“I peed.”
Another nurse joins her. They turn me on my left side, remove the bedding, wash me with warm towels, and spritz me with something. Then they turn me over to the right side and repeat. It feels nice. But I can’t move. I push hard with my brain, trying to wiggle my toes. I push so hard that I get a headache. My toes don’t move.
“I can’t move my legs,” I call out.
Many hours after the surgery, around 11:00 p.m., a nurse informed my dad, who had chosen to wait for news while everyone else went home at the staff’s insistence, that I had been moved from the recovery room into the ICU. They didn’t invite him in to see me, but he wandered into the unit anyway, unaccompanied. The floor consisted of a handful of bays, one patient in each. There were nurses everywhere, but no one even looked twice at him. He peeked into each bay until he spotted me.
There I was, semiconscious, propped up on pillows with my head wrapped in white gauze like some kind of sick Persian princess. I was attached to monitors and machines that beeped and groaned and had been wrapped up in nude compression stockings to keep my blood pressure normal. When he caught my eye, I instantly recognized him, which didn’t always happen. We hugged.
“The worst is behind you, Susannah.”
“Where’s Mom?” I asked.
“She will see you tomorrow,” he said. He could tell I was upset that my mother hadn’t come, even though it had been the right decision for her to go home that night. Then: “I can’t feel my legs, Dad.” I sounded convinced.
“Are you sure, Susannah?” my dad asked, turning white with fear. This had been the worry all along, that they would do permanent damage by messing with my brain.
“Yes. I can’t move them.”
My dad immediately called in a young resident, who came in and examined me, then rushed me out for an emergency MRI. My dad silently hurried beside the gurney, holding my hand until the MRI technician whisked me into the room, telling my dad to wait. In those thirty minutes, he would later sigh, he lost five years off his life. But the young resident eventually emerged to tell him that everything looked fine.
My dad stayed with me until I fell asleep. Then he went home and slid into bed, prayed, and fell into a restless slumber.
CHAPTER 28
SHADOWBOXER
After the surgery, I was reassigned to a shared room on the epilepsy unit. My roommate, a woman in her early thirties, suffered from seizures induced during alcohol consumption (though seizures occur commonly with alcohol withdrawal, sometimes drinking can induce seizures). She was constantly begging the staff to allow her to drink some wine so that they could record the seizure. They refused.
The results of the brain biopsy confirmed what the team had expected: my brain was inflamed. Dr. Najjar’s slides showed armies of angry inflammatory cells from my immune system attacking nerve cells in the brain, a signature of encephalitis.
There was a time, not so long ago, when neurologists believed that the brain was immunoprivileged, meaning it was completely separate from the immune system’s lymphocytes; now doctors use the careful phrasing “immuno-different.” The blood-brain barrier (BBB) is a dense patchwork quilt of vessels that serve as gates, regulating the passage of substances, like bacteria, chemicals, and drugs, from the blood to the brain.36 Researchers have discovered that the BBB does allow for certain B-cells and T-cells to squeeze through, in a process called diapedesis, to do regular “checkups.” But this was no routine checkup. The immune cells it had let through, which were supposed to protect the body, were in mid-blitz. This was the evidence Dr. Najjar had needed: I was in the grip of some kind of autoimmune disease.
Now that they had a hazy diagnosis, the doctors could move ahead with the first phase of treatment, intravenous steroids, a form of immunotherapy that suppresses inflammation created by the body’s immune system. A clear plastic bag of Solu-Medrol, an IV steroid, hung beside my bed for three days of intensive therapy. It was administered every six hours on an IV pump. These steroids, called corticosteroids, subdue the inflammation and quiet the immune system, which in turn quells future inflammation.37 As the steroids seeped into my system, they switched off inflammatory chemicals called cytokines. Dr. Najjar approved the highest dosage possible for three days. Then he would convert me to 60 milligrams of the oral steroid prednisone, which would continue, more gently, to quell the inflammation over time.
