In the late 1980s, French Canadian pediatric neurologist Dr. Guillaume Sébire noticed an unusual pattern among six children he treated from 1982 to 1990.51 They all had movement disorders, including involuntary tics or excessive restlessness, cognitive impairments, seizures, normal CT scans, and negative blood work results. The children were diagnosed with “encephalitis of an unknown origin” (or what was colloquially known as the Sébire syndrome), a disease that lasted on average ten months. Four of the six children made what could be called a full recovery. His hazy description of the disease persisted for another two decades.
An earlier paper, written in 1981 by Robert Delong and colleagues, described “acquired reversible autistic syndrome” in children.52 The disease presented like autism, but two of the three children studied (a five-year-old girl and a seven-year-old boy) recovered fully, while an eleven-year-old girl continued to endure severe memory and cognitive deficits, unable to remember three words provided to her after only a few minutes had elapsed. Now, studies show that roughly 40 percent of patients diagnosed with this disease are children (and this percentage is growing), but children present the disease differently from adults: afflicted children exhibit behaviors such as temper tantrums, mutism, hypersexuality, and violence.53 One parent described how her child tried to strangle her infant sibling; another heard low grunting noises from their normally angelic daughter; and another child clawed at her own eyes to communicate the inner turmoil that her toddler vocabulary could not convey. The disease in children has often been misdiagnosed as autism, but depending on where and when the person lived, it might have been described as supernatural, even something evil.
Evil. To the untrained eye, anti-NMDA-receptor autoimmune encephalitis can certainly appear malevolent. Afflicted sons and daughters suddenly became possessed, demonic, like creatures out of our most appalling nightmares. Imagine a young girl who, after several days of full-bodied convulsions that sent her flying into the air and off her bed—and after speaking in a strange, deep baritone—contorted her body and crab-walked down the staircase, hissing like a snake and spewing blood.
This chilling scene is, of course, from the unedited version of the blockbuster film The Exorcist, and though fictionalized, it depicts many of the same behaviors that children suffering from anti-NMDA-receptor autoimmune encephalitis do. The image is not as exaggerated as we might think. (Stephen, for one, can no longer watch The Exorcist; it brings him right back to those strange “panic attacks” I experienced in the hospital, and to my first seizure as we watched TV on the pullout couch.) In 2009, a thirteen-year-old girl from Tennessee displayed a “range of emotions and symptoms that varied by the hour, at times mirroring schizophrenia, and, at other times, autism or cerebral palsy.”54 She lashed out violently and would bite her tongue and mouth. She once insisted on crab-walking across the hospital floor. She also spoke in a bizarre, Cajun-inflected accent, according to the Chattanooga Times Free Press, which detailed her experience with anti-NMDA-receptor autoimmune encephalitis and subsequent recovery.
Many parents report that their children start speaking in a garbled foreign language or with an unusual accent, just like when the fictional Regan in The Exorcist begins to speak fluent Latin with the priest who has come to exorcise her. Likewise, those who suffer from this type of encephalitis will display what is known as echolalia, the repetition of sounds made by another person.55 That would explain the sudden ability to “speak in tongues,” though in real life those who are suffering from the illness typically do so illogically, not fluently.
How many children throughout history have been “exorcised” and then left to die when they did not improve? How many people currently are in psychiatric wards and nursing homes denied the relatively simple cure of steroids, plasma exchange, IVIG treatment, and, in the worst cases, more intense immunotherapy or chemotherapy? Dr. Najjar estimates that 90 percent of people suffering from this disease during the time when I was treated in 2009 went undiagnosed. Although this number is probably decreasing as the disease becomes better known, there are still people who are suffering from something treatable and not receiving the proper intervention. I couldn’t forget how close I had come to such a dangerous edge.
When I contacted her about my research, Dr. Dalmau’s colleague Dr. Rita Balice-Gordon brought up the old Indian proverb, often used by neuroscientists studying the brain, about six blind men trying to identify an elephant, offering it as a way of understanding how much more we have to learn about the disease.
