind of its own to create stomach problems, nor do the colon, the lungs, or the skin. Ulcers, asthma, psoriasis, eczema, once thought (incorrectly) to be psychosomatic, or originating in the mind, were all shown to have tangible causes, and have been reclassified as nonpsychological diseases.

Only one organ has a mind of its own, and it is constantly causing problems for itself. These problems, once termed “hysterical” and “psychosomatic,” are now called “functional” or “somatoform.” The conditions themselves are referred to as conversion disorders, implying the conversion of psychic distress into physical symptoms. It is one of the last vestiges of Sigmund Freud’s legacy still lurking in mainstream medicine.

Greta B is a thirty-eight-year-old woman, about five foot five inches, well-dressed, a bit overweight. She was referred by a neurologist north of Boston for an unknown type of walking disorder, and has come in with her husband, who is more distraught than she is.

“Hello. Thank you for waiting. I’m Dr. Allan Ropper. I’m at your service.”

“Doctor, you are our last resort. My wife just can’t function like this. It’s been a nightmare.”

“Okay. Tell me how it started.”

“Oh, it’s been going on for a very long time, and it’s getting worse.”

“How long?”

“What do you think, honey? Maybe six months?”

“Oh, longer than that,” she replies. “It’s just that it’s gotten so much worse, and I’m falling all the time, Doctor. You’ve got to do something to help me.”

“Did it start suddenly?”

“Well sort of, but sort of not,” she says, “because at first I caught my toe on the edge of the carpet, and then I began to notice that my legs were buckling going upstairs. That was mainly on the left side, right? And now I can’t even walk down the street or get in and out of a car, because as soon as I start, my knees buckle.”

“Okay, let me see you get out of the chair without using your hands.”

She puts her arms out in front of her, and starts to stand up from the chair, slowly and laboriously, like Frankenstein. Midway through, with her hips and knees bent, she pauses as if starting a dive off a diving board. She then walks forward in duck-step, bent at the hips and knees, like the undersecretary from the Ministry of Silly Walks. When she gets to the doorframe, she grabs both doorjambs, pulls herself to an erect position, and starts to walk down the hall. About every fifth step, her knees suddenly buckle, and she almost falls, like Charlie Chaplin tripping into the sunset, but recovers every time and starts walking again. It puts me in mind of how hard it was as a kid attempting the Cossack dance with my cousins at weddings and bar mitzvahs. Not only does it take a tremendous amount of power and balance to do what she is doing, but it must engage the frontal lobes, cerebellum, basal ganglia, and all levels of her spinal cord. With me in tow, she’s doing her own entire neurological examination, and it is super normal.

The effort required to take her by the arm and help her down the hall and then back to the chair, what with the dips and whirls I have to do to keep up with her, wears me out. She plops down into the chair before I can claim it for myself.

“You’ve just passed the physical, and you’re now cleared for the Olympic pentathalon.” That’s what I want to tell her. Instead I just say, “I think you’re going to be okay.”

Symptoms
are what a patient reports.
Signs
are what a physician sees in an examination. Symptoms are thus subjective, and signs objective. When a patient reports a symptom, we have to take it at face
value: a headache, dizziness, numbness, lower back pain. We have no tests for such things, and accept them as real until something in the patient’s behavior gives the game away.

The claim of blindness, on the other hand, can be tested. People follow the image of their own eyes in a mirror. Not only that, even if they don’t flinch when I bring my hand toward their face quickly, most will involuntarily glance at a $100 bill that I wave in front of them. An old-school neurologist tested this effect. What denomination will get a blind hysteric to follow the bill? A $1 bill doesn’t work; a $100 bill works almost all of the time. So he always carried a C-note in his wallet just for that purpose.

Lucinda H is a Latina female in her late teens, from Roxbury, with short-cropped and spiky hair, a bit blocky. She is half sitting on the edge of an Emergency Department gurney with her elbows propping her up. Healed slash marks on her wrists stand out against her dark skin. Her mother lurks at the bedside.