Because corticosteroids interact with blood-sugar levels, among other things, I developed a temporary form of type II diabetes. Though the doctors changed my menu, providing only sugar-free Jell-O as a sweet snack, my parents remained oblivious to the dangers of my Easter jellybeans, as I continued to munch away. Since I was placed on bed rest following surgery, the nurses applied thigh-high compression boots, which blow up and deflate, pumping blood through my legs and mimicking the act of contracting and expanding during physical activity. But they made my legs itchy and sweaty, as I explained to anyone who would listen, and I kicked them off every night.
Despite the new intensive steroid treatment, my condition did not seem to improve right away. In fact, it worsened; the abnormal nightly movements and undefined panic attacks increased. My father wrote about my continued difficulties in the logbook that he and my mom shared: “She had a strange smirking expression on her face. She tensed up,” he wrote. “Arms stretched out straight, grimace, tenseness, shakes.”
But I could still pull myself together for visitors. Hannah arrived soon after the surgery and stifled a laugh when she saw my strange white turban of bandages.
I was a good sport about it. “I’m going to be bald!” I said, smiling, and popped an Easter jellybean into my mouth.
“What do you mean? Did they shave your scalp?”
“Bald!”
“Maybe you need Propecia.” We both cracked up.
EEG video, April 12, 8:12 a.m., 7 minutes
I’m wearing a white surgical cap, reclining with my legs folded over as if I’m sunbathing. My pink backpack containing the EEG box rests on my lower stomach. I get up and walk to the door. My movements are halting and painfully slow. My left arm is outstretched.
“Would that be the little green button?” my mother asks a nurse from off-camera, referring to the seizure/event button tied to the bedside rail. She enters the frame and sits by the window.
I get back into bed. My mother gets up and hovers over me and then pushes the nurse’s button. Nurse Edward arrives moments later and starts a neurological exam, miming the action he wants me to follow, extending his arms out. Gradually I follow his lead. He taps on my left index finger and tells me to close my eyes and touch it to my face. After a moment, I do. He repeats it on the other side.
When Edward leaves, I reach for the sheets. It takes a full ten seconds for me to lie down. Meanwhile, my mother looks nervous. She checks her purse, crosses and uncrosses her legs, all the while keeping an eye on me.
End of video.
By our third night in the shared room, the woman next to me had a seizure. Somehow she had convinced the medical staff to allow her to drink wine. Since they had what they needed, a physical recording of a seizure, she was released shortly thereafter.
CHAPTER 29
DALMAU’S DISEASE
Dr. Russo arrived later that day to explain which diseases they could now tick off the list of possibilities, including hyperthyroidism, lymphoma, and Devic’s disease, a rare disease similar symptomatically to multiple sclerosis. They still suspected that I had been exposed to hepatitis, which can cause encephalitis, but they didn’t have proof.
After the conversation, my mother followed Dr. Russo into the hallway. “So what do you think it is?” my mom prodded.
“Actually, Dr. Najjar and I have a bet going.”
“What kind of a bet?”
“Well, Dr. Najjar thinks the inflammation is caused by autoimmune encephalitis; I think it’s paraneoplastic syndrome.”38 When my mother pressed for more details, Dr. Russo explained that paraneoplastic syndrom
e is a consequence of an underlying cancer, most often associated with lung, breast, or ovarian cancer. The symptoms—psychosis, catatonia, and so forth—are not associated with the cancer but with the immune system’s response to it. As the body gears up to attack the tumor, it sometimes begins to target healthy parts of the body, such as the spine or the brain. “I think because of her history of melanoma, it makes sense,” Dr. Russo concluded.
This was not what my mother wanted to hear. Cancer had always been the greatest fear, the word she dared not utter. Now this doctor was tossing it off casually as part of a bet.