Each man grabs hold of a different part of the animal and tries to identify the unnamed object. One man touches the tail and says, “rope”; one touches a leg and says, “pillar”; one feels a trunk and says, “tree”; one feels an ear and says, “fan”; one feels the belly and says, “wall”; the last one feels the tusk and is certain it’s a “pipe.” (The tale has been told so many times that the outcomes differ widely. In a Buddhist iteration, the men are told they are all correct and rejoice; in another, the men break out in violence when they can’t agree.)
Dr. Balice-Gordon has a hopeful interpretation of the analogy: “We’re sort of approaching the elephant from the front end and from the back end in the hopes of touching in the middle. We’re hoping to paint a detailed enough landscape of the elephant.”
Two particular fields of study, schizophrenia and autism, will likely gain the most from this landscaping of the elephant. Dr. Balice-Gordon believes that a percentage, albeit a small one, of those diagnosed with autism and schizophrenia might in fact have an autoimmune disease. Many children ultimately diagnosed with anti-NMDA autoimmune encephalitis were first determined to be autistic. How many children originally diagnosed with autism weren’t able to find their autoimmune diagnosis?
As she explained, out of a hypothetical 5 million people diagnosed with autism, 4,999,000 of them might indeed be autistic. But what about that tiny slice that in fact have anti-NMDA-receptor encephalitis or one of the other related disorders, and could be effectively treated by looking for a peripheral tumor or antibodies in the brain?
The same goes for schizophrenia. Many of the adults ultimately diagnosed with anti-NMDA-receptor autoimmune encephalitis first receive the diagnosis of schizophrenia (or other related mental disorders, such as schizoaffective disorder, in my case). Statistically there must be some people who receive a diagnosis of psychosis or schizophrenia and never get the proper help. Even if it’s only 0.01 percent of patients, it’s still too many.
Unfortunately, for most people suffering from severe psychiatric conditions, it’s nearly impossible to give everyone the proper testing to diagnose and treat autoimmune diseases. PET scans, CT scans, MRIs, IVIG treatment, and plasmapheresis can cost upwards of thousands of dollars each.
“How practical would this screening be?” asks professor of psychology Philip Harvey. “Lumbar punctures for everyone? That’s an impossibility.”
It had cost $1 million to treat me, a number that boggles the mind. Luckily, at the time I was a full-time employee at the Post, and my insurance covered most of the exorbitant price tag. I also had a support system in place. My family was in the fortunate situation of being able to pay out of pocket anything that the insurance company wouldn’t cover or reimburse. Unfortunately, there’s often not the same safety net in place for those with lifelong psychiatric conditions, who are unable to hold jobs and must make do with disability payments and Medicaid.
But this is all the more reason that psychiatrists and neurologists are finding ways to break down the barriers set in place between psychology and neurology, urging for one uniform look at mental illnesses as the neurochemical diseases that they are, and, in the process, perhaps getting more grant money to study the overlap.
“One thought is that this is just a coincidence, that [NMDA-receptor encephalitis] and schizophrenia are unrelated. But Mother Nature doesn’t work that way. The best hypothesis for schizophrenia is that at least some of those cases can also be explained by a [similar] dysfunctio
n,” said Dr. Balice-Gordon.
Dr. Najjar, for one, is taking the link between autoimmune diseases and mental illnesses one step further: through his cutting-edge research, he posits that some forms of schizophrenia, bipolar disorder, obsessive-compulsive disorder, and depression are actually caused by inflammatory conditions in the brain.
Dr. Najjar is in the midst of groundbreaking work that might finally sever the barrier separating immunology, neurology, and psychiatry. A recent case of his centers on a nineteen-year-old woman who had been diagnosed with schizophrenia by six leading psychiatrists over the course of two years.56 When she was seventeen, her symptoms began with auditory hallucinations—“people putting me down and thinking they’re better than I am,” she told Dr. Najjar—followed by visual ones. Late at night, she would see “people’s faces on the walls.”
