The Man Who Wasn't There: Investigations into the Strange New Science of the Self (4 page)

But in the devastation are clues to what makes us who we are. These maladies are to the study of the self what brain lesions are to
study of the brain: They are cracks in the façade of the self that let us examine an otherwise almost impenetrable, ongoing, unceasing neural process. And while what follows in the coming chapters is not an exhaustive list of all neuropsychological conditions that disturb the self, I have chosen conditions that satisfied at least two criteria: first, they were amenable to studying some distinct aspect of the self, and second, there is significant ongoing science that specifically addresses these conditions from the perspective of the self.

In Alzheimer’s we get a sense of one’s story unraveling. If you can’t answer the question “Who am I?” with declarative statements (“I am Richard,” “I am a retired professor,” and so on), either because your memory is failing you or the brain regions that let you reflect upon these characteristics are damaged, have you lost your sense of self? If so, have you lost all of it, or part of it? What if, despite the cognitive disintegration of your coherent story—what some call the narrative or autobiographical self—other aspects of you still functioned?

Ralph Waldo Emerson is thought to have suffered from Alzheimer’s. He also wrote eloquently about memory and its role in making us who we are. But
Emerson was curiously indifferent about his own dementia. It’s one of the characteristics of Alzheimer’s disease that sufferers are sometimes unaware of their own condition. Alzheimer’s was the unmaking of his identity, including identifying himself as diseased.

The next chapter examines Alzheimer’s and its role in the undoing of a person, while asking: is some essence of selfhood—despite a ravaged brain in the late stages of disease—preserved in the body? The celebrated American composer Aaron Copland (1900–1990) also suffered from Alzheimer’s disease. At times he wouldn’t know where he was, but
he could still conduct his signature orchestral suite
Appalachian Spring
. Who or what swung the conductor’s baton?

Body integrity identity disorder—a curious condition in which people feel that some part of their body, usually limbs, is not their own, often leading them to the horrific act of severing the body part—gives us a glimpse into how the brain constructs a sense of one’s own body, the bodily self.

Schizophrenia can fragment a person—and part of this fragmentation is due to a compromised sense of agency, the feeling we all have that we are the agents of our actions. What if this feeling—a crucial aspect of the self—goes awry? Could it lead to psychosis?

Then there is depersonalization disorder, which robs the self of its emotional substrate, making us strangers to ourselves, thus highlighting the role of emotions and feelings in creating the self.

Autism sheds light on the developing self. Children with autism are usually unable to instinctively “read” others’ minds, which then leads to problems relating socially to others, but is this ability also tied to reading one’s own mind and hence self-awareness? There’s tantalizing new work suggesting that the roots of this impairment lie in an autistic brain’s inability to make sense of the body and its interactions with the environment, leading first to an uncertain bodily self and then to behavioral problems.

Out-of-body experiences and the more complex doppelgänger effect (in which people perceive and interact with a duplicate of their own body) reveal that even the most basic things we take for granted—being grounded in a body, identifying with it, and viewing the world from behind our eyes—can be disrupted, thus giving us a glimpse of the components necessary for a low-level self that potentially precedes all else.

Ecstatic epilepsy begets a condition that borders on the mystical, when we are truly here and now, fully aware of our own being, yet
paradoxically bereft of boundaries, leading to a feeling of transcendental oneness. Is this condition bringing us closer to the essence of the self—a self that maybe endures for just moments and is at the heart of the debate about whether there is or there isn’t a self?

We conclude with a journey to Sarnath, India, where the Buddha, nearly 2,500 years ago, gave his first sermon. Buddhist ideas of no-self seem to resonate with what some modern philosophers are saying about the self—that it’s illusory. But is it really? Does empirical evidence support the idea that the self is a made-up entity? Insights gleaned from the maladies of the self will help us make sense of age-old questions and maybe even ask a few of our own.