Meanwhile, two plastic tubes, placed securely in Styrofoam boxes, had arrived at the University of Pennsylvania, transported in a refrigerator in the back of a FedEx truck; one contained transparent cerebrospinal fluid, as clear as unfiltered water, and another held blood, which started to look like dehydrated urine as, over time, the red blood cells dropped to the bottom. The test tubes were coded 0933, labeled with my initials, SC, and placed in a negative-80 degree freezer waiting for the lab to conduct its tests. They were addressed to the lab run by neuro-oncologist Dr. Josep Dalmau, whom Dr. Najjar had mentioned during his first visit and whom Dr. Russo had since e-mailed to ask if he would take a look at my case.
Four years earlier, in 2005, Dr. Dalmau had been the senior author on a paper in the neuroscience journal Annals of Neurology that focused on four young women who had developed prominent psychiatric symptoms and encephalitis. All had white blood cells in their cerebrospinal fluid, confusion, memory problems, hallucinations, delusions, and difficulty breathing, and they all had tumors called teratomas in their ovaries. But the most remarkable finding was that all four patients had similar antibodies that appeared to be reacting against specific areas of the brain, mainly the hippocampus. Something about the combination of the tumor and the antibodies was making these women very sick.
Dr. Dalmau had noticed a pattern in these four women; now he had to learn more about the antibody itself. He and his research team began to work night and day on an elaborate immunohistrochemistry experiment involving frozen sections of rat brains, which had been sliced into paper-thin pieces and then exposed to the cerebrospinal fluid of those four sick women. The hope was that the antibodies from the cerebrospinal fluid would bind directly to some receptors in the rat brain and reveal a characteristic design. It took eight months of tinkering before a pattern finally emerged.
Dr. Dalmau had prepared the rat brain slides all the same, placing a small amount of cerebrospinal fluid from each of the four patients on each. Twenty-four hours later:
A: Section of rat brain in the hippocampus that shows the reactivity of the cerebrospinal fluid of a patient with anti-NMDAR encephalitis. The brown staining corresponds to the patient’s antibodies that have bound to the NMDA receptors.
B: A similar section of hippocampus of an individual without NMDA receptor antibodies.
Four beautiful images, like cave drawings or abstract seashell patterns, revealed the antibodies’ binding to the naked eye. “It was a moment of great excitement,” Dr. Dalmau later recalled. “Everything had been negative. Now we became totally positive that all four not only had the same illness, but the same antibody.”
He had clarified that the pattern of reactivity was more intense in the hippocampus of the rat brain, but this was only the beginning. A far more difficult question now arose: Which receptors were these antibodies targeting? Through a combination of trial and error, plus a few educated guesses about which receptors are most common in the hippocampus, Dr. Dalmau and his colleagues eventually identified the target. Using a kidney cell line bought from a commercial lab that came with no receptors on their surfaces at all, a kind of “blank slate,” his lab introduced DNA sequences that direct the cells to make certain types of receptors, allowing the lab to control which receptors were available for binding. Dalmau chose to have them express only NMDA receptors, after figuring out that those were the most likely to have been present in high volume in the hippocampus. Sure enough, the antibodies in the cerebrospinal fluid of the four patients bound to the cells. There was his answer: the culprits were NMDA-receptor-seeking antibodies.
NMDA (N-methyl-D-aspartate acid) receptors are vital to learning, memory, and behavior, and they are a main staple of our brain chemistry.39 If these are incapacitated, mind and body fail. NMDA receptors are located all over the brain, but the majority are concentrated on neurons in the hippocampus, the brain’s primary learning and memory center, and in the frontal lobes, the seat of higher functions and personality. These receptors receive instructions from chemicals called neurotransmitters. All neurotransmitters carry only one of two messages: they can either “excite” a cell, encouraging it to fire an electrical impulse, or “inhibit” a cell, which hinders it from firing. These simple conversations between neurons are at the root of everything we do, from sipping a glass of wine to writing a newspaper lead.