Her parents did not believe the schizophrenia diagnosis and eventually made their way to New York University, where they met with Dr. Najjar. He ordered a right frontal brain biopsy—something he had learned from my case—that showed the presence of inflammation and antibodies targeting the glutamate receptors in the brain. She was treated with steroids, plasma exchange, and IVIG treatment, which helped with the hallucinations and paranoia, but because the treatment was started so late, it is unclear if she ever will return to her former self.
“Just because it seems like schizophrenia doesn’t mean that it is,” Dr. Najjar told me. “We have to keep humble and keep our eyes open.”
As I researched my article, I was curious to get the perspective of Dr. Bailey, the neurologist who had asserted that my problems stemmed from alcohol withdrawal and stress, to see what he thought about the ultimate diagnosis. When I reached him by phone, though, it turned out he still had never heard of the illness, even though my diagnosis had been discussed in almost every major medical journal, including the New England Journal of Medicine, and the New York Times.
In the spring of 2009, I was the 217th person ever to be diagnosed with anti-NMDA-receptor autoimmune encephalitis. Just a year later, that figure had doubled. Now the number is in the thousands. Yet Dr. Bailey, considered one of the best neurologists in the country, had never heard of it. When we live in a time when the rate of misdiagnoses in the United States has shown no improvement since the 1930s, the lesson here is that it’s important to always get a second opinion.57
While he may be an excellent doctor in many respects, Dr. Bailey is also, in some ways, a perfect example of what is wrong with medicine. I was just a number to him (and if he saw thirty-five patients a day, as he told me, that means I was one of a very large number). He is a by-product of a defective system that forces neurologists to spend five minutes with X number of patients a day to maintain their bottom line. It’s a bad system. Dr. Bailey is not the exception to the rule. He is the rule.
I’m the one who is an exception. I’m the one who is lucky. I did not slip through a system that is designed to miss cases just like my own—cases that require time and patience and individualized attention. Sure, when I talked to him, I was shocked that he knew nothing about the disease, but that wasn’t the really shocking part; I realize now that my survival, my recovery—my ability to write this book—is the shocking part.
Yet even after all of this, the most harrowing part of researching and writing the article about my illness was something that I had in no way prepared for: handing over the EEG tapes to my paper’s photo editor, who wanted to use some images of me in the hospital for the piece. I hadn’t yet watched them and at that point did not plan to.
But when he had trouble opening up the disk, he asked me for help. I got it to work and in the process caught a fleeting glimpse of myself in the hospital gown. I was outrageously skinny. Crazed. Angry. Reaching out aggressively toward the camera.
I shuddered and turned away from the image, trying to concentrate on breathing as I forced a smile. I had the intense urge to grab the videos from him and burn them or at least hide them away, safe from view. Even after everything I’d done and learned, maybe I wasn’t ready for this yet. Yet I felt compelled to keep watching.
I had enough distance from my own madness to view it as a hypothetical. But watching myself on screen, up close and personal, obliterated that journalistic distance. The girl in the video is a reminder about how fragile our hold on sanity and health is and how much we are at the utter whim of our Brutus bodies, which will inevitably, one day, turn on us for good. I am a prisoner, as we all are. And with that realization comes an aching sense of vulnerability.
That night I went home and passed a night of fitful dreams that blurred together. In one, I was with my mom and Allen in Summit.
“Remember when you were in the hospital,” my mom said, laughing really hard. “You were so crazy that . . .”
She was laughing so hard that she couldn’t complete the sentence.
“What happened?” I asked, grabbing a notebook and a tape recorder. She was laughing, gulping in air, too hysterical to talk, still laughing.