While visiting David Cohen in Paris, I asked him about May, his fifteen-year-old Cotard’s syndrome patient. “Who is it that is saying she doesn’t exist?”

“This is the mystery of psychiatry,” Cohen said. “We always say that there is something . . . that can still relate to the real world, even in the most crazy state.”

In Liège, Steven Laureys’s PhD student Athena Demertzi, who helped Laureys scan and study Graham, told me something about Graham that also reminded me that despite his delusion of being brain dead, there was an essence that remained. Graham had just come out of the scanner when Demertzi asked him, “Are you OK?”

“I’m OK,” replied Graham.

“Alive and kicking?” she asked.

“Kicking,” said Graham, pointedly.

The self is both remarkably robust and frighteningly fragile. This book, I hope, brings to life this essential paradox of who we are.

THE UNMAKING OF YOUR STORY

MEMORIES, A PERSON, A NARRATIVE—AND ITS UNRAVELING

Memory, connecting inconceivable mystery to inconceivable mystery, performs the impossible by the strength of her divine arms; holds together past and present,—beholding both,—existing in both . . . and gives continuity and dignity to human life. It holds us to our family, to our friends. Hereby a home is possible.

—Ralph Waldo Emerson

All those moments will be lost in time, like tears in rain.

—Replicant Roy Batty in
Blade Runner

A
llan, Michaele, and I are sitting in the living room of their home in California. Allan is settled into a large, high-backed, brown leather sofa, looking distinguished with his white beard and mustache and balding pate, and surprisingly dark eyebrows. At first glance I’m unable to tell anything’s amiss. Michaele sits on a chair next to him. I
ask Allan if he has any brothers or sisters. He says no, and then corrects himself immediately. “Oh, I had a brother who was demented,” he says.

“Retarded,” Michaele gently corrects him.

“Retarded,” Allan agrees. “No one knew he was retarded until he was [about] four. I was eighteen. I didn’t understand a lot.”

“But you were ten when he was four,” Michaele says.

“OK,” says Allan.

“Allan, do you remember much about your brother?” I ask.

“A sadness about him,” says Allan. “Because he couldn’t talk and stuff like that. I’d take him for a walk or something like that. He never said a word.”

After a small pause, he adds, “I don’t even know if he’s still alive.”

“No, honey, he died,” says Michaele. “He died the year you and I met.”

Allan and Michaele met nearly thirty years ago. Allan had been a philosophy professor at a community college, Michaele a forty-year-old working as a midwife, back at school after finding herself at a cusp in her life.

“Do you remember how he died?” asks Michaele.

“I thought he died in his sleep or something,” says Allan.

Actually, Allan’s brother had been hospitalized for a blood clot, and while at the hospital he fell out of an upper-floor window and died. At the time, thirty years ago, Allan had told Michaele that his brother, given his diminished mental capacities, would not have had the wherewithal to jump; he had probably wanted to get home and likely stepped out of the window thinking he was on the ground floor.

When Michaele reminds Allan of this during our conversation, he says, “Oh, that’s something I wanted to forget, but no . . . fell out of the window . . .” He mumbles; his words meander.

“What did they say at the hospital?” asks Michaele.

“I was too sad and too young to take it in,” says Allan.

Michaele turns to me and points out that Allan was fifty years old when his brother died.

On December 21, 1995, researchers in Germany found a blue cardboard file that had been missing for nearly ninety years. The file contained the case report for a patient named Auguste D, a fifty-one-year-old woman from Frankfurt.
A handwritten note in the file, dated November 26, 1901, captured an exchange between Auguste and her doctor, Aloysius “Alois” Alzheimer, which the German researchers published in the journal
Lancet
in 1997 (with Auguste’s answers italicized):

She sits on the bed with a helpless expression. What is your name?
Auguste
. Last name?
Auguste
. What is your husband’s name?
Auguste, I think
. Your husband?
Ah, my husband
. She looks as if she didn’t understand the question. Are you married?
To Auguste
. Mrs D?
Yes, yes, Auguste D
. How long have you been here? She seems to be trying to remember.
Three weeks
. What is this? I show her a pencil.
A pen
. A purse and key, diary, cigar are identified correctly. At lunch she eats cauliflower and pork. Asked what she is eating she answers
spinach
. When she was chewing meat and asked what she was doing, she answered
potatoes
and then
horseradish
. When objects are shown to her, she does not remember after a short time which objects have been shown. In between she always speaks about twins.