In those unfortunate patients with Dr. Dalmau’s anti-NMDA-receptor encephalitis, the antibodies, normally a force for good in the body, had become treasonous persona non grata in the brain. These receptor-seeking antibodies planted their death kiss on the surface of a neuron, handicapping the neuron’s receptors, making them unable to send and receive those important chemical signals. Though researchers are far from fully understanding how NMDA receptors (and their corresponding neurons) affect and alter behavior, it’s clear that when they are compromised the outcome can be disastrous, even deadly.
Still, a few experiments have offered up some clues as to their importance. Decrease NMDA receptors by, say, 40 percent, and you might get psychosis; decrease them by 70 percent, and you have catatonia. In “knockout mice” without NMDA receptors at all, even the most basic life functions are impossible: most die within ten hours of birth due to respiratory failure.40 Mice with a very small number of NMDA receptors don’t learn to suckle, and they simply starve to death within a day or so. Those mice with at least 5 percent of their NMDA receptors intact survive, but exhibit abnormal behavior and strange social and sexual interactions. Mice with half their receptors in working order also live, but they show memory deficits and abnormal social relationships.
As a result of this additional research, in 2007, Dr. Dalmau and his colleagues presented another paper, now introducing his new class of NMDA-receptor-seeking diseases to the world. This second article identified twelve women with the same profile of neurological symptoms, which could now be called a syndrome.41 They all had teratomas, and almost all of them were young women. Within a year after publication, one hundred more patients had been diagnosed; not all of them had ovarian teratomas and not all of them were young women (some were men and many were children), enabling Dr. Dalmau to do an even more thorough study on the newly discovered, but nameless, disease.
“Why not name it the Dalmau disease?” people often asked him. But he didn’t think “Dalmau disease” sounded right, and it was no longer customary to name a disease after its discoverer. “I didn’t think that would be wise. It’s not very humble.” He shrugged.
By the time I was a patient at NYU, Dr. Dalmau had fine-tuned his approach, designing two tests that could swiftly and accurately diagnose the disease. As soon as he received my samples, he could test the spinal fluid. If he found that I had anti-NMDA-receptor autoimmune encephalitis, it would make me the 217th person worldwide to be diagnosed since 2007. It just begged the question: If it took so long for one of the best hospitals in the world to get to this step, how many other people were going untreated, diagnosed with a mental illness or condemned to a life in a nursing home or a psychiatric ward?
CHAPTER 30
RHUBARB
By my twenty-fifth day in the hospital, two days after the biopsy, with a preliminary diagnosis in sight, my doctors thought it was a good time to officially assess my cognitive skills to record a baseline. This test would be a fulcrum, a turning point, that would measure what kind of progress they could expect in the future through the various s
tages of treatment. Beginning on the afternoon of April 15, a speech pathologist and a neuropsychologist visited me for two days in a row, each for separate assessments.
The speech pathologist, Karen Gendal, did the first assessment, starting with basic questions: “What’s your name?” “How old are you?” “Are you a woman?” “Do you live in California?” “Do you live in New York?” “Do you peel a banana before eating it?” and so forth. I was able to answer all of these questions, though I did so slowly. But when she asked the more open-ended question “Why are you in the hospital?” I could not explain. (To be fair, the doctors didn’t know either, but I could not provide even the basics.)
After some spotty and tangential answers, I finally said, “I can’t get my ideas from my head out.” She nodded: this was a typical response for people suffering from aphasia, a language impairment related to brain injury. I also had something called dysarthria, a motor speech impairment caused by a weakness in the muscles of the face, throat, or vocal cords.
Gendal asked me to stick out my tongue, which trembled from the effort. It had a reduced range of motion on both sides, which was contributing to my inability to articulate.
“Would you smile for me?”
I tried, but my facial muscles were so weak that no smile came. She wrote down “hypo-aroused,” a medical term for lethargic, and also noted that I was not fully alert. When I did talk, the words came out without any emotional register.
She moved on to cognitive abilities. Holding up her pen, she asked, “What is this?”
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