There was a second dream that blurred together with this first one. In it, I was on the epilepsy floor, but I was completely naked and in search of a bathroom to hide in. I heard a group of nurses walk by and tried to hide, but as I turned the corner, all of a sudden I saw Adeline, the Filipino nurse from the floor. Now I was fully clothed.
“Susannah,” she said. “I hear you’re not taking care of yourself. What a shame.”
Though I hesitate to draw any Freudian meanings from these dreams, they clearly represent the anxiety I felt about how I behaved in the hospital and how others perceived me during my recovery. This was not where I wanted to be psychologically as I started working on my first major assignment back at the Post. I didn’t want to be frazzled and upset, and these tapes had obviously upset my internal balance.
But, ready or not, on Sunday, October 4, the biggest story of my career ran in the Post under the headline: “My Mysterious Lost Month of Madness: I was a happy 24-year-old suddenly stricken by paranoia and seizures. Was I going crazy?”
CHAPTER 48
SURVIVOR’S GUILT
It is one thing to research your own condition and think abstractly about the other people who have suffered from the same condition; it’s another thing entirely to get to know the people themselves who have run the risk of being lost in the system.
Because I had been the only person ever to be diagnosed with anti-NMDA-receptor autoimmune encephalitis at NYU, I had felt as if I was in a rarified group of the walking wounded without any compatriots with whom to share war stories. I was wrong.
Although anti-NMDA-receptor autoimmune encephalitis is rare, it is one of the more than one hundred different kinds of autoimmune diseases that afflict an estimated 50 million people in the United States, a staggering figure that has more than tripled in the past three decades.58 An alarming majority of autoimmune diseases—around 75 percent—occur in women, affecting us more than all types of cancer combined. Autoimmune diseases are most likely the number one cause of disability in women of all ages. There are multiple theories about why women are so disproportionally affected, ranging from genetic, to environmental, to hormonal (most women are of childbearing age when they are diagnosed), to the fact that women’s immune systems are more complicated (they need to identify and safeguard fetuses, which are half-foreign entities, during pregnancy), and with everything more complex, malfunctions are all the more severe. For now, it’s just one more riddle in a series of question marks.
Dr. Dalmau and his lab have also identified other receptor-seeking autoimmune diseases that occur in the brain, making the anti-NMDA-receptor variety still rare but not unique. Now antibody-mediated autoimmune diseases have become a bonafide group of syndromes. Dr. Dalmau’s lab has identified six other types of antibodies that target various receptors in the brain, adding to the NMDA-receptor-preying kind, which struck me. This figure is growing. Dr. Dalmau estimates that when all is said and done, there could be twenty or more. These discoveries
finally will give names to diseases vaguely referred to as “encephalitis of an unknown origin,” or “psychosis not otherwise specified,” or not given any designation at all.
So it was no wonder that after the Post article ran, my inbox filled with hundreds of e-mails from mothers and fathers whose children had recently been diagnosed with all kinds of autoimmune diseases, women my age in the throes of the same disease, and people who suspected that their loved ones had it and wanted information on how best to treat it. Like any other major trauma, this disease bursts you wide open, and after surviving so much, you’re finally prepared to give back and willing to help anyone else who may be going through similar upheavals. But being so exposed, like a gushing wound, leaves you unprotected from the elements.
Many of the stories that I heard from that time were similar to my own, if not more harrowing. The words of people I spoke with kept me up at night: Why me? Why did my antibodies decide to attack? Why was I able to then recover?
I live with that constant refrain—not of self-pity but the real question of why my body decided to turn on itself. Then again, why does this happen to anyone? There are now thousands of cases of anti-NMDA receptor autoimmune encephalitis and many that have not ended well: an elderly woman who passed away because she had been misdiagnosed with a urinary tract infection; a woman who was pregnant when her symptoms progressed had lost her baby; several girls who had their ovaries removed when the doctors could not find a teratoma and the immune suppressants that had worked wonders on me didn’t help them.