Three days later, Alzheimer made further notes:

On what street do you live?
I can tell you, I must wait a bit.
What did I ask you?
Well, this is Frankfurt am Main.
On what street do you live?
Waldemarstreet, not, no. . . .
When did you marry?
I don’t know at present. The woman lives on the same floor.
Which woman?
The woman where we are living.
The patient calls
Mrs G, Mrs G, here a step deeper, she lives. . . .
I show her a key, a pencil and a book and she names them correctly. What did I show you?
I don’t know I don’t know
. It’s difficult isn’t it?
So anxious, so anxious.
I show her 3 fingers; how many fingers?
3.
Are you still anxious
Yes
. How many fingers did I show you?
Well this is Frankfurt am Main.

Auguste died on April 8, 1906. By then, Alzheimer had moved from Frankfurt to the Royal Psychiatric Clinic in Munich, so he had Auguste’s brain sent there, where he “
sampled thin slices of this brain tissue, [and] stained them with silver salts.” After affixing these slices between glass slides, “
Alzheimer put down his habitual cigar, removed his pince-nez, and peered into his state-of-the-art Zeiss microscope. Then, at a magnification of several hundred times, he finally saw her disease.”

Summer passed and in the fall, on November 4, Alzheimer presented his findings at the 37th Conference of South-West German Psychiatrists in Tübingen. Auguste, he said, had “
progressive cognitive impairment, focal symptoms, hallucinations, delusions, and psychosocial incompetence.” More to the point, the cells in her cerebral cortex showed weird abnormalities.

The following year, Alzheimer published a paper called “
A Characteristic Serious Disease of the Cerebral Cortex,” in which he detailed the abnormalities. One was found inside neurons: “
In the centre of an
otherwise almost normal cell there stands out one or several fibrils due to their characteristic thickness and peculiar impregnability.” Alzheimer also identified “
miliary foci,” places between cells where he saw aggregates of a strange substance.

It was a new form of dementia. In 1910, Emil Kraepelin, the director of the Royal Psychiatric Clinic, coined the term “Alzheimer’s disease” for such strange cases of dementia, and wrote, “
The clinical interpretation of this Alzheimer’s disease is still unclear. Although the anatomical findings suggest that we are dealing with a particularly serious form of senile dementia, the fact is that this disease sometimes starts as early as in the late forties.”

The abnormalities Alzheimer had identified in Auguste D’s brain were what are now called neurofibrillary tangles and plaques of beta-amyloid protein. While neuroscientists are still debating which comes first—the neurofibrillary tangles or the beta-amyloid plaques (with some wondering whether there are precursors to these neuropathologies)—it’s clear that these aberrant proteins are involved in the ruthless progression of the disease.

If Auguste D had come to see a neurologist today, she would have been diagnosed with Alzheimer’s disease.

Michaele had been working as a lay midwife, helping with home births, in the early 1980s when midwifery was not strictly regulated in California. But with the legal issues surrounding her work becoming more challenging, Michaele decided to go back to nursing school. As part of her studies, she took a class in philosophy being taught by a charismatic fifty-year-old professor. He sauntered into the classroom wearing a leather jacket and large horn-rimmed tortoiseshell glasses, with
white hair and a beard, and discussed philosophy and governments with dramatic flair. “I believe governments should be run by Romanian gypsies and ballet dancers, instead of dictators and greedy politicians,” Michaele recalled him saying. She was mesmerized.

