In his decades as a neurologist in Boston, Allan Ropper ’70, MD ’74, has seen his share of challenges and curiosities. There was the salesman who drove around the same traffic circle for an hour, then told the cop who finally pulled him over that he didn’t know how he got there. And the recent college grad whose violent arm tremors had no physical cause. And the elderly man who obsessively scribbled pages of incomprehensible notes and insisted that Ropper had been a football star at BU. (He hadn’t.)
Ropper, a Harvard professor whose patients include actor Michael J. Fox, revisits those cases and more in Reaching Down the Rabbit Hole: A Renowned Neurologist Explains the Mystery and Drama of Brain Disease, published in late September by St. Martin’s Press. In the same “narrative nonfiction” genre as works by Oliver Sacks, the book chronicles life on the front lines of neurology, which still relies heavily on personal observation even in the age of high-tech scans such as the MRI. “It’s a specialty in which the clinician can be of tremendous value,” observes Ropper, a former math major who co-authored the widely used textbook Principles of Neurology. “You have to communicate with another individual’s brain on a level that isn’t always tangible, using your cleverness, experience, and understanding of human nature. It’s ever-changing and infinitely interesting.”
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.”
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 Ross, the chief resident, 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.
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 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.
“Doctor, she can’t see! Oh my God, she’s blind, she can’t see! Dios.”
That’s the first red flag: Why is her mother speaking for her and why is the girl so calm?
“When did it start?”
“When I came over this morning to pick up the baby. She told me she couldn’t see.”
“It would be good if I could hear what happened from Lucinda. How about it, Lucinda, what exactly happened?”
“I don’t know . . . I’m blind. Isn’t that enough?”
“I know it must be frustrating to tell your story over and over to every doctor who comes in, but it’s important that I hear the details so that we can get your vision back.”
“I can’t see anything. How would you feel if you were blind? Tell me that, okay? It’s like I’m blind.”
“Are you blind in both eyes?”
“That’s what being blind means, doesn’t it?”
“What were you doing when it started?”
“Can you see my hand in front of your face?”
“Okay, let me hold your arm and get you up to see how you walk.”
I ease her off the gurney, and her feet hit the ground naturally. Without being asked, she makes a left turn and heads for the wall and bumps into it at full stride, but manages to stick her belly out just before hitting, so that neither her head nor knees make any contact. Even before this, I was thinking, Ho boy! Now I’m thinking, Oy, vey!
I help her back onto the gurney.
“Let’s try some other tests. Follow my finger.” She stares blankly ahead as I move my finger back and forth. I pull a small mirror out of my bag, and move it from left to right in front of her face about a foot away. Her eyes follow their own images in the mirror. It’s gimmicky, like the $100 bill trick, but seeing eyes almost always follow the mirror.
“You know,” I say to her, “I think you can see, but for some reason, maybe one you don’t want to talk about now, you are just upset or distracted and are shutting down your vision.”
“Well, you’re the crazy one, so screw you.”
Oy, vey, indeed.
“I’m not saying you are crazy, just that something is bothering your brain in a way that is beyond your control.”
Her mother blurts out, “So what are you going to do about it? I can’t take her home like this!” Hands are waving all over the room as the mother begins to pace alongside the stretcher, invoking the gods and the prophets at a high pitch. The temperature seems to be rising. I need to leave for a few minutes to cool off.
That’s the extent of my plan.
Most of these patients would rather see a neurologist than a psychiatrist anyway. In their minds, they are sick but not insane.
Nomenclature: Hysteria, psychosomatic, and pseudoseizure are OUT. Neurologists still use these words all of the time, just not in front of patients and their families. Other words we take pains to avoid are psychiatric and psychiatrist. People tend to hear these as crazy and shrink, and this rarely goes over well with anyone. Terms that are IN include: conversion disorder instead of hysteria, functional instead of psychosomatic (the two are not equivalent in any case), and psychological non-epileptic seizure—or P-NES (I kid you not)—instead of pseudoseizure, as in, “This lady has a P-NES.” That’s now a term of the art. It was coined either by someone with a very devious sense of humor, or no sense at all.
Susanna B is a nineteen-year-old woman from a devout Pentecostal family living in Plaistow, New Hampshire. She has just started nursing school. She is surrounded by five family members, all seated around her bed, including a hulking brother who has taken the lone easy chair, is fiddling on his laptop, and never once looks up from the screen.
As I rush into the room with the residents, Susanna’s arms are shaking, her whole body is shaking. Clearly this is a lovely young woman, but just as obviously, she’s in distress. She is fluttering her eyelids at about twice per second, her eyeballs are rolled upward, and her neck is arched backward.
“Susanna!” I say to her. “I’m Dr. Ropper. Can you hear me?”
Her violent movements continue, and her mother eventually stands up and leans over the side of her bed, getting her face as close to mine as she can, and says, “Why aren’t you stopping this? We’ve been here for hours, and we are going to call the patient-care representative. This is unacceptable. We have a lawyer we can talk to.”
“You’ll have to give me some time to sort this out. It might help me if I could examine her without so many people in the room. Would you mind giving me a few moments alone with her?”
“No way! We’re not leaving her alone. Who knows what might happen? She’s a virgin, you know.”
Wow! That is not high on my list of diagnostic questions, but it raises an entirely new concern. I want them out because this exotic motor performance is characteristic of a pseudoseizure, undoubtedly triggered by the unnerving family dynamic. The quickest way to stop it, I’m convinced, is to send the audience out to the lobby. The neck and back arching, in particular, are not characteristic of most epileptic seizures. Rapid eye blinking can be part of a true seizure, but the way she’s fluttering her eyelids suggests otherwise. When the spell finally stops, I speak to her, and find that she is naturally calm, soft-spoken, and quite polite. She knows that the spells are happening, but has no control over them. They were occurring up to several times an hour prior to her admission, and have become totally disabling over the last several days.