Soon, they began seeing each other (“lots of notes on the door and clandestine meetings after class,” said Michaele). He was in the process of separating from his wife, and drinking a lot; she too was struggling with a bad marriage, which had started disintegrating around the time she went back to school. They both had children. But none of that stopped them as they tumbled into love.

The day I met Allan, I asked him about what Michaele had said, about being blown away by him. “Well, we were both blown the same way,” he said, his voice surprisingly firm and confident. “It was . . .” He then struggled to find the right word. “Things that twirl up in the air.” Tornado, I offered as a suggestion. “Tornado,” he agreed.

Eventually, they bought a house together (the one I visited), got married, traveled together, often to Europe, and remade their lives around each other. Michaele recalled that one of her sons had pointed this out in his toast at their wedding: “It’s always been my mom and Allan against the world . . . They have made it work . . . They have made a life for themselves, despite the challenges.”

Nothing about Allan’s personality had prepared Michaele for what was to come. “When it started happening, I never imagined,
never
, that he’d ever be a person with dementia,” she said.

The first hints came in spring 2003. Michaele and Allan took a weekend break and went up the Eel River in Northern California, and stayed at the Benbow Historic Inn in Garberville. When they came back home on Monday, they found their answering machine overflowing with messages from Allan’s department secretary and students.
Allan had completely forgotten that he had scheduled a final exam that day. It was the first serious indication that something was wrong with his memory.

In September of that year, they went to Europe for a vacation, and Michaele found Allan unable to cope with anything new. He constantly got lost, could not navigate through the French countryside, would put his ATM card into a movie-rental machine, and even had trouble packing his own suitcase.

Back in California, Allan began showing further signs of dementia. He’d forget how to get to his daughter’s home, which was not too far from where they lived. There were other things that seemed amiss. “I’d come home and find him cleaning the hot tub with the circuit breaker still on, which was very dangerous. You can get an electric shock,” Michaele told me. “And when I’d tell him to go turn off the circuit breaker, he would go looking in the garage, when it was on the other side of the house.”

It took a year for them to see a neurologist. Allan failed some standard tests (counting backward from 100 in decrements of 7, for example, which requires the patient to concentrate and is a test for declining cognitive ability), but he still did reasonably well, which the neurologist attributed to Allan’s high intelligence. The MRI suggested some small occlusions of blood vessels. The neurologist diagnosed Allan as being in the early stages of vascular dementia (the decline of cognitive processes due to impaired blood flow in the brain). A few years later, Allan’s diagnosis was changed to Alzheimer’s disease.

Meanwhile, Allan’s personality was changing too. During their entire love affair and married life prior to his diagnosis, Allan had been a kind, sweet man. He and Michaele would have the usual arguments that all couples do, but they would resolve them almost
immediately by talking things through. “He was very present,” Michaele told me.

Not so once Alzheimer’s set in. The slightest argument and Allan would storm out of the house, slamming the door behind him, and go “tearing off in his car.” He also wrote notes incessantly—an old habit that now provided glimpses into Allan’s morphing personality. “Some of them were really mean,” said Michaele. “If I had called him on something, he’d go write a nasty note, ‘Bitch-fest number 5.’” Or simply, “Bitch, bitch, bitch . . .”

The notes also revealed his torment at having been diagnosed with dementia. Michaele recalled one note that said “Get me out of this fucking hole.” Allan read all the books he could find on the disease, and even read
Final Exit
, a book that offered those suffering from terminal illnesses a way out via assisted suicide (he kept the book on the end table near his bed). He told Michaele, “I don’t want to ever end up in diapers. I don’t want to ever end up in a nursing home. You have to take me down to the Bay and push me off the pier.” Michaele knew she would do no such thing. “I couldn’t do that, Allan, I would go to jail,” she recalled telling him. “I could be charged with murder. If you want to do it, I totally get it, but you are going to have to do it. I’d support you, but I won’t help you, at least not do it for you.”