From dribs and drabs I pick up from the family, I find out that she represents a great hope for the entire clan. Everything is riding on her success, and they make this expectation very clear at the young woman’s bedside. The virginity thing comes up again for no apparent reason, and it gives me the creeps. This is one of the most controlling families I’ve ever seen.
“Susanna, these are not epileptic seizures. They usually come from . . . “
“What are you saying, Doctor?” The mother again. “We’d like someone else to see her immediately, and if they don’t, we will be speaking to people who can make that happen.”
“As I was saying, Susanna, it is very important to start by making the correct diagnosis. If we felt that this was an epileptic problem, it would be treated very aggressively with medicines for seizures, but it is not. Somehow, this pattern of motor behavior has gotten into your head, and taken on a life of its own. After a while, movements like this seem to appear without you willing them. Knowing this is very powerful, since it opens up some ways of unlearning the movements, and eventually getting rid of them by retraining the mind.”
She tells me that the spells come out of nowhere, have no clear trigger, continue for days, sometimes lasting a minute, other times up to an hour, then suddenly go away. It is very telling, however, that they do not interrupt her sleep, and the video monitor attached to her continuous EEG recording will confirm it. The spells exhaust her, but she lacks any insight into their character. The family gets more and more aggressive, and on two occasions I’m on the verge of offering to transfer her to another hospital, particularly when they suggest they might sue me for malpractice. This is one of the few times that I feel more anger than sympathy toward an anguished family. I take the virgin business, coming up so repeatedly, to be both an admission and a warning: we know that childhood sexual abuse is closely connected to these types of dissociative states, and they are saying, “Don’t you dare go there.” I’m not going to bother.
The spells stop on the fourth day, and we send her home over the forceful protests of the family that there had been no resolution. They refuse to accept my diagnosis of conversion disorder. I don’t hear from them for about five months, when she returns with the same problem, to us no less, requiring another three-day hospitalization.
The Brits call this sort of thing Functional Neurological Symptoms, or FNS, the psychiatrists call it conversion disorder, and almost everyone else just calls it hysteria. There are three generally acknowledged, albeit uncodified, strategies for dealing with it. The Irish strategy is the most emphatic, and is epitomized by Matt O’Keefe, with whom I rounded a few years back on a stint in Ireland.
“What are you going to do?” I asked him about a young woman with pseudoseizures.
“What am I going to do?” he said. “I’ll tell you what I’m goin’ to do. I’m going to get her, and her family, and her husband, and the children, and even the feckin’ dog in a room, and tell ’em that they’re wasting my feckin’ time. I want ’em all to hear it so that there is enough feckin’ shame and guilt there that it’ll keep her the feck away from me. It might not cure her, but so what? As long as I get rid of them.” This approach has its adherents even on these shores.
The English strategy is less caustic, and can best be summarized by a popular slogan of World War II vintage currently enjoying a revival: “Keep Calm and Carry On.” It is dry, not overly explanatory, not psychological, and does not blame the patient: “Yes, you have something,” it says. “This is what it is [insert technical term here], but we will not be expending our time or a psychiatrist’s time on it. You will have to deal with it.”
Predictably, the American strategy holds no one accountable, involves a brain-centered euphemistic explanation coupled with some touchy-feely stuff, and ends with a recommendation for a therapeutic program that, very often, the patient will ignore. In its abdication of responsibility, motivated by the fear of a lawsuit, it closely mirrors the beginning of the end of a doomed relationship: “It’s not you, it’s . . . no wait, it’s not me, either. It just is what it is.” Not surprisingly, estimates of recurrence of symptoms range from a half to two-thirds of all cases, making this one of the most common conditions that a neurologist will face, again and again.
“I’m not saying you’re crazy, just that something is bothering your brain in a way that is beyond your control.” This is the basis of all non-Irish appeals. “The brain learns these patterns, sometimes they’re hard to unlearn, but it’s important to know that there’s no damage to your brain going on.” It doesn’t blame the patient, doesn’t give a psycho-dynamic explanation, but does give the patient an out.
“This pattern can be unlearned. It is within your power.” That’s the moderate tack, not necessarily the American one, because the American sensibility obliges us to add: “Why don’t we sit down and find out why you are doing this.”
If you pick up any conventional psychiatry book, it will advise you that there is a psychodynamic explanation, or there is a genetic susceptibility, and that by identifying the underlying psycho-problem, talking about it, getting it out in the open, and realizing what it has done to you, you can get rid of the symptoms. If the psychiatrists want to handle it that way, fine by me, but I’d rather not. Besides, most of these patients would rather see a neurologist than a psychiatrist anyway. In their minds, they are sick but not insane. Most of all, they resent the implication that they have a weak character, that they are faking an illness because they can’t deal with their lives. I happen to think nothing of the sort, but how do I tell them?
In many cases of hysteria, the ideal treatment would be hypnosis. We used it when I was a resident, and it worked, just as it worked for Sigmund Freud and his teacher, the French neurologist Jean-Martin Charcot over a century ago. It worked because patients with hysterical symptoms are suggestible, and, having fooled themselves into the symptoms, they can be fooled out of them. Deception works, but in the modern age, in the age of informed consent, we are not allowed to fool patients about anything, even if it is the only way we can help them.
From REACHING DOWN THE RABBIT HOLE by Dr. Allan H. Ropper and Brian David Burrell. Copyright © 2014 by the authors and reprinted by permission of St. Martin’s Press, LLC.